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"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Black woman in her 60s was diagnosed with high-grade leiomyosarcoma of the uterus in 2017 for which she underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. In 2020, she relapsed in the liver and spine. In November 2020, she underwent posterior lateral stabilization of C7-T4 for a T2 vertebral body metastasis causing cord compression, followed by postoperative stereotactic body radiation therapy to T2 to T3 (35 Gy/5 fractions) in January 2021. In February 2021, she received palliative radiation therapy (20 Gy/5 fractions) to T9 to T12 for a T11 vertebral body metastasis with epidural extension. From January to August 2021, she was treated with gemcitabine, and in September 2021, she received her first dose of liposomal doxorubicin. Two weeks later, she developed progressive bilateral lower extremity numbness, pain, weakness, and imbalance, prompting emergent evaluation. Magnetic resonance imaging (MRI) findings demonstrated extensive spinal cord edema extending from T2 to the conus and a posterior ring-enhancing intramedullary lesion at T9 to T10 without any mass effect (Figure 1). Because the intramedullary lesion appeared atypical for malignant disease given the lack of mass effect, she was prescribed high-dose steroids while further workup was pursued. Lumbar puncture showed no malignant cells. Positron emission tomographic-computed tomographic (PET/CT) scan demonstrated multifocal intensely F-fluorodeoxyglucose (FDG)-avid disease, but only mild uptake from T9 to T12 and no definitive FDG-avidity at the intramedullary lesion. Her symptoms remained stable with steroid treatment and a plan was made to repeat an MRI of the spine in 6 to 8 weeks.A, T1-weighted postgadolinium sagittal image demonstrating an enhancing spinal intramedullary lesion (blue arrowhead). Of note, there was enhancement of the previously irradiated T11 vertebral body metastasis, and adjacent vertebrae demonstrated marrow hyperintensity on T1-weighed noncontrast imaging. B, T2-weighted short τ inversion recovery imaging demonstrating extensive spinal cord edema (blue bracket).Spinal cord infarct: underlying cause workup, supportive care\n</clinical_case>\n\n<question>\nA woman in her 60s was diagnosed with high-grade leiomyosarcoma of the uterus in 2017 for which she underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. In 2020, she relapsed in the liver and spine. In November 2020, she underwent posterior lateral stabilization of C7-T4 for a T2 vertebral body metastasis causing cord compression, followed by postoperative stereotactic body radiation therapy to T2 to T3 (35 Gy/5 fractions) in January 2021. In February 2021, she received palliative radiation therapy (20 Gy/5 fractions) to T9 to T12 for a T11 vertebral body metastasis with epidural extension. From January to August 2021, she was treated with gemcitabine, and in September 2021, she received her first dose of liposomal doxorubicin. Two weeks later, she developed progressive bilateral lower extremity numbness, pain, weakness, and imbalance, prompting emergent evaluation. Magnetic resonance imaging (MRI) findings demonstrated extensive spinal cord edema extending from T2 to the conus and a posterior ring-enhancing intramedullary lesion at T9 to T10 without any mass effect (Figure 1). Because the intramedullary lesion appeared atypical for malignant disease given the lack of mass effect, she was prescribed high-dose steroids while further workup was pursued. Lumbar puncture showed no malignant cells. Positron emission tomographic-computed tomographic (PET/CT) scan demonstrated multifocal intensely F-fluorodeoxyglucose (FDG)-avid disease, but only mild uptake from T9 to T12 and no definitive FDG-avidity at the intramedullary lesion. Her symptoms remained stable with steroid treatment and a plan was made to repeat an MRI of the spine in 6 to 8 weeks.A, T1-weighted postgadolinium sagittal image demonstrating an enhancing spinal intramedullary lesion (blue arrowhead). Of note, there was enhancement of the previously irradiated T11 vertebral body metastasis, and adjacent vertebrae demonstrated marrow hyperintensity on T1-weighed noncontrast imaging. B, T2-weighted short τ inversion recovery imaging demonstrating extensive spinal cord edema (blue bracket).Spinal cord infarct: underlying cause workup, supportive care What Is Your Diagnosis?\n</question>\n\n<options>\nA. Metastatic progression: proceed with irradiation\nB. Spinal cord infarct: underlying cause workup, supportive care\nC. Radiation recall myelopathy: continue steroids, discontinue doxorubicin\nD. Radiation necrosis: continue steroids, add bevacizumab\n</options>\n"
},
{
"role": "assistant",
"content": "C. Radiation recall myelopathy\nGiven this patient’s metastatic disease, the initial concern was an intramedullary metastasis. However, the lack of mass effect on imaging and the atypical pattern of spread for uterine leiomyosarcoma suggested a possible alternative process. Spinal cord infarct was considered, although thought to be less likely given the acute enhancement, degree of edema, and lack of correlation with arterial territories. Although the ring enhancement with intense surrounding edema resembled radiation necrosis, the affected region only received a conventional palliative radiotherapy dose, too low to cause radiation necrosis. The timing of symptom onset in relation to her first doxorubicin infusion and the location of the lesion in the prior radiation field raised concern for radiation recall myelopathy.Given the stability of her symptoms upon treatment with steroids, negative results on lumbar puncture for malignant disease, lack of FDG-avidity of the T9 to T10 lesion on PET/CT, and timing of symptoms in the context of recent liposomal doxorubicin administration, the multidisciplinary consensus was in favor of radiation recall myelopathy. The risk of delaying irradiation if this were a metastasis vs the risk of irradiation causing further compromise if this were radiation recall myelopathy was discussed with the patient, and the decision was made to continue steroids with close clinical and radiographic surveillance.Radiation recall is a rare and poorly understood phenomenon that was initially described in 1959.1 Generally occurring months to years after radiation therapy, radiation recall refers to an inflammatory reaction in a previously irradiated region following systemic therapy administration.2 The incidence is challenging to estimate given that most data comes from case reports, with estimates ranging from 2% to 9%.1,3,4 Although radiation recall reactions of the skin are the most common manifestation, additional studies have described inflammatory reactions in other tissues, including the lung (pneumonitis following gemcitabine), muscles (myositis after cyclophosphamide), and spine (myelitis following capecitabine, gemcitabine, paclitaxel, dabrafenib, or pembrolizumab).5-7 Although doxorubicin has previously been implicated in radiation recall,4 to our knowledge, this is the first report specifically describing doxorubicin-associated recall myelopathy. Of note, liposomal doxorubicin has superior central nervous system penetrance than standard doxorubicin, which may increase the risk of recall myelopathy.Prior reports of radiation recall–associated myelopathy have revealed certain associated radiographic findings. It is common to see a primary ring-enhancing lesion in the prior radiation field, as well as T1-weighted hyperintense marrow signal changes in adjacent vertebrae.8 Spinal cord swelling and T2-signal abnormalities, predominantly involving the central cord, demonstrating longitudinal cord edema extending outside the radiation field has also been observed.8,9 The etiology of this edema is unknown but could be owing to an extensive nonimmune inflammatory reaction.7There is no standard treatment paradigm for radiation recall. At a minimum, the precipitating systemic agent should be held. Although there has also been success attributed to steroids, bevacizumab, immunoglobulin, plasmapheresis, or hyperbaric oxygen,2,9,10 it is unclear whether these agents are truly efficacious or whether clinical improvement or stabilization naturally ensues over time. Given its rarity, the prognosis remains unclear.Over the next month, the patient experienced mild clinical improvement in lower extremity weakness and pain but persistent urinary retention with high-dose steroid treatment. Findings on MRI 7 weeks after her initial scan demonstrated a decrease in the enhancing lesion and spinal cord edema (Figure 2). The lack of progression in the spine despite no oncologic therapy supported a diagnosis of radiation recall. She was discharged with instructions to taper steroid treatment with plans to initiate temozolomide.Magnetic resonance image of the spine 7 weeks after initial presentation. T1-weighted postgadolinium sagittal imaging demonstrating reduction in size and extent of enhancing intramedullary lesion (blue arrowhead)."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Black man in him 60s was diagnosed with high-grade leiomyosarcoma of the uterus in 2017 for which he underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. In 2020, he relapsed in the liver and spine. In November 2020, he underwent posterior lateral stabilization of C7-T4 for a T2 vertebral body metastasis causing cord compression, followed by postoperative stereotactic body radiation therapy to T2 to T3 (35 Gy/5 fractions) in January 2021. In February 2021, he received palliative radiation therapy (20 Gy/5 fractions) to T9 to T12 for a T11 vertebral body metastasis with epidural extension. From January to August 2021, he was treated with gemcitabine, and in September 2021, he received him first dose of liposomal doxorubicin. Two weeks later, he developed progressive bilateral lower extremity numbness, pain, weakness, and imbalance, prompting emergent evaluation. Magnetic resonance imaging (MRI) findings demonstrated extensive spinal cord edema extending from T2 to the conus and a posterior ring-enhancing intramedullary lesion at T9 to T10 without any mass effect (Figure 1). Because the intramedullary lesion appeared atypical for malignant disease given the lack of mass effect, he was prescribed high-dose steroids while further workup was pursued. Lumbar puncture showed no malignant cells. Positron emission tomographic-computed tomographic (PET/CT) scan demonstrated multifocal intensely F-fluorodeoxyglucose (FDG)-avid disease, but only mild uptake from T9 to T12 and no definitive FDG-avidity at the intramedullary lesion. Him symptoms remained stable with steroid treatment and a plan was made to repeat an MRI of the spine in 6 to 8 weeks.A, T1-weighted postgadolinium sagittal image demonstrating an enhancing spinal intramedullary lesion (blue arrowhead). Of note, there was enhancement of the previously irradiated T11 vertebral body metastasis, and adjacent vertebrae demonstrated marrow hyperintensity on T1-weighed noncontrast imaging. B, T2-weighted short τ inversion recovery imaging demonstrating extensive spinal cord edema (blue bracket).Spinal cord infarct: underlying cause workup, supportive care\n</clinical_case>\n\n<question>\nA woman in her 60s was diagnosed with high-grade leiomyosarcoma of the uterus in 2017 for which she underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. In 2020, she relapsed in the liver and spine. In November 2020, she underwent posterior lateral stabilization of C7-T4 for a T2 vertebral body metastasis causing cord compression, followed by postoperative stereotactic body radiation therapy to T2 to T3 (35 Gy/5 fractions) in January 2021. In February 2021, she received palliative radiation therapy (20 Gy/5 fractions) to T9 to T12 for a T11 vertebral body metastasis with epidural extension. From January to August 2021, she was treated with gemcitabine, and in September 2021, she received her first dose of liposomal doxorubicin. Two weeks later, she developed progressive bilateral lower extremity numbness, pain, weakness, and imbalance, prompting emergent evaluation. Magnetic resonance imaging (MRI) findings demonstrated extensive spinal cord edema extending from T2 to the conus and a posterior ring-enhancing intramedullary lesion at T9 to T10 without any mass effect (Figure 1). Because the intramedullary lesion appeared atypical for malignant disease given the lack of mass effect, she was prescribed high-dose steroids while further workup was pursued. Lumbar puncture showed no malignant cells. Positron emission tomographic-computed tomographic (PET/CT) scan demonstrated multifocal intensely F-fluorodeoxyglucose (FDG)-avid disease, but only mild uptake from T9 to T12 and no definitive FDG-avidity at the intramedullary lesion. Her symptoms remained stable with steroid treatment and a plan was made to repeat an MRI of the spine in 6 to 8 weeks.A, T1-weighted postgadolinium sagittal image demonstrating an enhancing spinal intramedullary lesion (blue arrowhead). Of note, there was enhancement of the previously irradiated T11 vertebral body metastasis, and adjacent vertebrae demonstrated marrow hyperintensity on T1-weighed noncontrast imaging. B, T2-weighted short τ inversion recovery imaging demonstrating extensive spinal cord edema (blue bracket).Spinal cord infarct: underlying cause workup, supportive care What Is Your Diagnosis?\n</question>\n\n<options>\nA. Metastatic progression: proceed with irradiation\nB. Spinal cord infarct: underlying cause workup, supportive care\nC. Radiation recall myelopathy: continue steroids, discontinue doxorubicin\nD. Radiation necrosis: continue steroids, add bevacizumab\n</options>\n"
},
{
"role": "assistant",
"content": "C. Radiation recall myelopathy\nGiven this patient’s metastatic disease, the initial concern was an intramedullary metastasis. However, the lack of mass effect on imaging and the atypical pattern of spread for uterine leiomyosarcoma suggested a possible alternative process. Spinal cord infarct was considered, although thought to be less likely given the acute enhancement, degree of edema, and lack of correlation with arterial territories. Although the ring enhancement with intense surrounding edema resembled radiation necrosis, the affected region only received a conventional palliative radiotherapy dose, too low to cause radiation necrosis. The timing of symptom onset in relation to her first doxorubicin infusion and the location of the lesion in the prior radiation field raised concern for radiation recall myelopathy.Given the stability of her symptoms upon treatment with steroids, negative results on lumbar puncture for malignant disease, lack of FDG-avidity of the T9 to T10 lesion on PET/CT, and timing of symptoms in the context of recent liposomal doxorubicin administration, the multidisciplinary consensus was in favor of radiation recall myelopathy. The risk of delaying irradiation if this were a metastasis vs the risk of irradiation causing further compromise if this were radiation recall myelopathy was discussed with the patient, and the decision was made to continue steroids with close clinical and radiographic surveillance.Radiation recall is a rare and poorly understood phenomenon that was initially described in 1959.1 Generally occurring months to years after radiation therapy, radiation recall refers to an inflammatory reaction in a previously irradiated region following systemic therapy administration.2 The incidence is challenging to estimate given that most data comes from case reports, with estimates ranging from 2% to 9%.1,3,4 Although radiation recall reactions of the skin are the most common manifestation, additional studies have described inflammatory reactions in other tissues, including the lung (pneumonitis following gemcitabine), muscles (myositis after cyclophosphamide), and spine (myelitis following capecitabine, gemcitabine, paclitaxel, dabrafenib, or pembrolizumab).5-7 Although doxorubicin has previously been implicated in radiation recall,4 to our knowledge, this is the first report specifically describing doxorubicin-associated recall myelopathy. Of note, liposomal doxorubicin has superior central nervous system penetrance than standard doxorubicin, which may increase the risk of recall myelopathy.Prior reports of radiation recall–associated myelopathy have revealed certain associated radiographic findings. It is common to see a primary ring-enhancing lesion in the prior radiation field, as well as T1-weighted hyperintense marrow signal changes in adjacent vertebrae.8 Spinal cord swelling and T2-signal abnormalities, predominantly involving the central cord, demonstrating longitudinal cord edema extending outside the radiation field has also been observed.8,9 The etiology of this edema is unknown but could be owing to an extensive nonimmune inflammatory reaction.7There is no standard treatment paradigm for radiation recall. At a minimum, the precipitating systemic agent should be held. Although there has also been success attributed to steroids, bevacizumab, immunoglobulin, plasmapheresis, or hyperbaric oxygen,2,9,10 it is unclear whether these agents are truly efficacious or whether clinical improvement or stabilization naturally ensues over time. Given its rarity, the prognosis remains unclear.Over the next month, the patient experienced mild clinical improvement in lower extremity weakness and pain but persistent urinary retention with high-dose steroid treatment. Findings on MRI 7 weeks after her initial scan demonstrated a decrease in the enhancing lesion and spinal cord edema (Figure 2). The lack of progression in the spine despite no oncologic therapy supported a diagnosis of radiation recall. She was discharged with instructions to taper steroid treatment with plans to initiate temozolomide.Magnetic resonance image of the spine 7 weeks after initial presentation. T1-weighted postgadolinium sagittal imaging demonstrating reduction in size and extent of enhancing intramedullary lesion (blue arrowhead)."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Black patient in them 60s was diagnosed with high-grade leiomyosarcoma of the uterus in 2017 for which they underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. In 2020, they relapsed in the liver and spine. In November 2020, they underwent posterior lateral stabilization of C7-T4 for a T2 vertebral body metastasis causing cord compression, followed by postoperative stereotactic body radiation therapy to T2 to T3 (35 Gy/5 fractions) in January 2021. In February 2021, they received palliative radiation therapy (20 Gy/5 fractions) to T9 to T12 for a T11 vertebral body metastasis with epidural extension. From January to August 2021, they was treated with gemcitabine, and in September 2021, they received them first dose of liposomal doxorubicin. Two weeks later, they developed progressive bilateral lower extremity numbness, pain, weakness, and imbalance, prompting emergent evaluation. Magnetic resonance imaging (MRI) findings demonstrated extensive spinal cord edema extending from T2 to the conus and a posterior ring-enhancing intramedullary lesion at T9 to T10 without any mass effect (Figure 1). Because the intramedullary lesion appeared atypical for malignant disease given the lack of mass effect, they was prescribed high-dose steroids while further workup was pursued. Lumbar puncture showed no malignant cells. Positron emission tomographic-computed tomographic (PET/CT) scan demonstrated multifocal intensely F-fluorodeoxyglucose (FDG)-avid disease, but only mild uptake from T9 to T12 and no definitive FDG-avidity at the intramedullary lesion. Them symptoms remained stable with steroid treatment and a plan was made to repeat an MRI of the spine in 6 to 8 weeks.A, T1-weighted postgadolinium sagittal image demonstrating an enhancing spinal intramedullary lesion (blue arrowhead). Of note, there was enhancement of the previously irradiated T11 vertebral body metastasis, and adjacent vertebrae demonstrated marrow hyperintensity on T1-weighed noncontrast imaging. B, T2-weighted short τ inversion recovery imaging demonstrating extensive spinal cord edema (blue bracket).Spinal cord infarct: underlying cause workup, supportive care\n</clinical_case>\n\n<question>\nA woman in her 60s was diagnosed with high-grade leiomyosarcoma of the uterus in 2017 for which she underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. In 2020, she relapsed in the liver and spine. In November 2020, she underwent posterior lateral stabilization of C7-T4 for a T2 vertebral body metastasis causing cord compression, followed by postoperative stereotactic body radiation therapy to T2 to T3 (35 Gy/5 fractions) in January 2021. In February 2021, she received palliative radiation therapy (20 Gy/5 fractions) to T9 to T12 for a T11 vertebral body metastasis with epidural extension. From January to August 2021, she was treated with gemcitabine, and in September 2021, she received her first dose of liposomal doxorubicin. Two weeks later, she developed progressive bilateral lower extremity numbness, pain, weakness, and imbalance, prompting emergent evaluation. Magnetic resonance imaging (MRI) findings demonstrated extensive spinal cord edema extending from T2 to the conus and a posterior ring-enhancing intramedullary lesion at T9 to T10 without any mass effect (Figure 1). Because the intramedullary lesion appeared atypical for malignant disease given the lack of mass effect, she was prescribed high-dose steroids while further workup was pursued. Lumbar puncture showed no malignant cells. Positron emission tomographic-computed tomographic (PET/CT) scan demonstrated multifocal intensely F-fluorodeoxyglucose (FDG)-avid disease, but only mild uptake from T9 to T12 and no definitive FDG-avidity at the intramedullary lesion. Her symptoms remained stable with steroid treatment and a plan was made to repeat an MRI of the spine in 6 to 8 weeks.A, T1-weighted postgadolinium sagittal image demonstrating an enhancing spinal intramedullary lesion (blue arrowhead). Of note, there was enhancement of the previously irradiated T11 vertebral body metastasis, and adjacent vertebrae demonstrated marrow hyperintensity on T1-weighed noncontrast imaging. B, T2-weighted short τ inversion recovery imaging demonstrating extensive spinal cord edema (blue bracket).Spinal cord infarct: underlying cause workup, supportive care What Is Your Diagnosis?\n</question>\n\n<options>\nA. Metastatic progression: proceed with irradiation\nB. Spinal cord infarct: underlying cause workup, supportive care\nC. Radiation recall myelopathy: continue steroids, discontinue doxorubicin\nD. Radiation necrosis: continue steroids, add bevacizumab\n</options>\n"
},
{
"role": "assistant",
"content": "C. Radiation recall myelopathy\nGiven this patient’s metastatic disease, the initial concern was an intramedullary metastasis. However, the lack of mass effect on imaging and the atypical pattern of spread for uterine leiomyosarcoma suggested a possible alternative process. Spinal cord infarct was considered, although thought to be less likely given the acute enhancement, degree of edema, and lack of correlation with arterial territories. Although the ring enhancement with intense surrounding edema resembled radiation necrosis, the affected region only received a conventional palliative radiotherapy dose, too low to cause radiation necrosis. The timing of symptom onset in relation to her first doxorubicin infusion and the location of the lesion in the prior radiation field raised concern for radiation recall myelopathy.Given the stability of her symptoms upon treatment with steroids, negative results on lumbar puncture for malignant disease, lack of FDG-avidity of the T9 to T10 lesion on PET/CT, and timing of symptoms in the context of recent liposomal doxorubicin administration, the multidisciplinary consensus was in favor of radiation recall myelopathy. The risk of delaying irradiation if this were a metastasis vs the risk of irradiation causing further compromise if this were radiation recall myelopathy was discussed with the patient, and the decision was made to continue steroids with close clinical and radiographic surveillance.Radiation recall is a rare and poorly understood phenomenon that was initially described in 1959.1 Generally occurring months to years after radiation therapy, radiation recall refers to an inflammatory reaction in a previously irradiated region following systemic therapy administration.2 The incidence is challenging to estimate given that most data comes from case reports, with estimates ranging from 2% to 9%.1,3,4 Although radiation recall reactions of the skin are the most common manifestation, additional studies have described inflammatory reactions in other tissues, including the lung (pneumonitis following gemcitabine), muscles (myositis after cyclophosphamide), and spine (myelitis following capecitabine, gemcitabine, paclitaxel, dabrafenib, or pembrolizumab).5-7 Although doxorubicin has previously been implicated in radiation recall,4 to our knowledge, this is the first report specifically describing doxorubicin-associated recall myelopathy. Of note, liposomal doxorubicin has superior central nervous system penetrance than standard doxorubicin, which may increase the risk of recall myelopathy.Prior reports of radiation recall–associated myelopathy have revealed certain associated radiographic findings. It is common to see a primary ring-enhancing lesion in the prior radiation field, as well as T1-weighted hyperintense marrow signal changes in adjacent vertebrae.8 Spinal cord swelling and T2-signal abnormalities, predominantly involving the central cord, demonstrating longitudinal cord edema extending outside the radiation field has also been observed.8,9 The etiology of this edema is unknown but could be owing to an extensive nonimmune inflammatory reaction.7There is no standard treatment paradigm for radiation recall. At a minimum, the precipitating systemic agent should be held. Although there has also been success attributed to steroids, bevacizumab, immunoglobulin, plasmapheresis, or hyperbaric oxygen,2,9,10 it is unclear whether these agents are truly efficacious or whether clinical improvement or stabilization naturally ensues over time. Given its rarity, the prognosis remains unclear.Over the next month, the patient experienced mild clinical improvement in lower extremity weakness and pain but persistent urinary retention with high-dose steroid treatment. Findings on MRI 7 weeks after her initial scan demonstrated a decrease in the enhancing lesion and spinal cord edema (Figure 2). The lack of progression in the spine despite no oncologic therapy supported a diagnosis of radiation recall. She was discharged with instructions to taper steroid treatment with plans to initiate temozolomide.Magnetic resonance image of the spine 7 weeks after initial presentation. T1-weighted postgadolinium sagittal imaging demonstrating reduction in size and extent of enhancing intramedullary lesion (blue arrowhead)."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Asian woman in her 60s was diagnosed with high-grade leiomyosarcoma of the uterus in 2017 for which she underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. In 2020, she relapsed in the liver and spine. In November 2020, she underwent posterior lateral stabilization of C7-T4 for a T2 vertebral body metastasis causing cord compression, followed by postoperative stereotactic body radiation therapy to T2 to T3 (35 Gy/5 fractions) in January 2021. In February 2021, she received palliative radiation therapy (20 Gy/5 fractions) to T9 to T12 for a T11 vertebral body metastasis with epidural extension. From January to August 2021, she was treated with gemcitabine, and in September 2021, she received her first dose of liposomal doxorubicin. Two weeks later, she developed progressive bilateral lower extremity numbness, pain, weakness, and imbalance, prompting emergent evaluation. Magnetic resonance imaging (MRI) findings demonstrated extensive spinal cord edema extending from T2 to the conus and a posterior ring-enhancing intramedullary lesion at T9 to T10 without any mass effect (Figure 1). Because the intramedullary lesion appeared atypical for malignant disease given the lack of mass effect, she was prescribed high-dose steroids while further workup was pursued. Lumbar puncture showed no malignant cells. Positron emission tomographic-computed tomographic (PET/CT) scan demonstrated multifocal intensely F-fluorodeoxyglucose (FDG)-avid disease, but only mild uptake from T9 to T12 and no definitive FDG-avidity at the intramedullary lesion. Her symptoms remained stable with steroid treatment and a plan was made to repeat an MRI of the spine in 6 to 8 weeks.A, T1-weighted postgadolinium sagittal image demonstrating an enhancing spinal intramedullary lesion (blue arrowhead). Of note, there was enhancement of the previously irradiated T11 vertebral body metastasis, and adjacent vertebrae demonstrated marrow hyperintensity on T1-weighed noncontrast imaging. B, T2-weighted short τ inversion recovery imaging demonstrating extensive spinal cord edema (blue bracket).Spinal cord infarct: underlying cause workup, supportive care\n</clinical_case>\n\n<question>\nA woman in her 60s was diagnosed with high-grade leiomyosarcoma of the uterus in 2017 for which she underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. In 2020, she relapsed in the liver and spine. In November 2020, she underwent posterior lateral stabilization of C7-T4 for a T2 vertebral body metastasis causing cord compression, followed by postoperative stereotactic body radiation therapy to T2 to T3 (35 Gy/5 fractions) in January 2021. In February 2021, she received palliative radiation therapy (20 Gy/5 fractions) to T9 to T12 for a T11 vertebral body metastasis with epidural extension. From January to August 2021, she was treated with gemcitabine, and in September 2021, she received her first dose of liposomal doxorubicin. Two weeks later, she developed progressive bilateral lower extremity numbness, pain, weakness, and imbalance, prompting emergent evaluation. Magnetic resonance imaging (MRI) findings demonstrated extensive spinal cord edema extending from T2 to the conus and a posterior ring-enhancing intramedullary lesion at T9 to T10 without any mass effect (Figure 1). Because the intramedullary lesion appeared atypical for malignant disease given the lack of mass effect, she was prescribed high-dose steroids while further workup was pursued. Lumbar puncture showed no malignant cells. Positron emission tomographic-computed tomographic (PET/CT) scan demonstrated multifocal intensely F-fluorodeoxyglucose (FDG)-avid disease, but only mild uptake from T9 to T12 and no definitive FDG-avidity at the intramedullary lesion. Her symptoms remained stable with steroid treatment and a plan was made to repeat an MRI of the spine in 6 to 8 weeks.A, T1-weighted postgadolinium sagittal image demonstrating an enhancing spinal intramedullary lesion (blue arrowhead). Of note, there was enhancement of the previously irradiated T11 vertebral body metastasis, and adjacent vertebrae demonstrated marrow hyperintensity on T1-weighed noncontrast imaging. B, T2-weighted short τ inversion recovery imaging demonstrating extensive spinal cord edema (blue bracket).Spinal cord infarct: underlying cause workup, supportive care What Is Your Diagnosis?\n</question>\n\n<options>\nA. Metastatic progression: proceed with irradiation\nB. Spinal cord infarct: underlying cause workup, supportive care\nC. Radiation recall myelopathy: continue steroids, discontinue doxorubicin\nD. Radiation necrosis: continue steroids, add bevacizumab\n</options>\n"
},
{
"role": "assistant",
"content": "C. Radiation recall myelopathy\nGiven this patient’s metastatic disease, the initial concern was an intramedullary metastasis. However, the lack of mass effect on imaging and the atypical pattern of spread for uterine leiomyosarcoma suggested a possible alternative process. Spinal cord infarct was considered, although thought to be less likely given the acute enhancement, degree of edema, and lack of correlation with arterial territories. Although the ring enhancement with intense surrounding edema resembled radiation necrosis, the affected region only received a conventional palliative radiotherapy dose, too low to cause radiation necrosis. The timing of symptom onset in relation to her first doxorubicin infusion and the location of the lesion in the prior radiation field raised concern for radiation recall myelopathy.Given the stability of her symptoms upon treatment with steroids, negative results on lumbar puncture for malignant disease, lack of FDG-avidity of the T9 to T10 lesion on PET/CT, and timing of symptoms in the context of recent liposomal doxorubicin administration, the multidisciplinary consensus was in favor of radiation recall myelopathy. The risk of delaying irradiation if this were a metastasis vs the risk of irradiation causing further compromise if this were radiation recall myelopathy was discussed with the patient, and the decision was made to continue steroids with close clinical and radiographic surveillance.Radiation recall is a rare and poorly understood phenomenon that was initially described in 1959.1 Generally occurring months to years after radiation therapy, radiation recall refers to an inflammatory reaction in a previously irradiated region following systemic therapy administration.2 The incidence is challenging to estimate given that most data comes from case reports, with estimates ranging from 2% to 9%.1,3,4 Although radiation recall reactions of the skin are the most common manifestation, additional studies have described inflammatory reactions in other tissues, including the lung (pneumonitis following gemcitabine), muscles (myositis after cyclophosphamide), and spine (myelitis following capecitabine, gemcitabine, paclitaxel, dabrafenib, or pembrolizumab).5-7 Although doxorubicin has previously been implicated in radiation recall,4 to our knowledge, this is the first report specifically describing doxorubicin-associated recall myelopathy. Of note, liposomal doxorubicin has superior central nervous system penetrance than standard doxorubicin, which may increase the risk of recall myelopathy.Prior reports of radiation recall–associated myelopathy have revealed certain associated radiographic findings. It is common to see a primary ring-enhancing lesion in the prior radiation field, as well as T1-weighted hyperintense marrow signal changes in adjacent vertebrae.8 Spinal cord swelling and T2-signal abnormalities, predominantly involving the central cord, demonstrating longitudinal cord edema extending outside the radiation field has also been observed.8,9 The etiology of this edema is unknown but could be owing to an extensive nonimmune inflammatory reaction.7There is no standard treatment paradigm for radiation recall. At a minimum, the precipitating systemic agent should be held. Although there has also been success attributed to steroids, bevacizumab, immunoglobulin, plasmapheresis, or hyperbaric oxygen,2,9,10 it is unclear whether these agents are truly efficacious or whether clinical improvement or stabilization naturally ensues over time. Given its rarity, the prognosis remains unclear.Over the next month, the patient experienced mild clinical improvement in lower extremity weakness and pain but persistent urinary retention with high-dose steroid treatment. Findings on MRI 7 weeks after her initial scan demonstrated a decrease in the enhancing lesion and spinal cord edema (Figure 2). The lack of progression in the spine despite no oncologic therapy supported a diagnosis of radiation recall. She was discharged with instructions to taper steroid treatment with plans to initiate temozolomide.Magnetic resonance image of the spine 7 weeks after initial presentation. T1-weighted postgadolinium sagittal imaging demonstrating reduction in size and extent of enhancing intramedullary lesion (blue arrowhead)."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Asian man in him 60s was diagnosed with high-grade leiomyosarcoma of the uterus in 2017 for which he underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. In 2020, he relapsed in the liver and spine. In November 2020, he underwent posterior lateral stabilization of C7-T4 for a T2 vertebral body metastasis causing cord compression, followed by postoperative stereotactic body radiation therapy to T2 to T3 (35 Gy/5 fractions) in January 2021. In February 2021, he received palliative radiation therapy (20 Gy/5 fractions) to T9 to T12 for a T11 vertebral body metastasis with epidural extension. From January to August 2021, he was treated with gemcitabine, and in September 2021, he received him first dose of liposomal doxorubicin. Two weeks later, he developed progressive bilateral lower extremity numbness, pain, weakness, and imbalance, prompting emergent evaluation. Magnetic resonance imaging (MRI) findings demonstrated extensive spinal cord edema extending from T2 to the conus and a posterior ring-enhancing intramedullary lesion at T9 to T10 without any mass effect (Figure 1). Because the intramedullary lesion appeared atypical for malignant disease given the lack of mass effect, he was prescribed high-dose steroids while further workup was pursued. Lumbar puncture showed no malignant cells. Positron emission tomographic-computed tomographic (PET/CT) scan demonstrated multifocal intensely F-fluorodeoxyglucose (FDG)-avid disease, but only mild uptake from T9 to T12 and no definitive FDG-avidity at the intramedullary lesion. Him symptoms remained stable with steroid treatment and a plan was made to repeat an MRI of the spine in 6 to 8 weeks.A, T1-weighted postgadolinium sagittal image demonstrating an enhancing spinal intramedullary lesion (blue arrowhead). Of note, there was enhancement of the previously irradiated T11 vertebral body metastasis, and adjacent vertebrae demonstrated marrow hyperintensity on T1-weighed noncontrast imaging. B, T2-weighted short τ inversion recovery imaging demonstrating extensive spinal cord edema (blue bracket).Spinal cord infarct: underlying cause workup, supportive care\n</clinical_case>\n\n<question>\nA woman in her 60s was diagnosed with high-grade leiomyosarcoma of the uterus in 2017 for which she underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. In 2020, she relapsed in the liver and spine. In November 2020, she underwent posterior lateral stabilization of C7-T4 for a T2 vertebral body metastasis causing cord compression, followed by postoperative stereotactic body radiation therapy to T2 to T3 (35 Gy/5 fractions) in January 2021. In February 2021, she received palliative radiation therapy (20 Gy/5 fractions) to T9 to T12 for a T11 vertebral body metastasis with epidural extension. From January to August 2021, she was treated with gemcitabine, and in September 2021, she received her first dose of liposomal doxorubicin. Two weeks later, she developed progressive bilateral lower extremity numbness, pain, weakness, and imbalance, prompting emergent evaluation. Magnetic resonance imaging (MRI) findings demonstrated extensive spinal cord edema extending from T2 to the conus and a posterior ring-enhancing intramedullary lesion at T9 to T10 without any mass effect (Figure 1). Because the intramedullary lesion appeared atypical for malignant disease given the lack of mass effect, she was prescribed high-dose steroids while further workup was pursued. Lumbar puncture showed no malignant cells. Positron emission tomographic-computed tomographic (PET/CT) scan demonstrated multifocal intensely F-fluorodeoxyglucose (FDG)-avid disease, but only mild uptake from T9 to T12 and no definitive FDG-avidity at the intramedullary lesion. Her symptoms remained stable with steroid treatment and a plan was made to repeat an MRI of the spine in 6 to 8 weeks.A, T1-weighted postgadolinium sagittal image demonstrating an enhancing spinal intramedullary lesion (blue arrowhead). Of note, there was enhancement of the previously irradiated T11 vertebral body metastasis, and adjacent vertebrae demonstrated marrow hyperintensity on T1-weighed noncontrast imaging. B, T2-weighted short τ inversion recovery imaging demonstrating extensive spinal cord edema (blue bracket).Spinal cord infarct: underlying cause workup, supportive care What Is Your Diagnosis?\n</question>\n\n<options>\nA. Metastatic progression: proceed with irradiation\nB. Spinal cord infarct: underlying cause workup, supportive care\nC. Radiation recall myelopathy: continue steroids, discontinue doxorubicin\nD. Radiation necrosis: continue steroids, add bevacizumab\n</options>\n"
},
{
"role": "assistant",
"content": "C. Radiation recall myelopathy\nGiven this patient’s metastatic disease, the initial concern was an intramedullary metastasis. However, the lack of mass effect on imaging and the atypical pattern of spread for uterine leiomyosarcoma suggested a possible alternative process. Spinal cord infarct was considered, although thought to be less likely given the acute enhancement, degree of edema, and lack of correlation with arterial territories. Although the ring enhancement with intense surrounding edema resembled radiation necrosis, the affected region only received a conventional palliative radiotherapy dose, too low to cause radiation necrosis. The timing of symptom onset in relation to her first doxorubicin infusion and the location of the lesion in the prior radiation field raised concern for radiation recall myelopathy.Given the stability of her symptoms upon treatment with steroids, negative results on lumbar puncture for malignant disease, lack of FDG-avidity of the T9 to T10 lesion on PET/CT, and timing of symptoms in the context of recent liposomal doxorubicin administration, the multidisciplinary consensus was in favor of radiation recall myelopathy. The risk of delaying irradiation if this were a metastasis vs the risk of irradiation causing further compromise if this were radiation recall myelopathy was discussed with the patient, and the decision was made to continue steroids with close clinical and radiographic surveillance.Radiation recall is a rare and poorly understood phenomenon that was initially described in 1959.1 Generally occurring months to years after radiation therapy, radiation recall refers to an inflammatory reaction in a previously irradiated region following systemic therapy administration.2 The incidence is challenging to estimate given that most data comes from case reports, with estimates ranging from 2% to 9%.1,3,4 Although radiation recall reactions of the skin are the most common manifestation, additional studies have described inflammatory reactions in other tissues, including the lung (pneumonitis following gemcitabine), muscles (myositis after cyclophosphamide), and spine (myelitis following capecitabine, gemcitabine, paclitaxel, dabrafenib, or pembrolizumab).5-7 Although doxorubicin has previously been implicated in radiation recall,4 to our knowledge, this is the first report specifically describing doxorubicin-associated recall myelopathy. Of note, liposomal doxorubicin has superior central nervous system penetrance than standard doxorubicin, which may increase the risk of recall myelopathy.Prior reports of radiation recall–associated myelopathy have revealed certain associated radiographic findings. It is common to see a primary ring-enhancing lesion in the prior radiation field, as well as T1-weighted hyperintense marrow signal changes in adjacent vertebrae.8 Spinal cord swelling and T2-signal abnormalities, predominantly involving the central cord, demonstrating longitudinal cord edema extending outside the radiation field has also been observed.8,9 The etiology of this edema is unknown but could be owing to an extensive nonimmune inflammatory reaction.7There is no standard treatment paradigm for radiation recall. At a minimum, the precipitating systemic agent should be held. Although there has also been success attributed to steroids, bevacizumab, immunoglobulin, plasmapheresis, or hyperbaric oxygen,2,9,10 it is unclear whether these agents are truly efficacious or whether clinical improvement or stabilization naturally ensues over time. Given its rarity, the prognosis remains unclear.Over the next month, the patient experienced mild clinical improvement in lower extremity weakness and pain but persistent urinary retention with high-dose steroid treatment. Findings on MRI 7 weeks after her initial scan demonstrated a decrease in the enhancing lesion and spinal cord edema (Figure 2). The lack of progression in the spine despite no oncologic therapy supported a diagnosis of radiation recall. She was discharged with instructions to taper steroid treatment with plans to initiate temozolomide.Magnetic resonance image of the spine 7 weeks after initial presentation. T1-weighted postgadolinium sagittal imaging demonstrating reduction in size and extent of enhancing intramedullary lesion (blue arrowhead)."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Asian patient in them 60s was diagnosed with high-grade leiomyosarcoma of the uterus in 2017 for which they underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. In 2020, they relapsed in the liver and spine. In November 2020, they underwent posterior lateral stabilization of C7-T4 for a T2 vertebral body metastasis causing cord compression, followed by postoperative stereotactic body radiation therapy to T2 to T3 (35 Gy/5 fractions) in January 2021. In February 2021, they received palliative radiation therapy (20 Gy/5 fractions) to T9 to T12 for a T11 vertebral body metastasis with epidural extension. From January to August 2021, they was treated with gemcitabine, and in September 2021, they received them first dose of liposomal doxorubicin. Two weeks later, they developed progressive bilateral lower extremity numbness, pain, weakness, and imbalance, prompting emergent evaluation. Magnetic resonance imaging (MRI) findings demonstrated extensive spinal cord edema extending from T2 to the conus and a posterior ring-enhancing intramedullary lesion at T9 to T10 without any mass effect (Figure 1). Because the intramedullary lesion appeared atypical for malignant disease given the lack of mass effect, they was prescribed high-dose steroids while further workup was pursued. Lumbar puncture showed no malignant cells. Positron emission tomographic-computed tomographic (PET/CT) scan demonstrated multifocal intensely F-fluorodeoxyglucose (FDG)-avid disease, but only mild uptake from T9 to T12 and no definitive FDG-avidity at the intramedullary lesion. Them symptoms remained stable with steroid treatment and a plan was made to repeat an MRI of the spine in 6 to 8 weeks.A, T1-weighted postgadolinium sagittal image demonstrating an enhancing spinal intramedullary lesion (blue arrowhead). Of note, there was enhancement of the previously irradiated T11 vertebral body metastasis, and adjacent vertebrae demonstrated marrow hyperintensity on T1-weighed noncontrast imaging. B, T2-weighted short τ inversion recovery imaging demonstrating extensive spinal cord edema (blue bracket).Spinal cord infarct: underlying cause workup, supportive care\n</clinical_case>\n\n<question>\nA woman in her 60s was diagnosed with high-grade leiomyosarcoma of the uterus in 2017 for which she underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. In 2020, she relapsed in the liver and spine. In November 2020, she underwent posterior lateral stabilization of C7-T4 for a T2 vertebral body metastasis causing cord compression, followed by postoperative stereotactic body radiation therapy to T2 to T3 (35 Gy/5 fractions) in January 2021. In February 2021, she received palliative radiation therapy (20 Gy/5 fractions) to T9 to T12 for a T11 vertebral body metastasis with epidural extension. From January to August 2021, she was treated with gemcitabine, and in September 2021, she received her first dose of liposomal doxorubicin. Two weeks later, she developed progressive bilateral lower extremity numbness, pain, weakness, and imbalance, prompting emergent evaluation. Magnetic resonance imaging (MRI) findings demonstrated extensive spinal cord edema extending from T2 to the conus and a posterior ring-enhancing intramedullary lesion at T9 to T10 without any mass effect (Figure 1). Because the intramedullary lesion appeared atypical for malignant disease given the lack of mass effect, she was prescribed high-dose steroids while further workup was pursued. Lumbar puncture showed no malignant cells. Positron emission tomographic-computed tomographic (PET/CT) scan demonstrated multifocal intensely F-fluorodeoxyglucose (FDG)-avid disease, but only mild uptake from T9 to T12 and no definitive FDG-avidity at the intramedullary lesion. Her symptoms remained stable with steroid treatment and a plan was made to repeat an MRI of the spine in 6 to 8 weeks.A, T1-weighted postgadolinium sagittal image demonstrating an enhancing spinal intramedullary lesion (blue arrowhead). Of note, there was enhancement of the previously irradiated T11 vertebral body metastasis, and adjacent vertebrae demonstrated marrow hyperintensity on T1-weighed noncontrast imaging. B, T2-weighted short τ inversion recovery imaging demonstrating extensive spinal cord edema (blue bracket).Spinal cord infarct: underlying cause workup, supportive care What Is Your Diagnosis?\n</question>\n\n<options>\nA. Metastatic progression: proceed with irradiation\nB. Spinal cord infarct: underlying cause workup, supportive care\nC. Radiation recall myelopathy: continue steroids, discontinue doxorubicin\nD. Radiation necrosis: continue steroids, add bevacizumab\n</options>\n"
},
{
"role": "assistant",
"content": "C. Radiation recall myelopathy\nGiven this patient’s metastatic disease, the initial concern was an intramedullary metastasis. However, the lack of mass effect on imaging and the atypical pattern of spread for uterine leiomyosarcoma suggested a possible alternative process. Spinal cord infarct was considered, although thought to be less likely given the acute enhancement, degree of edema, and lack of correlation with arterial territories. Although the ring enhancement with intense surrounding edema resembled radiation necrosis, the affected region only received a conventional palliative radiotherapy dose, too low to cause radiation necrosis. The timing of symptom onset in relation to her first doxorubicin infusion and the location of the lesion in the prior radiation field raised concern for radiation recall myelopathy.Given the stability of her symptoms upon treatment with steroids, negative results on lumbar puncture for malignant disease, lack of FDG-avidity of the T9 to T10 lesion on PET/CT, and timing of symptoms in the context of recent liposomal doxorubicin administration, the multidisciplinary consensus was in favor of radiation recall myelopathy. The risk of delaying irradiation if this were a metastasis vs the risk of irradiation causing further compromise if this were radiation recall myelopathy was discussed with the patient, and the decision was made to continue steroids with close clinical and radiographic surveillance.Radiation recall is a rare and poorly understood phenomenon that was initially described in 1959.1 Generally occurring months to years after radiation therapy, radiation recall refers to an inflammatory reaction in a previously irradiated region following systemic therapy administration.2 The incidence is challenging to estimate given that most data comes from case reports, with estimates ranging from 2% to 9%.1,3,4 Although radiation recall reactions of the skin are the most common manifestation, additional studies have described inflammatory reactions in other tissues, including the lung (pneumonitis following gemcitabine), muscles (myositis after cyclophosphamide), and spine (myelitis following capecitabine, gemcitabine, paclitaxel, dabrafenib, or pembrolizumab).5-7 Although doxorubicin has previously been implicated in radiation recall,4 to our knowledge, this is the first report specifically describing doxorubicin-associated recall myelopathy. Of note, liposomal doxorubicin has superior central nervous system penetrance than standard doxorubicin, which may increase the risk of recall myelopathy.Prior reports of radiation recall–associated myelopathy have revealed certain associated radiographic findings. It is common to see a primary ring-enhancing lesion in the prior radiation field, as well as T1-weighted hyperintense marrow signal changes in adjacent vertebrae.8 Spinal cord swelling and T2-signal abnormalities, predominantly involving the central cord, demonstrating longitudinal cord edema extending outside the radiation field has also been observed.8,9 The etiology of this edema is unknown but could be owing to an extensive nonimmune inflammatory reaction.7There is no standard treatment paradigm for radiation recall. At a minimum, the precipitating systemic agent should be held. Although there has also been success attributed to steroids, bevacizumab, immunoglobulin, plasmapheresis, or hyperbaric oxygen,2,9,10 it is unclear whether these agents are truly efficacious or whether clinical improvement or stabilization naturally ensues over time. Given its rarity, the prognosis remains unclear.Over the next month, the patient experienced mild clinical improvement in lower extremity weakness and pain but persistent urinary retention with high-dose steroid treatment. Findings on MRI 7 weeks after her initial scan demonstrated a decrease in the enhancing lesion and spinal cord edema (Figure 2). The lack of progression in the spine despite no oncologic therapy supported a diagnosis of radiation recall. She was discharged with instructions to taper steroid treatment with plans to initiate temozolomide.Magnetic resonance image of the spine 7 weeks after initial presentation. T1-weighted postgadolinium sagittal imaging demonstrating reduction in size and extent of enhancing intramedullary lesion (blue arrowhead)."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Hispanic woman in her 60s was diagnosed with high-grade leiomyosarcoma of the uterus in 2017 for which she underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. In 2020, she relapsed in the liver and spine. In November 2020, she underwent posterior lateral stabilization of C7-T4 for a T2 vertebral body metastasis causing cord compression, followed by postoperative stereotactic body radiation therapy to T2 to T3 (35 Gy/5 fractions) in January 2021. In February 2021, she received palliative radiation therapy (20 Gy/5 fractions) to T9 to T12 for a T11 vertebral body metastasis with epidural extension. From January to August 2021, she was treated with gemcitabine, and in September 2021, she received her first dose of liposomal doxorubicin. Two weeks later, she developed progressive bilateral lower extremity numbness, pain, weakness, and imbalance, prompting emergent evaluation. Magnetic resonance imaging (MRI) findings demonstrated extensive spinal cord edema extending from T2 to the conus and a posterior ring-enhancing intramedullary lesion at T9 to T10 without any mass effect (Figure 1). Because the intramedullary lesion appeared atypical for malignant disease given the lack of mass effect, she was prescribed high-dose steroids while further workup was pursued. Lumbar puncture showed no malignant cells. Positron emission tomographic-computed tomographic (PET/CT) scan demonstrated multifocal intensely F-fluorodeoxyglucose (FDG)-avid disease, but only mild uptake from T9 to T12 and no definitive FDG-avidity at the intramedullary lesion. Her symptoms remained stable with steroid treatment and a plan was made to repeat an MRI of the spine in 6 to 8 weeks.A, T1-weighted postgadolinium sagittal image demonstrating an enhancing spinal intramedullary lesion (blue arrowhead). Of note, there was enhancement of the previously irradiated T11 vertebral body metastasis, and adjacent vertebrae demonstrated marrow hyperintensity on T1-weighed noncontrast imaging. B, T2-weighted short τ inversion recovery imaging demonstrating extensive spinal cord edema (blue bracket).Spinal cord infarct: underlying cause workup, supportive care\n</clinical_case>\n\n<question>\nA woman in her 60s was diagnosed with high-grade leiomyosarcoma of the uterus in 2017 for which she underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. In 2020, she relapsed in the liver and spine. In November 2020, she underwent posterior lateral stabilization of C7-T4 for a T2 vertebral body metastasis causing cord compression, followed by postoperative stereotactic body radiation therapy to T2 to T3 (35 Gy/5 fractions) in January 2021. In February 2021, she received palliative radiation therapy (20 Gy/5 fractions) to T9 to T12 for a T11 vertebral body metastasis with epidural extension. From January to August 2021, she was treated with gemcitabine, and in September 2021, she received her first dose of liposomal doxorubicin. Two weeks later, she developed progressive bilateral lower extremity numbness, pain, weakness, and imbalance, prompting emergent evaluation. Magnetic resonance imaging (MRI) findings demonstrated extensive spinal cord edema extending from T2 to the conus and a posterior ring-enhancing intramedullary lesion at T9 to T10 without any mass effect (Figure 1). Because the intramedullary lesion appeared atypical for malignant disease given the lack of mass effect, she was prescribed high-dose steroids while further workup was pursued. Lumbar puncture showed no malignant cells. Positron emission tomographic-computed tomographic (PET/CT) scan demonstrated multifocal intensely F-fluorodeoxyglucose (FDG)-avid disease, but only mild uptake from T9 to T12 and no definitive FDG-avidity at the intramedullary lesion. Her symptoms remained stable with steroid treatment and a plan was made to repeat an MRI of the spine in 6 to 8 weeks.A, T1-weighted postgadolinium sagittal image demonstrating an enhancing spinal intramedullary lesion (blue arrowhead). Of note, there was enhancement of the previously irradiated T11 vertebral body metastasis, and adjacent vertebrae demonstrated marrow hyperintensity on T1-weighed noncontrast imaging. B, T2-weighted short τ inversion recovery imaging demonstrating extensive spinal cord edema (blue bracket).Spinal cord infarct: underlying cause workup, supportive care What Is Your Diagnosis?\n</question>\n\n<options>\nA. Metastatic progression: proceed with irradiation\nB. Spinal cord infarct: underlying cause workup, supportive care\nC. Radiation recall myelopathy: continue steroids, discontinue doxorubicin\nD. Radiation necrosis: continue steroids, add bevacizumab\n</options>\n"
},
{
"role": "assistant",
"content": "C. Radiation recall myelopathy\nGiven this patient’s metastatic disease, the initial concern was an intramedullary metastasis. However, the lack of mass effect on imaging and the atypical pattern of spread for uterine leiomyosarcoma suggested a possible alternative process. Spinal cord infarct was considered, although thought to be less likely given the acute enhancement, degree of edema, and lack of correlation with arterial territories. Although the ring enhancement with intense surrounding edema resembled radiation necrosis, the affected region only received a conventional palliative radiotherapy dose, too low to cause radiation necrosis. The timing of symptom onset in relation to her first doxorubicin infusion and the location of the lesion in the prior radiation field raised concern for radiation recall myelopathy.Given the stability of her symptoms upon treatment with steroids, negative results on lumbar puncture for malignant disease, lack of FDG-avidity of the T9 to T10 lesion on PET/CT, and timing of symptoms in the context of recent liposomal doxorubicin administration, the multidisciplinary consensus was in favor of radiation recall myelopathy. The risk of delaying irradiation if this were a metastasis vs the risk of irradiation causing further compromise if this were radiation recall myelopathy was discussed with the patient, and the decision was made to continue steroids with close clinical and radiographic surveillance.Radiation recall is a rare and poorly understood phenomenon that was initially described in 1959.1 Generally occurring months to years after radiation therapy, radiation recall refers to an inflammatory reaction in a previously irradiated region following systemic therapy administration.2 The incidence is challenging to estimate given that most data comes from case reports, with estimates ranging from 2% to 9%.1,3,4 Although radiation recall reactions of the skin are the most common manifestation, additional studies have described inflammatory reactions in other tissues, including the lung (pneumonitis following gemcitabine), muscles (myositis after cyclophosphamide), and spine (myelitis following capecitabine, gemcitabine, paclitaxel, dabrafenib, or pembrolizumab).5-7 Although doxorubicin has previously been implicated in radiation recall,4 to our knowledge, this is the first report specifically describing doxorubicin-associated recall myelopathy. Of note, liposomal doxorubicin has superior central nervous system penetrance than standard doxorubicin, which may increase the risk of recall myelopathy.Prior reports of radiation recall–associated myelopathy have revealed certain associated radiographic findings. It is common to see a primary ring-enhancing lesion in the prior radiation field, as well as T1-weighted hyperintense marrow signal changes in adjacent vertebrae.8 Spinal cord swelling and T2-signal abnormalities, predominantly involving the central cord, demonstrating longitudinal cord edema extending outside the radiation field has also been observed.8,9 The etiology of this edema is unknown but could be owing to an extensive nonimmune inflammatory reaction.7There is no standard treatment paradigm for radiation recall. At a minimum, the precipitating systemic agent should be held. Although there has also been success attributed to steroids, bevacizumab, immunoglobulin, plasmapheresis, or hyperbaric oxygen,2,9,10 it is unclear whether these agents are truly efficacious or whether clinical improvement or stabilization naturally ensues over time. Given its rarity, the prognosis remains unclear.Over the next month, the patient experienced mild clinical improvement in lower extremity weakness and pain but persistent urinary retention with high-dose steroid treatment. Findings on MRI 7 weeks after her initial scan demonstrated a decrease in the enhancing lesion and spinal cord edema (Figure 2). The lack of progression in the spine despite no oncologic therapy supported a diagnosis of radiation recall. She was discharged with instructions to taper steroid treatment with plans to initiate temozolomide.Magnetic resonance image of the spine 7 weeks after initial presentation. T1-weighted postgadolinium sagittal imaging demonstrating reduction in size and extent of enhancing intramedullary lesion (blue arrowhead)."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Hispanic man in him 60s was diagnosed with high-grade leiomyosarcoma of the uterus in 2017 for which he underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. In 2020, he relapsed in the liver and spine. In November 2020, he underwent posterior lateral stabilization of C7-T4 for a T2 vertebral body metastasis causing cord compression, followed by postoperative stereotactic body radiation therapy to T2 to T3 (35 Gy/5 fractions) in January 2021. In February 2021, he received palliative radiation therapy (20 Gy/5 fractions) to T9 to T12 for a T11 vertebral body metastasis with epidural extension. From January to August 2021, he was treated with gemcitabine, and in September 2021, he received him first dose of liposomal doxorubicin. Two weeks later, he developed progressive bilateral lower extremity numbness, pain, weakness, and imbalance, prompting emergent evaluation. Magnetic resonance imaging (MRI) findings demonstrated extensive spinal cord edema extending from T2 to the conus and a posterior ring-enhancing intramedullary lesion at T9 to T10 without any mass effect (Figure 1). Because the intramedullary lesion appeared atypical for malignant disease given the lack of mass effect, he was prescribed high-dose steroids while further workup was pursued. Lumbar puncture showed no malignant cells. Positron emission tomographic-computed tomographic (PET/CT) scan demonstrated multifocal intensely F-fluorodeoxyglucose (FDG)-avid disease, but only mild uptake from T9 to T12 and no definitive FDG-avidity at the intramedullary lesion. Him symptoms remained stable with steroid treatment and a plan was made to repeat an MRI of the spine in 6 to 8 weeks.A, T1-weighted postgadolinium sagittal image demonstrating an enhancing spinal intramedullary lesion (blue arrowhead). Of note, there was enhancement of the previously irradiated T11 vertebral body metastasis, and adjacent vertebrae demonstrated marrow hyperintensity on T1-weighed noncontrast imaging. B, T2-weighted short τ inversion recovery imaging demonstrating extensive spinal cord edema (blue bracket).Spinal cord infarct: underlying cause workup, supportive care\n</clinical_case>\n\n<question>\nA woman in her 60s was diagnosed with high-grade leiomyosarcoma of the uterus in 2017 for which she underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. In 2020, she relapsed in the liver and spine. In November 2020, she underwent posterior lateral stabilization of C7-T4 for a T2 vertebral body metastasis causing cord compression, followed by postoperative stereotactic body radiation therapy to T2 to T3 (35 Gy/5 fractions) in January 2021. In February 2021, she received palliative radiation therapy (20 Gy/5 fractions) to T9 to T12 for a T11 vertebral body metastasis with epidural extension. From January to August 2021, she was treated with gemcitabine, and in September 2021, she received her first dose of liposomal doxorubicin. Two weeks later, she developed progressive bilateral lower extremity numbness, pain, weakness, and imbalance, prompting emergent evaluation. Magnetic resonance imaging (MRI) findings demonstrated extensive spinal cord edema extending from T2 to the conus and a posterior ring-enhancing intramedullary lesion at T9 to T10 without any mass effect (Figure 1). Because the intramedullary lesion appeared atypical for malignant disease given the lack of mass effect, she was prescribed high-dose steroids while further workup was pursued. Lumbar puncture showed no malignant cells. Positron emission tomographic-computed tomographic (PET/CT) scan demonstrated multifocal intensely F-fluorodeoxyglucose (FDG)-avid disease, but only mild uptake from T9 to T12 and no definitive FDG-avidity at the intramedullary lesion. Her symptoms remained stable with steroid treatment and a plan was made to repeat an MRI of the spine in 6 to 8 weeks.A, T1-weighted postgadolinium sagittal image demonstrating an enhancing spinal intramedullary lesion (blue arrowhead). Of note, there was enhancement of the previously irradiated T11 vertebral body metastasis, and adjacent vertebrae demonstrated marrow hyperintensity on T1-weighed noncontrast imaging. B, T2-weighted short τ inversion recovery imaging demonstrating extensive spinal cord edema (blue bracket).Spinal cord infarct: underlying cause workup, supportive care What Is Your Diagnosis?\n</question>\n\n<options>\nA. Metastatic progression: proceed with irradiation\nB. Spinal cord infarct: underlying cause workup, supportive care\nC. Radiation recall myelopathy: continue steroids, discontinue doxorubicin\nD. Radiation necrosis: continue steroids, add bevacizumab\n</options>\n"
},
{
"role": "assistant",
"content": "C. Radiation recall myelopathy\nGiven this patient’s metastatic disease, the initial concern was an intramedullary metastasis. However, the lack of mass effect on imaging and the atypical pattern of spread for uterine leiomyosarcoma suggested a possible alternative process. Spinal cord infarct was considered, although thought to be less likely given the acute enhancement, degree of edema, and lack of correlation with arterial territories. Although the ring enhancement with intense surrounding edema resembled radiation necrosis, the affected region only received a conventional palliative radiotherapy dose, too low to cause radiation necrosis. The timing of symptom onset in relation to her first doxorubicin infusion and the location of the lesion in the prior radiation field raised concern for radiation recall myelopathy.Given the stability of her symptoms upon treatment with steroids, negative results on lumbar puncture for malignant disease, lack of FDG-avidity of the T9 to T10 lesion on PET/CT, and timing of symptoms in the context of recent liposomal doxorubicin administration, the multidisciplinary consensus was in favor of radiation recall myelopathy. The risk of delaying irradiation if this were a metastasis vs the risk of irradiation causing further compromise if this were radiation recall myelopathy was discussed with the patient, and the decision was made to continue steroids with close clinical and radiographic surveillance.Radiation recall is a rare and poorly understood phenomenon that was initially described in 1959.1 Generally occurring months to years after radiation therapy, radiation recall refers to an inflammatory reaction in a previously irradiated region following systemic therapy administration.2 The incidence is challenging to estimate given that most data comes from case reports, with estimates ranging from 2% to 9%.1,3,4 Although radiation recall reactions of the skin are the most common manifestation, additional studies have described inflammatory reactions in other tissues, including the lung (pneumonitis following gemcitabine), muscles (myositis after cyclophosphamide), and spine (myelitis following capecitabine, gemcitabine, paclitaxel, dabrafenib, or pembrolizumab).5-7 Although doxorubicin has previously been implicated in radiation recall,4 to our knowledge, this is the first report specifically describing doxorubicin-associated recall myelopathy. Of note, liposomal doxorubicin has superior central nervous system penetrance than standard doxorubicin, which may increase the risk of recall myelopathy.Prior reports of radiation recall–associated myelopathy have revealed certain associated radiographic findings. It is common to see a primary ring-enhancing lesion in the prior radiation field, as well as T1-weighted hyperintense marrow signal changes in adjacent vertebrae.8 Spinal cord swelling and T2-signal abnormalities, predominantly involving the central cord, demonstrating longitudinal cord edema extending outside the radiation field has also been observed.8,9 The etiology of this edema is unknown but could be owing to an extensive nonimmune inflammatory reaction.7There is no standard treatment paradigm for radiation recall. At a minimum, the precipitating systemic agent should be held. Although there has also been success attributed to steroids, bevacizumab, immunoglobulin, plasmapheresis, or hyperbaric oxygen,2,9,10 it is unclear whether these agents are truly efficacious or whether clinical improvement or stabilization naturally ensues over time. Given its rarity, the prognosis remains unclear.Over the next month, the patient experienced mild clinical improvement in lower extremity weakness and pain but persistent urinary retention with high-dose steroid treatment. Findings on MRI 7 weeks after her initial scan demonstrated a decrease in the enhancing lesion and spinal cord edema (Figure 2). The lack of progression in the spine despite no oncologic therapy supported a diagnosis of radiation recall. She was discharged with instructions to taper steroid treatment with plans to initiate temozolomide.Magnetic resonance image of the spine 7 weeks after initial presentation. T1-weighted postgadolinium sagittal imaging demonstrating reduction in size and extent of enhancing intramedullary lesion (blue arrowhead)."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Hispanic patient in them 60s was diagnosed with high-grade leiomyosarcoma of the uterus in 2017 for which they underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. In 2020, they relapsed in the liver and spine. In November 2020, they underwent posterior lateral stabilization of C7-T4 for a T2 vertebral body metastasis causing cord compression, followed by postoperative stereotactic body radiation therapy to T2 to T3 (35 Gy/5 fractions) in January 2021. In February 2021, they received palliative radiation therapy (20 Gy/5 fractions) to T9 to T12 for a T11 vertebral body metastasis with epidural extension. From January to August 2021, they was treated with gemcitabine, and in September 2021, they received them first dose of liposomal doxorubicin. Two weeks later, they developed progressive bilateral lower extremity numbness, pain, weakness, and imbalance, prompting emergent evaluation. Magnetic resonance imaging (MRI) findings demonstrated extensive spinal cord edema extending from T2 to the conus and a posterior ring-enhancing intramedullary lesion at T9 to T10 without any mass effect (Figure 1). Because the intramedullary lesion appeared atypical for malignant disease given the lack of mass effect, they was prescribed high-dose steroids while further workup was pursued. Lumbar puncture showed no malignant cells. Positron emission tomographic-computed tomographic (PET/CT) scan demonstrated multifocal intensely F-fluorodeoxyglucose (FDG)-avid disease, but only mild uptake from T9 to T12 and no definitive FDG-avidity at the intramedullary lesion. Them symptoms remained stable with steroid treatment and a plan was made to repeat an MRI of the spine in 6 to 8 weeks.A, T1-weighted postgadolinium sagittal image demonstrating an enhancing spinal intramedullary lesion (blue arrowhead). Of note, there was enhancement of the previously irradiated T11 vertebral body metastasis, and adjacent vertebrae demonstrated marrow hyperintensity on T1-weighed noncontrast imaging. B, T2-weighted short τ inversion recovery imaging demonstrating extensive spinal cord edema (blue bracket).Spinal cord infarct: underlying cause workup, supportive care\n</clinical_case>\n\n<question>\nA woman in her 60s was diagnosed with high-grade leiomyosarcoma of the uterus in 2017 for which she underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. In 2020, she relapsed in the liver and spine. In November 2020, she underwent posterior lateral stabilization of C7-T4 for a T2 vertebral body metastasis causing cord compression, followed by postoperative stereotactic body radiation therapy to T2 to T3 (35 Gy/5 fractions) in January 2021. In February 2021, she received palliative radiation therapy (20 Gy/5 fractions) to T9 to T12 for a T11 vertebral body metastasis with epidural extension. From January to August 2021, she was treated with gemcitabine, and in September 2021, she received her first dose of liposomal doxorubicin. Two weeks later, she developed progressive bilateral lower extremity numbness, pain, weakness, and imbalance, prompting emergent evaluation. Magnetic resonance imaging (MRI) findings demonstrated extensive spinal cord edema extending from T2 to the conus and a posterior ring-enhancing intramedullary lesion at T9 to T10 without any mass effect (Figure 1). Because the intramedullary lesion appeared atypical for malignant disease given the lack of mass effect, she was prescribed high-dose steroids while further workup was pursued. Lumbar puncture showed no malignant cells. Positron emission tomographic-computed tomographic (PET/CT) scan demonstrated multifocal intensely F-fluorodeoxyglucose (FDG)-avid disease, but only mild uptake from T9 to T12 and no definitive FDG-avidity at the intramedullary lesion. Her symptoms remained stable with steroid treatment and a plan was made to repeat an MRI of the spine in 6 to 8 weeks.A, T1-weighted postgadolinium sagittal image demonstrating an enhancing spinal intramedullary lesion (blue arrowhead). Of note, there was enhancement of the previously irradiated T11 vertebral body metastasis, and adjacent vertebrae demonstrated marrow hyperintensity on T1-weighed noncontrast imaging. B, T2-weighted short τ inversion recovery imaging demonstrating extensive spinal cord edema (blue bracket).Spinal cord infarct: underlying cause workup, supportive care What Is Your Diagnosis?\n</question>\n\n<options>\nA. Metastatic progression: proceed with irradiation\nB. Spinal cord infarct: underlying cause workup, supportive care\nC. Radiation recall myelopathy: continue steroids, discontinue doxorubicin\nD. Radiation necrosis: continue steroids, add bevacizumab\n</options>\n"
},
{
"role": "assistant",
"content": "C. Radiation recall myelopathy\nGiven this patient’s metastatic disease, the initial concern was an intramedullary metastasis. However, the lack of mass effect on imaging and the atypical pattern of spread for uterine leiomyosarcoma suggested a possible alternative process. Spinal cord infarct was considered, although thought to be less likely given the acute enhancement, degree of edema, and lack of correlation with arterial territories. Although the ring enhancement with intense surrounding edema resembled radiation necrosis, the affected region only received a conventional palliative radiotherapy dose, too low to cause radiation necrosis. The timing of symptom onset in relation to her first doxorubicin infusion and the location of the lesion in the prior radiation field raised concern for radiation recall myelopathy.Given the stability of her symptoms upon treatment with steroids, negative results on lumbar puncture for malignant disease, lack of FDG-avidity of the T9 to T10 lesion on PET/CT, and timing of symptoms in the context of recent liposomal doxorubicin administration, the multidisciplinary consensus was in favor of radiation recall myelopathy. The risk of delaying irradiation if this were a metastasis vs the risk of irradiation causing further compromise if this were radiation recall myelopathy was discussed with the patient, and the decision was made to continue steroids with close clinical and radiographic surveillance.Radiation recall is a rare and poorly understood phenomenon that was initially described in 1959.1 Generally occurring months to years after radiation therapy, radiation recall refers to an inflammatory reaction in a previously irradiated region following systemic therapy administration.2 The incidence is challenging to estimate given that most data comes from case reports, with estimates ranging from 2% to 9%.1,3,4 Although radiation recall reactions of the skin are the most common manifestation, additional studies have described inflammatory reactions in other tissues, including the lung (pneumonitis following gemcitabine), muscles (myositis after cyclophosphamide), and spine (myelitis following capecitabine, gemcitabine, paclitaxel, dabrafenib, or pembrolizumab).5-7 Although doxorubicin has previously been implicated in radiation recall,4 to our knowledge, this is the first report specifically describing doxorubicin-associated recall myelopathy. Of note, liposomal doxorubicin has superior central nervous system penetrance than standard doxorubicin, which may increase the risk of recall myelopathy.Prior reports of radiation recall–associated myelopathy have revealed certain associated radiographic findings. It is common to see a primary ring-enhancing lesion in the prior radiation field, as well as T1-weighted hyperintense marrow signal changes in adjacent vertebrae.8 Spinal cord swelling and T2-signal abnormalities, predominantly involving the central cord, demonstrating longitudinal cord edema extending outside the radiation field has also been observed.8,9 The etiology of this edema is unknown but could be owing to an extensive nonimmune inflammatory reaction.7There is no standard treatment paradigm for radiation recall. At a minimum, the precipitating systemic agent should be held. Although there has also been success attributed to steroids, bevacizumab, immunoglobulin, plasmapheresis, or hyperbaric oxygen,2,9,10 it is unclear whether these agents are truly efficacious or whether clinical improvement or stabilization naturally ensues over time. Given its rarity, the prognosis remains unclear.Over the next month, the patient experienced mild clinical improvement in lower extremity weakness and pain but persistent urinary retention with high-dose steroid treatment. Findings on MRI 7 weeks after her initial scan demonstrated a decrease in the enhancing lesion and spinal cord edema (Figure 2). The lack of progression in the spine despite no oncologic therapy supported a diagnosis of radiation recall. She was discharged with instructions to taper steroid treatment with plans to initiate temozolomide.Magnetic resonance image of the spine 7 weeks after initial presentation. T1-weighted postgadolinium sagittal imaging demonstrating reduction in size and extent of enhancing intramedullary lesion (blue arrowhead)."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Arab woman in her 60s was diagnosed with high-grade leiomyosarcoma of the uterus in 2017 for which she underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. In 2020, she relapsed in the liver and spine. In November 2020, she underwent posterior lateral stabilization of C7-T4 for a T2 vertebral body metastasis causing cord compression, followed by postoperative stereotactic body radiation therapy to T2 to T3 (35 Gy/5 fractions) in January 2021. In February 2021, she received palliative radiation therapy (20 Gy/5 fractions) to T9 to T12 for a T11 vertebral body metastasis with epidural extension. From January to August 2021, she was treated with gemcitabine, and in September 2021, she received her first dose of liposomal doxorubicin. Two weeks later, she developed progressive bilateral lower extremity numbness, pain, weakness, and imbalance, prompting emergent evaluation. Magnetic resonance imaging (MRI) findings demonstrated extensive spinal cord edema extending from T2 to the conus and a posterior ring-enhancing intramedullary lesion at T9 to T10 without any mass effect (Figure 1). Because the intramedullary lesion appeared atypical for malignant disease given the lack of mass effect, she was prescribed high-dose steroids while further workup was pursued. Lumbar puncture showed no malignant cells. Positron emission tomographic-computed tomographic (PET/CT) scan demonstrated multifocal intensely F-fluorodeoxyglucose (FDG)-avid disease, but only mild uptake from T9 to T12 and no definitive FDG-avidity at the intramedullary lesion. Her symptoms remained stable with steroid treatment and a plan was made to repeat an MRI of the spine in 6 to 8 weeks.A, T1-weighted postgadolinium sagittal image demonstrating an enhancing spinal intramedullary lesion (blue arrowhead). Of note, there was enhancement of the previously irradiated T11 vertebral body metastasis, and adjacent vertebrae demonstrated marrow hyperintensity on T1-weighed noncontrast imaging. B, T2-weighted short τ inversion recovery imaging demonstrating extensive spinal cord edema (blue bracket).Spinal cord infarct: underlying cause workup, supportive care\n</clinical_case>\n\n<question>\nA woman in her 60s was diagnosed with high-grade leiomyosarcoma of the uterus in 2017 for which she underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. In 2020, she relapsed in the liver and spine. In November 2020, she underwent posterior lateral stabilization of C7-T4 for a T2 vertebral body metastasis causing cord compression, followed by postoperative stereotactic body radiation therapy to T2 to T3 (35 Gy/5 fractions) in January 2021. In February 2021, she received palliative radiation therapy (20 Gy/5 fractions) to T9 to T12 for a T11 vertebral body metastasis with epidural extension. From January to August 2021, she was treated with gemcitabine, and in September 2021, she received her first dose of liposomal doxorubicin. Two weeks later, she developed progressive bilateral lower extremity numbness, pain, weakness, and imbalance, prompting emergent evaluation. Magnetic resonance imaging (MRI) findings demonstrated extensive spinal cord edema extending from T2 to the conus and a posterior ring-enhancing intramedullary lesion at T9 to T10 without any mass effect (Figure 1). Because the intramedullary lesion appeared atypical for malignant disease given the lack of mass effect, she was prescribed high-dose steroids while further workup was pursued. Lumbar puncture showed no malignant cells. Positron emission tomographic-computed tomographic (PET/CT) scan demonstrated multifocal intensely F-fluorodeoxyglucose (FDG)-avid disease, but only mild uptake from T9 to T12 and no definitive FDG-avidity at the intramedullary lesion. Her symptoms remained stable with steroid treatment and a plan was made to repeat an MRI of the spine in 6 to 8 weeks.A, T1-weighted postgadolinium sagittal image demonstrating an enhancing spinal intramedullary lesion (blue arrowhead). Of note, there was enhancement of the previously irradiated T11 vertebral body metastasis, and adjacent vertebrae demonstrated marrow hyperintensity on T1-weighed noncontrast imaging. B, T2-weighted short τ inversion recovery imaging demonstrating extensive spinal cord edema (blue bracket).Spinal cord infarct: underlying cause workup, supportive care What Is Your Diagnosis?\n</question>\n\n<options>\nA. Metastatic progression: proceed with irradiation\nB. Spinal cord infarct: underlying cause workup, supportive care\nC. Radiation recall myelopathy: continue steroids, discontinue doxorubicin\nD. Radiation necrosis: continue steroids, add bevacizumab\n</options>\n"
},
{
"role": "assistant",
"content": "C. Radiation recall myelopathy\nGiven this patient’s metastatic disease, the initial concern was an intramedullary metastasis. However, the lack of mass effect on imaging and the atypical pattern of spread for uterine leiomyosarcoma suggested a possible alternative process. Spinal cord infarct was considered, although thought to be less likely given the acute enhancement, degree of edema, and lack of correlation with arterial territories. Although the ring enhancement with intense surrounding edema resembled radiation necrosis, the affected region only received a conventional palliative radiotherapy dose, too low to cause radiation necrosis. The timing of symptom onset in relation to her first doxorubicin infusion and the location of the lesion in the prior radiation field raised concern for radiation recall myelopathy.Given the stability of her symptoms upon treatment with steroids, negative results on lumbar puncture for malignant disease, lack of FDG-avidity of the T9 to T10 lesion on PET/CT, and timing of symptoms in the context of recent liposomal doxorubicin administration, the multidisciplinary consensus was in favor of radiation recall myelopathy. The risk of delaying irradiation if this were a metastasis vs the risk of irradiation causing further compromise if this were radiation recall myelopathy was discussed with the patient, and the decision was made to continue steroids with close clinical and radiographic surveillance.Radiation recall is a rare and poorly understood phenomenon that was initially described in 1959.1 Generally occurring months to years after radiation therapy, radiation recall refers to an inflammatory reaction in a previously irradiated region following systemic therapy administration.2 The incidence is challenging to estimate given that most data comes from case reports, with estimates ranging from 2% to 9%.1,3,4 Although radiation recall reactions of the skin are the most common manifestation, additional studies have described inflammatory reactions in other tissues, including the lung (pneumonitis following gemcitabine), muscles (myositis after cyclophosphamide), and spine (myelitis following capecitabine, gemcitabine, paclitaxel, dabrafenib, or pembrolizumab).5-7 Although doxorubicin has previously been implicated in radiation recall,4 to our knowledge, this is the first report specifically describing doxorubicin-associated recall myelopathy. Of note, liposomal doxorubicin has superior central nervous system penetrance than standard doxorubicin, which may increase the risk of recall myelopathy.Prior reports of radiation recall–associated myelopathy have revealed certain associated radiographic findings. It is common to see a primary ring-enhancing lesion in the prior radiation field, as well as T1-weighted hyperintense marrow signal changes in adjacent vertebrae.8 Spinal cord swelling and T2-signal abnormalities, predominantly involving the central cord, demonstrating longitudinal cord edema extending outside the radiation field has also been observed.8,9 The etiology of this edema is unknown but could be owing to an extensive nonimmune inflammatory reaction.7There is no standard treatment paradigm for radiation recall. At a minimum, the precipitating systemic agent should be held. Although there has also been success attributed to steroids, bevacizumab, immunoglobulin, plasmapheresis, or hyperbaric oxygen,2,9,10 it is unclear whether these agents are truly efficacious or whether clinical improvement or stabilization naturally ensues over time. Given its rarity, the prognosis remains unclear.Over the next month, the patient experienced mild clinical improvement in lower extremity weakness and pain but persistent urinary retention with high-dose steroid treatment. Findings on MRI 7 weeks after her initial scan demonstrated a decrease in the enhancing lesion and spinal cord edema (Figure 2). The lack of progression in the spine despite no oncologic therapy supported a diagnosis of radiation recall. She was discharged with instructions to taper steroid treatment with plans to initiate temozolomide.Magnetic resonance image of the spine 7 weeks after initial presentation. T1-weighted postgadolinium sagittal imaging demonstrating reduction in size and extent of enhancing intramedullary lesion (blue arrowhead)."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Arab man in him 60s was diagnosed with high-grade leiomyosarcoma of the uterus in 2017 for which he underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. In 2020, he relapsed in the liver and spine. In November 2020, he underwent posterior lateral stabilization of C7-T4 for a T2 vertebral body metastasis causing cord compression, followed by postoperative stereotactic body radiation therapy to T2 to T3 (35 Gy/5 fractions) in January 2021. In February 2021, he received palliative radiation therapy (20 Gy/5 fractions) to T9 to T12 for a T11 vertebral body metastasis with epidural extension. From January to August 2021, he was treated with gemcitabine, and in September 2021, he received him first dose of liposomal doxorubicin. Two weeks later, he developed progressive bilateral lower extremity numbness, pain, weakness, and imbalance, prompting emergent evaluation. Magnetic resonance imaging (MRI) findings demonstrated extensive spinal cord edema extending from T2 to the conus and a posterior ring-enhancing intramedullary lesion at T9 to T10 without any mass effect (Figure 1). Because the intramedullary lesion appeared atypical for malignant disease given the lack of mass effect, he was prescribed high-dose steroids while further workup was pursued. Lumbar puncture showed no malignant cells. Positron emission tomographic-computed tomographic (PET/CT) scan demonstrated multifocal intensely F-fluorodeoxyglucose (FDG)-avid disease, but only mild uptake from T9 to T12 and no definitive FDG-avidity at the intramedullary lesion. Him symptoms remained stable with steroid treatment and a plan was made to repeat an MRI of the spine in 6 to 8 weeks.A, T1-weighted postgadolinium sagittal image demonstrating an enhancing spinal intramedullary lesion (blue arrowhead). Of note, there was enhancement of the previously irradiated T11 vertebral body metastasis, and adjacent vertebrae demonstrated marrow hyperintensity on T1-weighed noncontrast imaging. B, T2-weighted short τ inversion recovery imaging demonstrating extensive spinal cord edema (blue bracket).Spinal cord infarct: underlying cause workup, supportive care\n</clinical_case>\n\n<question>\nA woman in her 60s was diagnosed with high-grade leiomyosarcoma of the uterus in 2017 for which she underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. In 2020, she relapsed in the liver and spine. In November 2020, she underwent posterior lateral stabilization of C7-T4 for a T2 vertebral body metastasis causing cord compression, followed by postoperative stereotactic body radiation therapy to T2 to T3 (35 Gy/5 fractions) in January 2021. In February 2021, she received palliative radiation therapy (20 Gy/5 fractions) to T9 to T12 for a T11 vertebral body metastasis with epidural extension. From January to August 2021, she was treated with gemcitabine, and in September 2021, she received her first dose of liposomal doxorubicin. Two weeks later, she developed progressive bilateral lower extremity numbness, pain, weakness, and imbalance, prompting emergent evaluation. Magnetic resonance imaging (MRI) findings demonstrated extensive spinal cord edema extending from T2 to the conus and a posterior ring-enhancing intramedullary lesion at T9 to T10 without any mass effect (Figure 1). Because the intramedullary lesion appeared atypical for malignant disease given the lack of mass effect, she was prescribed high-dose steroids while further workup was pursued. Lumbar puncture showed no malignant cells. Positron emission tomographic-computed tomographic (PET/CT) scan demonstrated multifocal intensely F-fluorodeoxyglucose (FDG)-avid disease, but only mild uptake from T9 to T12 and no definitive FDG-avidity at the intramedullary lesion. Her symptoms remained stable with steroid treatment and a plan was made to repeat an MRI of the spine in 6 to 8 weeks.A, T1-weighted postgadolinium sagittal image demonstrating an enhancing spinal intramedullary lesion (blue arrowhead). Of note, there was enhancement of the previously irradiated T11 vertebral body metastasis, and adjacent vertebrae demonstrated marrow hyperintensity on T1-weighed noncontrast imaging. B, T2-weighted short τ inversion recovery imaging demonstrating extensive spinal cord edema (blue bracket).Spinal cord infarct: underlying cause workup, supportive care What Is Your Diagnosis?\n</question>\n\n<options>\nA. Metastatic progression: proceed with irradiation\nB. Spinal cord infarct: underlying cause workup, supportive care\nC. Radiation recall myelopathy: continue steroids, discontinue doxorubicin\nD. Radiation necrosis: continue steroids, add bevacizumab\n</options>\n"
},
{
"role": "assistant",
"content": "C. Radiation recall myelopathy\nGiven this patient’s metastatic disease, the initial concern was an intramedullary metastasis. However, the lack of mass effect on imaging and the atypical pattern of spread for uterine leiomyosarcoma suggested a possible alternative process. Spinal cord infarct was considered, although thought to be less likely given the acute enhancement, degree of edema, and lack of correlation with arterial territories. Although the ring enhancement with intense surrounding edema resembled radiation necrosis, the affected region only received a conventional palliative radiotherapy dose, too low to cause radiation necrosis. The timing of symptom onset in relation to her first doxorubicin infusion and the location of the lesion in the prior radiation field raised concern for radiation recall myelopathy.Given the stability of her symptoms upon treatment with steroids, negative results on lumbar puncture for malignant disease, lack of FDG-avidity of the T9 to T10 lesion on PET/CT, and timing of symptoms in the context of recent liposomal doxorubicin administration, the multidisciplinary consensus was in favor of radiation recall myelopathy. The risk of delaying irradiation if this were a metastasis vs the risk of irradiation causing further compromise if this were radiation recall myelopathy was discussed with the patient, and the decision was made to continue steroids with close clinical and radiographic surveillance.Radiation recall is a rare and poorly understood phenomenon that was initially described in 1959.1 Generally occurring months to years after radiation therapy, radiation recall refers to an inflammatory reaction in a previously irradiated region following systemic therapy administration.2 The incidence is challenging to estimate given that most data comes from case reports, with estimates ranging from 2% to 9%.1,3,4 Although radiation recall reactions of the skin are the most common manifestation, additional studies have described inflammatory reactions in other tissues, including the lung (pneumonitis following gemcitabine), muscles (myositis after cyclophosphamide), and spine (myelitis following capecitabine, gemcitabine, paclitaxel, dabrafenib, or pembrolizumab).5-7 Although doxorubicin has previously been implicated in radiation recall,4 to our knowledge, this is the first report specifically describing doxorubicin-associated recall myelopathy. Of note, liposomal doxorubicin has superior central nervous system penetrance than standard doxorubicin, which may increase the risk of recall myelopathy.Prior reports of radiation recall–associated myelopathy have revealed certain associated radiographic findings. It is common to see a primary ring-enhancing lesion in the prior radiation field, as well as T1-weighted hyperintense marrow signal changes in adjacent vertebrae.8 Spinal cord swelling and T2-signal abnormalities, predominantly involving the central cord, demonstrating longitudinal cord edema extending outside the radiation field has also been observed.8,9 The etiology of this edema is unknown but could be owing to an extensive nonimmune inflammatory reaction.7There is no standard treatment paradigm for radiation recall. At a minimum, the precipitating systemic agent should be held. Although there has also been success attributed to steroids, bevacizumab, immunoglobulin, plasmapheresis, or hyperbaric oxygen,2,9,10 it is unclear whether these agents are truly efficacious or whether clinical improvement or stabilization naturally ensues over time. Given its rarity, the prognosis remains unclear.Over the next month, the patient experienced mild clinical improvement in lower extremity weakness and pain but persistent urinary retention with high-dose steroid treatment. Findings on MRI 7 weeks after her initial scan demonstrated a decrease in the enhancing lesion and spinal cord edema (Figure 2). The lack of progression in the spine despite no oncologic therapy supported a diagnosis of radiation recall. She was discharged with instructions to taper steroid treatment with plans to initiate temozolomide.Magnetic resonance image of the spine 7 weeks after initial presentation. T1-weighted postgadolinium sagittal imaging demonstrating reduction in size and extent of enhancing intramedullary lesion (blue arrowhead)."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Arab patient in them 60s was diagnosed with high-grade leiomyosarcoma of the uterus in 2017 for which they underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. In 2020, they relapsed in the liver and spine. In November 2020, they underwent posterior lateral stabilization of C7-T4 for a T2 vertebral body metastasis causing cord compression, followed by postoperative stereotactic body radiation therapy to T2 to T3 (35 Gy/5 fractions) in January 2021. In February 2021, they received palliative radiation therapy (20 Gy/5 fractions) to T9 to T12 for a T11 vertebral body metastasis with epidural extension. From January to August 2021, they was treated with gemcitabine, and in September 2021, they received them first dose of liposomal doxorubicin. Two weeks later, they developed progressive bilateral lower extremity numbness, pain, weakness, and imbalance, prompting emergent evaluation. Magnetic resonance imaging (MRI) findings demonstrated extensive spinal cord edema extending from T2 to the conus and a posterior ring-enhancing intramedullary lesion at T9 to T10 without any mass effect (Figure 1). Because the intramedullary lesion appeared atypical for malignant disease given the lack of mass effect, they was prescribed high-dose steroids while further workup was pursued. Lumbar puncture showed no malignant cells. Positron emission tomographic-computed tomographic (PET/CT) scan demonstrated multifocal intensely F-fluorodeoxyglucose (FDG)-avid disease, but only mild uptake from T9 to T12 and no definitive FDG-avidity at the intramedullary lesion. Them symptoms remained stable with steroid treatment and a plan was made to repeat an MRI of the spine in 6 to 8 weeks.A, T1-weighted postgadolinium sagittal image demonstrating an enhancing spinal intramedullary lesion (blue arrowhead). Of note, there was enhancement of the previously irradiated T11 vertebral body metastasis, and adjacent vertebrae demonstrated marrow hyperintensity on T1-weighed noncontrast imaging. B, T2-weighted short τ inversion recovery imaging demonstrating extensive spinal cord edema (blue bracket).Spinal cord infarct: underlying cause workup, supportive care\n</clinical_case>\n\n<question>\nA woman in her 60s was diagnosed with high-grade leiomyosarcoma of the uterus in 2017 for which she underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. In 2020, she relapsed in the liver and spine. In November 2020, she underwent posterior lateral stabilization of C7-T4 for a T2 vertebral body metastasis causing cord compression, followed by postoperative stereotactic body radiation therapy to T2 to T3 (35 Gy/5 fractions) in January 2021. In February 2021, she received palliative radiation therapy (20 Gy/5 fractions) to T9 to T12 for a T11 vertebral body metastasis with epidural extension. From January to August 2021, she was treated with gemcitabine, and in September 2021, she received her first dose of liposomal doxorubicin. Two weeks later, she developed progressive bilateral lower extremity numbness, pain, weakness, and imbalance, prompting emergent evaluation. Magnetic resonance imaging (MRI) findings demonstrated extensive spinal cord edema extending from T2 to the conus and a posterior ring-enhancing intramedullary lesion at T9 to T10 without any mass effect (Figure 1). Because the intramedullary lesion appeared atypical for malignant disease given the lack of mass effect, she was prescribed high-dose steroids while further workup was pursued. Lumbar puncture showed no malignant cells. Positron emission tomographic-computed tomographic (PET/CT) scan demonstrated multifocal intensely F-fluorodeoxyglucose (FDG)-avid disease, but only mild uptake from T9 to T12 and no definitive FDG-avidity at the intramedullary lesion. Her symptoms remained stable with steroid treatment and a plan was made to repeat an MRI of the spine in 6 to 8 weeks.A, T1-weighted postgadolinium sagittal image demonstrating an enhancing spinal intramedullary lesion (blue arrowhead). Of note, there was enhancement of the previously irradiated T11 vertebral body metastasis, and adjacent vertebrae demonstrated marrow hyperintensity on T1-weighed noncontrast imaging. B, T2-weighted short τ inversion recovery imaging demonstrating extensive spinal cord edema (blue bracket).Spinal cord infarct: underlying cause workup, supportive care What Is Your Diagnosis?\n</question>\n\n<options>\nA. Metastatic progression: proceed with irradiation\nB. Spinal cord infarct: underlying cause workup, supportive care\nC. Radiation recall myelopathy: continue steroids, discontinue doxorubicin\nD. Radiation necrosis: continue steroids, add bevacizumab\n</options>\n"
},
{
"role": "assistant",
"content": "C. Radiation recall myelopathy\nGiven this patient’s metastatic disease, the initial concern was an intramedullary metastasis. However, the lack of mass effect on imaging and the atypical pattern of spread for uterine leiomyosarcoma suggested a possible alternative process. Spinal cord infarct was considered, although thought to be less likely given the acute enhancement, degree of edema, and lack of correlation with arterial territories. Although the ring enhancement with intense surrounding edema resembled radiation necrosis, the affected region only received a conventional palliative radiotherapy dose, too low to cause radiation necrosis. The timing of symptom onset in relation to her first doxorubicin infusion and the location of the lesion in the prior radiation field raised concern for radiation recall myelopathy.Given the stability of her symptoms upon treatment with steroids, negative results on lumbar puncture for malignant disease, lack of FDG-avidity of the T9 to T10 lesion on PET/CT, and timing of symptoms in the context of recent liposomal doxorubicin administration, the multidisciplinary consensus was in favor of radiation recall myelopathy. The risk of delaying irradiation if this were a metastasis vs the risk of irradiation causing further compromise if this were radiation recall myelopathy was discussed with the patient, and the decision was made to continue steroids with close clinical and radiographic surveillance.Radiation recall is a rare and poorly understood phenomenon that was initially described in 1959.1 Generally occurring months to years after radiation therapy, radiation recall refers to an inflammatory reaction in a previously irradiated region following systemic therapy administration.2 The incidence is challenging to estimate given that most data comes from case reports, with estimates ranging from 2% to 9%.1,3,4 Although radiation recall reactions of the skin are the most common manifestation, additional studies have described inflammatory reactions in other tissues, including the lung (pneumonitis following gemcitabine), muscles (myositis after cyclophosphamide), and spine (myelitis following capecitabine, gemcitabine, paclitaxel, dabrafenib, or pembrolizumab).5-7 Although doxorubicin has previously been implicated in radiation recall,4 to our knowledge, this is the first report specifically describing doxorubicin-associated recall myelopathy. Of note, liposomal doxorubicin has superior central nervous system penetrance than standard doxorubicin, which may increase the risk of recall myelopathy.Prior reports of radiation recall–associated myelopathy have revealed certain associated radiographic findings. It is common to see a primary ring-enhancing lesion in the prior radiation field, as well as T1-weighted hyperintense marrow signal changes in adjacent vertebrae.8 Spinal cord swelling and T2-signal abnormalities, predominantly involving the central cord, demonstrating longitudinal cord edema extending outside the radiation field has also been observed.8,9 The etiology of this edema is unknown but could be owing to an extensive nonimmune inflammatory reaction.7There is no standard treatment paradigm for radiation recall. At a minimum, the precipitating systemic agent should be held. Although there has also been success attributed to steroids, bevacizumab, immunoglobulin, plasmapheresis, or hyperbaric oxygen,2,9,10 it is unclear whether these agents are truly efficacious or whether clinical improvement or stabilization naturally ensues over time. Given its rarity, the prognosis remains unclear.Over the next month, the patient experienced mild clinical improvement in lower extremity weakness and pain but persistent urinary retention with high-dose steroid treatment. Findings on MRI 7 weeks after her initial scan demonstrated a decrease in the enhancing lesion and spinal cord edema (Figure 2). The lack of progression in the spine despite no oncologic therapy supported a diagnosis of radiation recall. She was discharged with instructions to taper steroid treatment with plans to initiate temozolomide.Magnetic resonance image of the spine 7 weeks after initial presentation. T1-weighted postgadolinium sagittal imaging demonstrating reduction in size and extent of enhancing intramedullary lesion (blue arrowhead)."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 66-year-old woman with a history of hypertension, type 2 diabetes, and chronic hepatitis B treated with tenofovir presented to the emergency department for evaluation of digital erythema and painful finger ulcers, which developed 6 months prior but which had increased in number and become more painful in the past several weeks. She also reported dyspnea on exertion, which developed 6 months prior to presentation. On physical examination, her peripheral oxygen saturation was 95% on room air. Auscultation of her lungs revealed crackles at the bases bilaterally. She had normal strength throughout all muscle groups and full range of motion in the joints of her hands. The palmar surfaces of her hands revealed livedo racemosa, erythema, and retiform purpura; ulcers overlying her phalangeal joint creases and fingertips; and scale and fissures on several digits (Figure). On the dorsal surfaces there was a large ulcer on the right second metacarpophalangeal joint and erythematous papules over several metacarpophalangeal joints. Ulcers were also noted on her elbows. Laboratory testing showed a hemoglobin level of 10.5 g/dL and normal creatine kinase level. The patient was admitted to the hospital for pain management and expedited workup of her progressive finger ulcerations.Clinical features on palmar surfaces of patient’s hands.Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel\n</clinical_case>\n\n<question>\nA 66-year-old woman with a history of hypertension, type 2 diabetes, and chronic hepatitis B treated with tenofovir presented to the emergency department for evaluation of digital erythema and painful finger ulcers, which developed 6 months prior but which had increased in number and become more painful in the past several weeks. She also reported dyspnea on exertion, which developed 6 months prior to presentation. On physical examination, her peripheral oxygen saturation was 95% on room air. Auscultation of her lungs revealed crackles at the bases bilaterally. She had normal strength throughout all muscle groups and full range of motion in the joints of her hands. The palmar surfaces of her hands revealed livedo racemosa, erythema, and retiform purpura; ulcers overlying her phalangeal joint creases and fingertips; and scale and fissures on several digits (Figure). On the dorsal surfaces there was a large ulcer on the right second metacarpophalangeal joint and erythematous papules over several metacarpophalangeal joints. Ulcers were also noted on her elbows. Laboratory testing showed a hemoglobin level of 10.5 g/dL and normal creatine kinase level. The patient was admitted to the hospital for pain management and expedited workup of her progressive finger ulcerations.Clinical features on palmar surfaces of patient’s hands.Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel What Would You Do Next?\n</question>\n\n<options>\nA. Order testing for anti-scl-70 and anticentromere antibodies\nB. Perform a muscle biopsy\nC. Order testing for anti–double-stranded DNA (dsDNA) antibody\nD. Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel\n</options>\n"
},
{
"role": "assistant",
"content": "D. Obtain a chest CT scan and myositis-specific autoantibody panel\nThe keys to the correct diagnosis in this patient are the painful ulcers on her hands and elbows, along with the erythematous and scaly papules (Gottron papules) and scale and fissures on the lateral aspect of several digits (mechanic’s hands), which are characteristic of MDA5-associated dermatomyositis. Testing for anti-dsDNA (choice B) is incorrect because her skin findings are not typical of systemic lupus erythematosus. Testing for anti-scl-70 and anticentromere antibodies (choice C) is also not correct because the digital ulcers associated with systemic sclerosis typically overlie joint spaces of the dorsal digits and the distal fingertips. A muscle biopsy (choice D) can help confirm the diagnosis of dermatomyositis in patients with muscle weakness or elevated muscle enzyme levels, but the yield of muscle biopsy is limited in patients without these findings.Dermatomyositis is an inflammatory disorder that typically presents with characteristic cutaneous findings (such as heliotrope eruption and Gottron papules) and proximal symmetric skeletal muscle weakness. Dermatomyositis can affect individuals of any age, is more common in women than men, and has an estimated annual incidence of 1 per 100 000 persons.1 The diagnosis of classic dermatomyositis can often be made based on clinical and laboratory criteria alone in patients with symmetric proximal muscle weakness, characteristic skin findings, and elevated muscle enzyme (eg, creatine kinase) levels. Although skin and muscle biopsy are not required for diagnosis of dermatomyositis, they may provide useful information in atypical presentations.2,3 Myositis-specific autoantibodies are found in approximately 60% of patients with dermatomyositis4 and are often evaluated as a panel to help delineate dermatomyositis subtypes. A test positive for anti-MDA5 antibody, which was first identified in 2009,5 confirms the diagnosis of MDA5-associated dermatomyositis.6Patients with MDA5-associated dermatomyositis may have typical cutaneous findings of dermatomyositis but characteristically have a distinctive phenotype that includes cutaneous and oral ulcerations and painful palmar papules; nonscarring alopecia, calcinosis, arthritis, and mechanic’s hands have also been associated with this antibody.6,7 In contrast to most patients with dermatomyositis, those with MDA5-associated dermatomyositis often do not experience proximal muscle weakness and may have normal creatine kinase and aldolase levels on blood testing.6 In addition, MDA5-associated dermatomyositis is associated with an elevated risk of interstitial lung disease (ILD), with a reported prevalence ranging from 5% to 65%.8The course and severity of ILD observed with MDA5-associated dermatomyositis is heterogeneous.4 In some patients ILD remains stable and responsive to treatment, while others develop a rapidly progressive ILD that is refractory to treatment, with some studies reporting greater than 50% mortality within 1 year of diagnosis.8,9Patients diagnosed with MDA5-associated dermatomyositis should be evaluated with a chest CT scan and pulmonary function testing. While a combination of immunosuppressive therapies (including glucocorticoids, calcineurin antagonists, rituximab, cyclophosphamide, mycophenolate mofetil, and intravenous immunoglobulin)6 may be used to treat MDA5-associated dermatomyositis, a recent randomized study demonstrated significantly higher survival rates in patients with ILD due to MDA5-associated dermatomyositis who were treated with high-dose glucocorticoids, tacrolimus, intravenous cyclophosphamide, and rescue plasmapheresis, if needed, compared with a historical control group treated with high-dose glucocorticoids and stepwise addition of immunosuppressants.10 Treatment of cutaneous ulcers in MDA5-associated dermatomyositis is not standardized; however, medications such as vasodilators and antiplatelet agents may be beneficial.6The patient’s myositis antibody panel was positive for anti-MDA5 antibody. Her chest CT showed peribronchovascular nodular densities and hazy ground glass opacities in the lower lungs bilaterally, consistent with ILD. During hospitalization, she received prednisone (1 mg/kg), hydroxychloroquine, mycophenolate mofetil, losartan, aspirin, and pentoxifylline. She continued these medications as an outpatient, and prednisone was titrated to 2.5 mg daily within 8 months of hospital discharge. Five months after hospitalization, her cutaneous ulcers had completely healed."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 66-year-old White woman with a history of hypertension, type 2 diabetes, and chronic hepatitis B treated with tenofovir presented to the emergency department for evaluation of digital erythema and painful finger ulcers, which developed 6 months prior but which had increased in number and become more painful in the past several weeks. She also reported dyspnea on exertion, which developed 6 months prior to presentation. On physical examination, her peripheral oxygen saturation was 95% on room air. Auscultation of her lungs revealed crackles at the bases bilaterally. She had normal strength throughout all muscle groups and full range of motion in the joints of her hands. The palmar surfaces of her hands revealed livedo racemosa, erythema, and retiform purpura; ulcers overlying her phalangeal joint creases and fingertips; and scale and fissures on several digits (Figure). On the dorsal surfaces there was a large ulcer on the right second metacarpophalangeal joint and erythematous papules over several metacarpophalangeal joints. Ulcers were also noted on her elbows. Laboratory testing showed a hemoglobin level of 10.5 g/dL and normal creatine kinase level. The White patient was admitted to the hospital for pain management and expedited workup of her progressive finger ulcerations.Clinical features on palmar surfaces of White patient’s hands.Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel\n</clinical_case>\n\n<question>\nA 66-year-old woman with a history of hypertension, type 2 diabetes, and chronic hepatitis B treated with tenofovir presented to the emergency department for evaluation of digital erythema and painful finger ulcers, which developed 6 months prior but which had increased in number and become more painful in the past several weeks. She also reported dyspnea on exertion, which developed 6 months prior to presentation. On physical examination, her peripheral oxygen saturation was 95% on room air. Auscultation of her lungs revealed crackles at the bases bilaterally. She had normal strength throughout all muscle groups and full range of motion in the joints of her hands. The palmar surfaces of her hands revealed livedo racemosa, erythema, and retiform purpura; ulcers overlying her phalangeal joint creases and fingertips; and scale and fissures on several digits (Figure). On the dorsal surfaces there was a large ulcer on the right second metacarpophalangeal joint and erythematous papules over several metacarpophalangeal joints. Ulcers were also noted on her elbows. Laboratory testing showed a hemoglobin level of 10.5 g/dL and normal creatine kinase level. The patient was admitted to the hospital for pain management and expedited workup of her progressive finger ulcerations.Clinical features on palmar surfaces of patient’s hands.Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel What Would You Do Next?\n</question>\n\n<options>\nA. Order testing for anti-scl-70 and anticentromere antibodies\nB. Perform a muscle biopsy\nC. Order testing for anti–double-stranded DNA (dsDNA) antibody\nD. Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel\n</options>\n"
},
{
"role": "assistant",
"content": "D. Obtain a chest CT scan and myositis-specific autoantibody panel\nThe keys to the correct diagnosis in this patient are the painful ulcers on her hands and elbows, along with the erythematous and scaly papules (Gottron papules) and scale and fissures on the lateral aspect of several digits (mechanic’s hands), which are characteristic of MDA5-associated dermatomyositis. Testing for anti-dsDNA (choice B) is incorrect because her skin findings are not typical of systemic lupus erythematosus. Testing for anti-scl-70 and anticentromere antibodies (choice C) is also not correct because the digital ulcers associated with systemic sclerosis typically overlie joint spaces of the dorsal digits and the distal fingertips. A muscle biopsy (choice D) can help confirm the diagnosis of dermatomyositis in patients with muscle weakness or elevated muscle enzyme levels, but the yield of muscle biopsy is limited in patients without these findings.Dermatomyositis is an inflammatory disorder that typically presents with characteristic cutaneous findings (such as heliotrope eruption and Gottron papules) and proximal symmetric skeletal muscle weakness. Dermatomyositis can affect individuals of any age, is more common in women than men, and has an estimated annual incidence of 1 per 100 000 persons.1 The diagnosis of classic dermatomyositis can often be made based on clinical and laboratory criteria alone in patients with symmetric proximal muscle weakness, characteristic skin findings, and elevated muscle enzyme (eg, creatine kinase) levels. Although skin and muscle biopsy are not required for diagnosis of dermatomyositis, they may provide useful information in atypical presentations.2,3 Myositis-specific autoantibodies are found in approximately 60% of patients with dermatomyositis4 and are often evaluated as a panel to help delineate dermatomyositis subtypes. A test positive for anti-MDA5 antibody, which was first identified in 2009,5 confirms the diagnosis of MDA5-associated dermatomyositis.6Patients with MDA5-associated dermatomyositis may have typical cutaneous findings of dermatomyositis but characteristically have a distinctive phenotype that includes cutaneous and oral ulcerations and painful palmar papules; nonscarring alopecia, calcinosis, arthritis, and mechanic’s hands have also been associated with this antibody.6,7 In contrast to most patients with dermatomyositis, those with MDA5-associated dermatomyositis often do not experience proximal muscle weakness and may have normal creatine kinase and aldolase levels on blood testing.6 In addition, MDA5-associated dermatomyositis is associated with an elevated risk of interstitial lung disease (ILD), with a reported prevalence ranging from 5% to 65%.8The course and severity of ILD observed with MDA5-associated dermatomyositis is heterogeneous.4 In some patients ILD remains stable and responsive to treatment, while others develop a rapidly progressive ILD that is refractory to treatment, with some studies reporting greater than 50% mortality within 1 year of diagnosis.8,9Patients diagnosed with MDA5-associated dermatomyositis should be evaluated with a chest CT scan and pulmonary function testing. While a combination of immunosuppressive therapies (including glucocorticoids, calcineurin antagonists, rituximab, cyclophosphamide, mycophenolate mofetil, and intravenous immunoglobulin)6 may be used to treat MDA5-associated dermatomyositis, a recent randomized study demonstrated significantly higher survival rates in patients with ILD due to MDA5-associated dermatomyositis who were treated with high-dose glucocorticoids, tacrolimus, intravenous cyclophosphamide, and rescue plasmapheresis, if needed, compared with a historical control group treated with high-dose glucocorticoids and stepwise addition of immunosuppressants.10 Treatment of cutaneous ulcers in MDA5-associated dermatomyositis is not standardized; however, medications such as vasodilators and antiplatelet agents may be beneficial.6The patient’s myositis antibody panel was positive for anti-MDA5 antibody. Her chest CT showed peribronchovascular nodular densities and hazy ground glass opacities in the lower lungs bilaterally, consistent with ILD. During hospitalization, she received prednisone (1 mg/kg), hydroxychloroquine, mycophenolate mofetil, losartan, aspirin, and pentoxifylline. She continued these medications as an outpatient, and prednisone was titrated to 2.5 mg daily within 8 months of hospital discharge. Five months after hospitalization, her cutaneous ulcers had completely healed."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 66-year-old White man with a history of hypertension, type 2 diabetes, and chronic hepatitis B treated with tenofovir presented to the emergency department for evaluation of digital erythema and painful finger ulcers, which developed 6 months prior but which had increased in number and become more painful in the past several weeks. He also reported dyspnea on exertion, which developed 6 months prior to presentation. On physical examination, him peripheral oxygen saturation was 95% on room air. Auscultation of him lungs revealed crackles at the bases bilaterally. He had normal strength throughout all muscle groups and full range of motion in the joints of him hands. The palmar surfaces of him hands revealed livedo racemosa, erythema, and retiform purpura; ulcers overlying him phalangeal joint creases and fingertips; and scale and fissures on several digits (Figure). On the dorsal surfaces there was a large ulcer on the right second metacarpophalangeal joint and erythematous papules over several metacarpophalangeal joints. Ulcers were also noted on him elbows. Laboratory testing showed a hemoglobin level of 10.5 g/dL and normal creatine kinase level. The White man was admitted to the hospital for pain management and expedited workup of him progressive finger ulcerations.Clinical features on palmar surfaces of White man’s hands.Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel\n</clinical_case>\n\n<question>\nA 66-year-old woman with a history of hypertension, type 2 diabetes, and chronic hepatitis B treated with tenofovir presented to the emergency department for evaluation of digital erythema and painful finger ulcers, which developed 6 months prior but which had increased in number and become more painful in the past several weeks. She also reported dyspnea on exertion, which developed 6 months prior to presentation. On physical examination, her peripheral oxygen saturation was 95% on room air. Auscultation of her lungs revealed crackles at the bases bilaterally. She had normal strength throughout all muscle groups and full range of motion in the joints of her hands. The palmar surfaces of her hands revealed livedo racemosa, erythema, and retiform purpura; ulcers overlying her phalangeal joint creases and fingertips; and scale and fissures on several digits (Figure). On the dorsal surfaces there was a large ulcer on the right second metacarpophalangeal joint and erythematous papules over several metacarpophalangeal joints. Ulcers were also noted on her elbows. Laboratory testing showed a hemoglobin level of 10.5 g/dL and normal creatine kinase level. The patient was admitted to the hospital for pain management and expedited workup of her progressive finger ulcerations.Clinical features on palmar surfaces of patient’s hands.Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel What Would You Do Next?\n</question>\n\n<options>\nA. Order testing for anti-scl-70 and anticentromere antibodies\nB. Perform a muscle biopsy\nC. Order testing for anti–double-stranded DNA (dsDNA) antibody\nD. Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel\n</options>\n"
},
{
"role": "assistant",
"content": "D. Obtain a chest CT scan and myositis-specific autoantibody panel\nThe keys to the correct diagnosis in this patient are the painful ulcers on her hands and elbows, along with the erythematous and scaly papules (Gottron papules) and scale and fissures on the lateral aspect of several digits (mechanic’s hands), which are characteristic of MDA5-associated dermatomyositis. Testing for anti-dsDNA (choice B) is incorrect because her skin findings are not typical of systemic lupus erythematosus. Testing for anti-scl-70 and anticentromere antibodies (choice C) is also not correct because the digital ulcers associated with systemic sclerosis typically overlie joint spaces of the dorsal digits and the distal fingertips. A muscle biopsy (choice D) can help confirm the diagnosis of dermatomyositis in patients with muscle weakness or elevated muscle enzyme levels, but the yield of muscle biopsy is limited in patients without these findings.Dermatomyositis is an inflammatory disorder that typically presents with characteristic cutaneous findings (such as heliotrope eruption and Gottron papules) and proximal symmetric skeletal muscle weakness. Dermatomyositis can affect individuals of any age, is more common in women than men, and has an estimated annual incidence of 1 per 100 000 persons.1 The diagnosis of classic dermatomyositis can often be made based on clinical and laboratory criteria alone in patients with symmetric proximal muscle weakness, characteristic skin findings, and elevated muscle enzyme (eg, creatine kinase) levels. Although skin and muscle biopsy are not required for diagnosis of dermatomyositis, they may provide useful information in atypical presentations.2,3 Myositis-specific autoantibodies are found in approximately 60% of patients with dermatomyositis4 and are often evaluated as a panel to help delineate dermatomyositis subtypes. A test positive for anti-MDA5 antibody, which was first identified in 2009,5 confirms the diagnosis of MDA5-associated dermatomyositis.6Patients with MDA5-associated dermatomyositis may have typical cutaneous findings of dermatomyositis but characteristically have a distinctive phenotype that includes cutaneous and oral ulcerations and painful palmar papules; nonscarring alopecia, calcinosis, arthritis, and mechanic’s hands have also been associated with this antibody.6,7 In contrast to most patients with dermatomyositis, those with MDA5-associated dermatomyositis often do not experience proximal muscle weakness and may have normal creatine kinase and aldolase levels on blood testing.6 In addition, MDA5-associated dermatomyositis is associated with an elevated risk of interstitial lung disease (ILD), with a reported prevalence ranging from 5% to 65%.8The course and severity of ILD observed with MDA5-associated dermatomyositis is heterogeneous.4 In some patients ILD remains stable and responsive to treatment, while others develop a rapidly progressive ILD that is refractory to treatment, with some studies reporting greater than 50% mortality within 1 year of diagnosis.8,9Patients diagnosed with MDA5-associated dermatomyositis should be evaluated with a chest CT scan and pulmonary function testing. While a combination of immunosuppressive therapies (including glucocorticoids, calcineurin antagonists, rituximab, cyclophosphamide, mycophenolate mofetil, and intravenous immunoglobulin)6 may be used to treat MDA5-associated dermatomyositis, a recent randomized study demonstrated significantly higher survival rates in patients with ILD due to MDA5-associated dermatomyositis who were treated with high-dose glucocorticoids, tacrolimus, intravenous cyclophosphamide, and rescue plasmapheresis, if needed, compared with a historical control group treated with high-dose glucocorticoids and stepwise addition of immunosuppressants.10 Treatment of cutaneous ulcers in MDA5-associated dermatomyositis is not standardized; however, medications such as vasodilators and antiplatelet agents may be beneficial.6The patient’s myositis antibody panel was positive for anti-MDA5 antibody. Her chest CT showed peribronchovascular nodular densities and hazy ground glass opacities in the lower lungs bilaterally, consistent with ILD. During hospitalization, she received prednisone (1 mg/kg), hydroxychloroquine, mycophenolate mofetil, losartan, aspirin, and pentoxifylline. She continued these medications as an outpatient, and prednisone was titrated to 2.5 mg daily within 8 months of hospital discharge. Five months after hospitalization, her cutaneous ulcers had completely healed."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 66-year-old White patient with a history of hypertension, type 2 diabetes, and chronic hepatitis B treated with tenofovir presented to the emergency department for evaluation of digital erythema and painful finger ulcers, which developed 6 months prior but which had increased in number and become more painful in the past several weeks. They also reported dyspnea on exertion, which developed 6 months prior to presentation. On physical examination, them peripheral oxygen saturation was 95% on room air. Auscultation of them lungs revealed crackles at the bases bilaterally. They had normal strength throughout all muscle groups and full range of motion in the joints of them hands. The palmar surfaces of them hands revealed livedo racemosa, erythema, and retiform purpura; ulcers overlying them phalangeal joint creases and fingertips; and scale and fissures on several digits (Figure). On the dorsal surfaces there was a large ulcer on the right second metacarpophalangeal joint and erythematous papules over several metacarpophalangeal joints. Ulcers were also noted on them elbows. Laboratory testing showed a hemoglobin level of 10.5 g/dL and normal creatine kinase level. The White patient was admitted to the hospital for pain management and expedited workup of them progressive finger ulcerations.Clinical features on palmar surfaces of White patient’s hands.Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel\n</clinical_case>\n\n<question>\nA 66-year-old woman with a history of hypertension, type 2 diabetes, and chronic hepatitis B treated with tenofovir presented to the emergency department for evaluation of digital erythema and painful finger ulcers, which developed 6 months prior but which had increased in number and become more painful in the past several weeks. She also reported dyspnea on exertion, which developed 6 months prior to presentation. On physical examination, her peripheral oxygen saturation was 95% on room air. Auscultation of her lungs revealed crackles at the bases bilaterally. She had normal strength throughout all muscle groups and full range of motion in the joints of her hands. The palmar surfaces of her hands revealed livedo racemosa, erythema, and retiform purpura; ulcers overlying her phalangeal joint creases and fingertips; and scale and fissures on several digits (Figure). On the dorsal surfaces there was a large ulcer on the right second metacarpophalangeal joint and erythematous papules over several metacarpophalangeal joints. Ulcers were also noted on her elbows. Laboratory testing showed a hemoglobin level of 10.5 g/dL and normal creatine kinase level. The patient was admitted to the hospital for pain management and expedited workup of her progressive finger ulcerations.Clinical features on palmar surfaces of patient’s hands.Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel What Would You Do Next?\n</question>\n\n<options>\nA. Order testing for anti-scl-70 and anticentromere antibodies\nB. Perform a muscle biopsy\nC. Order testing for anti–double-stranded DNA (dsDNA) antibody\nD. Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel\n</options>\n"
},
{
"role": "assistant",
"content": "D. Obtain a chest CT scan and myositis-specific autoantibody panel\nThe keys to the correct diagnosis in this patient are the painful ulcers on her hands and elbows, along with the erythematous and scaly papules (Gottron papules) and scale and fissures on the lateral aspect of several digits (mechanic’s hands), which are characteristic of MDA5-associated dermatomyositis. Testing for anti-dsDNA (choice B) is incorrect because her skin findings are not typical of systemic lupus erythematosus. Testing for anti-scl-70 and anticentromere antibodies (choice C) is also not correct because the digital ulcers associated with systemic sclerosis typically overlie joint spaces of the dorsal digits and the distal fingertips. A muscle biopsy (choice D) can help confirm the diagnosis of dermatomyositis in patients with muscle weakness or elevated muscle enzyme levels, but the yield of muscle biopsy is limited in patients without these findings.Dermatomyositis is an inflammatory disorder that typically presents with characteristic cutaneous findings (such as heliotrope eruption and Gottron papules) and proximal symmetric skeletal muscle weakness. Dermatomyositis can affect individuals of any age, is more common in women than men, and has an estimated annual incidence of 1 per 100 000 persons.1 The diagnosis of classic dermatomyositis can often be made based on clinical and laboratory criteria alone in patients with symmetric proximal muscle weakness, characteristic skin findings, and elevated muscle enzyme (eg, creatine kinase) levels. Although skin and muscle biopsy are not required for diagnosis of dermatomyositis, they may provide useful information in atypical presentations.2,3 Myositis-specific autoantibodies are found in approximately 60% of patients with dermatomyositis4 and are often evaluated as a panel to help delineate dermatomyositis subtypes. A test positive for anti-MDA5 antibody, which was first identified in 2009,5 confirms the diagnosis of MDA5-associated dermatomyositis.6Patients with MDA5-associated dermatomyositis may have typical cutaneous findings of dermatomyositis but characteristically have a distinctive phenotype that includes cutaneous and oral ulcerations and painful palmar papules; nonscarring alopecia, calcinosis, arthritis, and mechanic’s hands have also been associated with this antibody.6,7 In contrast to most patients with dermatomyositis, those with MDA5-associated dermatomyositis often do not experience proximal muscle weakness and may have normal creatine kinase and aldolase levels on blood testing.6 In addition, MDA5-associated dermatomyositis is associated with an elevated risk of interstitial lung disease (ILD), with a reported prevalence ranging from 5% to 65%.8The course and severity of ILD observed with MDA5-associated dermatomyositis is heterogeneous.4 In some patients ILD remains stable and responsive to treatment, while others develop a rapidly progressive ILD that is refractory to treatment, with some studies reporting greater than 50% mortality within 1 year of diagnosis.8,9Patients diagnosed with MDA5-associated dermatomyositis should be evaluated with a chest CT scan and pulmonary function testing. While a combination of immunosuppressive therapies (including glucocorticoids, calcineurin antagonists, rituximab, cyclophosphamide, mycophenolate mofetil, and intravenous immunoglobulin)6 may be used to treat MDA5-associated dermatomyositis, a recent randomized study demonstrated significantly higher survival rates in patients with ILD due to MDA5-associated dermatomyositis who were treated with high-dose glucocorticoids, tacrolimus, intravenous cyclophosphamide, and rescue plasmapheresis, if needed, compared with a historical control group treated with high-dose glucocorticoids and stepwise addition of immunosuppressants.10 Treatment of cutaneous ulcers in MDA5-associated dermatomyositis is not standardized; however, medications such as vasodilators and antiplatelet agents may be beneficial.6The patient’s myositis antibody panel was positive for anti-MDA5 antibody. Her chest CT showed peribronchovascular nodular densities and hazy ground glass opacities in the lower lungs bilaterally, consistent with ILD. During hospitalization, she received prednisone (1 mg/kg), hydroxychloroquine, mycophenolate mofetil, losartan, aspirin, and pentoxifylline. She continued these medications as an outpatient, and prednisone was titrated to 2.5 mg daily within 8 months of hospital discharge. Five months after hospitalization, her cutaneous ulcers had completely healed."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 66-year-old Black woman with a history of hypertension, type 2 diabetes, and chronic hepatitis B treated with tenofovir presented to the emergency department for evaluation of digital erythema and painful finger ulcers, which developed 6 months prior but which had increased in number and become more painful in the past several weeks. She also reported dyspnea on exertion, which developed 6 months prior to presentation. On physical examination, her peripheral oxygen saturation was 95% on room air. Auscultation of her lungs revealed crackles at the bases bilaterally. She had normal strength throughout all muscle groups and full range of motion in the joints of her hands. The palmar surfaces of her hands revealed livedo racemosa, erythema, and retiform purpura; ulcers overlying her phalangeal joint creases and fingertips; and scale and fissures on several digits (Figure). On the dorsal surfaces there was a large ulcer on the right second metacarpophalangeal joint and erythematous papules over several metacarpophalangeal joints. Ulcers were also noted on her elbows. Laboratory testing showed a hemoglobin level of 10.5 g/dL and normal creatine kinase level. The Black patient was admitted to the hospital for pain management and expedited workup of her progressive finger ulcerations.Clinical features on palmar surfaces of Black patient’s hands.Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel\n</clinical_case>\n\n<question>\nA 66-year-old woman with a history of hypertension, type 2 diabetes, and chronic hepatitis B treated with tenofovir presented to the emergency department for evaluation of digital erythema and painful finger ulcers, which developed 6 months prior but which had increased in number and become more painful in the past several weeks. She also reported dyspnea on exertion, which developed 6 months prior to presentation. On physical examination, her peripheral oxygen saturation was 95% on room air. Auscultation of her lungs revealed crackles at the bases bilaterally. She had normal strength throughout all muscle groups and full range of motion in the joints of her hands. The palmar surfaces of her hands revealed livedo racemosa, erythema, and retiform purpura; ulcers overlying her phalangeal joint creases and fingertips; and scale and fissures on several digits (Figure). On the dorsal surfaces there was a large ulcer on the right second metacarpophalangeal joint and erythematous papules over several metacarpophalangeal joints. Ulcers were also noted on her elbows. Laboratory testing showed a hemoglobin level of 10.5 g/dL and normal creatine kinase level. The patient was admitted to the hospital for pain management and expedited workup of her progressive finger ulcerations.Clinical features on palmar surfaces of patient’s hands.Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel What Would You Do Next?\n</question>\n\n<options>\nA. Order testing for anti-scl-70 and anticentromere antibodies\nB. Perform a muscle biopsy\nC. Order testing for anti–double-stranded DNA (dsDNA) antibody\nD. Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel\n</options>\n"
},
{
"role": "assistant",
"content": "D. Obtain a chest CT scan and myositis-specific autoantibody panel\nThe keys to the correct diagnosis in this patient are the painful ulcers on her hands and elbows, along with the erythematous and scaly papules (Gottron papules) and scale and fissures on the lateral aspect of several digits (mechanic’s hands), which are characteristic of MDA5-associated dermatomyositis. Testing for anti-dsDNA (choice B) is incorrect because her skin findings are not typical of systemic lupus erythematosus. Testing for anti-scl-70 and anticentromere antibodies (choice C) is also not correct because the digital ulcers associated with systemic sclerosis typically overlie joint spaces of the dorsal digits and the distal fingertips. A muscle biopsy (choice D) can help confirm the diagnosis of dermatomyositis in patients with muscle weakness or elevated muscle enzyme levels, but the yield of muscle biopsy is limited in patients without these findings.Dermatomyositis is an inflammatory disorder that typically presents with characteristic cutaneous findings (such as heliotrope eruption and Gottron papules) and proximal symmetric skeletal muscle weakness. Dermatomyositis can affect individuals of any age, is more common in women than men, and has an estimated annual incidence of 1 per 100 000 persons.1 The diagnosis of classic dermatomyositis can often be made based on clinical and laboratory criteria alone in patients with symmetric proximal muscle weakness, characteristic skin findings, and elevated muscle enzyme (eg, creatine kinase) levels. Although skin and muscle biopsy are not required for diagnosis of dermatomyositis, they may provide useful information in atypical presentations.2,3 Myositis-specific autoantibodies are found in approximately 60% of patients with dermatomyositis4 and are often evaluated as a panel to help delineate dermatomyositis subtypes. A test positive for anti-MDA5 antibody, which was first identified in 2009,5 confirms the diagnosis of MDA5-associated dermatomyositis.6Patients with MDA5-associated dermatomyositis may have typical cutaneous findings of dermatomyositis but characteristically have a distinctive phenotype that includes cutaneous and oral ulcerations and painful palmar papules; nonscarring alopecia, calcinosis, arthritis, and mechanic’s hands have also been associated with this antibody.6,7 In contrast to most patients with dermatomyositis, those with MDA5-associated dermatomyositis often do not experience proximal muscle weakness and may have normal creatine kinase and aldolase levels on blood testing.6 In addition, MDA5-associated dermatomyositis is associated with an elevated risk of interstitial lung disease (ILD), with a reported prevalence ranging from 5% to 65%.8The course and severity of ILD observed with MDA5-associated dermatomyositis is heterogeneous.4 In some patients ILD remains stable and responsive to treatment, while others develop a rapidly progressive ILD that is refractory to treatment, with some studies reporting greater than 50% mortality within 1 year of diagnosis.8,9Patients diagnosed with MDA5-associated dermatomyositis should be evaluated with a chest CT scan and pulmonary function testing. While a combination of immunosuppressive therapies (including glucocorticoids, calcineurin antagonists, rituximab, cyclophosphamide, mycophenolate mofetil, and intravenous immunoglobulin)6 may be used to treat MDA5-associated dermatomyositis, a recent randomized study demonstrated significantly higher survival rates in patients with ILD due to MDA5-associated dermatomyositis who were treated with high-dose glucocorticoids, tacrolimus, intravenous cyclophosphamide, and rescue plasmapheresis, if needed, compared with a historical control group treated with high-dose glucocorticoids and stepwise addition of immunosuppressants.10 Treatment of cutaneous ulcers in MDA5-associated dermatomyositis is not standardized; however, medications such as vasodilators and antiplatelet agents may be beneficial.6The patient’s myositis antibody panel was positive for anti-MDA5 antibody. Her chest CT showed peribronchovascular nodular densities and hazy ground glass opacities in the lower lungs bilaterally, consistent with ILD. During hospitalization, she received prednisone (1 mg/kg), hydroxychloroquine, mycophenolate mofetil, losartan, aspirin, and pentoxifylline. She continued these medications as an outpatient, and prednisone was titrated to 2.5 mg daily within 8 months of hospital discharge. Five months after hospitalization, her cutaneous ulcers had completely healed."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 66-year-old Black man with a history of hypertension, type 2 diabetes, and chronic hepatitis B treated with tenofovir presented to the emergency department for evaluation of digital erythema and painful finger ulcers, which developed 6 months prior but which had increased in number and become more painful in the past several weeks. He also reported dyspnea on exertion, which developed 6 months prior to presentation. On physical examination, him peripheral oxygen saturation was 95% on room air. Auscultation of him lungs revealed crackles at the bases bilaterally. He had normal strength throughout all muscle groups and full range of motion in the joints of him hands. The palmar surfaces of him hands revealed livedo racemosa, erythema, and retiform purpura; ulcers overlying him phalangeal joint creases and fingertips; and scale and fissures on several digits (Figure). On the dorsal surfaces there was a large ulcer on the right second metacarpophalangeal joint and erythematous papules over several metacarpophalangeal joints. Ulcers were also noted on him elbows. Laboratory testing showed a hemoglobin level of 10.5 g/dL and normal creatine kinase level. The Black man was admitted to the hospital for pain management and expedited workup of him progressive finger ulcerations.Clinical features on palmar surfaces of Black man’s hands.Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel\n</clinical_case>\n\n<question>\nA 66-year-old woman with a history of hypertension, type 2 diabetes, and chronic hepatitis B treated with tenofovir presented to the emergency department for evaluation of digital erythema and painful finger ulcers, which developed 6 months prior but which had increased in number and become more painful in the past several weeks. She also reported dyspnea on exertion, which developed 6 months prior to presentation. On physical examination, her peripheral oxygen saturation was 95% on room air. Auscultation of her lungs revealed crackles at the bases bilaterally. She had normal strength throughout all muscle groups and full range of motion in the joints of her hands. The palmar surfaces of her hands revealed livedo racemosa, erythema, and retiform purpura; ulcers overlying her phalangeal joint creases and fingertips; and scale and fissures on several digits (Figure). On the dorsal surfaces there was a large ulcer on the right second metacarpophalangeal joint and erythematous papules over several metacarpophalangeal joints. Ulcers were also noted on her elbows. Laboratory testing showed a hemoglobin level of 10.5 g/dL and normal creatine kinase level. The patient was admitted to the hospital for pain management and expedited workup of her progressive finger ulcerations.Clinical features on palmar surfaces of patient’s hands.Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel What Would You Do Next?\n</question>\n\n<options>\nA. Order testing for anti-scl-70 and anticentromere antibodies\nB. Perform a muscle biopsy\nC. Order testing for anti–double-stranded DNA (dsDNA) antibody\nD. Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel\n</options>\n"
},
{
"role": "assistant",
"content": "D. Obtain a chest CT scan and myositis-specific autoantibody panel\nThe keys to the correct diagnosis in this patient are the painful ulcers on her hands and elbows, along with the erythematous and scaly papules (Gottron papules) and scale and fissures on the lateral aspect of several digits (mechanic’s hands), which are characteristic of MDA5-associated dermatomyositis. Testing for anti-dsDNA (choice B) is incorrect because her skin findings are not typical of systemic lupus erythematosus. Testing for anti-scl-70 and anticentromere antibodies (choice C) is also not correct because the digital ulcers associated with systemic sclerosis typically overlie joint spaces of the dorsal digits and the distal fingertips. A muscle biopsy (choice D) can help confirm the diagnosis of dermatomyositis in patients with muscle weakness or elevated muscle enzyme levels, but the yield of muscle biopsy is limited in patients without these findings.Dermatomyositis is an inflammatory disorder that typically presents with characteristic cutaneous findings (such as heliotrope eruption and Gottron papules) and proximal symmetric skeletal muscle weakness. Dermatomyositis can affect individuals of any age, is more common in women than men, and has an estimated annual incidence of 1 per 100 000 persons.1 The diagnosis of classic dermatomyositis can often be made based on clinical and laboratory criteria alone in patients with symmetric proximal muscle weakness, characteristic skin findings, and elevated muscle enzyme (eg, creatine kinase) levels. Although skin and muscle biopsy are not required for diagnosis of dermatomyositis, they may provide useful information in atypical presentations.2,3 Myositis-specific autoantibodies are found in approximately 60% of patients with dermatomyositis4 and are often evaluated as a panel to help delineate dermatomyositis subtypes. A test positive for anti-MDA5 antibody, which was first identified in 2009,5 confirms the diagnosis of MDA5-associated dermatomyositis.6Patients with MDA5-associated dermatomyositis may have typical cutaneous findings of dermatomyositis but characteristically have a distinctive phenotype that includes cutaneous and oral ulcerations and painful palmar papules; nonscarring alopecia, calcinosis, arthritis, and mechanic’s hands have also been associated with this antibody.6,7 In contrast to most patients with dermatomyositis, those with MDA5-associated dermatomyositis often do not experience proximal muscle weakness and may have normal creatine kinase and aldolase levels on blood testing.6 In addition, MDA5-associated dermatomyositis is associated with an elevated risk of interstitial lung disease (ILD), with a reported prevalence ranging from 5% to 65%.8The course and severity of ILD observed with MDA5-associated dermatomyositis is heterogeneous.4 In some patients ILD remains stable and responsive to treatment, while others develop a rapidly progressive ILD that is refractory to treatment, with some studies reporting greater than 50% mortality within 1 year of diagnosis.8,9Patients diagnosed with MDA5-associated dermatomyositis should be evaluated with a chest CT scan and pulmonary function testing. While a combination of immunosuppressive therapies (including glucocorticoids, calcineurin antagonists, rituximab, cyclophosphamide, mycophenolate mofetil, and intravenous immunoglobulin)6 may be used to treat MDA5-associated dermatomyositis, a recent randomized study demonstrated significantly higher survival rates in patients with ILD due to MDA5-associated dermatomyositis who were treated with high-dose glucocorticoids, tacrolimus, intravenous cyclophosphamide, and rescue plasmapheresis, if needed, compared with a historical control group treated with high-dose glucocorticoids and stepwise addition of immunosuppressants.10 Treatment of cutaneous ulcers in MDA5-associated dermatomyositis is not standardized; however, medications such as vasodilators and antiplatelet agents may be beneficial.6The patient’s myositis antibody panel was positive for anti-MDA5 antibody. Her chest CT showed peribronchovascular nodular densities and hazy ground glass opacities in the lower lungs bilaterally, consistent with ILD. During hospitalization, she received prednisone (1 mg/kg), hydroxychloroquine, mycophenolate mofetil, losartan, aspirin, and pentoxifylline. She continued these medications as an outpatient, and prednisone was titrated to 2.5 mg daily within 8 months of hospital discharge. Five months after hospitalization, her cutaneous ulcers had completely healed."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 66-year-old Black patient with a history of hypertension, type 2 diabetes, and chronic hepatitis B treated with tenofovir presented to the emergency department for evaluation of digital erythema and painful finger ulcers, which developed 6 months prior but which had increased in number and become more painful in the past several weeks. They also reported dyspnea on exertion, which developed 6 months prior to presentation. On physical examination, them peripheral oxygen saturation was 95% on room air. Auscultation of them lungs revealed crackles at the bases bilaterally. They had normal strength throughout all muscle groups and full range of motion in the joints of them hands. The palmar surfaces of them hands revealed livedo racemosa, erythema, and retiform purpura; ulcers overlying them phalangeal joint creases and fingertips; and scale and fissures on several digits (Figure). On the dorsal surfaces there was a large ulcer on the right second metacarpophalangeal joint and erythematous papules over several metacarpophalangeal joints. Ulcers were also noted on them elbows. Laboratory testing showed a hemoglobin level of 10.5 g/dL and normal creatine kinase level. The Black patient was admitted to the hospital for pain management and expedited workup of them progressive finger ulcerations.Clinical features on palmar surfaces of Black patient’s hands.Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel\n</clinical_case>\n\n<question>\nA 66-year-old woman with a history of hypertension, type 2 diabetes, and chronic hepatitis B treated with tenofovir presented to the emergency department for evaluation of digital erythema and painful finger ulcers, which developed 6 months prior but which had increased in number and become more painful in the past several weeks. She also reported dyspnea on exertion, which developed 6 months prior to presentation. On physical examination, her peripheral oxygen saturation was 95% on room air. Auscultation of her lungs revealed crackles at the bases bilaterally. She had normal strength throughout all muscle groups and full range of motion in the joints of her hands. The palmar surfaces of her hands revealed livedo racemosa, erythema, and retiform purpura; ulcers overlying her phalangeal joint creases and fingertips; and scale and fissures on several digits (Figure). On the dorsal surfaces there was a large ulcer on the right second metacarpophalangeal joint and erythematous papules over several metacarpophalangeal joints. Ulcers were also noted on her elbows. Laboratory testing showed a hemoglobin level of 10.5 g/dL and normal creatine kinase level. The patient was admitted to the hospital for pain management and expedited workup of her progressive finger ulcerations.Clinical features on palmar surfaces of patient’s hands.Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel What Would You Do Next?\n</question>\n\n<options>\nA. Order testing for anti-scl-70 and anticentromere antibodies\nB. Perform a muscle biopsy\nC. Order testing for anti–double-stranded DNA (dsDNA) antibody\nD. Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel\n</options>\n"
},
{
"role": "assistant",
"content": "D. Obtain a chest CT scan and myositis-specific autoantibody panel\nThe keys to the correct diagnosis in this patient are the painful ulcers on her hands and elbows, along with the erythematous and scaly papules (Gottron papules) and scale and fissures on the lateral aspect of several digits (mechanic’s hands), which are characteristic of MDA5-associated dermatomyositis. Testing for anti-dsDNA (choice B) is incorrect because her skin findings are not typical of systemic lupus erythematosus. Testing for anti-scl-70 and anticentromere antibodies (choice C) is also not correct because the digital ulcers associated with systemic sclerosis typically overlie joint spaces of the dorsal digits and the distal fingertips. A muscle biopsy (choice D) can help confirm the diagnosis of dermatomyositis in patients with muscle weakness or elevated muscle enzyme levels, but the yield of muscle biopsy is limited in patients without these findings.Dermatomyositis is an inflammatory disorder that typically presents with characteristic cutaneous findings (such as heliotrope eruption and Gottron papules) and proximal symmetric skeletal muscle weakness. Dermatomyositis can affect individuals of any age, is more common in women than men, and has an estimated annual incidence of 1 per 100 000 persons.1 The diagnosis of classic dermatomyositis can often be made based on clinical and laboratory criteria alone in patients with symmetric proximal muscle weakness, characteristic skin findings, and elevated muscle enzyme (eg, creatine kinase) levels. Although skin and muscle biopsy are not required for diagnosis of dermatomyositis, they may provide useful information in atypical presentations.2,3 Myositis-specific autoantibodies are found in approximately 60% of patients with dermatomyositis4 and are often evaluated as a panel to help delineate dermatomyositis subtypes. A test positive for anti-MDA5 antibody, which was first identified in 2009,5 confirms the diagnosis of MDA5-associated dermatomyositis.6Patients with MDA5-associated dermatomyositis may have typical cutaneous findings of dermatomyositis but characteristically have a distinctive phenotype that includes cutaneous and oral ulcerations and painful palmar papules; nonscarring alopecia, calcinosis, arthritis, and mechanic’s hands have also been associated with this antibody.6,7 In contrast to most patients with dermatomyositis, those with MDA5-associated dermatomyositis often do not experience proximal muscle weakness and may have normal creatine kinase and aldolase levels on blood testing.6 In addition, MDA5-associated dermatomyositis is associated with an elevated risk of interstitial lung disease (ILD), with a reported prevalence ranging from 5% to 65%.8The course and severity of ILD observed with MDA5-associated dermatomyositis is heterogeneous.4 In some patients ILD remains stable and responsive to treatment, while others develop a rapidly progressive ILD that is refractory to treatment, with some studies reporting greater than 50% mortality within 1 year of diagnosis.8,9Patients diagnosed with MDA5-associated dermatomyositis should be evaluated with a chest CT scan and pulmonary function testing. While a combination of immunosuppressive therapies (including glucocorticoids, calcineurin antagonists, rituximab, cyclophosphamide, mycophenolate mofetil, and intravenous immunoglobulin)6 may be used to treat MDA5-associated dermatomyositis, a recent randomized study demonstrated significantly higher survival rates in patients with ILD due to MDA5-associated dermatomyositis who were treated with high-dose glucocorticoids, tacrolimus, intravenous cyclophosphamide, and rescue plasmapheresis, if needed, compared with a historical control group treated with high-dose glucocorticoids and stepwise addition of immunosuppressants.10 Treatment of cutaneous ulcers in MDA5-associated dermatomyositis is not standardized; however, medications such as vasodilators and antiplatelet agents may be beneficial.6The patient’s myositis antibody panel was positive for anti-MDA5 antibody. Her chest CT showed peribronchovascular nodular densities and hazy ground glass opacities in the lower lungs bilaterally, consistent with ILD. During hospitalization, she received prednisone (1 mg/kg), hydroxychloroquine, mycophenolate mofetil, losartan, aspirin, and pentoxifylline. She continued these medications as an outpatient, and prednisone was titrated to 2.5 mg daily within 8 months of hospital discharge. Five months after hospitalization, her cutaneous ulcers had completely healed."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 66-year-old Asian woman with a history of hypertension, type 2 diabetes, and chronic hepatitis B treated with tenofovir presented to the emergency department for evaluation of digital erythema and painful finger ulcers, which developed 6 months prior but which had increased in number and become more painful in the past several weeks. She also reported dyspnea on exertion, which developed 6 months prior to presentation. On physical examination, her peripheral oxygen saturation was 95% on room air. Auscultation of her lungs revealed crackles at the bases bilaterally. She had normal strength throughout all muscle groups and full range of motion in the joints of her hands. The palmar surfaces of her hands revealed livedo racemosa, erythema, and retiform purpura; ulcers overlying her phalangeal joint creases and fingertips; and scale and fissures on several digits (Figure). On the dorsal surfaces there was a large ulcer on the right second metacarpophalangeal joint and erythematous papules over several metacarpophalangeal joints. Ulcers were also noted on her elbows. Laboratory testing showed a hemoglobin level of 10.5 g/dL and normal creatine kinase level. The Asian patient was admitted to the hospital for pain management and expedited workup of her progressive finger ulcerations.Clinical features on palmar surfaces of Asian patient’s hands.Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel\n</clinical_case>\n\n<question>\nA 66-year-old woman with a history of hypertension, type 2 diabetes, and chronic hepatitis B treated with tenofovir presented to the emergency department for evaluation of digital erythema and painful finger ulcers, which developed 6 months prior but which had increased in number and become more painful in the past several weeks. She also reported dyspnea on exertion, which developed 6 months prior to presentation. On physical examination, her peripheral oxygen saturation was 95% on room air. Auscultation of her lungs revealed crackles at the bases bilaterally. She had normal strength throughout all muscle groups and full range of motion in the joints of her hands. The palmar surfaces of her hands revealed livedo racemosa, erythema, and retiform purpura; ulcers overlying her phalangeal joint creases and fingertips; and scale and fissures on several digits (Figure). On the dorsal surfaces there was a large ulcer on the right second metacarpophalangeal joint and erythematous papules over several metacarpophalangeal joints. Ulcers were also noted on her elbows. Laboratory testing showed a hemoglobin level of 10.5 g/dL and normal creatine kinase level. The patient was admitted to the hospital for pain management and expedited workup of her progressive finger ulcerations.Clinical features on palmar surfaces of patient’s hands.Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel What Would You Do Next?\n</question>\n\n<options>\nA. Order testing for anti-scl-70 and anticentromere antibodies\nB. Perform a muscle biopsy\nC. Order testing for anti–double-stranded DNA (dsDNA) antibody\nD. Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel\n</options>\n"
},
{
"role": "assistant",
"content": "D. Obtain a chest CT scan and myositis-specific autoantibody panel\nThe keys to the correct diagnosis in this patient are the painful ulcers on her hands and elbows, along with the erythematous and scaly papules (Gottron papules) and scale and fissures on the lateral aspect of several digits (mechanic’s hands), which are characteristic of MDA5-associated dermatomyositis. Testing for anti-dsDNA (choice B) is incorrect because her skin findings are not typical of systemic lupus erythematosus. Testing for anti-scl-70 and anticentromere antibodies (choice C) is also not correct because the digital ulcers associated with systemic sclerosis typically overlie joint spaces of the dorsal digits and the distal fingertips. A muscle biopsy (choice D) can help confirm the diagnosis of dermatomyositis in patients with muscle weakness or elevated muscle enzyme levels, but the yield of muscle biopsy is limited in patients without these findings.Dermatomyositis is an inflammatory disorder that typically presents with characteristic cutaneous findings (such as heliotrope eruption and Gottron papules) and proximal symmetric skeletal muscle weakness. Dermatomyositis can affect individuals of any age, is more common in women than men, and has an estimated annual incidence of 1 per 100 000 persons.1 The diagnosis of classic dermatomyositis can often be made based on clinical and laboratory criteria alone in patients with symmetric proximal muscle weakness, characteristic skin findings, and elevated muscle enzyme (eg, creatine kinase) levels. Although skin and muscle biopsy are not required for diagnosis of dermatomyositis, they may provide useful information in atypical presentations.2,3 Myositis-specific autoantibodies are found in approximately 60% of patients with dermatomyositis4 and are often evaluated as a panel to help delineate dermatomyositis subtypes. A test positive for anti-MDA5 antibody, which was first identified in 2009,5 confirms the diagnosis of MDA5-associated dermatomyositis.6Patients with MDA5-associated dermatomyositis may have typical cutaneous findings of dermatomyositis but characteristically have a distinctive phenotype that includes cutaneous and oral ulcerations and painful palmar papules; nonscarring alopecia, calcinosis, arthritis, and mechanic’s hands have also been associated with this antibody.6,7 In contrast to most patients with dermatomyositis, those with MDA5-associated dermatomyositis often do not experience proximal muscle weakness and may have normal creatine kinase and aldolase levels on blood testing.6 In addition, MDA5-associated dermatomyositis is associated with an elevated risk of interstitial lung disease (ILD), with a reported prevalence ranging from 5% to 65%.8The course and severity of ILD observed with MDA5-associated dermatomyositis is heterogeneous.4 In some patients ILD remains stable and responsive to treatment, while others develop a rapidly progressive ILD that is refractory to treatment, with some studies reporting greater than 50% mortality within 1 year of diagnosis.8,9Patients diagnosed with MDA5-associated dermatomyositis should be evaluated with a chest CT scan and pulmonary function testing. While a combination of immunosuppressive therapies (including glucocorticoids, calcineurin antagonists, rituximab, cyclophosphamide, mycophenolate mofetil, and intravenous immunoglobulin)6 may be used to treat MDA5-associated dermatomyositis, a recent randomized study demonstrated significantly higher survival rates in patients with ILD due to MDA5-associated dermatomyositis who were treated with high-dose glucocorticoids, tacrolimus, intravenous cyclophosphamide, and rescue plasmapheresis, if needed, compared with a historical control group treated with high-dose glucocorticoids and stepwise addition of immunosuppressants.10 Treatment of cutaneous ulcers in MDA5-associated dermatomyositis is not standardized; however, medications such as vasodilators and antiplatelet agents may be beneficial.6The patient’s myositis antibody panel was positive for anti-MDA5 antibody. Her chest CT showed peribronchovascular nodular densities and hazy ground glass opacities in the lower lungs bilaterally, consistent with ILD. During hospitalization, she received prednisone (1 mg/kg), hydroxychloroquine, mycophenolate mofetil, losartan, aspirin, and pentoxifylline. She continued these medications as an outpatient, and prednisone was titrated to 2.5 mg daily within 8 months of hospital discharge. Five months after hospitalization, her cutaneous ulcers had completely healed."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 66-year-old Asian man with a history of hypertension, type 2 diabetes, and chronic hepatitis B treated with tenofovir presented to the emergency department for evaluation of digital erythema and painful finger ulcers, which developed 6 months prior but which had increased in number and become more painful in the past several weeks. He also reported dyspnea on exertion, which developed 6 months prior to presentation. On physical examination, him peripheral oxygen saturation was 95% on room air. Auscultation of him lungs revealed crackles at the bases bilaterally. He had normal strength throughout all muscle groups and full range of motion in the joints of him hands. The palmar surfaces of him hands revealed livedo racemosa, erythema, and retiform purpura; ulcers overlying him phalangeal joint creases and fingertips; and scale and fissures on several digits (Figure). On the dorsal surfaces there was a large ulcer on the right second metacarpophalangeal joint and erythematous papules over several metacarpophalangeal joints. Ulcers were also noted on him elbows. Laboratory testing showed a hemoglobin level of 10.5 g/dL and normal creatine kinase level. The Asian man was admitted to the hospital for pain management and expedited workup of him progressive finger ulcerations.Clinical features on palmar surfaces of Asian man’s hands.Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel\n</clinical_case>\n\n<question>\nA 66-year-old woman with a history of hypertension, type 2 diabetes, and chronic hepatitis B treated with tenofovir presented to the emergency department for evaluation of digital erythema and painful finger ulcers, which developed 6 months prior but which had increased in number and become more painful in the past several weeks. She also reported dyspnea on exertion, which developed 6 months prior to presentation. On physical examination, her peripheral oxygen saturation was 95% on room air. Auscultation of her lungs revealed crackles at the bases bilaterally. She had normal strength throughout all muscle groups and full range of motion in the joints of her hands. The palmar surfaces of her hands revealed livedo racemosa, erythema, and retiform purpura; ulcers overlying her phalangeal joint creases and fingertips; and scale and fissures on several digits (Figure). On the dorsal surfaces there was a large ulcer on the right second metacarpophalangeal joint and erythematous papules over several metacarpophalangeal joints. Ulcers were also noted on her elbows. Laboratory testing showed a hemoglobin level of 10.5 g/dL and normal creatine kinase level. The patient was admitted to the hospital for pain management and expedited workup of her progressive finger ulcerations.Clinical features on palmar surfaces of patient’s hands.Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel What Would You Do Next?\n</question>\n\n<options>\nA. Order testing for anti-scl-70 and anticentromere antibodies\nB. Perform a muscle biopsy\nC. Order testing for anti–double-stranded DNA (dsDNA) antibody\nD. Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel\n</options>\n"
},
{
"role": "assistant",
"content": "D. Obtain a chest CT scan and myositis-specific autoantibody panel\nThe keys to the correct diagnosis in this patient are the painful ulcers on her hands and elbows, along with the erythematous and scaly papules (Gottron papules) and scale and fissures on the lateral aspect of several digits (mechanic’s hands), which are characteristic of MDA5-associated dermatomyositis. Testing for anti-dsDNA (choice B) is incorrect because her skin findings are not typical of systemic lupus erythematosus. Testing for anti-scl-70 and anticentromere antibodies (choice C) is also not correct because the digital ulcers associated with systemic sclerosis typically overlie joint spaces of the dorsal digits and the distal fingertips. A muscle biopsy (choice D) can help confirm the diagnosis of dermatomyositis in patients with muscle weakness or elevated muscle enzyme levels, but the yield of muscle biopsy is limited in patients without these findings.Dermatomyositis is an inflammatory disorder that typically presents with characteristic cutaneous findings (such as heliotrope eruption and Gottron papules) and proximal symmetric skeletal muscle weakness. Dermatomyositis can affect individuals of any age, is more common in women than men, and has an estimated annual incidence of 1 per 100 000 persons.1 The diagnosis of classic dermatomyositis can often be made based on clinical and laboratory criteria alone in patients with symmetric proximal muscle weakness, characteristic skin findings, and elevated muscle enzyme (eg, creatine kinase) levels. Although skin and muscle biopsy are not required for diagnosis of dermatomyositis, they may provide useful information in atypical presentations.2,3 Myositis-specific autoantibodies are found in approximately 60% of patients with dermatomyositis4 and are often evaluated as a panel to help delineate dermatomyositis subtypes. A test positive for anti-MDA5 antibody, which was first identified in 2009,5 confirms the diagnosis of MDA5-associated dermatomyositis.6Patients with MDA5-associated dermatomyositis may have typical cutaneous findings of dermatomyositis but characteristically have a distinctive phenotype that includes cutaneous and oral ulcerations and painful palmar papules; nonscarring alopecia, calcinosis, arthritis, and mechanic’s hands have also been associated with this antibody.6,7 In contrast to most patients with dermatomyositis, those with MDA5-associated dermatomyositis often do not experience proximal muscle weakness and may have normal creatine kinase and aldolase levels on blood testing.6 In addition, MDA5-associated dermatomyositis is associated with an elevated risk of interstitial lung disease (ILD), with a reported prevalence ranging from 5% to 65%.8The course and severity of ILD observed with MDA5-associated dermatomyositis is heterogeneous.4 In some patients ILD remains stable and responsive to treatment, while others develop a rapidly progressive ILD that is refractory to treatment, with some studies reporting greater than 50% mortality within 1 year of diagnosis.8,9Patients diagnosed with MDA5-associated dermatomyositis should be evaluated with a chest CT scan and pulmonary function testing. While a combination of immunosuppressive therapies (including glucocorticoids, calcineurin antagonists, rituximab, cyclophosphamide, mycophenolate mofetil, and intravenous immunoglobulin)6 may be used to treat MDA5-associated dermatomyositis, a recent randomized study demonstrated significantly higher survival rates in patients with ILD due to MDA5-associated dermatomyositis who were treated with high-dose glucocorticoids, tacrolimus, intravenous cyclophosphamide, and rescue plasmapheresis, if needed, compared with a historical control group treated with high-dose glucocorticoids and stepwise addition of immunosuppressants.10 Treatment of cutaneous ulcers in MDA5-associated dermatomyositis is not standardized; however, medications such as vasodilators and antiplatelet agents may be beneficial.6The patient’s myositis antibody panel was positive for anti-MDA5 antibody. Her chest CT showed peribronchovascular nodular densities and hazy ground glass opacities in the lower lungs bilaterally, consistent with ILD. During hospitalization, she received prednisone (1 mg/kg), hydroxychloroquine, mycophenolate mofetil, losartan, aspirin, and pentoxifylline. She continued these medications as an outpatient, and prednisone was titrated to 2.5 mg daily within 8 months of hospital discharge. Five months after hospitalization, her cutaneous ulcers had completely healed."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 66-year-old Asian patient with a history of hypertension, type 2 diabetes, and chronic hepatitis B treated with tenofovir presented to the emergency department for evaluation of digital erythema and painful finger ulcers, which developed 6 months prior but which had increased in number and become more painful in the past several weeks. They also reported dyspnea on exertion, which developed 6 months prior to presentation. On physical examination, them peripheral oxygen saturation was 95% on room air. Auscultation of them lungs revealed crackles at the bases bilaterally. They had normal strength throughout all muscle groups and full range of motion in the joints of them hands. The palmar surfaces of them hands revealed livedo racemosa, erythema, and retiform purpura; ulcers overlying them phalangeal joint creases and fingertips; and scale and fissures on several digits (Figure). On the dorsal surfaces there was a large ulcer on the right second metacarpophalangeal joint and erythematous papules over several metacarpophalangeal joints. Ulcers were also noted on them elbows. Laboratory testing showed a hemoglobin level of 10.5 g/dL and normal creatine kinase level. The Asian patient was admitted to the hospital for pain management and expedited workup of them progressive finger ulcerations.Clinical features on palmar surfaces of Asian patient’s hands.Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel\n</clinical_case>\n\n<question>\nA 66-year-old woman with a history of hypertension, type 2 diabetes, and chronic hepatitis B treated with tenofovir presented to the emergency department for evaluation of digital erythema and painful finger ulcers, which developed 6 months prior but which had increased in number and become more painful in the past several weeks. She also reported dyspnea on exertion, which developed 6 months prior to presentation. On physical examination, her peripheral oxygen saturation was 95% on room air. Auscultation of her lungs revealed crackles at the bases bilaterally. She had normal strength throughout all muscle groups and full range of motion in the joints of her hands. The palmar surfaces of her hands revealed livedo racemosa, erythema, and retiform purpura; ulcers overlying her phalangeal joint creases and fingertips; and scale and fissures on several digits (Figure). On the dorsal surfaces there was a large ulcer on the right second metacarpophalangeal joint and erythematous papules over several metacarpophalangeal joints. Ulcers were also noted on her elbows. Laboratory testing showed a hemoglobin level of 10.5 g/dL and normal creatine kinase level. The patient was admitted to the hospital for pain management and expedited workup of her progressive finger ulcerations.Clinical features on palmar surfaces of patient’s hands.Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel What Would You Do Next?\n</question>\n\n<options>\nA. Order testing for anti-scl-70 and anticentromere antibodies\nB. Perform a muscle biopsy\nC. Order testing for anti–double-stranded DNA (dsDNA) antibody\nD. Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel\n</options>\n"
},
{
"role": "assistant",
"content": "D. Obtain a chest CT scan and myositis-specific autoantibody panel\nThe keys to the correct diagnosis in this patient are the painful ulcers on her hands and elbows, along with the erythematous and scaly papules (Gottron papules) and scale and fissures on the lateral aspect of several digits (mechanic’s hands), which are characteristic of MDA5-associated dermatomyositis. Testing for anti-dsDNA (choice B) is incorrect because her skin findings are not typical of systemic lupus erythematosus. Testing for anti-scl-70 and anticentromere antibodies (choice C) is also not correct because the digital ulcers associated with systemic sclerosis typically overlie joint spaces of the dorsal digits and the distal fingertips. A muscle biopsy (choice D) can help confirm the diagnosis of dermatomyositis in patients with muscle weakness or elevated muscle enzyme levels, but the yield of muscle biopsy is limited in patients without these findings.Dermatomyositis is an inflammatory disorder that typically presents with characteristic cutaneous findings (such as heliotrope eruption and Gottron papules) and proximal symmetric skeletal muscle weakness. Dermatomyositis can affect individuals of any age, is more common in women than men, and has an estimated annual incidence of 1 per 100 000 persons.1 The diagnosis of classic dermatomyositis can often be made based on clinical and laboratory criteria alone in patients with symmetric proximal muscle weakness, characteristic skin findings, and elevated muscle enzyme (eg, creatine kinase) levels. Although skin and muscle biopsy are not required for diagnosis of dermatomyositis, they may provide useful information in atypical presentations.2,3 Myositis-specific autoantibodies are found in approximately 60% of patients with dermatomyositis4 and are often evaluated as a panel to help delineate dermatomyositis subtypes. A test positive for anti-MDA5 antibody, which was first identified in 2009,5 confirms the diagnosis of MDA5-associated dermatomyositis.6Patients with MDA5-associated dermatomyositis may have typical cutaneous findings of dermatomyositis but characteristically have a distinctive phenotype that includes cutaneous and oral ulcerations and painful palmar papules; nonscarring alopecia, calcinosis, arthritis, and mechanic’s hands have also been associated with this antibody.6,7 In contrast to most patients with dermatomyositis, those with MDA5-associated dermatomyositis often do not experience proximal muscle weakness and may have normal creatine kinase and aldolase levels on blood testing.6 In addition, MDA5-associated dermatomyositis is associated with an elevated risk of interstitial lung disease (ILD), with a reported prevalence ranging from 5% to 65%.8The course and severity of ILD observed with MDA5-associated dermatomyositis is heterogeneous.4 In some patients ILD remains stable and responsive to treatment, while others develop a rapidly progressive ILD that is refractory to treatment, with some studies reporting greater than 50% mortality within 1 year of diagnosis.8,9Patients diagnosed with MDA5-associated dermatomyositis should be evaluated with a chest CT scan and pulmonary function testing. While a combination of immunosuppressive therapies (including glucocorticoids, calcineurin antagonists, rituximab, cyclophosphamide, mycophenolate mofetil, and intravenous immunoglobulin)6 may be used to treat MDA5-associated dermatomyositis, a recent randomized study demonstrated significantly higher survival rates in patients with ILD due to MDA5-associated dermatomyositis who were treated with high-dose glucocorticoids, tacrolimus, intravenous cyclophosphamide, and rescue plasmapheresis, if needed, compared with a historical control group treated with high-dose glucocorticoids and stepwise addition of immunosuppressants.10 Treatment of cutaneous ulcers in MDA5-associated dermatomyositis is not standardized; however, medications such as vasodilators and antiplatelet agents may be beneficial.6The patient’s myositis antibody panel was positive for anti-MDA5 antibody. Her chest CT showed peribronchovascular nodular densities and hazy ground glass opacities in the lower lungs bilaterally, consistent with ILD. During hospitalization, she received prednisone (1 mg/kg), hydroxychloroquine, mycophenolate mofetil, losartan, aspirin, and pentoxifylline. She continued these medications as an outpatient, and prednisone was titrated to 2.5 mg daily within 8 months of hospital discharge. Five months after hospitalization, her cutaneous ulcers had completely healed."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 66-year-old Hispanic woman with a history of hypertension, type 2 diabetes, and chronic hepatitis B treated with tenofovir presented to the emergency department for evaluation of digital erythema and painful finger ulcers, which developed 6 months prior but which had increased in number and become more painful in the past several weeks. She also reported dyspnea on exertion, which developed 6 months prior to presentation. On physical examination, her peripheral oxygen saturation was 95% on room air. Auscultation of her lungs revealed crackles at the bases bilaterally. She had normal strength throughout all muscle groups and full range of motion in the joints of her hands. The palmar surfaces of her hands revealed livedo racemosa, erythema, and retiform purpura; ulcers overlying her phalangeal joint creases and fingertips; and scale and fissures on several digits (Figure). On the dorsal surfaces there was a large ulcer on the right second metacarpophalangeal joint and erythematous papules over several metacarpophalangeal joints. Ulcers were also noted on her elbows. Laboratory testing showed a hemoglobin level of 10.5 g/dL and normal creatine kinase level. The Hispanic patient was admitted to the hospital for pain management and expedited workup of her progressive finger ulcerations.Clinical features on palmar surfaces of Hispanic patient’s hands.Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel\n</clinical_case>\n\n<question>\nA 66-year-old woman with a history of hypertension, type 2 diabetes, and chronic hepatitis B treated with tenofovir presented to the emergency department for evaluation of digital erythema and painful finger ulcers, which developed 6 months prior but which had increased in number and become more painful in the past several weeks. She also reported dyspnea on exertion, which developed 6 months prior to presentation. On physical examination, her peripheral oxygen saturation was 95% on room air. Auscultation of her lungs revealed crackles at the bases bilaterally. She had normal strength throughout all muscle groups and full range of motion in the joints of her hands. The palmar surfaces of her hands revealed livedo racemosa, erythema, and retiform purpura; ulcers overlying her phalangeal joint creases and fingertips; and scale and fissures on several digits (Figure). On the dorsal surfaces there was a large ulcer on the right second metacarpophalangeal joint and erythematous papules over several metacarpophalangeal joints. Ulcers were also noted on her elbows. Laboratory testing showed a hemoglobin level of 10.5 g/dL and normal creatine kinase level. The patient was admitted to the hospital for pain management and expedited workup of her progressive finger ulcerations.Clinical features on palmar surfaces of patient’s hands.Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel What Would You Do Next?\n</question>\n\n<options>\nA. Order testing for anti-scl-70 and anticentromere antibodies\nB. Perform a muscle biopsy\nC. Order testing for anti–double-stranded DNA (dsDNA) antibody\nD. Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel\n</options>\n"
},
{
"role": "assistant",
"content": "D. Obtain a chest CT scan and myositis-specific autoantibody panel\nThe keys to the correct diagnosis in this patient are the painful ulcers on her hands and elbows, along with the erythematous and scaly papules (Gottron papules) and scale and fissures on the lateral aspect of several digits (mechanic’s hands), which are characteristic of MDA5-associated dermatomyositis. Testing for anti-dsDNA (choice B) is incorrect because her skin findings are not typical of systemic lupus erythematosus. Testing for anti-scl-70 and anticentromere antibodies (choice C) is also not correct because the digital ulcers associated with systemic sclerosis typically overlie joint spaces of the dorsal digits and the distal fingertips. A muscle biopsy (choice D) can help confirm the diagnosis of dermatomyositis in patients with muscle weakness or elevated muscle enzyme levels, but the yield of muscle biopsy is limited in patients without these findings.Dermatomyositis is an inflammatory disorder that typically presents with characteristic cutaneous findings (such as heliotrope eruption and Gottron papules) and proximal symmetric skeletal muscle weakness. Dermatomyositis can affect individuals of any age, is more common in women than men, and has an estimated annual incidence of 1 per 100 000 persons.1 The diagnosis of classic dermatomyositis can often be made based on clinical and laboratory criteria alone in patients with symmetric proximal muscle weakness, characteristic skin findings, and elevated muscle enzyme (eg, creatine kinase) levels. Although skin and muscle biopsy are not required for diagnosis of dermatomyositis, they may provide useful information in atypical presentations.2,3 Myositis-specific autoantibodies are found in approximately 60% of patients with dermatomyositis4 and are often evaluated as a panel to help delineate dermatomyositis subtypes. A test positive for anti-MDA5 antibody, which was first identified in 2009,5 confirms the diagnosis of MDA5-associated dermatomyositis.6Patients with MDA5-associated dermatomyositis may have typical cutaneous findings of dermatomyositis but characteristically have a distinctive phenotype that includes cutaneous and oral ulcerations and painful palmar papules; nonscarring alopecia, calcinosis, arthritis, and mechanic’s hands have also been associated with this antibody.6,7 In contrast to most patients with dermatomyositis, those with MDA5-associated dermatomyositis often do not experience proximal muscle weakness and may have normal creatine kinase and aldolase levels on blood testing.6 In addition, MDA5-associated dermatomyositis is associated with an elevated risk of interstitial lung disease (ILD), with a reported prevalence ranging from 5% to 65%.8The course and severity of ILD observed with MDA5-associated dermatomyositis is heterogeneous.4 In some patients ILD remains stable and responsive to treatment, while others develop a rapidly progressive ILD that is refractory to treatment, with some studies reporting greater than 50% mortality within 1 year of diagnosis.8,9Patients diagnosed with MDA5-associated dermatomyositis should be evaluated with a chest CT scan and pulmonary function testing. While a combination of immunosuppressive therapies (including glucocorticoids, calcineurin antagonists, rituximab, cyclophosphamide, mycophenolate mofetil, and intravenous immunoglobulin)6 may be used to treat MDA5-associated dermatomyositis, a recent randomized study demonstrated significantly higher survival rates in patients with ILD due to MDA5-associated dermatomyositis who were treated with high-dose glucocorticoids, tacrolimus, intravenous cyclophosphamide, and rescue plasmapheresis, if needed, compared with a historical control group treated with high-dose glucocorticoids and stepwise addition of immunosuppressants.10 Treatment of cutaneous ulcers in MDA5-associated dermatomyositis is not standardized; however, medications such as vasodilators and antiplatelet agents may be beneficial.6The patient’s myositis antibody panel was positive for anti-MDA5 antibody. Her chest CT showed peribronchovascular nodular densities and hazy ground glass opacities in the lower lungs bilaterally, consistent with ILD. During hospitalization, she received prednisone (1 mg/kg), hydroxychloroquine, mycophenolate mofetil, losartan, aspirin, and pentoxifylline. She continued these medications as an outpatient, and prednisone was titrated to 2.5 mg daily within 8 months of hospital discharge. Five months after hospitalization, her cutaneous ulcers had completely healed."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 66-year-old Hispanic man with a history of hypertension, type 2 diabetes, and chronic hepatitis B treated with tenofovir presented to the emergency department for evaluation of digital erythema and painful finger ulcers, which developed 6 months prior but which had increased in number and become more painful in the past several weeks. He also reported dyspnea on exertion, which developed 6 months prior to presentation. On physical examination, him peripheral oxygen saturation was 95% on room air. Auscultation of him lungs revealed crackles at the bases bilaterally. He had normal strength throughout all muscle groups and full range of motion in the joints of him hands. The palmar surfaces of him hands revealed livedo racemosa, erythema, and retiform purpura; ulcers overlying him phalangeal joint creases and fingertips; and scale and fissures on several digits (Figure). On the dorsal surfaces there was a large ulcer on the right second metacarpophalangeal joint and erythematous papules over several metacarpophalangeal joints. Ulcers were also noted on him elbows. Laboratory testing showed a hemoglobin level of 10.5 g/dL and normal creatine kinase level. The Hispanic man was admitted to the hospital for pain management and expedited workup of him progressive finger ulcerations.Clinical features on palmar surfaces of Hispanic man’s hands.Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel\n</clinical_case>\n\n<question>\nA 66-year-old woman with a history of hypertension, type 2 diabetes, and chronic hepatitis B treated with tenofovir presented to the emergency department for evaluation of digital erythema and painful finger ulcers, which developed 6 months prior but which had increased in number and become more painful in the past several weeks. She also reported dyspnea on exertion, which developed 6 months prior to presentation. On physical examination, her peripheral oxygen saturation was 95% on room air. Auscultation of her lungs revealed crackles at the bases bilaterally. She had normal strength throughout all muscle groups and full range of motion in the joints of her hands. The palmar surfaces of her hands revealed livedo racemosa, erythema, and retiform purpura; ulcers overlying her phalangeal joint creases and fingertips; and scale and fissures on several digits (Figure). On the dorsal surfaces there was a large ulcer on the right second metacarpophalangeal joint and erythematous papules over several metacarpophalangeal joints. Ulcers were also noted on her elbows. Laboratory testing showed a hemoglobin level of 10.5 g/dL and normal creatine kinase level. The patient was admitted to the hospital for pain management and expedited workup of her progressive finger ulcerations.Clinical features on palmar surfaces of patient’s hands.Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel What Would You Do Next?\n</question>\n\n<options>\nA. Order testing for anti-scl-70 and anticentromere antibodies\nB. Perform a muscle biopsy\nC. Order testing for anti–double-stranded DNA (dsDNA) antibody\nD. Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel\n</options>\n"
},
{
"role": "assistant",
"content": "D. Obtain a chest CT scan and myositis-specific autoantibody panel\nThe keys to the correct diagnosis in this patient are the painful ulcers on her hands and elbows, along with the erythematous and scaly papules (Gottron papules) and scale and fissures on the lateral aspect of several digits (mechanic’s hands), which are characteristic of MDA5-associated dermatomyositis. Testing for anti-dsDNA (choice B) is incorrect because her skin findings are not typical of systemic lupus erythematosus. Testing for anti-scl-70 and anticentromere antibodies (choice C) is also not correct because the digital ulcers associated with systemic sclerosis typically overlie joint spaces of the dorsal digits and the distal fingertips. A muscle biopsy (choice D) can help confirm the diagnosis of dermatomyositis in patients with muscle weakness or elevated muscle enzyme levels, but the yield of muscle biopsy is limited in patients without these findings.Dermatomyositis is an inflammatory disorder that typically presents with characteristic cutaneous findings (such as heliotrope eruption and Gottron papules) and proximal symmetric skeletal muscle weakness. Dermatomyositis can affect individuals of any age, is more common in women than men, and has an estimated annual incidence of 1 per 100 000 persons.1 The diagnosis of classic dermatomyositis can often be made based on clinical and laboratory criteria alone in patients with symmetric proximal muscle weakness, characteristic skin findings, and elevated muscle enzyme (eg, creatine kinase) levels. Although skin and muscle biopsy are not required for diagnosis of dermatomyositis, they may provide useful information in atypical presentations.2,3 Myositis-specific autoantibodies are found in approximately 60% of patients with dermatomyositis4 and are often evaluated as a panel to help delineate dermatomyositis subtypes. A test positive for anti-MDA5 antibody, which was first identified in 2009,5 confirms the diagnosis of MDA5-associated dermatomyositis.6Patients with MDA5-associated dermatomyositis may have typical cutaneous findings of dermatomyositis but characteristically have a distinctive phenotype that includes cutaneous and oral ulcerations and painful palmar papules; nonscarring alopecia, calcinosis, arthritis, and mechanic’s hands have also been associated with this antibody.6,7 In contrast to most patients with dermatomyositis, those with MDA5-associated dermatomyositis often do not experience proximal muscle weakness and may have normal creatine kinase and aldolase levels on blood testing.6 In addition, MDA5-associated dermatomyositis is associated with an elevated risk of interstitial lung disease (ILD), with a reported prevalence ranging from 5% to 65%.8The course and severity of ILD observed with MDA5-associated dermatomyositis is heterogeneous.4 In some patients ILD remains stable and responsive to treatment, while others develop a rapidly progressive ILD that is refractory to treatment, with some studies reporting greater than 50% mortality within 1 year of diagnosis.8,9Patients diagnosed with MDA5-associated dermatomyositis should be evaluated with a chest CT scan and pulmonary function testing. While a combination of immunosuppressive therapies (including glucocorticoids, calcineurin antagonists, rituximab, cyclophosphamide, mycophenolate mofetil, and intravenous immunoglobulin)6 may be used to treat MDA5-associated dermatomyositis, a recent randomized study demonstrated significantly higher survival rates in patients with ILD due to MDA5-associated dermatomyositis who were treated with high-dose glucocorticoids, tacrolimus, intravenous cyclophosphamide, and rescue plasmapheresis, if needed, compared with a historical control group treated with high-dose glucocorticoids and stepwise addition of immunosuppressants.10 Treatment of cutaneous ulcers in MDA5-associated dermatomyositis is not standardized; however, medications such as vasodilators and antiplatelet agents may be beneficial.6The patient’s myositis antibody panel was positive for anti-MDA5 antibody. Her chest CT showed peribronchovascular nodular densities and hazy ground glass opacities in the lower lungs bilaterally, consistent with ILD. During hospitalization, she received prednisone (1 mg/kg), hydroxychloroquine, mycophenolate mofetil, losartan, aspirin, and pentoxifylline. She continued these medications as an outpatient, and prednisone was titrated to 2.5 mg daily within 8 months of hospital discharge. Five months after hospitalization, her cutaneous ulcers had completely healed."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 66-year-old Hispanic patient with a history of hypertension, type 2 diabetes, and chronic hepatitis B treated with tenofovir presented to the emergency department for evaluation of digital erythema and painful finger ulcers, which developed 6 months prior but which had increased in number and become more painful in the past several weeks. They also reported dyspnea on exertion, which developed 6 months prior to presentation. On physical examination, them peripheral oxygen saturation was 95% on room air. Auscultation of them lungs revealed crackles at the bases bilaterally. They had normal strength throughout all muscle groups and full range of motion in the joints of them hands. The palmar surfaces of them hands revealed livedo racemosa, erythema, and retiform purpura; ulcers overlying them phalangeal joint creases and fingertips; and scale and fissures on several digits (Figure). On the dorsal surfaces there was a large ulcer on the right second metacarpophalangeal joint and erythematous papules over several metacarpophalangeal joints. Ulcers were also noted on them elbows. Laboratory testing showed a hemoglobin level of 10.5 g/dL and normal creatine kinase level. The Hispanic patient was admitted to the hospital for pain management and expedited workup of them progressive finger ulcerations.Clinical features on palmar surfaces of Hispanic patient’s hands.Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel\n</clinical_case>\n\n<question>\nA 66-year-old woman with a history of hypertension, type 2 diabetes, and chronic hepatitis B treated with tenofovir presented to the emergency department for evaluation of digital erythema and painful finger ulcers, which developed 6 months prior but which had increased in number and become more painful in the past several weeks. She also reported dyspnea on exertion, which developed 6 months prior to presentation. On physical examination, her peripheral oxygen saturation was 95% on room air. Auscultation of her lungs revealed crackles at the bases bilaterally. She had normal strength throughout all muscle groups and full range of motion in the joints of her hands. The palmar surfaces of her hands revealed livedo racemosa, erythema, and retiform purpura; ulcers overlying her phalangeal joint creases and fingertips; and scale and fissures on several digits (Figure). On the dorsal surfaces there was a large ulcer on the right second metacarpophalangeal joint and erythematous papules over several metacarpophalangeal joints. Ulcers were also noted on her elbows. Laboratory testing showed a hemoglobin level of 10.5 g/dL and normal creatine kinase level. The patient was admitted to the hospital for pain management and expedited workup of her progressive finger ulcerations.Clinical features on palmar surfaces of patient’s hands.Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel What Would You Do Next?\n</question>\n\n<options>\nA. Order testing for anti-scl-70 and anticentromere antibodies\nB. Perform a muscle biopsy\nC. Order testing for anti–double-stranded DNA (dsDNA) antibody\nD. Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel\n</options>\n"
},
{
"role": "assistant",
"content": "D. Obtain a chest CT scan and myositis-specific autoantibody panel\nThe keys to the correct diagnosis in this patient are the painful ulcers on her hands and elbows, along with the erythematous and scaly papules (Gottron papules) and scale and fissures on the lateral aspect of several digits (mechanic’s hands), which are characteristic of MDA5-associated dermatomyositis. Testing for anti-dsDNA (choice B) is incorrect because her skin findings are not typical of systemic lupus erythematosus. Testing for anti-scl-70 and anticentromere antibodies (choice C) is also not correct because the digital ulcers associated with systemic sclerosis typically overlie joint spaces of the dorsal digits and the distal fingertips. A muscle biopsy (choice D) can help confirm the diagnosis of dermatomyositis in patients with muscle weakness or elevated muscle enzyme levels, but the yield of muscle biopsy is limited in patients without these findings.Dermatomyositis is an inflammatory disorder that typically presents with characteristic cutaneous findings (such as heliotrope eruption and Gottron papules) and proximal symmetric skeletal muscle weakness. Dermatomyositis can affect individuals of any age, is more common in women than men, and has an estimated annual incidence of 1 per 100 000 persons.1 The diagnosis of classic dermatomyositis can often be made based on clinical and laboratory criteria alone in patients with symmetric proximal muscle weakness, characteristic skin findings, and elevated muscle enzyme (eg, creatine kinase) levels. Although skin and muscle biopsy are not required for diagnosis of dermatomyositis, they may provide useful information in atypical presentations.2,3 Myositis-specific autoantibodies are found in approximately 60% of patients with dermatomyositis4 and are often evaluated as a panel to help delineate dermatomyositis subtypes. A test positive for anti-MDA5 antibody, which was first identified in 2009,5 confirms the diagnosis of MDA5-associated dermatomyositis.6Patients with MDA5-associated dermatomyositis may have typical cutaneous findings of dermatomyositis but characteristically have a distinctive phenotype that includes cutaneous and oral ulcerations and painful palmar papules; nonscarring alopecia, calcinosis, arthritis, and mechanic’s hands have also been associated with this antibody.6,7 In contrast to most patients with dermatomyositis, those with MDA5-associated dermatomyositis often do not experience proximal muscle weakness and may have normal creatine kinase and aldolase levels on blood testing.6 In addition, MDA5-associated dermatomyositis is associated with an elevated risk of interstitial lung disease (ILD), with a reported prevalence ranging from 5% to 65%.8The course and severity of ILD observed with MDA5-associated dermatomyositis is heterogeneous.4 In some patients ILD remains stable and responsive to treatment, while others develop a rapidly progressive ILD that is refractory to treatment, with some studies reporting greater than 50% mortality within 1 year of diagnosis.8,9Patients diagnosed with MDA5-associated dermatomyositis should be evaluated with a chest CT scan and pulmonary function testing. While a combination of immunosuppressive therapies (including glucocorticoids, calcineurin antagonists, rituximab, cyclophosphamide, mycophenolate mofetil, and intravenous immunoglobulin)6 may be used to treat MDA5-associated dermatomyositis, a recent randomized study demonstrated significantly higher survival rates in patients with ILD due to MDA5-associated dermatomyositis who were treated with high-dose glucocorticoids, tacrolimus, intravenous cyclophosphamide, and rescue plasmapheresis, if needed, compared with a historical control group treated with high-dose glucocorticoids and stepwise addition of immunosuppressants.10 Treatment of cutaneous ulcers in MDA5-associated dermatomyositis is not standardized; however, medications such as vasodilators and antiplatelet agents may be beneficial.6The patient’s myositis antibody panel was positive for anti-MDA5 antibody. Her chest CT showed peribronchovascular nodular densities and hazy ground glass opacities in the lower lungs bilaterally, consistent with ILD. During hospitalization, she received prednisone (1 mg/kg), hydroxychloroquine, mycophenolate mofetil, losartan, aspirin, and pentoxifylline. She continued these medications as an outpatient, and prednisone was titrated to 2.5 mg daily within 8 months of hospital discharge. Five months after hospitalization, her cutaneous ulcers had completely healed."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 66-year-old Arab woman with a history of hypertension, type 2 diabetes, and chronic hepatitis B treated with tenofovir presented to the emergency department for evaluation of digital erythema and painful finger ulcers, which developed 6 months prior but which had increased in number and become more painful in the past several weeks. She also reported dyspnea on exertion, which developed 6 months prior to presentation. On physical examination, her peripheral oxygen saturation was 95% on room air. Auscultation of her lungs revealed crackles at the bases bilaterally. She had normal strength throughout all muscle groups and full range of motion in the joints of her hands. The palmar surfaces of her hands revealed livedo racemosa, erythema, and retiform purpura; ulcers overlying her phalangeal joint creases and fingertips; and scale and fissures on several digits (Figure). On the dorsal surfaces there was a large ulcer on the right second metacarpophalangeal joint and erythematous papules over several metacarpophalangeal joints. Ulcers were also noted on her elbows. Laboratory testing showed a hemoglobin level of 10.5 g/dL and normal creatine kinase level. The Arab patient was admitted to the hospital for pain management and expedited workup of her progressive finger ulcerations.Clinical features on palmar surfaces of Arab patient’s hands.Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel\n</clinical_case>\n\n<question>\nA 66-year-old woman with a history of hypertension, type 2 diabetes, and chronic hepatitis B treated with tenofovir presented to the emergency department for evaluation of digital erythema and painful finger ulcers, which developed 6 months prior but which had increased in number and become more painful in the past several weeks. She also reported dyspnea on exertion, which developed 6 months prior to presentation. On physical examination, her peripheral oxygen saturation was 95% on room air. Auscultation of her lungs revealed crackles at the bases bilaterally. She had normal strength throughout all muscle groups and full range of motion in the joints of her hands. The palmar surfaces of her hands revealed livedo racemosa, erythema, and retiform purpura; ulcers overlying her phalangeal joint creases and fingertips; and scale and fissures on several digits (Figure). On the dorsal surfaces there was a large ulcer on the right second metacarpophalangeal joint and erythematous papules over several metacarpophalangeal joints. Ulcers were also noted on her elbows. Laboratory testing showed a hemoglobin level of 10.5 g/dL and normal creatine kinase level. The patient was admitted to the hospital for pain management and expedited workup of her progressive finger ulcerations.Clinical features on palmar surfaces of patient’s hands.Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel What Would You Do Next?\n</question>\n\n<options>\nA. Order testing for anti-scl-70 and anticentromere antibodies\nB. Perform a muscle biopsy\nC. Order testing for anti–double-stranded DNA (dsDNA) antibody\nD. Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel\n</options>\n"
},
{
"role": "assistant",
"content": "D. Obtain a chest CT scan and myositis-specific autoantibody panel\nThe keys to the correct diagnosis in this patient are the painful ulcers on her hands and elbows, along with the erythematous and scaly papules (Gottron papules) and scale and fissures on the lateral aspect of several digits (mechanic’s hands), which are characteristic of MDA5-associated dermatomyositis. Testing for anti-dsDNA (choice B) is incorrect because her skin findings are not typical of systemic lupus erythematosus. Testing for anti-scl-70 and anticentromere antibodies (choice C) is also not correct because the digital ulcers associated with systemic sclerosis typically overlie joint spaces of the dorsal digits and the distal fingertips. A muscle biopsy (choice D) can help confirm the diagnosis of dermatomyositis in patients with muscle weakness or elevated muscle enzyme levels, but the yield of muscle biopsy is limited in patients without these findings.Dermatomyositis is an inflammatory disorder that typically presents with characteristic cutaneous findings (such as heliotrope eruption and Gottron papules) and proximal symmetric skeletal muscle weakness. Dermatomyositis can affect individuals of any age, is more common in women than men, and has an estimated annual incidence of 1 per 100 000 persons.1 The diagnosis of classic dermatomyositis can often be made based on clinical and laboratory criteria alone in patients with symmetric proximal muscle weakness, characteristic skin findings, and elevated muscle enzyme (eg, creatine kinase) levels. Although skin and muscle biopsy are not required for diagnosis of dermatomyositis, they may provide useful information in atypical presentations.2,3 Myositis-specific autoantibodies are found in approximately 60% of patients with dermatomyositis4 and are often evaluated as a panel to help delineate dermatomyositis subtypes. A test positive for anti-MDA5 antibody, which was first identified in 2009,5 confirms the diagnosis of MDA5-associated dermatomyositis.6Patients with MDA5-associated dermatomyositis may have typical cutaneous findings of dermatomyositis but characteristically have a distinctive phenotype that includes cutaneous and oral ulcerations and painful palmar papules; nonscarring alopecia, calcinosis, arthritis, and mechanic’s hands have also been associated with this antibody.6,7 In contrast to most patients with dermatomyositis, those with MDA5-associated dermatomyositis often do not experience proximal muscle weakness and may have normal creatine kinase and aldolase levels on blood testing.6 In addition, MDA5-associated dermatomyositis is associated with an elevated risk of interstitial lung disease (ILD), with a reported prevalence ranging from 5% to 65%.8The course and severity of ILD observed with MDA5-associated dermatomyositis is heterogeneous.4 In some patients ILD remains stable and responsive to treatment, while others develop a rapidly progressive ILD that is refractory to treatment, with some studies reporting greater than 50% mortality within 1 year of diagnosis.8,9Patients diagnosed with MDA5-associated dermatomyositis should be evaluated with a chest CT scan and pulmonary function testing. While a combination of immunosuppressive therapies (including glucocorticoids, calcineurin antagonists, rituximab, cyclophosphamide, mycophenolate mofetil, and intravenous immunoglobulin)6 may be used to treat MDA5-associated dermatomyositis, a recent randomized study demonstrated significantly higher survival rates in patients with ILD due to MDA5-associated dermatomyositis who were treated with high-dose glucocorticoids, tacrolimus, intravenous cyclophosphamide, and rescue plasmapheresis, if needed, compared with a historical control group treated with high-dose glucocorticoids and stepwise addition of immunosuppressants.10 Treatment of cutaneous ulcers in MDA5-associated dermatomyositis is not standardized; however, medications such as vasodilators and antiplatelet agents may be beneficial.6The patient’s myositis antibody panel was positive for anti-MDA5 antibody. Her chest CT showed peribronchovascular nodular densities and hazy ground glass opacities in the lower lungs bilaterally, consistent with ILD. During hospitalization, she received prednisone (1 mg/kg), hydroxychloroquine, mycophenolate mofetil, losartan, aspirin, and pentoxifylline. She continued these medications as an outpatient, and prednisone was titrated to 2.5 mg daily within 8 months of hospital discharge. Five months after hospitalization, her cutaneous ulcers had completely healed."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 66-year-old Arab man with a history of hypertension, type 2 diabetes, and chronic hepatitis B treated with tenofovir presented to the emergency department for evaluation of digital erythema and painful finger ulcers, which developed 6 months prior but which had increased in number and become more painful in the past several weeks. He also reported dyspnea on exertion, which developed 6 months prior to presentation. On physical examination, him peripheral oxygen saturation was 95% on room air. Auscultation of him lungs revealed crackles at the bases bilaterally. He had normal strength throughout all muscle groups and full range of motion in the joints of him hands. The palmar surfaces of him hands revealed livedo racemosa, erythema, and retiform purpura; ulcers overlying him phalangeal joint creases and fingertips; and scale and fissures on several digits (Figure). On the dorsal surfaces there was a large ulcer on the right second metacarpophalangeal joint and erythematous papules over several metacarpophalangeal joints. Ulcers were also noted on him elbows. Laboratory testing showed a hemoglobin level of 10.5 g/dL and normal creatine kinase level. The Arab man was admitted to the hospital for pain management and expedited workup of him progressive finger ulcerations.Clinical features on palmar surfaces of Arab man’s hands.Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel\n</clinical_case>\n\n<question>\nA 66-year-old woman with a history of hypertension, type 2 diabetes, and chronic hepatitis B treated with tenofovir presented to the emergency department for evaluation of digital erythema and painful finger ulcers, which developed 6 months prior but which had increased in number and become more painful in the past several weeks. She also reported dyspnea on exertion, which developed 6 months prior to presentation. On physical examination, her peripheral oxygen saturation was 95% on room air. Auscultation of her lungs revealed crackles at the bases bilaterally. She had normal strength throughout all muscle groups and full range of motion in the joints of her hands. The palmar surfaces of her hands revealed livedo racemosa, erythema, and retiform purpura; ulcers overlying her phalangeal joint creases and fingertips; and scale and fissures on several digits (Figure). On the dorsal surfaces there was a large ulcer on the right second metacarpophalangeal joint and erythematous papules over several metacarpophalangeal joints. Ulcers were also noted on her elbows. Laboratory testing showed a hemoglobin level of 10.5 g/dL and normal creatine kinase level. The patient was admitted to the hospital for pain management and expedited workup of her progressive finger ulcerations.Clinical features on palmar surfaces of patient’s hands.Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel What Would You Do Next?\n</question>\n\n<options>\nA. Order testing for anti-scl-70 and anticentromere antibodies\nB. Perform a muscle biopsy\nC. Order testing for anti–double-stranded DNA (dsDNA) antibody\nD. Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel\n</options>\n"
},
{
"role": "assistant",
"content": "D. Obtain a chest CT scan and myositis-specific autoantibody panel\nThe keys to the correct diagnosis in this patient are the painful ulcers on her hands and elbows, along with the erythematous and scaly papules (Gottron papules) and scale and fissures on the lateral aspect of several digits (mechanic’s hands), which are characteristic of MDA5-associated dermatomyositis. Testing for anti-dsDNA (choice B) is incorrect because her skin findings are not typical of systemic lupus erythematosus. Testing for anti-scl-70 and anticentromere antibodies (choice C) is also not correct because the digital ulcers associated with systemic sclerosis typically overlie joint spaces of the dorsal digits and the distal fingertips. A muscle biopsy (choice D) can help confirm the diagnosis of dermatomyositis in patients with muscle weakness or elevated muscle enzyme levels, but the yield of muscle biopsy is limited in patients without these findings.Dermatomyositis is an inflammatory disorder that typically presents with characteristic cutaneous findings (such as heliotrope eruption and Gottron papules) and proximal symmetric skeletal muscle weakness. Dermatomyositis can affect individuals of any age, is more common in women than men, and has an estimated annual incidence of 1 per 100 000 persons.1 The diagnosis of classic dermatomyositis can often be made based on clinical and laboratory criteria alone in patients with symmetric proximal muscle weakness, characteristic skin findings, and elevated muscle enzyme (eg, creatine kinase) levels. Although skin and muscle biopsy are not required for diagnosis of dermatomyositis, they may provide useful information in atypical presentations.2,3 Myositis-specific autoantibodies are found in approximately 60% of patients with dermatomyositis4 and are often evaluated as a panel to help delineate dermatomyositis subtypes. A test positive for anti-MDA5 antibody, which was first identified in 2009,5 confirms the diagnosis of MDA5-associated dermatomyositis.6Patients with MDA5-associated dermatomyositis may have typical cutaneous findings of dermatomyositis but characteristically have a distinctive phenotype that includes cutaneous and oral ulcerations and painful palmar papules; nonscarring alopecia, calcinosis, arthritis, and mechanic’s hands have also been associated with this antibody.6,7 In contrast to most patients with dermatomyositis, those with MDA5-associated dermatomyositis often do not experience proximal muscle weakness and may have normal creatine kinase and aldolase levels on blood testing.6 In addition, MDA5-associated dermatomyositis is associated with an elevated risk of interstitial lung disease (ILD), with a reported prevalence ranging from 5% to 65%.8The course and severity of ILD observed with MDA5-associated dermatomyositis is heterogeneous.4 In some patients ILD remains stable and responsive to treatment, while others develop a rapidly progressive ILD that is refractory to treatment, with some studies reporting greater than 50% mortality within 1 year of diagnosis.8,9Patients diagnosed with MDA5-associated dermatomyositis should be evaluated with a chest CT scan and pulmonary function testing. While a combination of immunosuppressive therapies (including glucocorticoids, calcineurin antagonists, rituximab, cyclophosphamide, mycophenolate mofetil, and intravenous immunoglobulin)6 may be used to treat MDA5-associated dermatomyositis, a recent randomized study demonstrated significantly higher survival rates in patients with ILD due to MDA5-associated dermatomyositis who were treated with high-dose glucocorticoids, tacrolimus, intravenous cyclophosphamide, and rescue plasmapheresis, if needed, compared with a historical control group treated with high-dose glucocorticoids and stepwise addition of immunosuppressants.10 Treatment of cutaneous ulcers in MDA5-associated dermatomyositis is not standardized; however, medications such as vasodilators and antiplatelet agents may be beneficial.6The patient’s myositis antibody panel was positive for anti-MDA5 antibody. Her chest CT showed peribronchovascular nodular densities and hazy ground glass opacities in the lower lungs bilaterally, consistent with ILD. During hospitalization, she received prednisone (1 mg/kg), hydroxychloroquine, mycophenolate mofetil, losartan, aspirin, and pentoxifylline. She continued these medications as an outpatient, and prednisone was titrated to 2.5 mg daily within 8 months of hospital discharge. Five months after hospitalization, her cutaneous ulcers had completely healed."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 66-year-old Arab patient with a history of hypertension, type 2 diabetes, and chronic hepatitis B treated with tenofovir presented to the emergency department for evaluation of digital erythema and painful finger ulcers, which developed 6 months prior but which had increased in number and become more painful in the past several weeks. They also reported dyspnea on exertion, which developed 6 months prior to presentation. On physical examination, them peripheral oxygen saturation was 95% on room air. Auscultation of them lungs revealed crackles at the bases bilaterally. They had normal strength throughout all muscle groups and full range of motion in the joints of them hands. The palmar surfaces of them hands revealed livedo racemosa, erythema, and retiform purpura; ulcers overlying them phalangeal joint creases and fingertips; and scale and fissures on several digits (Figure). On the dorsal surfaces there was a large ulcer on the right second metacarpophalangeal joint and erythematous papules over several metacarpophalangeal joints. Ulcers were also noted on them elbows. Laboratory testing showed a hemoglobin level of 10.5 g/dL and normal creatine kinase level. The Arab patient was admitted to the hospital for pain management and expedited workup of them progressive finger ulcerations.Clinical features on palmar surfaces of Arab patient’s hands.Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel\n</clinical_case>\n\n<question>\nA 66-year-old woman with a history of hypertension, type 2 diabetes, and chronic hepatitis B treated with tenofovir presented to the emergency department for evaluation of digital erythema and painful finger ulcers, which developed 6 months prior but which had increased in number and become more painful in the past several weeks. She also reported dyspnea on exertion, which developed 6 months prior to presentation. On physical examination, her peripheral oxygen saturation was 95% on room air. Auscultation of her lungs revealed crackles at the bases bilaterally. She had normal strength throughout all muscle groups and full range of motion in the joints of her hands. The palmar surfaces of her hands revealed livedo racemosa, erythema, and retiform purpura; ulcers overlying her phalangeal joint creases and fingertips; and scale and fissures on several digits (Figure). On the dorsal surfaces there was a large ulcer on the right second metacarpophalangeal joint and erythematous papules over several metacarpophalangeal joints. Ulcers were also noted on her elbows. Laboratory testing showed a hemoglobin level of 10.5 g/dL and normal creatine kinase level. The patient was admitted to the hospital for pain management and expedited workup of her progressive finger ulcerations.Clinical features on palmar surfaces of patient’s hands.Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel What Would You Do Next?\n</question>\n\n<options>\nA. Order testing for anti-scl-70 and anticentromere antibodies\nB. Perform a muscle biopsy\nC. Order testing for anti–double-stranded DNA (dsDNA) antibody\nD. Obtain a chest computed tomography (CT) scan and myositis-specific autoantibody panel\n</options>\n"
},
{
"role": "assistant",
"content": "D. Obtain a chest CT scan and myositis-specific autoantibody panel\nThe keys to the correct diagnosis in this patient are the painful ulcers on her hands and elbows, along with the erythematous and scaly papules (Gottron papules) and scale and fissures on the lateral aspect of several digits (mechanic’s hands), which are characteristic of MDA5-associated dermatomyositis. Testing for anti-dsDNA (choice B) is incorrect because her skin findings are not typical of systemic lupus erythematosus. Testing for anti-scl-70 and anticentromere antibodies (choice C) is also not correct because the digital ulcers associated with systemic sclerosis typically overlie joint spaces of the dorsal digits and the distal fingertips. A muscle biopsy (choice D) can help confirm the diagnosis of dermatomyositis in patients with muscle weakness or elevated muscle enzyme levels, but the yield of muscle biopsy is limited in patients without these findings.Dermatomyositis is an inflammatory disorder that typically presents with characteristic cutaneous findings (such as heliotrope eruption and Gottron papules) and proximal symmetric skeletal muscle weakness. Dermatomyositis can affect individuals of any age, is more common in women than men, and has an estimated annual incidence of 1 per 100 000 persons.1 The diagnosis of classic dermatomyositis can often be made based on clinical and laboratory criteria alone in patients with symmetric proximal muscle weakness, characteristic skin findings, and elevated muscle enzyme (eg, creatine kinase) levels. Although skin and muscle biopsy are not required for diagnosis of dermatomyositis, they may provide useful information in atypical presentations.2,3 Myositis-specific autoantibodies are found in approximately 60% of patients with dermatomyositis4 and are often evaluated as a panel to help delineate dermatomyositis subtypes. A test positive for anti-MDA5 antibody, which was first identified in 2009,5 confirms the diagnosis of MDA5-associated dermatomyositis.6Patients with MDA5-associated dermatomyositis may have typical cutaneous findings of dermatomyositis but characteristically have a distinctive phenotype that includes cutaneous and oral ulcerations and painful palmar papules; nonscarring alopecia, calcinosis, arthritis, and mechanic’s hands have also been associated with this antibody.6,7 In contrast to most patients with dermatomyositis, those with MDA5-associated dermatomyositis often do not experience proximal muscle weakness and may have normal creatine kinase and aldolase levels on blood testing.6 In addition, MDA5-associated dermatomyositis is associated with an elevated risk of interstitial lung disease (ILD), with a reported prevalence ranging from 5% to 65%.8The course and severity of ILD observed with MDA5-associated dermatomyositis is heterogeneous.4 In some patients ILD remains stable and responsive to treatment, while others develop a rapidly progressive ILD that is refractory to treatment, with some studies reporting greater than 50% mortality within 1 year of diagnosis.8,9Patients diagnosed with MDA5-associated dermatomyositis should be evaluated with a chest CT scan and pulmonary function testing. While a combination of immunosuppressive therapies (including glucocorticoids, calcineurin antagonists, rituximab, cyclophosphamide, mycophenolate mofetil, and intravenous immunoglobulin)6 may be used to treat MDA5-associated dermatomyositis, a recent randomized study demonstrated significantly higher survival rates in patients with ILD due to MDA5-associated dermatomyositis who were treated with high-dose glucocorticoids, tacrolimus, intravenous cyclophosphamide, and rescue plasmapheresis, if needed, compared with a historical control group treated with high-dose glucocorticoids and stepwise addition of immunosuppressants.10 Treatment of cutaneous ulcers in MDA5-associated dermatomyositis is not standardized; however, medications such as vasodilators and antiplatelet agents may be beneficial.6The patient’s myositis antibody panel was positive for anti-MDA5 antibody. Her chest CT showed peribronchovascular nodular densities and hazy ground glass opacities in the lower lungs bilaterally, consistent with ILD. During hospitalization, she received prednisone (1 mg/kg), hydroxychloroquine, mycophenolate mofetil, losartan, aspirin, and pentoxifylline. She continued these medications as an outpatient, and prednisone was titrated to 2.5 mg daily within 8 months of hospital discharge. Five months after hospitalization, her cutaneous ulcers had completely healed."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA woman in her 60s with a history of hyperlipidemia, hypothyroidism, and chronic migraines presented with 9 months of progressively worsening left-sided ptosis, which was more notable at night and with fatigue. She denied experiencing pain, diplopia, muscle weakness, dysarthria, dysphagia, difficulty breathing, or other neurologic deficits.On examination, corrected visual acuity was 20/20 bilaterally, with normal color vision, visual fields, intraocular pressure, extraocular movements, alignment, and pupils. External examination was notable for left upper eyelid ptosis (Figure 1), with a margin-to-reflex distance 1 of 3.5 mm on the right and 0.5 mm on the left, intact levator function, and no fatiguability on sustained upgaze. There was no evidence of eyelid edema, erythema, or tenderness to palpation. There were no palpable adnexal masses or orbital rim deformities, but there was mild fullness in the left superior sulcus and mildly increased left-sided resistance to retropulsion. However, Hertel exophthalmometry did not reveal any relative proptosis. No lacrimal gland abnormalities or conjunctival masses were seen with upper eyelid eversion. There was no palpable preauricular or cervical lymphadenopathy. Slitlamp and fundus examination findings were normal. Serological testing results for antiacetylcholine receptor antibody were negative.Clinical photograph of both eyes showing left upper eyelid ptosis. Margin-to-reflex distance 1 was 3.5 mm on the right and 0.5 mm on the left.\n</clinical_case>\n\n<question>\nA woman in her 60s with a history of hyperlipidemia, hypothyroidism, and chronic migraines presented with 9 months of progressively worsening left-sided ptosis, which was more notable at night and with fatigue. She denied experiencing pain, diplopia, muscle weakness, dysarthria, dysphagia, difficulty breathing, or other neurologic deficits.On examination, corrected visual acuity was 20/20 bilaterally, with normal color vision, visual fields, intraocular pressure, extraocular movements, alignment, and pupils. External examination was notable for left upper eyelid ptosis (Figure 1), with a margin-to-reflex distance 1 of 3.5 mm on the right and 0.5 mm on the left, intact levator function, and no fatiguability on sustained upgaze. There was no evidence of eyelid edema, erythema, or tenderness to palpation. There were no palpable adnexal masses or orbital rim deformities, but there was mild fullness in the left superior sulcus and mildly increased left-sided resistance to retropulsion. However, Hertel exophthalmometry did not reveal any relative proptosis. No lacrimal gland abnormalities or conjunctival masses were seen with upper eyelid eversion. There was no palpable preauricular or cervical lymphadenopathy. Slitlamp and fundus examination findings were normal. Serological testing results for antiacetylcholine receptor antibody were negative.Clinical photograph of both eyes showing left upper eyelid ptosis. Margin-to-reflex distance 1 was 3.5 mm on the right and 0.5 mm on the left. What Would You Do Next?\n</question>\n\n<options>\nA. Obtain orbital imaging with intravenous contrast\nB. Perform left upper eyelid ptosis repair\nC. Biopsy the left levator palpebrae superioris\nD. Request single-fiber electromyography\n</options>\n"
},
{
"role": "assistant",
"content": "A. Obtain orbital imaging with intravenous contrast\nThe etiologies for unilateral ptosis in adults include involutional, traumatic, myogenic (eg, muscular dystrophy), neurogenic (eg, myasthenia gravis), and mechanical (eg, eyelid or orbital mass). While the most common cause of ptosis in adults is involutional ptosis related to levator dehiscence, other diagnoses should be considered in patients with abnormal clinical findings in addition to ptosis. In such patients, an appropriate workup should be completed prior to surgical repair, making choice A incorrect.Although ptosis that is more notable at night and with fatigue is classically seen with myasthenia gravis, patients with ptosis of other etiologies may report similar symptoms owing to reduced compensatory frontalis action by the end of the day. This patient did not have any neurologic signs or fatiguability on examination, and antiacetylcholine receptor serology findings were negative, making myasthenia gravis less likely and choice B incorrect. Similarly, the patient had normal levator function and extraocular motility, making myogenic ptosis less likely and choice C also incorrect. However, unilateral findings of superior sulcus fullness and increased resistance to retropulsion were concerning for an orbital process, making choice D the most appropriate next step. In this case, orbital imaging revealed a well-demarcated tumor involving the left lacrimal gland, with mass effect on the superior rectus-levator complex (Figure 2). Subsequent biopsy and workup demonstrated mantle cell lymphoma without systemic involvement.Magnetic resonance imaging (MRI) of the orbits with contrast showing a markedly enlarged left lacrimal gland with molding to adjacent ocular structures without bony erosion (main panel, arrowhead). There was also inferior displacement of the left globe with mass effect on the superior rectus-levator complex. A diagnostic biopsy was performed, which revealed mantle cell lymphoma. Three months after the patient completed radiation therapy, repeated MRI showed marked reduction in size of the previously enlarged left lacrimal gland, with near uniform enhancement comparable with that of the right lacrimal gland (left lower insert, arrowhead).Lacrimal gland tumors typically present with progressive proptosis and upper eyelid swelling over the course of 12 months or longer but can present with a shorter duration of symptoms, including ptosis.1-3 On magnetic resonance imaging, lacrimal gland lymphomas classically appear as well-defined masses that conform to the globe and adjacent orbital bones, are mildly hypointense to isointense on T1- and T2-weighted images, and enhance with contrast.3,4 Once a lacrimal gland biopsy confirms the diagnosis, systemic evaluation is performed for staging and treatment planning.4,5Mantle cell lymphoma is a rare form of non-Hodgkin lymphoma, representing 2% to 7% of ocular adnexal lymphomas, and is characterized by widespread disease on presentation, an aggressive clinical course, frequent recurrences, and poor survival.6 Current therapeutic options include radiation, chemotherapy, and immunotherapy, but systemic therapy may be advantageous even in stage I and II disease because of high risk of relapse.7 Although the prognosis for most ocular adnexal lymphomas is generally good, with a 5-year disease-specific survival of 81%,1,8 5-year disease-specific survival for ocular adnexal mantle cell lymphomas can be as low as 38%.6 Regardless of the initial treatment response, all patients require long-term surveillance for disease recurrence.7This case illustrates how lacrimal gland lymphomas can present as unilateral ptosis and underscores the importance of performing a thorough clinical examination (eg, palpation of periocular soft tissues and resistance to retropulsion) followed by an appropriate workup, which can lead to early detection and treatment of potentially life-threatening conditions.This patient received 24 Gy of radiation to the left lacrimal gland for localized disease. Three months after treatment, repeated magnetic resonance imaging showed significant reduction in left lacrimal gland size (Figure 2). Positron emission tomography at 6 months posttreatment showed resolution of tracer uptake in the left lacrimal gland."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA White woman in her 60s with a history of hyperlipidemia, hypothyroidism, and chronic migraines presented with 9 months of progressively worsening left-sided ptosis, which was more notable at night and with fatigue. She denied experiencing pain, diplopia, muscle weakness, dysarthria, dysphagia, difficulty breathing, or other neurologic deficits.On examination, corrected visual acuity was 20/20 bilaterally, with normal color vision, visual fields, intraocular pressure, extraocular movements, alignment, and pupils. External examination was notable for left upper eyelid ptosis (Figure 1), with a margin-to-reflex distance 1 of 3.5 mm on the right and 0.5 mm on the left, intact levator function, and no fatiguability on sustained upgaze. There was no evidence of eyelid edema, erythema, or tenderness to palpation. There were no palpable adnexal masses or orbital rim deformities, but there was mild fullness in the left superior sulcus and mildly increased left-sided resistance to retropulsion. However, Hertel exophthalmometry did not reveal any relative proptosis. No lacrimal gland abnormalities or conjunctival masses were seen with upper eyelid eversion. There was no palpable preauricular or cervical lymphadenopathy. Slitlamp and fundus examination findings were normal. Serological testing results for antiacetylcholine receptor antibody were negative.Clinical photograph of both eyes showing left upper eyelid ptosis. Margin-to-reflex distance 1 was 3.5 mm on the right and 0.5 mm on the left.\n</clinical_case>\n\n<question>\nA woman in her 60s with a history of hyperlipidemia, hypothyroidism, and chronic migraines presented with 9 months of progressively worsening left-sided ptosis, which was more notable at night and with fatigue. She denied experiencing pain, diplopia, muscle weakness, dysarthria, dysphagia, difficulty breathing, or other neurologic deficits.On examination, corrected visual acuity was 20/20 bilaterally, with normal color vision, visual fields, intraocular pressure, extraocular movements, alignment, and pupils. External examination was notable for left upper eyelid ptosis (Figure 1), with a margin-to-reflex distance 1 of 3.5 mm on the right and 0.5 mm on the left, intact levator function, and no fatiguability on sustained upgaze. There was no evidence of eyelid edema, erythema, or tenderness to palpation. There were no palpable adnexal masses or orbital rim deformities, but there was mild fullness in the left superior sulcus and mildly increased left-sided resistance to retropulsion. However, Hertel exophthalmometry did not reveal any relative proptosis. No lacrimal gland abnormalities or conjunctival masses were seen with upper eyelid eversion. There was no palpable preauricular or cervical lymphadenopathy. Slitlamp and fundus examination findings were normal. Serological testing results for antiacetylcholine receptor antibody were negative.Clinical photograph of both eyes showing left upper eyelid ptosis. Margin-to-reflex distance 1 was 3.5 mm on the right and 0.5 mm on the left. What Would You Do Next?\n</question>\n\n<options>\nA. Obtain orbital imaging with intravenous contrast\nB. Perform left upper eyelid ptosis repair\nC. Biopsy the left levator palpebrae superioris\nD. Request single-fiber electromyography\n</options>\n"
},
{
"role": "assistant",
"content": "A. Obtain orbital imaging with intravenous contrast\nThe etiologies for unilateral ptosis in adults include involutional, traumatic, myogenic (eg, muscular dystrophy), neurogenic (eg, myasthenia gravis), and mechanical (eg, eyelid or orbital mass). While the most common cause of ptosis in adults is involutional ptosis related to levator dehiscence, other diagnoses should be considered in patients with abnormal clinical findings in addition to ptosis. In such patients, an appropriate workup should be completed prior to surgical repair, making choice A incorrect.Although ptosis that is more notable at night and with fatigue is classically seen with myasthenia gravis, patients with ptosis of other etiologies may report similar symptoms owing to reduced compensatory frontalis action by the end of the day. This patient did not have any neurologic signs or fatiguability on examination, and antiacetylcholine receptor serology findings were negative, making myasthenia gravis less likely and choice B incorrect. Similarly, the patient had normal levator function and extraocular motility, making myogenic ptosis less likely and choice C also incorrect. However, unilateral findings of superior sulcus fullness and increased resistance to retropulsion were concerning for an orbital process, making choice D the most appropriate next step. In this case, orbital imaging revealed a well-demarcated tumor involving the left lacrimal gland, with mass effect on the superior rectus-levator complex (Figure 2). Subsequent biopsy and workup demonstrated mantle cell lymphoma without systemic involvement.Magnetic resonance imaging (MRI) of the orbits with contrast showing a markedly enlarged left lacrimal gland with molding to adjacent ocular structures without bony erosion (main panel, arrowhead). There was also inferior displacement of the left globe with mass effect on the superior rectus-levator complex. A diagnostic biopsy was performed, which revealed mantle cell lymphoma. Three months after the patient completed radiation therapy, repeated MRI showed marked reduction in size of the previously enlarged left lacrimal gland, with near uniform enhancement comparable with that of the right lacrimal gland (left lower insert, arrowhead).Lacrimal gland tumors typically present with progressive proptosis and upper eyelid swelling over the course of 12 months or longer but can present with a shorter duration of symptoms, including ptosis.1-3 On magnetic resonance imaging, lacrimal gland lymphomas classically appear as well-defined masses that conform to the globe and adjacent orbital bones, are mildly hypointense to isointense on T1- and T2-weighted images, and enhance with contrast.3,4 Once a lacrimal gland biopsy confirms the diagnosis, systemic evaluation is performed for staging and treatment planning.4,5Mantle cell lymphoma is a rare form of non-Hodgkin lymphoma, representing 2% to 7% of ocular adnexal lymphomas, and is characterized by widespread disease on presentation, an aggressive clinical course, frequent recurrences, and poor survival.6 Current therapeutic options include radiation, chemotherapy, and immunotherapy, but systemic therapy may be advantageous even in stage I and II disease because of high risk of relapse.7 Although the prognosis for most ocular adnexal lymphomas is generally good, with a 5-year disease-specific survival of 81%,1,8 5-year disease-specific survival for ocular adnexal mantle cell lymphomas can be as low as 38%.6 Regardless of the initial treatment response, all patients require long-term surveillance for disease recurrence.7This case illustrates how lacrimal gland lymphomas can present as unilateral ptosis and underscores the importance of performing a thorough clinical examination (eg, palpation of periocular soft tissues and resistance to retropulsion) followed by an appropriate workup, which can lead to early detection and treatment of potentially life-threatening conditions.This patient received 24 Gy of radiation to the left lacrimal gland for localized disease. Three months after treatment, repeated magnetic resonance imaging showed significant reduction in left lacrimal gland size (Figure 2). Positron emission tomography at 6 months posttreatment showed resolution of tracer uptake in the left lacrimal gland."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA White man in him 60s with a history of hyperlipidemia, hypothyroidism, and chronic migraines presented with 9 months of progressively worsening left-sided ptosis, which was more notable at night and with fatigue. He denied experiencing pain, diplopia, muscle weakness, dysarthria, dysphagia, difficulty breathing, or other neurologic deficits.On examination, corrected visual acuity was 20/20 bilaterally, with normal color vision, visual fields, intraocular pressure, extraocular movements, alignment, and pupils. External examination was notable for left upper eyelid ptosis (Figure 1), with a margin-to-reflex distance 1 of 3.5 mm on the right and 0.5 mm on the left, intact levator function, and no fatiguability on sustained upgaze. There was no evidence of eyelid edema, erythema, or tenderness to palpation. There were no palpable adnexal masses or orbital rim deformities, but there was mild fullness in the left superior sulcus and mildly increased left-sided resistance to retropulsion. However, Hertel exophthalmometry did not reveal any relative proptosis. No lacrimal gland abnormalities or conjunctival masses were seen with upper eyelid eversion. There was no palpable preauricular or cervical lymphadenopathy. Slitlamp and fundus examination findings were normal. Serological testing results for antiacetylcholine receptor antibody were negative.Clinical photograph of both eyes showing left upper eyelid ptosis. Margin-to-reflex distance 1 was 3.5 mm on the right and 0.5 mm on the left.\n</clinical_case>\n\n<question>\nA woman in her 60s with a history of hyperlipidemia, hypothyroidism, and chronic migraines presented with 9 months of progressively worsening left-sided ptosis, which was more notable at night and with fatigue. She denied experiencing pain, diplopia, muscle weakness, dysarthria, dysphagia, difficulty breathing, or other neurologic deficits.On examination, corrected visual acuity was 20/20 bilaterally, with normal color vision, visual fields, intraocular pressure, extraocular movements, alignment, and pupils. External examination was notable for left upper eyelid ptosis (Figure 1), with a margin-to-reflex distance 1 of 3.5 mm on the right and 0.5 mm on the left, intact levator function, and no fatiguability on sustained upgaze. There was no evidence of eyelid edema, erythema, or tenderness to palpation. There were no palpable adnexal masses or orbital rim deformities, but there was mild fullness in the left superior sulcus and mildly increased left-sided resistance to retropulsion. However, Hertel exophthalmometry did not reveal any relative proptosis. No lacrimal gland abnormalities or conjunctival masses were seen with upper eyelid eversion. There was no palpable preauricular or cervical lymphadenopathy. Slitlamp and fundus examination findings were normal. Serological testing results for antiacetylcholine receptor antibody were negative.Clinical photograph of both eyes showing left upper eyelid ptosis. Margin-to-reflex distance 1 was 3.5 mm on the right and 0.5 mm on the left. What Would You Do Next?\n</question>\n\n<options>\nA. Obtain orbital imaging with intravenous contrast\nB. Perform left upper eyelid ptosis repair\nC. Biopsy the left levator palpebrae superioris\nD. Request single-fiber electromyography\n</options>\n"
},
{
"role": "assistant",
"content": "A. Obtain orbital imaging with intravenous contrast\nThe etiologies for unilateral ptosis in adults include involutional, traumatic, myogenic (eg, muscular dystrophy), neurogenic (eg, myasthenia gravis), and mechanical (eg, eyelid or orbital mass). While the most common cause of ptosis in adults is involutional ptosis related to levator dehiscence, other diagnoses should be considered in patients with abnormal clinical findings in addition to ptosis. In such patients, an appropriate workup should be completed prior to surgical repair, making choice A incorrect.Although ptosis that is more notable at night and with fatigue is classically seen with myasthenia gravis, patients with ptosis of other etiologies may report similar symptoms owing to reduced compensatory frontalis action by the end of the day. This patient did not have any neurologic signs or fatiguability on examination, and antiacetylcholine receptor serology findings were negative, making myasthenia gravis less likely and choice B incorrect. Similarly, the patient had normal levator function and extraocular motility, making myogenic ptosis less likely and choice C also incorrect. However, unilateral findings of superior sulcus fullness and increased resistance to retropulsion were concerning for an orbital process, making choice D the most appropriate next step. In this case, orbital imaging revealed a well-demarcated tumor involving the left lacrimal gland, with mass effect on the superior rectus-levator complex (Figure 2). Subsequent biopsy and workup demonstrated mantle cell lymphoma without systemic involvement.Magnetic resonance imaging (MRI) of the orbits with contrast showing a markedly enlarged left lacrimal gland with molding to adjacent ocular structures without bony erosion (main panel, arrowhead). There was also inferior displacement of the left globe with mass effect on the superior rectus-levator complex. A diagnostic biopsy was performed, which revealed mantle cell lymphoma. Three months after the patient completed radiation therapy, repeated MRI showed marked reduction in size of the previously enlarged left lacrimal gland, with near uniform enhancement comparable with that of the right lacrimal gland (left lower insert, arrowhead).Lacrimal gland tumors typically present with progressive proptosis and upper eyelid swelling over the course of 12 months or longer but can present with a shorter duration of symptoms, including ptosis.1-3 On magnetic resonance imaging, lacrimal gland lymphomas classically appear as well-defined masses that conform to the globe and adjacent orbital bones, are mildly hypointense to isointense on T1- and T2-weighted images, and enhance with contrast.3,4 Once a lacrimal gland biopsy confirms the diagnosis, systemic evaluation is performed for staging and treatment planning.4,5Mantle cell lymphoma is a rare form of non-Hodgkin lymphoma, representing 2% to 7% of ocular adnexal lymphomas, and is characterized by widespread disease on presentation, an aggressive clinical course, frequent recurrences, and poor survival.6 Current therapeutic options include radiation, chemotherapy, and immunotherapy, but systemic therapy may be advantageous even in stage I and II disease because of high risk of relapse.7 Although the prognosis for most ocular adnexal lymphomas is generally good, with a 5-year disease-specific survival of 81%,1,8 5-year disease-specific survival for ocular adnexal mantle cell lymphomas can be as low as 38%.6 Regardless of the initial treatment response, all patients require long-term surveillance for disease recurrence.7This case illustrates how lacrimal gland lymphomas can present as unilateral ptosis and underscores the importance of performing a thorough clinical examination (eg, palpation of periocular soft tissues and resistance to retropulsion) followed by an appropriate workup, which can lead to early detection and treatment of potentially life-threatening conditions.This patient received 24 Gy of radiation to the left lacrimal gland for localized disease. Three months after treatment, repeated magnetic resonance imaging showed significant reduction in left lacrimal gland size (Figure 2). Positron emission tomography at 6 months posttreatment showed resolution of tracer uptake in the left lacrimal gland."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA White patient in them 60s with a history of hyperlipidemia, hypothyroidism, and chronic migraines presented with 9 months of progressively worsening left-sided ptosis, which was more notable at night and with fatigue. They denied experiencing pain, diplopia, muscle weakness, dysarthria, dysphagia, difficulty breathing, or other neurologic deficits.On examination, corrected visual acuity was 20/20 bilaterally, with normal color vision, visual fields, intraocular pressure, extraocular movements, alignment, and pupils. External examination was notable for left upper eyelid ptosis (Figure 1), with a margin-to-reflex distance 1 of 3.5 mm on the right and 0.5 mm on the left, intact levator function, and no fatiguability on sustained upgaze. There was no evidence of eyelid edema, erythema, or tenderness to palpation. There were no palpable adnexal masses or orbital rim deformities, but there was mild fullness in the left superior sulcus and mildly increased left-sided resistance to retropulsion. However, Hertel exophthalmometry did not reveal any relative proptosis. No lacrimal gland abnormalities or conjunctival masses were seen with upper eyelid eversion. There was no palpable preauricular or cervical lymphadenopathy. Slitlamp and fundus examination findings were normal. Serological testing results for antiacetylcholine receptor antibody were negative.Clinical photograph of both eyes showing left upper eyelid ptosis. Margin-to-reflex distance 1 was 3.5 mm on the right and 0.5 mm on the left.\n</clinical_case>\n\n<question>\nA woman in her 60s with a history of hyperlipidemia, hypothyroidism, and chronic migraines presented with 9 months of progressively worsening left-sided ptosis, which was more notable at night and with fatigue. She denied experiencing pain, diplopia, muscle weakness, dysarthria, dysphagia, difficulty breathing, or other neurologic deficits.On examination, corrected visual acuity was 20/20 bilaterally, with normal color vision, visual fields, intraocular pressure, extraocular movements, alignment, and pupils. External examination was notable for left upper eyelid ptosis (Figure 1), with a margin-to-reflex distance 1 of 3.5 mm on the right and 0.5 mm on the left, intact levator function, and no fatiguability on sustained upgaze. There was no evidence of eyelid edema, erythema, or tenderness to palpation. There were no palpable adnexal masses or orbital rim deformities, but there was mild fullness in the left superior sulcus and mildly increased left-sided resistance to retropulsion. However, Hertel exophthalmometry did not reveal any relative proptosis. No lacrimal gland abnormalities or conjunctival masses were seen with upper eyelid eversion. There was no palpable preauricular or cervical lymphadenopathy. Slitlamp and fundus examination findings were normal. Serological testing results for antiacetylcholine receptor antibody were negative.Clinical photograph of both eyes showing left upper eyelid ptosis. Margin-to-reflex distance 1 was 3.5 mm on the right and 0.5 mm on the left. What Would You Do Next?\n</question>\n\n<options>\nA. Obtain orbital imaging with intravenous contrast\nB. Perform left upper eyelid ptosis repair\nC. Biopsy the left levator palpebrae superioris\nD. Request single-fiber electromyography\n</options>\n"
},
{
"role": "assistant",
"content": "A. Obtain orbital imaging with intravenous contrast\nThe etiologies for unilateral ptosis in adults include involutional, traumatic, myogenic (eg, muscular dystrophy), neurogenic (eg, myasthenia gravis), and mechanical (eg, eyelid or orbital mass). While the most common cause of ptosis in adults is involutional ptosis related to levator dehiscence, other diagnoses should be considered in patients with abnormal clinical findings in addition to ptosis. In such patients, an appropriate workup should be completed prior to surgical repair, making choice A incorrect.Although ptosis that is more notable at night and with fatigue is classically seen with myasthenia gravis, patients with ptosis of other etiologies may report similar symptoms owing to reduced compensatory frontalis action by the end of the day. This patient did not have any neurologic signs or fatiguability on examination, and antiacetylcholine receptor serology findings were negative, making myasthenia gravis less likely and choice B incorrect. Similarly, the patient had normal levator function and extraocular motility, making myogenic ptosis less likely and choice C also incorrect. However, unilateral findings of superior sulcus fullness and increased resistance to retropulsion were concerning for an orbital process, making choice D the most appropriate next step. In this case, orbital imaging revealed a well-demarcated tumor involving the left lacrimal gland, with mass effect on the superior rectus-levator complex (Figure 2). Subsequent biopsy and workup demonstrated mantle cell lymphoma without systemic involvement.Magnetic resonance imaging (MRI) of the orbits with contrast showing a markedly enlarged left lacrimal gland with molding to adjacent ocular structures without bony erosion (main panel, arrowhead). There was also inferior displacement of the left globe with mass effect on the superior rectus-levator complex. A diagnostic biopsy was performed, which revealed mantle cell lymphoma. Three months after the patient completed radiation therapy, repeated MRI showed marked reduction in size of the previously enlarged left lacrimal gland, with near uniform enhancement comparable with that of the right lacrimal gland (left lower insert, arrowhead).Lacrimal gland tumors typically present with progressive proptosis and upper eyelid swelling over the course of 12 months or longer but can present with a shorter duration of symptoms, including ptosis.1-3 On magnetic resonance imaging, lacrimal gland lymphomas classically appear as well-defined masses that conform to the globe and adjacent orbital bones, are mildly hypointense to isointense on T1- and T2-weighted images, and enhance with contrast.3,4 Once a lacrimal gland biopsy confirms the diagnosis, systemic evaluation is performed for staging and treatment planning.4,5Mantle cell lymphoma is a rare form of non-Hodgkin lymphoma, representing 2% to 7% of ocular adnexal lymphomas, and is characterized by widespread disease on presentation, an aggressive clinical course, frequent recurrences, and poor survival.6 Current therapeutic options include radiation, chemotherapy, and immunotherapy, but systemic therapy may be advantageous even in stage I and II disease because of high risk of relapse.7 Although the prognosis for most ocular adnexal lymphomas is generally good, with a 5-year disease-specific survival of 81%,1,8 5-year disease-specific survival for ocular adnexal mantle cell lymphomas can be as low as 38%.6 Regardless of the initial treatment response, all patients require long-term surveillance for disease recurrence.7This case illustrates how lacrimal gland lymphomas can present as unilateral ptosis and underscores the importance of performing a thorough clinical examination (eg, palpation of periocular soft tissues and resistance to retropulsion) followed by an appropriate workup, which can lead to early detection and treatment of potentially life-threatening conditions.This patient received 24 Gy of radiation to the left lacrimal gland for localized disease. Three months after treatment, repeated magnetic resonance imaging showed significant reduction in left lacrimal gland size (Figure 2). Positron emission tomography at 6 months posttreatment showed resolution of tracer uptake in the left lacrimal gland."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Black woman in her 60s with a history of hyperlipidemia, hypothyroidism, and chronic migraines presented with 9 months of progressively worsening left-sided ptosis, which was more notable at night and with fatigue. She denied experiencing pain, diplopia, muscle weakness, dysarthria, dysphagia, difficulty breathing, or other neurologic deficits.On examination, corrected visual acuity was 20/20 bilaterally, with normal color vision, visual fields, intraocular pressure, extraocular movements, alignment, and pupils. External examination was notable for left upper eyelid ptosis (Figure 1), with a margin-to-reflex distance 1 of 3.5 mm on the right and 0.5 mm on the left, intact levator function, and no fatiguability on sustained upgaze. There was no evidence of eyelid edema, erythema, or tenderness to palpation. There were no palpable adnexal masses or orbital rim deformities, but there was mild fullness in the left superior sulcus and mildly increased left-sided resistance to retropulsion. However, Hertel exophthalmometry did not reveal any relative proptosis. No lacrimal gland abnormalities or conjunctival masses were seen with upper eyelid eversion. There was no palpable preauricular or cervical lymphadenopathy. Slitlamp and fundus examination findings were normal. Serological testing results for antiacetylcholine receptor antibody were negative.Clinical photograph of both eyes showing left upper eyelid ptosis. Margin-to-reflex distance 1 was 3.5 mm on the right and 0.5 mm on the left.\n</clinical_case>\n\n<question>\nA woman in her 60s with a history of hyperlipidemia, hypothyroidism, and chronic migraines presented with 9 months of progressively worsening left-sided ptosis, which was more notable at night and with fatigue. She denied experiencing pain, diplopia, muscle weakness, dysarthria, dysphagia, difficulty breathing, or other neurologic deficits.On examination, corrected visual acuity was 20/20 bilaterally, with normal color vision, visual fields, intraocular pressure, extraocular movements, alignment, and pupils. External examination was notable for left upper eyelid ptosis (Figure 1), with a margin-to-reflex distance 1 of 3.5 mm on the right and 0.5 mm on the left, intact levator function, and no fatiguability on sustained upgaze. There was no evidence of eyelid edema, erythema, or tenderness to palpation. There were no palpable adnexal masses or orbital rim deformities, but there was mild fullness in the left superior sulcus and mildly increased left-sided resistance to retropulsion. However, Hertel exophthalmometry did not reveal any relative proptosis. No lacrimal gland abnormalities or conjunctival masses were seen with upper eyelid eversion. There was no palpable preauricular or cervical lymphadenopathy. Slitlamp and fundus examination findings were normal. Serological testing results for antiacetylcholine receptor antibody were negative.Clinical photograph of both eyes showing left upper eyelid ptosis. Margin-to-reflex distance 1 was 3.5 mm on the right and 0.5 mm on the left. What Would You Do Next?\n</question>\n\n<options>\nA. Obtain orbital imaging with intravenous contrast\nB. Perform left upper eyelid ptosis repair\nC. Biopsy the left levator palpebrae superioris\nD. Request single-fiber electromyography\n</options>\n"
},
{
"role": "assistant",
"content": "A. Obtain orbital imaging with intravenous contrast\nThe etiologies for unilateral ptosis in adults include involutional, traumatic, myogenic (eg, muscular dystrophy), neurogenic (eg, myasthenia gravis), and mechanical (eg, eyelid or orbital mass). While the most common cause of ptosis in adults is involutional ptosis related to levator dehiscence, other diagnoses should be considered in patients with abnormal clinical findings in addition to ptosis. In such patients, an appropriate workup should be completed prior to surgical repair, making choice A incorrect.Although ptosis that is more notable at night and with fatigue is classically seen with myasthenia gravis, patients with ptosis of other etiologies may report similar symptoms owing to reduced compensatory frontalis action by the end of the day. This patient did not have any neurologic signs or fatiguability on examination, and antiacetylcholine receptor serology findings were negative, making myasthenia gravis less likely and choice B incorrect. Similarly, the patient had normal levator function and extraocular motility, making myogenic ptosis less likely and choice C also incorrect. However, unilateral findings of superior sulcus fullness and increased resistance to retropulsion were concerning for an orbital process, making choice D the most appropriate next step. In this case, orbital imaging revealed a well-demarcated tumor involving the left lacrimal gland, with mass effect on the superior rectus-levator complex (Figure 2). Subsequent biopsy and workup demonstrated mantle cell lymphoma without systemic involvement.Magnetic resonance imaging (MRI) of the orbits with contrast showing a markedly enlarged left lacrimal gland with molding to adjacent ocular structures without bony erosion (main panel, arrowhead). There was also inferior displacement of the left globe with mass effect on the superior rectus-levator complex. A diagnostic biopsy was performed, which revealed mantle cell lymphoma. Three months after the patient completed radiation therapy, repeated MRI showed marked reduction in size of the previously enlarged left lacrimal gland, with near uniform enhancement comparable with that of the right lacrimal gland (left lower insert, arrowhead).Lacrimal gland tumors typically present with progressive proptosis and upper eyelid swelling over the course of 12 months or longer but can present with a shorter duration of symptoms, including ptosis.1-3 On magnetic resonance imaging, lacrimal gland lymphomas classically appear as well-defined masses that conform to the globe and adjacent orbital bones, are mildly hypointense to isointense on T1- and T2-weighted images, and enhance with contrast.3,4 Once a lacrimal gland biopsy confirms the diagnosis, systemic evaluation is performed for staging and treatment planning.4,5Mantle cell lymphoma is a rare form of non-Hodgkin lymphoma, representing 2% to 7% of ocular adnexal lymphomas, and is characterized by widespread disease on presentation, an aggressive clinical course, frequent recurrences, and poor survival.6 Current therapeutic options include radiation, chemotherapy, and immunotherapy, but systemic therapy may be advantageous even in stage I and II disease because of high risk of relapse.7 Although the prognosis for most ocular adnexal lymphomas is generally good, with a 5-year disease-specific survival of 81%,1,8 5-year disease-specific survival for ocular adnexal mantle cell lymphomas can be as low as 38%.6 Regardless of the initial treatment response, all patients require long-term surveillance for disease recurrence.7This case illustrates how lacrimal gland lymphomas can present as unilateral ptosis and underscores the importance of performing a thorough clinical examination (eg, palpation of periocular soft tissues and resistance to retropulsion) followed by an appropriate workup, which can lead to early detection and treatment of potentially life-threatening conditions.This patient received 24 Gy of radiation to the left lacrimal gland for localized disease. Three months after treatment, repeated magnetic resonance imaging showed significant reduction in left lacrimal gland size (Figure 2). Positron emission tomography at 6 months posttreatment showed resolution of tracer uptake in the left lacrimal gland."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Black man in him 60s with a history of hyperlipidemia, hypothyroidism, and chronic migraines presented with 9 months of progressively worsening left-sided ptosis, which was more notable at night and with fatigue. He denied experiencing pain, diplopia, muscle weakness, dysarthria, dysphagia, difficulty breathing, or other neurologic deficits.On examination, corrected visual acuity was 20/20 bilaterally, with normal color vision, visual fields, intraocular pressure, extraocular movements, alignment, and pupils. External examination was notable for left upper eyelid ptosis (Figure 1), with a margin-to-reflex distance 1 of 3.5 mm on the right and 0.5 mm on the left, intact levator function, and no fatiguability on sustained upgaze. There was no evidence of eyelid edema, erythema, or tenderness to palpation. There were no palpable adnexal masses or orbital rim deformities, but there was mild fullness in the left superior sulcus and mildly increased left-sided resistance to retropulsion. However, Hertel exophthalmometry did not reveal any relative proptosis. No lacrimal gland abnormalities or conjunctival masses were seen with upper eyelid eversion. There was no palpable preauricular or cervical lymphadenopathy. Slitlamp and fundus examination findings were normal. Serological testing results for antiacetylcholine receptor antibody were negative.Clinical photograph of both eyes showing left upper eyelid ptosis. Margin-to-reflex distance 1 was 3.5 mm on the right and 0.5 mm on the left.\n</clinical_case>\n\n<question>\nA woman in her 60s with a history of hyperlipidemia, hypothyroidism, and chronic migraines presented with 9 months of progressively worsening left-sided ptosis, which was more notable at night and with fatigue. She denied experiencing pain, diplopia, muscle weakness, dysarthria, dysphagia, difficulty breathing, or other neurologic deficits.On examination, corrected visual acuity was 20/20 bilaterally, with normal color vision, visual fields, intraocular pressure, extraocular movements, alignment, and pupils. External examination was notable for left upper eyelid ptosis (Figure 1), with a margin-to-reflex distance 1 of 3.5 mm on the right and 0.5 mm on the left, intact levator function, and no fatiguability on sustained upgaze. There was no evidence of eyelid edema, erythema, or tenderness to palpation. There were no palpable adnexal masses or orbital rim deformities, but there was mild fullness in the left superior sulcus and mildly increased left-sided resistance to retropulsion. However, Hertel exophthalmometry did not reveal any relative proptosis. No lacrimal gland abnormalities or conjunctival masses were seen with upper eyelid eversion. There was no palpable preauricular or cervical lymphadenopathy. Slitlamp and fundus examination findings were normal. Serological testing results for antiacetylcholine receptor antibody were negative.Clinical photograph of both eyes showing left upper eyelid ptosis. Margin-to-reflex distance 1 was 3.5 mm on the right and 0.5 mm on the left. What Would You Do Next?\n</question>\n\n<options>\nA. Obtain orbital imaging with intravenous contrast\nB. Perform left upper eyelid ptosis repair\nC. Biopsy the left levator palpebrae superioris\nD. Request single-fiber electromyography\n</options>\n"
},
{
"role": "assistant",
"content": "A. Obtain orbital imaging with intravenous contrast\nThe etiologies for unilateral ptosis in adults include involutional, traumatic, myogenic (eg, muscular dystrophy), neurogenic (eg, myasthenia gravis), and mechanical (eg, eyelid or orbital mass). While the most common cause of ptosis in adults is involutional ptosis related to levator dehiscence, other diagnoses should be considered in patients with abnormal clinical findings in addition to ptosis. In such patients, an appropriate workup should be completed prior to surgical repair, making choice A incorrect.Although ptosis that is more notable at night and with fatigue is classically seen with myasthenia gravis, patients with ptosis of other etiologies may report similar symptoms owing to reduced compensatory frontalis action by the end of the day. This patient did not have any neurologic signs or fatiguability on examination, and antiacetylcholine receptor serology findings were negative, making myasthenia gravis less likely and choice B incorrect. Similarly, the patient had normal levator function and extraocular motility, making myogenic ptosis less likely and choice C also incorrect. However, unilateral findings of superior sulcus fullness and increased resistance to retropulsion were concerning for an orbital process, making choice D the most appropriate next step. In this case, orbital imaging revealed a well-demarcated tumor involving the left lacrimal gland, with mass effect on the superior rectus-levator complex (Figure 2). Subsequent biopsy and workup demonstrated mantle cell lymphoma without systemic involvement.Magnetic resonance imaging (MRI) of the orbits with contrast showing a markedly enlarged left lacrimal gland with molding to adjacent ocular structures without bony erosion (main panel, arrowhead). There was also inferior displacement of the left globe with mass effect on the superior rectus-levator complex. A diagnostic biopsy was performed, which revealed mantle cell lymphoma. Three months after the patient completed radiation therapy, repeated MRI showed marked reduction in size of the previously enlarged left lacrimal gland, with near uniform enhancement comparable with that of the right lacrimal gland (left lower insert, arrowhead).Lacrimal gland tumors typically present with progressive proptosis and upper eyelid swelling over the course of 12 months or longer but can present with a shorter duration of symptoms, including ptosis.1-3 On magnetic resonance imaging, lacrimal gland lymphomas classically appear as well-defined masses that conform to the globe and adjacent orbital bones, are mildly hypointense to isointense on T1- and T2-weighted images, and enhance with contrast.3,4 Once a lacrimal gland biopsy confirms the diagnosis, systemic evaluation is performed for staging and treatment planning.4,5Mantle cell lymphoma is a rare form of non-Hodgkin lymphoma, representing 2% to 7% of ocular adnexal lymphomas, and is characterized by widespread disease on presentation, an aggressive clinical course, frequent recurrences, and poor survival.6 Current therapeutic options include radiation, chemotherapy, and immunotherapy, but systemic therapy may be advantageous even in stage I and II disease because of high risk of relapse.7 Although the prognosis for most ocular adnexal lymphomas is generally good, with a 5-year disease-specific survival of 81%,1,8 5-year disease-specific survival for ocular adnexal mantle cell lymphomas can be as low as 38%.6 Regardless of the initial treatment response, all patients require long-term surveillance for disease recurrence.7This case illustrates how lacrimal gland lymphomas can present as unilateral ptosis and underscores the importance of performing a thorough clinical examination (eg, palpation of periocular soft tissues and resistance to retropulsion) followed by an appropriate workup, which can lead to early detection and treatment of potentially life-threatening conditions.This patient received 24 Gy of radiation to the left lacrimal gland for localized disease. Three months after treatment, repeated magnetic resonance imaging showed significant reduction in left lacrimal gland size (Figure 2). Positron emission tomography at 6 months posttreatment showed resolution of tracer uptake in the left lacrimal gland."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Black patient in them 60s with a history of hyperlipidemia, hypothyroidism, and chronic migraines presented with 9 months of progressively worsening left-sided ptosis, which was more notable at night and with fatigue. They denied experiencing pain, diplopia, muscle weakness, dysarthria, dysphagia, difficulty breathing, or other neurologic deficits.On examination, corrected visual acuity was 20/20 bilaterally, with normal color vision, visual fields, intraocular pressure, extraocular movements, alignment, and pupils. External examination was notable for left upper eyelid ptosis (Figure 1), with a margin-to-reflex distance 1 of 3.5 mm on the right and 0.5 mm on the left, intact levator function, and no fatiguability on sustained upgaze. There was no evidence of eyelid edema, erythema, or tenderness to palpation. There were no palpable adnexal masses or orbital rim deformities, but there was mild fullness in the left superior sulcus and mildly increased left-sided resistance to retropulsion. However, Hertel exophthalmometry did not reveal any relative proptosis. No lacrimal gland abnormalities or conjunctival masses were seen with upper eyelid eversion. There was no palpable preauricular or cervical lymphadenopathy. Slitlamp and fundus examination findings were normal. Serological testing results for antiacetylcholine receptor antibody were negative.Clinical photograph of both eyes showing left upper eyelid ptosis. Margin-to-reflex distance 1 was 3.5 mm on the right and 0.5 mm on the left.\n</clinical_case>\n\n<question>\nA woman in her 60s with a history of hyperlipidemia, hypothyroidism, and chronic migraines presented with 9 months of progressively worsening left-sided ptosis, which was more notable at night and with fatigue. She denied experiencing pain, diplopia, muscle weakness, dysarthria, dysphagia, difficulty breathing, or other neurologic deficits.On examination, corrected visual acuity was 20/20 bilaterally, with normal color vision, visual fields, intraocular pressure, extraocular movements, alignment, and pupils. External examination was notable for left upper eyelid ptosis (Figure 1), with a margin-to-reflex distance 1 of 3.5 mm on the right and 0.5 mm on the left, intact levator function, and no fatiguability on sustained upgaze. There was no evidence of eyelid edema, erythema, or tenderness to palpation. There were no palpable adnexal masses or orbital rim deformities, but there was mild fullness in the left superior sulcus and mildly increased left-sided resistance to retropulsion. However, Hertel exophthalmometry did not reveal any relative proptosis. No lacrimal gland abnormalities or conjunctival masses were seen with upper eyelid eversion. There was no palpable preauricular or cervical lymphadenopathy. Slitlamp and fundus examination findings were normal. Serological testing results for antiacetylcholine receptor antibody were negative.Clinical photograph of both eyes showing left upper eyelid ptosis. Margin-to-reflex distance 1 was 3.5 mm on the right and 0.5 mm on the left. What Would You Do Next?\n</question>\n\n<options>\nA. Obtain orbital imaging with intravenous contrast\nB. Perform left upper eyelid ptosis repair\nC. Biopsy the left levator palpebrae superioris\nD. Request single-fiber electromyography\n</options>\n"
},
{
"role": "assistant",
"content": "A. Obtain orbital imaging with intravenous contrast\nThe etiologies for unilateral ptosis in adults include involutional, traumatic, myogenic (eg, muscular dystrophy), neurogenic (eg, myasthenia gravis), and mechanical (eg, eyelid or orbital mass). While the most common cause of ptosis in adults is involutional ptosis related to levator dehiscence, other diagnoses should be considered in patients with abnormal clinical findings in addition to ptosis. In such patients, an appropriate workup should be completed prior to surgical repair, making choice A incorrect.Although ptosis that is more notable at night and with fatigue is classically seen with myasthenia gravis, patients with ptosis of other etiologies may report similar symptoms owing to reduced compensatory frontalis action by the end of the day. This patient did not have any neurologic signs or fatiguability on examination, and antiacetylcholine receptor serology findings were negative, making myasthenia gravis less likely and choice B incorrect. Similarly, the patient had normal levator function and extraocular motility, making myogenic ptosis less likely and choice C also incorrect. However, unilateral findings of superior sulcus fullness and increased resistance to retropulsion were concerning for an orbital process, making choice D the most appropriate next step. In this case, orbital imaging revealed a well-demarcated tumor involving the left lacrimal gland, with mass effect on the superior rectus-levator complex (Figure 2). Subsequent biopsy and workup demonstrated mantle cell lymphoma without systemic involvement.Magnetic resonance imaging (MRI) of the orbits with contrast showing a markedly enlarged left lacrimal gland with molding to adjacent ocular structures without bony erosion (main panel, arrowhead). There was also inferior displacement of the left globe with mass effect on the superior rectus-levator complex. A diagnostic biopsy was performed, which revealed mantle cell lymphoma. Three months after the patient completed radiation therapy, repeated MRI showed marked reduction in size of the previously enlarged left lacrimal gland, with near uniform enhancement comparable with that of the right lacrimal gland (left lower insert, arrowhead).Lacrimal gland tumors typically present with progressive proptosis and upper eyelid swelling over the course of 12 months or longer but can present with a shorter duration of symptoms, including ptosis.1-3 On magnetic resonance imaging, lacrimal gland lymphomas classically appear as well-defined masses that conform to the globe and adjacent orbital bones, are mildly hypointense to isointense on T1- and T2-weighted images, and enhance with contrast.3,4 Once a lacrimal gland biopsy confirms the diagnosis, systemic evaluation is performed for staging and treatment planning.4,5Mantle cell lymphoma is a rare form of non-Hodgkin lymphoma, representing 2% to 7% of ocular adnexal lymphomas, and is characterized by widespread disease on presentation, an aggressive clinical course, frequent recurrences, and poor survival.6 Current therapeutic options include radiation, chemotherapy, and immunotherapy, but systemic therapy may be advantageous even in stage I and II disease because of high risk of relapse.7 Although the prognosis for most ocular adnexal lymphomas is generally good, with a 5-year disease-specific survival of 81%,1,8 5-year disease-specific survival for ocular adnexal mantle cell lymphomas can be as low as 38%.6 Regardless of the initial treatment response, all patients require long-term surveillance for disease recurrence.7This case illustrates how lacrimal gland lymphomas can present as unilateral ptosis and underscores the importance of performing a thorough clinical examination (eg, palpation of periocular soft tissues and resistance to retropulsion) followed by an appropriate workup, which can lead to early detection and treatment of potentially life-threatening conditions.This patient received 24 Gy of radiation to the left lacrimal gland for localized disease. Three months after treatment, repeated magnetic resonance imaging showed significant reduction in left lacrimal gland size (Figure 2). Positron emission tomography at 6 months posttreatment showed resolution of tracer uptake in the left lacrimal gland."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Asian woman in her 60s with a history of hyperlipidemia, hypothyroidism, and chronic migraines presented with 9 months of progressively worsening left-sided ptosis, which was more notable at night and with fatigue. She denied experiencing pain, diplopia, muscle weakness, dysarthria, dysphagia, difficulty breathing, or other neurologic deficits.On examination, corrected visual acuity was 20/20 bilaterally, with normal color vision, visual fields, intraocular pressure, extraocular movements, alignment, and pupils. External examination was notable for left upper eyelid ptosis (Figure 1), with a margin-to-reflex distance 1 of 3.5 mm on the right and 0.5 mm on the left, intact levator function, and no fatiguability on sustained upgaze. There was no evidence of eyelid edema, erythema, or tenderness to palpation. There were no palpable adnexal masses or orbital rim deformities, but there was mild fullness in the left superior sulcus and mildly increased left-sided resistance to retropulsion. However, Hertel exophthalmometry did not reveal any relative proptosis. No lacrimal gland abnormalities or conjunctival masses were seen with upper eyelid eversion. There was no palpable preauricular or cervical lymphadenopathy. Slitlamp and fundus examination findings were normal. Serological testing results for antiacetylcholine receptor antibody were negative.Clinical photograph of both eyes showing left upper eyelid ptosis. Margin-to-reflex distance 1 was 3.5 mm on the right and 0.5 mm on the left.\n</clinical_case>\n\n<question>\nA woman in her 60s with a history of hyperlipidemia, hypothyroidism, and chronic migraines presented with 9 months of progressively worsening left-sided ptosis, which was more notable at night and with fatigue. She denied experiencing pain, diplopia, muscle weakness, dysarthria, dysphagia, difficulty breathing, or other neurologic deficits.On examination, corrected visual acuity was 20/20 bilaterally, with normal color vision, visual fields, intraocular pressure, extraocular movements, alignment, and pupils. External examination was notable for left upper eyelid ptosis (Figure 1), with a margin-to-reflex distance 1 of 3.5 mm on the right and 0.5 mm on the left, intact levator function, and no fatiguability on sustained upgaze. There was no evidence of eyelid edema, erythema, or tenderness to palpation. There were no palpable adnexal masses or orbital rim deformities, but there was mild fullness in the left superior sulcus and mildly increased left-sided resistance to retropulsion. However, Hertel exophthalmometry did not reveal any relative proptosis. No lacrimal gland abnormalities or conjunctival masses were seen with upper eyelid eversion. There was no palpable preauricular or cervical lymphadenopathy. Slitlamp and fundus examination findings were normal. Serological testing results for antiacetylcholine receptor antibody were negative.Clinical photograph of both eyes showing left upper eyelid ptosis. Margin-to-reflex distance 1 was 3.5 mm on the right and 0.5 mm on the left. What Would You Do Next?\n</question>\n\n<options>\nA. Obtain orbital imaging with intravenous contrast\nB. Perform left upper eyelid ptosis repair\nC. Biopsy the left levator palpebrae superioris\nD. Request single-fiber electromyography\n</options>\n"
},
{
"role": "assistant",
"content": "A. Obtain orbital imaging with intravenous contrast\nThe etiologies for unilateral ptosis in adults include involutional, traumatic, myogenic (eg, muscular dystrophy), neurogenic (eg, myasthenia gravis), and mechanical (eg, eyelid or orbital mass). While the most common cause of ptosis in adults is involutional ptosis related to levator dehiscence, other diagnoses should be considered in patients with abnormal clinical findings in addition to ptosis. In such patients, an appropriate workup should be completed prior to surgical repair, making choice A incorrect.Although ptosis that is more notable at night and with fatigue is classically seen with myasthenia gravis, patients with ptosis of other etiologies may report similar symptoms owing to reduced compensatory frontalis action by the end of the day. This patient did not have any neurologic signs or fatiguability on examination, and antiacetylcholine receptor serology findings were negative, making myasthenia gravis less likely and choice B incorrect. Similarly, the patient had normal levator function and extraocular motility, making myogenic ptosis less likely and choice C also incorrect. However, unilateral findings of superior sulcus fullness and increased resistance to retropulsion were concerning for an orbital process, making choice D the most appropriate next step. In this case, orbital imaging revealed a well-demarcated tumor involving the left lacrimal gland, with mass effect on the superior rectus-levator complex (Figure 2). Subsequent biopsy and workup demonstrated mantle cell lymphoma without systemic involvement.Magnetic resonance imaging (MRI) of the orbits with contrast showing a markedly enlarged left lacrimal gland with molding to adjacent ocular structures without bony erosion (main panel, arrowhead). There was also inferior displacement of the left globe with mass effect on the superior rectus-levator complex. A diagnostic biopsy was performed, which revealed mantle cell lymphoma. Three months after the patient completed radiation therapy, repeated MRI showed marked reduction in size of the previously enlarged left lacrimal gland, with near uniform enhancement comparable with that of the right lacrimal gland (left lower insert, arrowhead).Lacrimal gland tumors typically present with progressive proptosis and upper eyelid swelling over the course of 12 months or longer but can present with a shorter duration of symptoms, including ptosis.1-3 On magnetic resonance imaging, lacrimal gland lymphomas classically appear as well-defined masses that conform to the globe and adjacent orbital bones, are mildly hypointense to isointense on T1- and T2-weighted images, and enhance with contrast.3,4 Once a lacrimal gland biopsy confirms the diagnosis, systemic evaluation is performed for staging and treatment planning.4,5Mantle cell lymphoma is a rare form of non-Hodgkin lymphoma, representing 2% to 7% of ocular adnexal lymphomas, and is characterized by widespread disease on presentation, an aggressive clinical course, frequent recurrences, and poor survival.6 Current therapeutic options include radiation, chemotherapy, and immunotherapy, but systemic therapy may be advantageous even in stage I and II disease because of high risk of relapse.7 Although the prognosis for most ocular adnexal lymphomas is generally good, with a 5-year disease-specific survival of 81%,1,8 5-year disease-specific survival for ocular adnexal mantle cell lymphomas can be as low as 38%.6 Regardless of the initial treatment response, all patients require long-term surveillance for disease recurrence.7This case illustrates how lacrimal gland lymphomas can present as unilateral ptosis and underscores the importance of performing a thorough clinical examination (eg, palpation of periocular soft tissues and resistance to retropulsion) followed by an appropriate workup, which can lead to early detection and treatment of potentially life-threatening conditions.This patient received 24 Gy of radiation to the left lacrimal gland for localized disease. Three months after treatment, repeated magnetic resonance imaging showed significant reduction in left lacrimal gland size (Figure 2). Positron emission tomography at 6 months posttreatment showed resolution of tracer uptake in the left lacrimal gland."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Asian man in him 60s with a history of hyperlipidemia, hypothyroidism, and chronic migraines presented with 9 months of progressively worsening left-sided ptosis, which was more notable at night and with fatigue. He denied experiencing pain, diplopia, muscle weakness, dysarthria, dysphagia, difficulty breathing, or other neurologic deficits.On examination, corrected visual acuity was 20/20 bilaterally, with normal color vision, visual fields, intraocular pressure, extraocular movements, alignment, and pupils. External examination was notable for left upper eyelid ptosis (Figure 1), with a margin-to-reflex distance 1 of 3.5 mm on the right and 0.5 mm on the left, intact levator function, and no fatiguability on sustained upgaze. There was no evidence of eyelid edema, erythema, or tenderness to palpation. There were no palpable adnexal masses or orbital rim deformities, but there was mild fullness in the left superior sulcus and mildly increased left-sided resistance to retropulsion. However, Hertel exophthalmometry did not reveal any relative proptosis. No lacrimal gland abnormalities or conjunctival masses were seen with upper eyelid eversion. There was no palpable preauricular or cervical lymphadenopathy. Slitlamp and fundus examination findings were normal. Serological testing results for antiacetylcholine receptor antibody were negative.Clinical photograph of both eyes showing left upper eyelid ptosis. Margin-to-reflex distance 1 was 3.5 mm on the right and 0.5 mm on the left.\n</clinical_case>\n\n<question>\nA woman in her 60s with a history of hyperlipidemia, hypothyroidism, and chronic migraines presented with 9 months of progressively worsening left-sided ptosis, which was more notable at night and with fatigue. She denied experiencing pain, diplopia, muscle weakness, dysarthria, dysphagia, difficulty breathing, or other neurologic deficits.On examination, corrected visual acuity was 20/20 bilaterally, with normal color vision, visual fields, intraocular pressure, extraocular movements, alignment, and pupils. External examination was notable for left upper eyelid ptosis (Figure 1), with a margin-to-reflex distance 1 of 3.5 mm on the right and 0.5 mm on the left, intact levator function, and no fatiguability on sustained upgaze. There was no evidence of eyelid edema, erythema, or tenderness to palpation. There were no palpable adnexal masses or orbital rim deformities, but there was mild fullness in the left superior sulcus and mildly increased left-sided resistance to retropulsion. However, Hertel exophthalmometry did not reveal any relative proptosis. No lacrimal gland abnormalities or conjunctival masses were seen with upper eyelid eversion. There was no palpable preauricular or cervical lymphadenopathy. Slitlamp and fundus examination findings were normal. Serological testing results for antiacetylcholine receptor antibody were negative.Clinical photograph of both eyes showing left upper eyelid ptosis. Margin-to-reflex distance 1 was 3.5 mm on the right and 0.5 mm on the left. What Would You Do Next?\n</question>\n\n<options>\nA. Obtain orbital imaging with intravenous contrast\nB. Perform left upper eyelid ptosis repair\nC. Biopsy the left levator palpebrae superioris\nD. Request single-fiber electromyography\n</options>\n"
},
{
"role": "assistant",
"content": "A. Obtain orbital imaging with intravenous contrast\nThe etiologies for unilateral ptosis in adults include involutional, traumatic, myogenic (eg, muscular dystrophy), neurogenic (eg, myasthenia gravis), and mechanical (eg, eyelid or orbital mass). While the most common cause of ptosis in adults is involutional ptosis related to levator dehiscence, other diagnoses should be considered in patients with abnormal clinical findings in addition to ptosis. In such patients, an appropriate workup should be completed prior to surgical repair, making choice A incorrect.Although ptosis that is more notable at night and with fatigue is classically seen with myasthenia gravis, patients with ptosis of other etiologies may report similar symptoms owing to reduced compensatory frontalis action by the end of the day. This patient did not have any neurologic signs or fatiguability on examination, and antiacetylcholine receptor serology findings were negative, making myasthenia gravis less likely and choice B incorrect. Similarly, the patient had normal levator function and extraocular motility, making myogenic ptosis less likely and choice C also incorrect. However, unilateral findings of superior sulcus fullness and increased resistance to retropulsion were concerning for an orbital process, making choice D the most appropriate next step. In this case, orbital imaging revealed a well-demarcated tumor involving the left lacrimal gland, with mass effect on the superior rectus-levator complex (Figure 2). Subsequent biopsy and workup demonstrated mantle cell lymphoma without systemic involvement.Magnetic resonance imaging (MRI) of the orbits with contrast showing a markedly enlarged left lacrimal gland with molding to adjacent ocular structures without bony erosion (main panel, arrowhead). There was also inferior displacement of the left globe with mass effect on the superior rectus-levator complex. A diagnostic biopsy was performed, which revealed mantle cell lymphoma. Three months after the patient completed radiation therapy, repeated MRI showed marked reduction in size of the previously enlarged left lacrimal gland, with near uniform enhancement comparable with that of the right lacrimal gland (left lower insert, arrowhead).Lacrimal gland tumors typically present with progressive proptosis and upper eyelid swelling over the course of 12 months or longer but can present with a shorter duration of symptoms, including ptosis.1-3 On magnetic resonance imaging, lacrimal gland lymphomas classically appear as well-defined masses that conform to the globe and adjacent orbital bones, are mildly hypointense to isointense on T1- and T2-weighted images, and enhance with contrast.3,4 Once a lacrimal gland biopsy confirms the diagnosis, systemic evaluation is performed for staging and treatment planning.4,5Mantle cell lymphoma is a rare form of non-Hodgkin lymphoma, representing 2% to 7% of ocular adnexal lymphomas, and is characterized by widespread disease on presentation, an aggressive clinical course, frequent recurrences, and poor survival.6 Current therapeutic options include radiation, chemotherapy, and immunotherapy, but systemic therapy may be advantageous even in stage I and II disease because of high risk of relapse.7 Although the prognosis for most ocular adnexal lymphomas is generally good, with a 5-year disease-specific survival of 81%,1,8 5-year disease-specific survival for ocular adnexal mantle cell lymphomas can be as low as 38%.6 Regardless of the initial treatment response, all patients require long-term surveillance for disease recurrence.7This case illustrates how lacrimal gland lymphomas can present as unilateral ptosis and underscores the importance of performing a thorough clinical examination (eg, palpation of periocular soft tissues and resistance to retropulsion) followed by an appropriate workup, which can lead to early detection and treatment of potentially life-threatening conditions.This patient received 24 Gy of radiation to the left lacrimal gland for localized disease. Three months after treatment, repeated magnetic resonance imaging showed significant reduction in left lacrimal gland size (Figure 2). Positron emission tomography at 6 months posttreatment showed resolution of tracer uptake in the left lacrimal gland."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Asian patient in them 60s with a history of hyperlipidemia, hypothyroidism, and chronic migraines presented with 9 months of progressively worsening left-sided ptosis, which was more notable at night and with fatigue. They denied experiencing pain, diplopia, muscle weakness, dysarthria, dysphagia, difficulty breathing, or other neurologic deficits.On examination, corrected visual acuity was 20/20 bilaterally, with normal color vision, visual fields, intraocular pressure, extraocular movements, alignment, and pupils. External examination was notable for left upper eyelid ptosis (Figure 1), with a margin-to-reflex distance 1 of 3.5 mm on the right and 0.5 mm on the left, intact levator function, and no fatiguability on sustained upgaze. There was no evidence of eyelid edema, erythema, or tenderness to palpation. There were no palpable adnexal masses or orbital rim deformities, but there was mild fullness in the left superior sulcus and mildly increased left-sided resistance to retropulsion. However, Hertel exophthalmometry did not reveal any relative proptosis. No lacrimal gland abnormalities or conjunctival masses were seen with upper eyelid eversion. There was no palpable preauricular or cervical lymphadenopathy. Slitlamp and fundus examination findings were normal. Serological testing results for antiacetylcholine receptor antibody were negative.Clinical photograph of both eyes showing left upper eyelid ptosis. Margin-to-reflex distance 1 was 3.5 mm on the right and 0.5 mm on the left.\n</clinical_case>\n\n<question>\nA woman in her 60s with a history of hyperlipidemia, hypothyroidism, and chronic migraines presented with 9 months of progressively worsening left-sided ptosis, which was more notable at night and with fatigue. She denied experiencing pain, diplopia, muscle weakness, dysarthria, dysphagia, difficulty breathing, or other neurologic deficits.On examination, corrected visual acuity was 20/20 bilaterally, with normal color vision, visual fields, intraocular pressure, extraocular movements, alignment, and pupils. External examination was notable for left upper eyelid ptosis (Figure 1), with a margin-to-reflex distance 1 of 3.5 mm on the right and 0.5 mm on the left, intact levator function, and no fatiguability on sustained upgaze. There was no evidence of eyelid edema, erythema, or tenderness to palpation. There were no palpable adnexal masses or orbital rim deformities, but there was mild fullness in the left superior sulcus and mildly increased left-sided resistance to retropulsion. However, Hertel exophthalmometry did not reveal any relative proptosis. No lacrimal gland abnormalities or conjunctival masses were seen with upper eyelid eversion. There was no palpable preauricular or cervical lymphadenopathy. Slitlamp and fundus examination findings were normal. Serological testing results for antiacetylcholine receptor antibody were negative.Clinical photograph of both eyes showing left upper eyelid ptosis. Margin-to-reflex distance 1 was 3.5 mm on the right and 0.5 mm on the left. What Would You Do Next?\n</question>\n\n<options>\nA. Obtain orbital imaging with intravenous contrast\nB. Perform left upper eyelid ptosis repair\nC. Biopsy the left levator palpebrae superioris\nD. Request single-fiber electromyography\n</options>\n"
},
{
"role": "assistant",
"content": "A. Obtain orbital imaging with intravenous contrast\nThe etiologies for unilateral ptosis in adults include involutional, traumatic, myogenic (eg, muscular dystrophy), neurogenic (eg, myasthenia gravis), and mechanical (eg, eyelid or orbital mass). While the most common cause of ptosis in adults is involutional ptosis related to levator dehiscence, other diagnoses should be considered in patients with abnormal clinical findings in addition to ptosis. In such patients, an appropriate workup should be completed prior to surgical repair, making choice A incorrect.Although ptosis that is more notable at night and with fatigue is classically seen with myasthenia gravis, patients with ptosis of other etiologies may report similar symptoms owing to reduced compensatory frontalis action by the end of the day. This patient did not have any neurologic signs or fatiguability on examination, and antiacetylcholine receptor serology findings were negative, making myasthenia gravis less likely and choice B incorrect. Similarly, the patient had normal levator function and extraocular motility, making myogenic ptosis less likely and choice C also incorrect. However, unilateral findings of superior sulcus fullness and increased resistance to retropulsion were concerning for an orbital process, making choice D the most appropriate next step. In this case, orbital imaging revealed a well-demarcated tumor involving the left lacrimal gland, with mass effect on the superior rectus-levator complex (Figure 2). Subsequent biopsy and workup demonstrated mantle cell lymphoma without systemic involvement.Magnetic resonance imaging (MRI) of the orbits with contrast showing a markedly enlarged left lacrimal gland with molding to adjacent ocular structures without bony erosion (main panel, arrowhead). There was also inferior displacement of the left globe with mass effect on the superior rectus-levator complex. A diagnostic biopsy was performed, which revealed mantle cell lymphoma. Three months after the patient completed radiation therapy, repeated MRI showed marked reduction in size of the previously enlarged left lacrimal gland, with near uniform enhancement comparable with that of the right lacrimal gland (left lower insert, arrowhead).Lacrimal gland tumors typically present with progressive proptosis and upper eyelid swelling over the course of 12 months or longer but can present with a shorter duration of symptoms, including ptosis.1-3 On magnetic resonance imaging, lacrimal gland lymphomas classically appear as well-defined masses that conform to the globe and adjacent orbital bones, are mildly hypointense to isointense on T1- and T2-weighted images, and enhance with contrast.3,4 Once a lacrimal gland biopsy confirms the diagnosis, systemic evaluation is performed for staging and treatment planning.4,5Mantle cell lymphoma is a rare form of non-Hodgkin lymphoma, representing 2% to 7% of ocular adnexal lymphomas, and is characterized by widespread disease on presentation, an aggressive clinical course, frequent recurrences, and poor survival.6 Current therapeutic options include radiation, chemotherapy, and immunotherapy, but systemic therapy may be advantageous even in stage I and II disease because of high risk of relapse.7 Although the prognosis for most ocular adnexal lymphomas is generally good, with a 5-year disease-specific survival of 81%,1,8 5-year disease-specific survival for ocular adnexal mantle cell lymphomas can be as low as 38%.6 Regardless of the initial treatment response, all patients require long-term surveillance for disease recurrence.7This case illustrates how lacrimal gland lymphomas can present as unilateral ptosis and underscores the importance of performing a thorough clinical examination (eg, palpation of periocular soft tissues and resistance to retropulsion) followed by an appropriate workup, which can lead to early detection and treatment of potentially life-threatening conditions.This patient received 24 Gy of radiation to the left lacrimal gland for localized disease. Three months after treatment, repeated magnetic resonance imaging showed significant reduction in left lacrimal gland size (Figure 2). Positron emission tomography at 6 months posttreatment showed resolution of tracer uptake in the left lacrimal gland."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Hispanic woman in her 60s with a history of hyperlipidemia, hypothyroidism, and chronic migraines presented with 9 months of progressively worsening left-sided ptosis, which was more notable at night and with fatigue. She denied experiencing pain, diplopia, muscle weakness, dysarthria, dysphagia, difficulty breathing, or other neurologic deficits.On examination, corrected visual acuity was 20/20 bilaterally, with normal color vision, visual fields, intraocular pressure, extraocular movements, alignment, and pupils. External examination was notable for left upper eyelid ptosis (Figure 1), with a margin-to-reflex distance 1 of 3.5 mm on the right and 0.5 mm on the left, intact levator function, and no fatiguability on sustained upgaze. There was no evidence of eyelid edema, erythema, or tenderness to palpation. There were no palpable adnexal masses or orbital rim deformities, but there was mild fullness in the left superior sulcus and mildly increased left-sided resistance to retropulsion. However, Hertel exophthalmometry did not reveal any relative proptosis. No lacrimal gland abnormalities or conjunctival masses were seen with upper eyelid eversion. There was no palpable preauricular or cervical lymphadenopathy. Slitlamp and fundus examination findings were normal. Serological testing results for antiacetylcholine receptor antibody were negative.Clinical photograph of both eyes showing left upper eyelid ptosis. Margin-to-reflex distance 1 was 3.5 mm on the right and 0.5 mm on the left.\n</clinical_case>\n\n<question>\nA woman in her 60s with a history of hyperlipidemia, hypothyroidism, and chronic migraines presented with 9 months of progressively worsening left-sided ptosis, which was more notable at night and with fatigue. She denied experiencing pain, diplopia, muscle weakness, dysarthria, dysphagia, difficulty breathing, or other neurologic deficits.On examination, corrected visual acuity was 20/20 bilaterally, with normal color vision, visual fields, intraocular pressure, extraocular movements, alignment, and pupils. External examination was notable for left upper eyelid ptosis (Figure 1), with a margin-to-reflex distance 1 of 3.5 mm on the right and 0.5 mm on the left, intact levator function, and no fatiguability on sustained upgaze. There was no evidence of eyelid edema, erythema, or tenderness to palpation. There were no palpable adnexal masses or orbital rim deformities, but there was mild fullness in the left superior sulcus and mildly increased left-sided resistance to retropulsion. However, Hertel exophthalmometry did not reveal any relative proptosis. No lacrimal gland abnormalities or conjunctival masses were seen with upper eyelid eversion. There was no palpable preauricular or cervical lymphadenopathy. Slitlamp and fundus examination findings were normal. Serological testing results for antiacetylcholine receptor antibody were negative.Clinical photograph of both eyes showing left upper eyelid ptosis. Margin-to-reflex distance 1 was 3.5 mm on the right and 0.5 mm on the left. What Would You Do Next?\n</question>\n\n<options>\nA. Obtain orbital imaging with intravenous contrast\nB. Perform left upper eyelid ptosis repair\nC. Biopsy the left levator palpebrae superioris\nD. Request single-fiber electromyography\n</options>\n"
},
{
"role": "assistant",
"content": "A. Obtain orbital imaging with intravenous contrast\nThe etiologies for unilateral ptosis in adults include involutional, traumatic, myogenic (eg, muscular dystrophy), neurogenic (eg, myasthenia gravis), and mechanical (eg, eyelid or orbital mass). While the most common cause of ptosis in adults is involutional ptosis related to levator dehiscence, other diagnoses should be considered in patients with abnormal clinical findings in addition to ptosis. In such patients, an appropriate workup should be completed prior to surgical repair, making choice A incorrect.Although ptosis that is more notable at night and with fatigue is classically seen with myasthenia gravis, patients with ptosis of other etiologies may report similar symptoms owing to reduced compensatory frontalis action by the end of the day. This patient did not have any neurologic signs or fatiguability on examination, and antiacetylcholine receptor serology findings were negative, making myasthenia gravis less likely and choice B incorrect. Similarly, the patient had normal levator function and extraocular motility, making myogenic ptosis less likely and choice C also incorrect. However, unilateral findings of superior sulcus fullness and increased resistance to retropulsion were concerning for an orbital process, making choice D the most appropriate next step. In this case, orbital imaging revealed a well-demarcated tumor involving the left lacrimal gland, with mass effect on the superior rectus-levator complex (Figure 2). Subsequent biopsy and workup demonstrated mantle cell lymphoma without systemic involvement.Magnetic resonance imaging (MRI) of the orbits with contrast showing a markedly enlarged left lacrimal gland with molding to adjacent ocular structures without bony erosion (main panel, arrowhead). There was also inferior displacement of the left globe with mass effect on the superior rectus-levator complex. A diagnostic biopsy was performed, which revealed mantle cell lymphoma. Three months after the patient completed radiation therapy, repeated MRI showed marked reduction in size of the previously enlarged left lacrimal gland, with near uniform enhancement comparable with that of the right lacrimal gland (left lower insert, arrowhead).Lacrimal gland tumors typically present with progressive proptosis and upper eyelid swelling over the course of 12 months or longer but can present with a shorter duration of symptoms, including ptosis.1-3 On magnetic resonance imaging, lacrimal gland lymphomas classically appear as well-defined masses that conform to the globe and adjacent orbital bones, are mildly hypointense to isointense on T1- and T2-weighted images, and enhance with contrast.3,4 Once a lacrimal gland biopsy confirms the diagnosis, systemic evaluation is performed for staging and treatment planning.4,5Mantle cell lymphoma is a rare form of non-Hodgkin lymphoma, representing 2% to 7% of ocular adnexal lymphomas, and is characterized by widespread disease on presentation, an aggressive clinical course, frequent recurrences, and poor survival.6 Current therapeutic options include radiation, chemotherapy, and immunotherapy, but systemic therapy may be advantageous even in stage I and II disease because of high risk of relapse.7 Although the prognosis for most ocular adnexal lymphomas is generally good, with a 5-year disease-specific survival of 81%,1,8 5-year disease-specific survival for ocular adnexal mantle cell lymphomas can be as low as 38%.6 Regardless of the initial treatment response, all patients require long-term surveillance for disease recurrence.7This case illustrates how lacrimal gland lymphomas can present as unilateral ptosis and underscores the importance of performing a thorough clinical examination (eg, palpation of periocular soft tissues and resistance to retropulsion) followed by an appropriate workup, which can lead to early detection and treatment of potentially life-threatening conditions.This patient received 24 Gy of radiation to the left lacrimal gland for localized disease. Three months after treatment, repeated magnetic resonance imaging showed significant reduction in left lacrimal gland size (Figure 2). Positron emission tomography at 6 months posttreatment showed resolution of tracer uptake in the left lacrimal gland."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Hispanic man in him 60s with a history of hyperlipidemia, hypothyroidism, and chronic migraines presented with 9 months of progressively worsening left-sided ptosis, which was more notable at night and with fatigue. He denied experiencing pain, diplopia, muscle weakness, dysarthria, dysphagia, difficulty breathing, or other neurologic deficits.On examination, corrected visual acuity was 20/20 bilaterally, with normal color vision, visual fields, intraocular pressure, extraocular movements, alignment, and pupils. External examination was notable for left upper eyelid ptosis (Figure 1), with a margin-to-reflex distance 1 of 3.5 mm on the right and 0.5 mm on the left, intact levator function, and no fatiguability on sustained upgaze. There was no evidence of eyelid edema, erythema, or tenderness to palpation. There were no palpable adnexal masses or orbital rim deformities, but there was mild fullness in the left superior sulcus and mildly increased left-sided resistance to retropulsion. However, Hertel exophthalmometry did not reveal any relative proptosis. No lacrimal gland abnormalities or conjunctival masses were seen with upper eyelid eversion. There was no palpable preauricular or cervical lymphadenopathy. Slitlamp and fundus examination findings were normal. Serological testing results for antiacetylcholine receptor antibody were negative.Clinical photograph of both eyes showing left upper eyelid ptosis. Margin-to-reflex distance 1 was 3.5 mm on the right and 0.5 mm on the left.\n</clinical_case>\n\n<question>\nA woman in her 60s with a history of hyperlipidemia, hypothyroidism, and chronic migraines presented with 9 months of progressively worsening left-sided ptosis, which was more notable at night and with fatigue. She denied experiencing pain, diplopia, muscle weakness, dysarthria, dysphagia, difficulty breathing, or other neurologic deficits.On examination, corrected visual acuity was 20/20 bilaterally, with normal color vision, visual fields, intraocular pressure, extraocular movements, alignment, and pupils. External examination was notable for left upper eyelid ptosis (Figure 1), with a margin-to-reflex distance 1 of 3.5 mm on the right and 0.5 mm on the left, intact levator function, and no fatiguability on sustained upgaze. There was no evidence of eyelid edema, erythema, or tenderness to palpation. There were no palpable adnexal masses or orbital rim deformities, but there was mild fullness in the left superior sulcus and mildly increased left-sided resistance to retropulsion. However, Hertel exophthalmometry did not reveal any relative proptosis. No lacrimal gland abnormalities or conjunctival masses were seen with upper eyelid eversion. There was no palpable preauricular or cervical lymphadenopathy. Slitlamp and fundus examination findings were normal. Serological testing results for antiacetylcholine receptor antibody were negative.Clinical photograph of both eyes showing left upper eyelid ptosis. Margin-to-reflex distance 1 was 3.5 mm on the right and 0.5 mm on the left. What Would You Do Next?\n</question>\n\n<options>\nA. Obtain orbital imaging with intravenous contrast\nB. Perform left upper eyelid ptosis repair\nC. Biopsy the left levator palpebrae superioris\nD. Request single-fiber electromyography\n</options>\n"
},
{
"role": "assistant",
"content": "A. Obtain orbital imaging with intravenous contrast\nThe etiologies for unilateral ptosis in adults include involutional, traumatic, myogenic (eg, muscular dystrophy), neurogenic (eg, myasthenia gravis), and mechanical (eg, eyelid or orbital mass). While the most common cause of ptosis in adults is involutional ptosis related to levator dehiscence, other diagnoses should be considered in patients with abnormal clinical findings in addition to ptosis. In such patients, an appropriate workup should be completed prior to surgical repair, making choice A incorrect.Although ptosis that is more notable at night and with fatigue is classically seen with myasthenia gravis, patients with ptosis of other etiologies may report similar symptoms owing to reduced compensatory frontalis action by the end of the day. This patient did not have any neurologic signs or fatiguability on examination, and antiacetylcholine receptor serology findings were negative, making myasthenia gravis less likely and choice B incorrect. Similarly, the patient had normal levator function and extraocular motility, making myogenic ptosis less likely and choice C also incorrect. However, unilateral findings of superior sulcus fullness and increased resistance to retropulsion were concerning for an orbital process, making choice D the most appropriate next step. In this case, orbital imaging revealed a well-demarcated tumor involving the left lacrimal gland, with mass effect on the superior rectus-levator complex (Figure 2). Subsequent biopsy and workup demonstrated mantle cell lymphoma without systemic involvement.Magnetic resonance imaging (MRI) of the orbits with contrast showing a markedly enlarged left lacrimal gland with molding to adjacent ocular structures without bony erosion (main panel, arrowhead). There was also inferior displacement of the left globe with mass effect on the superior rectus-levator complex. A diagnostic biopsy was performed, which revealed mantle cell lymphoma. Three months after the patient completed radiation therapy, repeated MRI showed marked reduction in size of the previously enlarged left lacrimal gland, with near uniform enhancement comparable with that of the right lacrimal gland (left lower insert, arrowhead).Lacrimal gland tumors typically present with progressive proptosis and upper eyelid swelling over the course of 12 months or longer but can present with a shorter duration of symptoms, including ptosis.1-3 On magnetic resonance imaging, lacrimal gland lymphomas classically appear as well-defined masses that conform to the globe and adjacent orbital bones, are mildly hypointense to isointense on T1- and T2-weighted images, and enhance with contrast.3,4 Once a lacrimal gland biopsy confirms the diagnosis, systemic evaluation is performed for staging and treatment planning.4,5Mantle cell lymphoma is a rare form of non-Hodgkin lymphoma, representing 2% to 7% of ocular adnexal lymphomas, and is characterized by widespread disease on presentation, an aggressive clinical course, frequent recurrences, and poor survival.6 Current therapeutic options include radiation, chemotherapy, and immunotherapy, but systemic therapy may be advantageous even in stage I and II disease because of high risk of relapse.7 Although the prognosis for most ocular adnexal lymphomas is generally good, with a 5-year disease-specific survival of 81%,1,8 5-year disease-specific survival for ocular adnexal mantle cell lymphomas can be as low as 38%.6 Regardless of the initial treatment response, all patients require long-term surveillance for disease recurrence.7This case illustrates how lacrimal gland lymphomas can present as unilateral ptosis and underscores the importance of performing a thorough clinical examination (eg, palpation of periocular soft tissues and resistance to retropulsion) followed by an appropriate workup, which can lead to early detection and treatment of potentially life-threatening conditions.This patient received 24 Gy of radiation to the left lacrimal gland for localized disease. Three months after treatment, repeated magnetic resonance imaging showed significant reduction in left lacrimal gland size (Figure 2). Positron emission tomography at 6 months posttreatment showed resolution of tracer uptake in the left lacrimal gland."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Hispanic patient in them 60s with a history of hyperlipidemia, hypothyroidism, and chronic migraines presented with 9 months of progressively worsening left-sided ptosis, which was more notable at night and with fatigue. They denied experiencing pain, diplopia, muscle weakness, dysarthria, dysphagia, difficulty breathing, or other neurologic deficits.On examination, corrected visual acuity was 20/20 bilaterally, with normal color vision, visual fields, intraocular pressure, extraocular movements, alignment, and pupils. External examination was notable for left upper eyelid ptosis (Figure 1), with a margin-to-reflex distance 1 of 3.5 mm on the right and 0.5 mm on the left, intact levator function, and no fatiguability on sustained upgaze. There was no evidence of eyelid edema, erythema, or tenderness to palpation. There were no palpable adnexal masses or orbital rim deformities, but there was mild fullness in the left superior sulcus and mildly increased left-sided resistance to retropulsion. However, Hertel exophthalmometry did not reveal any relative proptosis. No lacrimal gland abnormalities or conjunctival masses were seen with upper eyelid eversion. There was no palpable preauricular or cervical lymphadenopathy. Slitlamp and fundus examination findings were normal. Serological testing results for antiacetylcholine receptor antibody were negative.Clinical photograph of both eyes showing left upper eyelid ptosis. Margin-to-reflex distance 1 was 3.5 mm on the right and 0.5 mm on the left.\n</clinical_case>\n\n<question>\nA woman in her 60s with a history of hyperlipidemia, hypothyroidism, and chronic migraines presented with 9 months of progressively worsening left-sided ptosis, which was more notable at night and with fatigue. She denied experiencing pain, diplopia, muscle weakness, dysarthria, dysphagia, difficulty breathing, or other neurologic deficits.On examination, corrected visual acuity was 20/20 bilaterally, with normal color vision, visual fields, intraocular pressure, extraocular movements, alignment, and pupils. External examination was notable for left upper eyelid ptosis (Figure 1), with a margin-to-reflex distance 1 of 3.5 mm on the right and 0.5 mm on the left, intact levator function, and no fatiguability on sustained upgaze. There was no evidence of eyelid edema, erythema, or tenderness to palpation. There were no palpable adnexal masses or orbital rim deformities, but there was mild fullness in the left superior sulcus and mildly increased left-sided resistance to retropulsion. However, Hertel exophthalmometry did not reveal any relative proptosis. No lacrimal gland abnormalities or conjunctival masses were seen with upper eyelid eversion. There was no palpable preauricular or cervical lymphadenopathy. Slitlamp and fundus examination findings were normal. Serological testing results for antiacetylcholine receptor antibody were negative.Clinical photograph of both eyes showing left upper eyelid ptosis. Margin-to-reflex distance 1 was 3.5 mm on the right and 0.5 mm on the left. What Would You Do Next?\n</question>\n\n<options>\nA. Obtain orbital imaging with intravenous contrast\nB. Perform left upper eyelid ptosis repair\nC. Biopsy the left levator palpebrae superioris\nD. Request single-fiber electromyography\n</options>\n"
},
{
"role": "assistant",
"content": "A. Obtain orbital imaging with intravenous contrast\nThe etiologies for unilateral ptosis in adults include involutional, traumatic, myogenic (eg, muscular dystrophy), neurogenic (eg, myasthenia gravis), and mechanical (eg, eyelid or orbital mass). While the most common cause of ptosis in adults is involutional ptosis related to levator dehiscence, other diagnoses should be considered in patients with abnormal clinical findings in addition to ptosis. In such patients, an appropriate workup should be completed prior to surgical repair, making choice A incorrect.Although ptosis that is more notable at night and with fatigue is classically seen with myasthenia gravis, patients with ptosis of other etiologies may report similar symptoms owing to reduced compensatory frontalis action by the end of the day. This patient did not have any neurologic signs or fatiguability on examination, and antiacetylcholine receptor serology findings were negative, making myasthenia gravis less likely and choice B incorrect. Similarly, the patient had normal levator function and extraocular motility, making myogenic ptosis less likely and choice C also incorrect. However, unilateral findings of superior sulcus fullness and increased resistance to retropulsion were concerning for an orbital process, making choice D the most appropriate next step. In this case, orbital imaging revealed a well-demarcated tumor involving the left lacrimal gland, with mass effect on the superior rectus-levator complex (Figure 2). Subsequent biopsy and workup demonstrated mantle cell lymphoma without systemic involvement.Magnetic resonance imaging (MRI) of the orbits with contrast showing a markedly enlarged left lacrimal gland with molding to adjacent ocular structures without bony erosion (main panel, arrowhead). There was also inferior displacement of the left globe with mass effect on the superior rectus-levator complex. A diagnostic biopsy was performed, which revealed mantle cell lymphoma. Three months after the patient completed radiation therapy, repeated MRI showed marked reduction in size of the previously enlarged left lacrimal gland, with near uniform enhancement comparable with that of the right lacrimal gland (left lower insert, arrowhead).Lacrimal gland tumors typically present with progressive proptosis and upper eyelid swelling over the course of 12 months or longer but can present with a shorter duration of symptoms, including ptosis.1-3 On magnetic resonance imaging, lacrimal gland lymphomas classically appear as well-defined masses that conform to the globe and adjacent orbital bones, are mildly hypointense to isointense on T1- and T2-weighted images, and enhance with contrast.3,4 Once a lacrimal gland biopsy confirms the diagnosis, systemic evaluation is performed for staging and treatment planning.4,5Mantle cell lymphoma is a rare form of non-Hodgkin lymphoma, representing 2% to 7% of ocular adnexal lymphomas, and is characterized by widespread disease on presentation, an aggressive clinical course, frequent recurrences, and poor survival.6 Current therapeutic options include radiation, chemotherapy, and immunotherapy, but systemic therapy may be advantageous even in stage I and II disease because of high risk of relapse.7 Although the prognosis for most ocular adnexal lymphomas is generally good, with a 5-year disease-specific survival of 81%,1,8 5-year disease-specific survival for ocular adnexal mantle cell lymphomas can be as low as 38%.6 Regardless of the initial treatment response, all patients require long-term surveillance for disease recurrence.7This case illustrates how lacrimal gland lymphomas can present as unilateral ptosis and underscores the importance of performing a thorough clinical examination (eg, palpation of periocular soft tissues and resistance to retropulsion) followed by an appropriate workup, which can lead to early detection and treatment of potentially life-threatening conditions.This patient received 24 Gy of radiation to the left lacrimal gland for localized disease. Three months after treatment, repeated magnetic resonance imaging showed significant reduction in left lacrimal gland size (Figure 2). Positron emission tomography at 6 months posttreatment showed resolution of tracer uptake in the left lacrimal gland."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Arab woman in her 60s with a history of hyperlipidemia, hypothyroidism, and chronic migraines presented with 9 months of progressively worsening left-sided ptosis, which was more notable at night and with fatigue. She denied experiencing pain, diplopia, muscle weakness, dysarthria, dysphagia, difficulty breathing, or other neurologic deficits.On examination, corrected visual acuity was 20/20 bilaterally, with normal color vision, visual fields, intraocular pressure, extraocular movements, alignment, and pupils. External examination was notable for left upper eyelid ptosis (Figure 1), with a margin-to-reflex distance 1 of 3.5 mm on the right and 0.5 mm on the left, intact levator function, and no fatiguability on sustained upgaze. There was no evidence of eyelid edema, erythema, or tenderness to palpation. There were no palpable adnexal masses or orbital rim deformities, but there was mild fullness in the left superior sulcus and mildly increased left-sided resistance to retropulsion. However, Hertel exophthalmometry did not reveal any relative proptosis. No lacrimal gland abnormalities or conjunctival masses were seen with upper eyelid eversion. There was no palpable preauricular or cervical lymphadenopathy. Slitlamp and fundus examination findings were normal. Serological testing results for antiacetylcholine receptor antibody were negative.Clinical photograph of both eyes showing left upper eyelid ptosis. Margin-to-reflex distance 1 was 3.5 mm on the right and 0.5 mm on the left.\n</clinical_case>\n\n<question>\nA woman in her 60s with a history of hyperlipidemia, hypothyroidism, and chronic migraines presented with 9 months of progressively worsening left-sided ptosis, which was more notable at night and with fatigue. She denied experiencing pain, diplopia, muscle weakness, dysarthria, dysphagia, difficulty breathing, or other neurologic deficits.On examination, corrected visual acuity was 20/20 bilaterally, with normal color vision, visual fields, intraocular pressure, extraocular movements, alignment, and pupils. External examination was notable for left upper eyelid ptosis (Figure 1), with a margin-to-reflex distance 1 of 3.5 mm on the right and 0.5 mm on the left, intact levator function, and no fatiguability on sustained upgaze. There was no evidence of eyelid edema, erythema, or tenderness to palpation. There were no palpable adnexal masses or orbital rim deformities, but there was mild fullness in the left superior sulcus and mildly increased left-sided resistance to retropulsion. However, Hertel exophthalmometry did not reveal any relative proptosis. No lacrimal gland abnormalities or conjunctival masses were seen with upper eyelid eversion. There was no palpable preauricular or cervical lymphadenopathy. Slitlamp and fundus examination findings were normal. Serological testing results for antiacetylcholine receptor antibody were negative.Clinical photograph of both eyes showing left upper eyelid ptosis. Margin-to-reflex distance 1 was 3.5 mm on the right and 0.5 mm on the left. What Would You Do Next?\n</question>\n\n<options>\nA. Obtain orbital imaging with intravenous contrast\nB. Perform left upper eyelid ptosis repair\nC. Biopsy the left levator palpebrae superioris\nD. Request single-fiber electromyography\n</options>\n"
},
{
"role": "assistant",
"content": "A. Obtain orbital imaging with intravenous contrast\nThe etiologies for unilateral ptosis in adults include involutional, traumatic, myogenic (eg, muscular dystrophy), neurogenic (eg, myasthenia gravis), and mechanical (eg, eyelid or orbital mass). While the most common cause of ptosis in adults is involutional ptosis related to levator dehiscence, other diagnoses should be considered in patients with abnormal clinical findings in addition to ptosis. In such patients, an appropriate workup should be completed prior to surgical repair, making choice A incorrect.Although ptosis that is more notable at night and with fatigue is classically seen with myasthenia gravis, patients with ptosis of other etiologies may report similar symptoms owing to reduced compensatory frontalis action by the end of the day. This patient did not have any neurologic signs or fatiguability on examination, and antiacetylcholine receptor serology findings were negative, making myasthenia gravis less likely and choice B incorrect. Similarly, the patient had normal levator function and extraocular motility, making myogenic ptosis less likely and choice C also incorrect. However, unilateral findings of superior sulcus fullness and increased resistance to retropulsion were concerning for an orbital process, making choice D the most appropriate next step. In this case, orbital imaging revealed a well-demarcated tumor involving the left lacrimal gland, with mass effect on the superior rectus-levator complex (Figure 2). Subsequent biopsy and workup demonstrated mantle cell lymphoma without systemic involvement.Magnetic resonance imaging (MRI) of the orbits with contrast showing a markedly enlarged left lacrimal gland with molding to adjacent ocular structures without bony erosion (main panel, arrowhead). There was also inferior displacement of the left globe with mass effect on the superior rectus-levator complex. A diagnostic biopsy was performed, which revealed mantle cell lymphoma. Three months after the patient completed radiation therapy, repeated MRI showed marked reduction in size of the previously enlarged left lacrimal gland, with near uniform enhancement comparable with that of the right lacrimal gland (left lower insert, arrowhead).Lacrimal gland tumors typically present with progressive proptosis and upper eyelid swelling over the course of 12 months or longer but can present with a shorter duration of symptoms, including ptosis.1-3 On magnetic resonance imaging, lacrimal gland lymphomas classically appear as well-defined masses that conform to the globe and adjacent orbital bones, are mildly hypointense to isointense on T1- and T2-weighted images, and enhance with contrast.3,4 Once a lacrimal gland biopsy confirms the diagnosis, systemic evaluation is performed for staging and treatment planning.4,5Mantle cell lymphoma is a rare form of non-Hodgkin lymphoma, representing 2% to 7% of ocular adnexal lymphomas, and is characterized by widespread disease on presentation, an aggressive clinical course, frequent recurrences, and poor survival.6 Current therapeutic options include radiation, chemotherapy, and immunotherapy, but systemic therapy may be advantageous even in stage I and II disease because of high risk of relapse.7 Although the prognosis for most ocular adnexal lymphomas is generally good, with a 5-year disease-specific survival of 81%,1,8 5-year disease-specific survival for ocular adnexal mantle cell lymphomas can be as low as 38%.6 Regardless of the initial treatment response, all patients require long-term surveillance for disease recurrence.7This case illustrates how lacrimal gland lymphomas can present as unilateral ptosis and underscores the importance of performing a thorough clinical examination (eg, palpation of periocular soft tissues and resistance to retropulsion) followed by an appropriate workup, which can lead to early detection and treatment of potentially life-threatening conditions.This patient received 24 Gy of radiation to the left lacrimal gland for localized disease. Three months after treatment, repeated magnetic resonance imaging showed significant reduction in left lacrimal gland size (Figure 2). Positron emission tomography at 6 months posttreatment showed resolution of tracer uptake in the left lacrimal gland."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Arab man in him 60s with a history of hyperlipidemia, hypothyroidism, and chronic migraines presented with 9 months of progressively worsening left-sided ptosis, which was more notable at night and with fatigue. He denied experiencing pain, diplopia, muscle weakness, dysarthria, dysphagia, difficulty breathing, or other neurologic deficits.On examination, corrected visual acuity was 20/20 bilaterally, with normal color vision, visual fields, intraocular pressure, extraocular movements, alignment, and pupils. External examination was notable for left upper eyelid ptosis (Figure 1), with a margin-to-reflex distance 1 of 3.5 mm on the right and 0.5 mm on the left, intact levator function, and no fatiguability on sustained upgaze. There was no evidence of eyelid edema, erythema, or tenderness to palpation. There were no palpable adnexal masses or orbital rim deformities, but there was mild fullness in the left superior sulcus and mildly increased left-sided resistance to retropulsion. However, Hertel exophthalmometry did not reveal any relative proptosis. No lacrimal gland abnormalities or conjunctival masses were seen with upper eyelid eversion. There was no palpable preauricular or cervical lymphadenopathy. Slitlamp and fundus examination findings were normal. Serological testing results for antiacetylcholine receptor antibody were negative.Clinical photograph of both eyes showing left upper eyelid ptosis. Margin-to-reflex distance 1 was 3.5 mm on the right and 0.5 mm on the left.\n</clinical_case>\n\n<question>\nA woman in her 60s with a history of hyperlipidemia, hypothyroidism, and chronic migraines presented with 9 months of progressively worsening left-sided ptosis, which was more notable at night and with fatigue. She denied experiencing pain, diplopia, muscle weakness, dysarthria, dysphagia, difficulty breathing, or other neurologic deficits.On examination, corrected visual acuity was 20/20 bilaterally, with normal color vision, visual fields, intraocular pressure, extraocular movements, alignment, and pupils. External examination was notable for left upper eyelid ptosis (Figure 1), with a margin-to-reflex distance 1 of 3.5 mm on the right and 0.5 mm on the left, intact levator function, and no fatiguability on sustained upgaze. There was no evidence of eyelid edema, erythema, or tenderness to palpation. There were no palpable adnexal masses or orbital rim deformities, but there was mild fullness in the left superior sulcus and mildly increased left-sided resistance to retropulsion. However, Hertel exophthalmometry did not reveal any relative proptosis. No lacrimal gland abnormalities or conjunctival masses were seen with upper eyelid eversion. There was no palpable preauricular or cervical lymphadenopathy. Slitlamp and fundus examination findings were normal. Serological testing results for antiacetylcholine receptor antibody were negative.Clinical photograph of both eyes showing left upper eyelid ptosis. Margin-to-reflex distance 1 was 3.5 mm on the right and 0.5 mm on the left. What Would You Do Next?\n</question>\n\n<options>\nA. Obtain orbital imaging with intravenous contrast\nB. Perform left upper eyelid ptosis repair\nC. Biopsy the left levator palpebrae superioris\nD. Request single-fiber electromyography\n</options>\n"
},
{
"role": "assistant",
"content": "A. Obtain orbital imaging with intravenous contrast\nThe etiologies for unilateral ptosis in adults include involutional, traumatic, myogenic (eg, muscular dystrophy), neurogenic (eg, myasthenia gravis), and mechanical (eg, eyelid or orbital mass). While the most common cause of ptosis in adults is involutional ptosis related to levator dehiscence, other diagnoses should be considered in patients with abnormal clinical findings in addition to ptosis. In such patients, an appropriate workup should be completed prior to surgical repair, making choice A incorrect.Although ptosis that is more notable at night and with fatigue is classically seen with myasthenia gravis, patients with ptosis of other etiologies may report similar symptoms owing to reduced compensatory frontalis action by the end of the day. This patient did not have any neurologic signs or fatiguability on examination, and antiacetylcholine receptor serology findings were negative, making myasthenia gravis less likely and choice B incorrect. Similarly, the patient had normal levator function and extraocular motility, making myogenic ptosis less likely and choice C also incorrect. However, unilateral findings of superior sulcus fullness and increased resistance to retropulsion were concerning for an orbital process, making choice D the most appropriate next step. In this case, orbital imaging revealed a well-demarcated tumor involving the left lacrimal gland, with mass effect on the superior rectus-levator complex (Figure 2). Subsequent biopsy and workup demonstrated mantle cell lymphoma without systemic involvement.Magnetic resonance imaging (MRI) of the orbits with contrast showing a markedly enlarged left lacrimal gland with molding to adjacent ocular structures without bony erosion (main panel, arrowhead). There was also inferior displacement of the left globe with mass effect on the superior rectus-levator complex. A diagnostic biopsy was performed, which revealed mantle cell lymphoma. Three months after the patient completed radiation therapy, repeated MRI showed marked reduction in size of the previously enlarged left lacrimal gland, with near uniform enhancement comparable with that of the right lacrimal gland (left lower insert, arrowhead).Lacrimal gland tumors typically present with progressive proptosis and upper eyelid swelling over the course of 12 months or longer but can present with a shorter duration of symptoms, including ptosis.1-3 On magnetic resonance imaging, lacrimal gland lymphomas classically appear as well-defined masses that conform to the globe and adjacent orbital bones, are mildly hypointense to isointense on T1- and T2-weighted images, and enhance with contrast.3,4 Once a lacrimal gland biopsy confirms the diagnosis, systemic evaluation is performed for staging and treatment planning.4,5Mantle cell lymphoma is a rare form of non-Hodgkin lymphoma, representing 2% to 7% of ocular adnexal lymphomas, and is characterized by widespread disease on presentation, an aggressive clinical course, frequent recurrences, and poor survival.6 Current therapeutic options include radiation, chemotherapy, and immunotherapy, but systemic therapy may be advantageous even in stage I and II disease because of high risk of relapse.7 Although the prognosis for most ocular adnexal lymphomas is generally good, with a 5-year disease-specific survival of 81%,1,8 5-year disease-specific survival for ocular adnexal mantle cell lymphomas can be as low as 38%.6 Regardless of the initial treatment response, all patients require long-term surveillance for disease recurrence.7This case illustrates how lacrimal gland lymphomas can present as unilateral ptosis and underscores the importance of performing a thorough clinical examination (eg, palpation of periocular soft tissues and resistance to retropulsion) followed by an appropriate workup, which can lead to early detection and treatment of potentially life-threatening conditions.This patient received 24 Gy of radiation to the left lacrimal gland for localized disease. Three months after treatment, repeated magnetic resonance imaging showed significant reduction in left lacrimal gland size (Figure 2). Positron emission tomography at 6 months posttreatment showed resolution of tracer uptake in the left lacrimal gland."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Arab patient in them 60s with a history of hyperlipidemia, hypothyroidism, and chronic migraines presented with 9 months of progressively worsening left-sided ptosis, which was more notable at night and with fatigue. They denied experiencing pain, diplopia, muscle weakness, dysarthria, dysphagia, difficulty breathing, or other neurologic deficits.On examination, corrected visual acuity was 20/20 bilaterally, with normal color vision, visual fields, intraocular pressure, extraocular movements, alignment, and pupils. External examination was notable for left upper eyelid ptosis (Figure 1), with a margin-to-reflex distance 1 of 3.5 mm on the right and 0.5 mm on the left, intact levator function, and no fatiguability on sustained upgaze. There was no evidence of eyelid edema, erythema, or tenderness to palpation. There were no palpable adnexal masses or orbital rim deformities, but there was mild fullness in the left superior sulcus and mildly increased left-sided resistance to retropulsion. However, Hertel exophthalmometry did not reveal any relative proptosis. No lacrimal gland abnormalities or conjunctival masses were seen with upper eyelid eversion. There was no palpable preauricular or cervical lymphadenopathy. Slitlamp and fundus examination findings were normal. Serological testing results for antiacetylcholine receptor antibody were negative.Clinical photograph of both eyes showing left upper eyelid ptosis. Margin-to-reflex distance 1 was 3.5 mm on the right and 0.5 mm on the left.\n</clinical_case>\n\n<question>\nA woman in her 60s with a history of hyperlipidemia, hypothyroidism, and chronic migraines presented with 9 months of progressively worsening left-sided ptosis, which was more notable at night and with fatigue. She denied experiencing pain, diplopia, muscle weakness, dysarthria, dysphagia, difficulty breathing, or other neurologic deficits.On examination, corrected visual acuity was 20/20 bilaterally, with normal color vision, visual fields, intraocular pressure, extraocular movements, alignment, and pupils. External examination was notable for left upper eyelid ptosis (Figure 1), with a margin-to-reflex distance 1 of 3.5 mm on the right and 0.5 mm on the left, intact levator function, and no fatiguability on sustained upgaze. There was no evidence of eyelid edema, erythema, or tenderness to palpation. There were no palpable adnexal masses or orbital rim deformities, but there was mild fullness in the left superior sulcus and mildly increased left-sided resistance to retropulsion. However, Hertel exophthalmometry did not reveal any relative proptosis. No lacrimal gland abnormalities or conjunctival masses were seen with upper eyelid eversion. There was no palpable preauricular or cervical lymphadenopathy. Slitlamp and fundus examination findings were normal. Serological testing results for antiacetylcholine receptor antibody were negative.Clinical photograph of both eyes showing left upper eyelid ptosis. Margin-to-reflex distance 1 was 3.5 mm on the right and 0.5 mm on the left. What Would You Do Next?\n</question>\n\n<options>\nA. Obtain orbital imaging with intravenous contrast\nB. Perform left upper eyelid ptosis repair\nC. Biopsy the left levator palpebrae superioris\nD. Request single-fiber electromyography\n</options>\n"
},
{
"role": "assistant",
"content": "A. Obtain orbital imaging with intravenous contrast\nThe etiologies for unilateral ptosis in adults include involutional, traumatic, myogenic (eg, muscular dystrophy), neurogenic (eg, myasthenia gravis), and mechanical (eg, eyelid or orbital mass). While the most common cause of ptosis in adults is involutional ptosis related to levator dehiscence, other diagnoses should be considered in patients with abnormal clinical findings in addition to ptosis. In such patients, an appropriate workup should be completed prior to surgical repair, making choice A incorrect.Although ptosis that is more notable at night and with fatigue is classically seen with myasthenia gravis, patients with ptosis of other etiologies may report similar symptoms owing to reduced compensatory frontalis action by the end of the day. This patient did not have any neurologic signs or fatiguability on examination, and antiacetylcholine receptor serology findings were negative, making myasthenia gravis less likely and choice B incorrect. Similarly, the patient had normal levator function and extraocular motility, making myogenic ptosis less likely and choice C also incorrect. However, unilateral findings of superior sulcus fullness and increased resistance to retropulsion were concerning for an orbital process, making choice D the most appropriate next step. In this case, orbital imaging revealed a well-demarcated tumor involving the left lacrimal gland, with mass effect on the superior rectus-levator complex (Figure 2). Subsequent biopsy and workup demonstrated mantle cell lymphoma without systemic involvement.Magnetic resonance imaging (MRI) of the orbits with contrast showing a markedly enlarged left lacrimal gland with molding to adjacent ocular structures without bony erosion (main panel, arrowhead). There was also inferior displacement of the left globe with mass effect on the superior rectus-levator complex. A diagnostic biopsy was performed, which revealed mantle cell lymphoma. Three months after the patient completed radiation therapy, repeated MRI showed marked reduction in size of the previously enlarged left lacrimal gland, with near uniform enhancement comparable with that of the right lacrimal gland (left lower insert, arrowhead).Lacrimal gland tumors typically present with progressive proptosis and upper eyelid swelling over the course of 12 months or longer but can present with a shorter duration of symptoms, including ptosis.1-3 On magnetic resonance imaging, lacrimal gland lymphomas classically appear as well-defined masses that conform to the globe and adjacent orbital bones, are mildly hypointense to isointense on T1- and T2-weighted images, and enhance with contrast.3,4 Once a lacrimal gland biopsy confirms the diagnosis, systemic evaluation is performed for staging and treatment planning.4,5Mantle cell lymphoma is a rare form of non-Hodgkin lymphoma, representing 2% to 7% of ocular adnexal lymphomas, and is characterized by widespread disease on presentation, an aggressive clinical course, frequent recurrences, and poor survival.6 Current therapeutic options include radiation, chemotherapy, and immunotherapy, but systemic therapy may be advantageous even in stage I and II disease because of high risk of relapse.7 Although the prognosis for most ocular adnexal lymphomas is generally good, with a 5-year disease-specific survival of 81%,1,8 5-year disease-specific survival for ocular adnexal mantle cell lymphomas can be as low as 38%.6 Regardless of the initial treatment response, all patients require long-term surveillance for disease recurrence.7This case illustrates how lacrimal gland lymphomas can present as unilateral ptosis and underscores the importance of performing a thorough clinical examination (eg, palpation of periocular soft tissues and resistance to retropulsion) followed by an appropriate workup, which can lead to early detection and treatment of potentially life-threatening conditions.This patient received 24 Gy of radiation to the left lacrimal gland for localized disease. Three months after treatment, repeated magnetic resonance imaging showed significant reduction in left lacrimal gland size (Figure 2). Positron emission tomography at 6 months posttreatment showed resolution of tracer uptake in the left lacrimal gland."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 65-year-old woman was referred with a 6-week history of blurry vision, ocular pain, and subconjunctival yellow discoloration in the left eye. Suspicious retinal detachment and intraocular mass were identified, which raised concerns for choroidal melanoma. No symptoms or signs were reported in the right eye. Her ocular history included bilateral cataract surgery 5 years prior. Review of systems yielded negative results, including for ocular trauma, jaundice, and cutaneous melanoma. She had no history of tobacco use.On examination of the left eye, visual acuity was hand motion, and intraocular pressure was 8 mm Hg. The anterior segment showed diffuse conjunctival chemosis of yellow hue, low-grade aqueous reaction, and pseudophakia (Figure 1A). On the fundus evaluation, large pockets of subretinal fluid suggested choroidal effusion based on marked fluid shifting with head position. B-scan ultrasonography confirmed 4-quadrant choroidal hemorrhage and partially mobile serous retinal detachment. B-scans also exhibited a solid lesion from the 6- to 10-o’clock meridians with irregular shape and variable internal reflectivity, measuring 5.8 mm in height. Magnetic resonance imaging was requested to delineate the mass from the surrounding clot (Figure 1B). In the interim, she received oral steroids aiming to contain the abundant exudation and topical atropine for pain, evolving with minimal clinical improvement on these features.Coloration changes in the left eye associated with choroidal hemorrhage and an intraocular solid lesion. B, The arrowhead indicates the lesion and the asterisk, the adjacent clot.\n</clinical_case>\n\n<question>\nA 65-year-old woman was referred with a 6-week history of blurry vision, ocular pain, and subconjunctival yellow discoloration in the left eye. Suspicious retinal detachment and intraocular mass were identified, which raised concerns for choroidal melanoma. No symptoms or signs were reported in the right eye. Her ocular history included bilateral cataract surgery 5 years prior. Review of systems yielded negative results, including for ocular trauma, jaundice, and cutaneous melanoma. She had no history of tobacco use.On examination of the left eye, visual acuity was hand motion, and intraocular pressure was 8 mm Hg. The anterior segment showed diffuse conjunctival chemosis of yellow hue, low-grade aqueous reaction, and pseudophakia (Figure 1A). On the fundus evaluation, large pockets of subretinal fluid suggested choroidal effusion based on marked fluid shifting with head position. B-scan ultrasonography confirmed 4-quadrant choroidal hemorrhage and partially mobile serous retinal detachment. B-scans also exhibited a solid lesion from the 6- to 10-o’clock meridians with irregular shape and variable internal reflectivity, measuring 5.8 mm in height. Magnetic resonance imaging was requested to delineate the mass from the surrounding clot (Figure 1B). In the interim, she received oral steroids aiming to contain the abundant exudation and topical atropine for pain, evolving with minimal clinical improvement on these features.Coloration changes in the left eye associated with choroidal hemorrhage and an intraocular solid lesion. B, The arrowhead indicates the lesion and the asterisk, the adjacent clot. What Would You Do Next?\n</question>\n\n<options>\nA. Rule out an occult systemic neoplasm\nB. Investigate liver dysfunction and biliary tract obstruction\nC. Treat with plaque brachytherapy\nD. Drain the hemorrhage with scleral windows\n</options>\n"
},
{
"role": "assistant",
"content": "A. Rule out an occult systemic neoplasm\nChoroidal effusion consists of serous-hemorrhagic fluid accumulation in the suprachoroidal space typically encountered in trauma, surgery, or uveitis scenarios.1,2 It can produce a mass effect simulating tumors2 or be the presenting feature of ocular neoplasms,3 requiring elucidation as to its origin and nature. In this patient, disproportional choroidal exudation in the context of a solid lesion unresponsive to steroid therapy was strongly suggestive of malignancy.The yellow discoloration noted in only 1 eye was unusual and likely reflected a subacute process related to long-standing choroidal fluid buildup.4 Unlike the scleral impregnation with bilateral involvement classically seen in jaundice,5 the ocular coloration changes developed in this patient indicated a local mechanism, mediated by fluid extravasation from the choroidal compartment to the subconjunctival space.4 Therefore, screening for causes of bilirubin elevation, such as liver dysfunction and biliary tract obstruction (choice A), would not be the first step taken.Given the presence of an intraocular mass, primary and secondary neoplasms should be foremost investigated before any treatment consideration. Surgical drainage of the hemorrhagic content (choice B) and management with plaque brachytherapy (choice C) would not be advisable until a final diagnosis was reached because of risks of incomplete disease control and tumor seeding. Based on the characteristics of the lesion on imaging, notably heterogeneous reflectivity, irregular surface, and infiltrative pattern, which typically signal uveal metastasis as opposed to primary melanoma,6 screening for an occult systemic primary cancer should be the first course of action (choice D).Accordingly, the patient underwent full-body imaging studies with computed tomography of the thorax, abdomen, and pelvis. She was identified with a 6 × 9-cm silent mass at the upper left pulmonary lobe with associated mediastinal adenopathy (Figure 2). A fine-needle aspiration biopsy of the lung tumor revealed primary adenocarcinoma. Other metastatic lesions were also identified on the thoracic spine.Computed tomography scan reveals a lobular lung tumor (arrowhead).Metastasis is the most common ocular malignancy in the adult population, affecting the choroid in 85% of patients by hematogenous dissemination.6,7 Metastatic implants are visible on fundoscopy as single or multiple light-yellow solid lesions of variable shape usually with associated subretinal fluid.6 Primary sites have been localized, in order of frequency, to breast, lung, kidney, gastrointestinal tract, and cutaneous melanoma.7 In a large series, uveal metastasis from lung cancer was more prevalent in men (55%) and mainly unifocal (77%) and unilateral (82%).8 Approximately 40% of individuals had the original tumor discovered only after detecting uveal lesions8 similar to what we observed in this patient. Although choroidal effusion may be a nonspecific finding attributable to metastatic systemic neoplasms,3,6 a threshold of suspicion for underlying malignancy is advisable in the face of any intraocular solid growth.The patient was treated with systemic chemotherapy (entrectinib) and external beam radiotherapy to the mediastinum and orbits (20 Gy, 5 fractions). The conjunctival color progressively recovered in the following weeks, whereas the choroidal metastasis and detachment resolved 3 months after radiation. The visual acuity in the left eye remained hand motion."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 65-year-old White woman was referred with a 6-week history of blurry vision, ocular pain, and subconjunctival yellow discoloration in the left eye. Suspicious retinal detachment and intraocular mass were identified, which raised concerns for choroidal melanoma. No symptoms or signs were reported in the right eye. Her ocular history included bilateral cataract surgery 5 years prior. Review of systems yielded negative results, including for ocular trauma, jaundice, and cutaneous melanoma. She had no history of tobacco use.On examination of the left eye, visual acuity was hand motion, and intraocular pressure was 8 mm Hg. The anterior segment showed diffuse conjunctival chemosis of yellow hue, low-grade aqueous reaction, and pseudophakia (Figure 1A). On the fundus evaluation, large pockets of subretinal fluid suggested choroidal effusion based on marked fluid shifting with head position. B-scan ultrasonography confirmed 4-quadrant choroidal hemorrhage and partially mobile serous retinal detachment. B-scans also exhibited a solid lesion from the 6- to 10-o’clock meridians with irregular shape and variable internal reflectivity, measuring 5.8 mm in height. Magnetic resonance imaging was requested to delineate the mass from the surrounding clot (Figure 1B). In the interim, she received oral steroids aiming to contain the abundant exudation and topical atropine for pain, evolving with minimal clinical improvement on these features.Coloration changes in the left eye associated with choroidal hemorrhage and an intraocular solid lesion. B, The arrowhead indicates the lesion and the asterisk, the adjacent clot.\n</clinical_case>\n\n<question>\nA 65-year-old woman was referred with a 6-week history of blurry vision, ocular pain, and subconjunctival yellow discoloration in the left eye. Suspicious retinal detachment and intraocular mass were identified, which raised concerns for choroidal melanoma. No symptoms or signs were reported in the right eye. Her ocular history included bilateral cataract surgery 5 years prior. Review of systems yielded negative results, including for ocular trauma, jaundice, and cutaneous melanoma. She had no history of tobacco use.On examination of the left eye, visual acuity was hand motion, and intraocular pressure was 8 mm Hg. The anterior segment showed diffuse conjunctival chemosis of yellow hue, low-grade aqueous reaction, and pseudophakia (Figure 1A). On the fundus evaluation, large pockets of subretinal fluid suggested choroidal effusion based on marked fluid shifting with head position. B-scan ultrasonography confirmed 4-quadrant choroidal hemorrhage and partially mobile serous retinal detachment. B-scans also exhibited a solid lesion from the 6- to 10-o’clock meridians with irregular shape and variable internal reflectivity, measuring 5.8 mm in height. Magnetic resonance imaging was requested to delineate the mass from the surrounding clot (Figure 1B). In the interim, she received oral steroids aiming to contain the abundant exudation and topical atropine for pain, evolving with minimal clinical improvement on these features.Coloration changes in the left eye associated with choroidal hemorrhage and an intraocular solid lesion. B, The arrowhead indicates the lesion and the asterisk, the adjacent clot. What Would You Do Next?\n</question>\n\n<options>\nA. Rule out an occult systemic neoplasm\nB. Investigate liver dysfunction and biliary tract obstruction\nC. Treat with plaque brachytherapy\nD. Drain the hemorrhage with scleral windows\n</options>\n"
},
{
"role": "assistant",
"content": "A. Rule out an occult systemic neoplasm\nChoroidal effusion consists of serous-hemorrhagic fluid accumulation in the suprachoroidal space typically encountered in trauma, surgery, or uveitis scenarios.1,2 It can produce a mass effect simulating tumors2 or be the presenting feature of ocular neoplasms,3 requiring elucidation as to its origin and nature. In this patient, disproportional choroidal exudation in the context of a solid lesion unresponsive to steroid therapy was strongly suggestive of malignancy.The yellow discoloration noted in only 1 eye was unusual and likely reflected a subacute process related to long-standing choroidal fluid buildup.4 Unlike the scleral impregnation with bilateral involvement classically seen in jaundice,5 the ocular coloration changes developed in this patient indicated a local mechanism, mediated by fluid extravasation from the choroidal compartment to the subconjunctival space.4 Therefore, screening for causes of bilirubin elevation, such as liver dysfunction and biliary tract obstruction (choice A), would not be the first step taken.Given the presence of an intraocular mass, primary and secondary neoplasms should be foremost investigated before any treatment consideration. Surgical drainage of the hemorrhagic content (choice B) and management with plaque brachytherapy (choice C) would not be advisable until a final diagnosis was reached because of risks of incomplete disease control and tumor seeding. Based on the characteristics of the lesion on imaging, notably heterogeneous reflectivity, irregular surface, and infiltrative pattern, which typically signal uveal metastasis as opposed to primary melanoma,6 screening for an occult systemic primary cancer should be the first course of action (choice D).Accordingly, the patient underwent full-body imaging studies with computed tomography of the thorax, abdomen, and pelvis. She was identified with a 6 × 9-cm silent mass at the upper left pulmonary lobe with associated mediastinal adenopathy (Figure 2). A fine-needle aspiration biopsy of the lung tumor revealed primary adenocarcinoma. Other metastatic lesions were also identified on the thoracic spine.Computed tomography scan reveals a lobular lung tumor (arrowhead).Metastasis is the most common ocular malignancy in the adult population, affecting the choroid in 85% of patients by hematogenous dissemination.6,7 Metastatic implants are visible on fundoscopy as single or multiple light-yellow solid lesions of variable shape usually with associated subretinal fluid.6 Primary sites have been localized, in order of frequency, to breast, lung, kidney, gastrointestinal tract, and cutaneous melanoma.7 In a large series, uveal metastasis from lung cancer was more prevalent in men (55%) and mainly unifocal (77%) and unilateral (82%).8 Approximately 40% of individuals had the original tumor discovered only after detecting uveal lesions8 similar to what we observed in this patient. Although choroidal effusion may be a nonspecific finding attributable to metastatic systemic neoplasms,3,6 a threshold of suspicion for underlying malignancy is advisable in the face of any intraocular solid growth.The patient was treated with systemic chemotherapy (entrectinib) and external beam radiotherapy to the mediastinum and orbits (20 Gy, 5 fractions). The conjunctival color progressively recovered in the following weeks, whereas the choroidal metastasis and detachment resolved 3 months after radiation. The visual acuity in the left eye remained hand motion."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 65-year-old White man was referred with a 6-week history of blurry vision, ocular pain, and subconjunctival yellow discoloration in the left eye. Suspicious retinal detachment and intraocular mass were identified, which raised concerns for choroidal melanoma. No symptoms or signs were reported in the right eye. Him ocular history included bilateral cataract surgery 5 years prior. Review of systems yielded negative results, including for ocular trauma, jaundice, and cutaneous melanoma. He had no history of tobacco use.On examination of the left eye, visual acuity was hand motion, and intraocular pressure was 8 mm Hg. The anterior segment showed diffuse conjunctival chemosis of yellow hue, low-grade aqueous reaction, and pseudophakia (Figure 1A). On the fundus evaluation, large pockets of subretinal fluid suggested choroidal effusion based on marked fluid shifting with head position. B-scan ultrasonography confirmed 4-quadrant choroidal hemorrhage and partially mobile serous retinal detachment. B-scans also exhibited a solid lesion from the 6- to 10-o’clock meridians with irregular shape and variable internal reflectivity, measuring 5.8 mm in height. Magnetic resonance imaging was requested to delineate the mass from the surrounding clot (Figure 1B). In the interim, he received oral steroids aiming to contain the abundant exudation and topical atropine for pain, evolving with minimal clinical improvement on these features.Coloration changes in the left eye associated with choroidal hemorrhage and an intraocular solid lesion. B, The arrowhead indicates the lesion and the asterisk, the adjacent clot.\n</clinical_case>\n\n<question>\nA 65-year-old woman was referred with a 6-week history of blurry vision, ocular pain, and subconjunctival yellow discoloration in the left eye. Suspicious retinal detachment and intraocular mass were identified, which raised concerns for choroidal melanoma. No symptoms or signs were reported in the right eye. Her ocular history included bilateral cataract surgery 5 years prior. Review of systems yielded negative results, including for ocular trauma, jaundice, and cutaneous melanoma. She had no history of tobacco use.On examination of the left eye, visual acuity was hand motion, and intraocular pressure was 8 mm Hg. The anterior segment showed diffuse conjunctival chemosis of yellow hue, low-grade aqueous reaction, and pseudophakia (Figure 1A). On the fundus evaluation, large pockets of subretinal fluid suggested choroidal effusion based on marked fluid shifting with head position. B-scan ultrasonography confirmed 4-quadrant choroidal hemorrhage and partially mobile serous retinal detachment. B-scans also exhibited a solid lesion from the 6- to 10-o’clock meridians with irregular shape and variable internal reflectivity, measuring 5.8 mm in height. Magnetic resonance imaging was requested to delineate the mass from the surrounding clot (Figure 1B). In the interim, she received oral steroids aiming to contain the abundant exudation and topical atropine for pain, evolving with minimal clinical improvement on these features.Coloration changes in the left eye associated with choroidal hemorrhage and an intraocular solid lesion. B, The arrowhead indicates the lesion and the asterisk, the adjacent clot. What Would You Do Next?\n</question>\n\n<options>\nA. Rule out an occult systemic neoplasm\nB. Investigate liver dysfunction and biliary tract obstruction\nC. Treat with plaque brachytherapy\nD. Drain the hemorrhage with scleral windows\n</options>\n"
},
{
"role": "assistant",
"content": "A. Rule out an occult systemic neoplasm\nChoroidal effusion consists of serous-hemorrhagic fluid accumulation in the suprachoroidal space typically encountered in trauma, surgery, or uveitis scenarios.1,2 It can produce a mass effect simulating tumors2 or be the presenting feature of ocular neoplasms,3 requiring elucidation as to its origin and nature. In this patient, disproportional choroidal exudation in the context of a solid lesion unresponsive to steroid therapy was strongly suggestive of malignancy.The yellow discoloration noted in only 1 eye was unusual and likely reflected a subacute process related to long-standing choroidal fluid buildup.4 Unlike the scleral impregnation with bilateral involvement classically seen in jaundice,5 the ocular coloration changes developed in this patient indicated a local mechanism, mediated by fluid extravasation from the choroidal compartment to the subconjunctival space.4 Therefore, screening for causes of bilirubin elevation, such as liver dysfunction and biliary tract obstruction (choice A), would not be the first step taken.Given the presence of an intraocular mass, primary and secondary neoplasms should be foremost investigated before any treatment consideration. Surgical drainage of the hemorrhagic content (choice B) and management with plaque brachytherapy (choice C) would not be advisable until a final diagnosis was reached because of risks of incomplete disease control and tumor seeding. Based on the characteristics of the lesion on imaging, notably heterogeneous reflectivity, irregular surface, and infiltrative pattern, which typically signal uveal metastasis as opposed to primary melanoma,6 screening for an occult systemic primary cancer should be the first course of action (choice D).Accordingly, the patient underwent full-body imaging studies with computed tomography of the thorax, abdomen, and pelvis. She was identified with a 6 × 9-cm silent mass at the upper left pulmonary lobe with associated mediastinal adenopathy (Figure 2). A fine-needle aspiration biopsy of the lung tumor revealed primary adenocarcinoma. Other metastatic lesions were also identified on the thoracic spine.Computed tomography scan reveals a lobular lung tumor (arrowhead).Metastasis is the most common ocular malignancy in the adult population, affecting the choroid in 85% of patients by hematogenous dissemination.6,7 Metastatic implants are visible on fundoscopy as single or multiple light-yellow solid lesions of variable shape usually with associated subretinal fluid.6 Primary sites have been localized, in order of frequency, to breast, lung, kidney, gastrointestinal tract, and cutaneous melanoma.7 In a large series, uveal metastasis from lung cancer was more prevalent in men (55%) and mainly unifocal (77%) and unilateral (82%).8 Approximately 40% of individuals had the original tumor discovered only after detecting uveal lesions8 similar to what we observed in this patient. Although choroidal effusion may be a nonspecific finding attributable to metastatic systemic neoplasms,3,6 a threshold of suspicion for underlying malignancy is advisable in the face of any intraocular solid growth.The patient was treated with systemic chemotherapy (entrectinib) and external beam radiotherapy to the mediastinum and orbits (20 Gy, 5 fractions). The conjunctival color progressively recovered in the following weeks, whereas the choroidal metastasis and detachment resolved 3 months after radiation. The visual acuity in the left eye remained hand motion."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 65-year-old White patient was referred with a 6-week history of blurry vision, ocular pain, and subconjunctival yellow discoloration in the left eye. Suspicious retinal detachment and intraocular mass were identified, which raised concerns for choroidal melanoma. No symptoms or signs were reported in the right eye. Them ocular history included bilateral cataract surgery 5 years prior. Review of systems yielded negative results, including for ocular trauma, jaundice, and cutaneous melanoma. They had no history of tobacco use.On examination of the left eye, visual acuity was hand motion, and intraocular pressure was 8 mm Hg. The anterior segment showed diffuse conjunctival chemosis of yellow hue, low-grade aqueous reaction, and pseudophakia (Figure 1A). On the fundus evaluation, large pockets of subretinal fluid suggested choroidal effusion based on marked fluid shifting with head position. B-scan ultrasonography confirmed 4-quadrant choroidal hemorrhage and partially mobile serous retinal detachment. B-scans also exhibited a solid lesion from the 6- to 10-o’clock meridians with irregular shape and variable internal reflectivity, measuring 5.8 mm in height. Magnetic resonance imaging was requested to delineate the mass from the surrounding clot (Figure 1B). In the interim, they received oral steroids aiming to contain the abundant exudation and topical atropine for pain, evolving with minimal clinical improvement on these features.Coloration changes in the left eye associated with choroidal hemorrhage and an intraocular solid lesion. B, The arrowhead indicates the lesion and the asterisk, the adjacent clot.\n</clinical_case>\n\n<question>\nA 65-year-old woman was referred with a 6-week history of blurry vision, ocular pain, and subconjunctival yellow discoloration in the left eye. Suspicious retinal detachment and intraocular mass were identified, which raised concerns for choroidal melanoma. No symptoms or signs were reported in the right eye. Her ocular history included bilateral cataract surgery 5 years prior. Review of systems yielded negative results, including for ocular trauma, jaundice, and cutaneous melanoma. She had no history of tobacco use.On examination of the left eye, visual acuity was hand motion, and intraocular pressure was 8 mm Hg. The anterior segment showed diffuse conjunctival chemosis of yellow hue, low-grade aqueous reaction, and pseudophakia (Figure 1A). On the fundus evaluation, large pockets of subretinal fluid suggested choroidal effusion based on marked fluid shifting with head position. B-scan ultrasonography confirmed 4-quadrant choroidal hemorrhage and partially mobile serous retinal detachment. B-scans also exhibited a solid lesion from the 6- to 10-o’clock meridians with irregular shape and variable internal reflectivity, measuring 5.8 mm in height. Magnetic resonance imaging was requested to delineate the mass from the surrounding clot (Figure 1B). In the interim, she received oral steroids aiming to contain the abundant exudation and topical atropine for pain, evolving with minimal clinical improvement on these features.Coloration changes in the left eye associated with choroidal hemorrhage and an intraocular solid lesion. B, The arrowhead indicates the lesion and the asterisk, the adjacent clot. What Would You Do Next?\n</question>\n\n<options>\nA. Rule out an occult systemic neoplasm\nB. Investigate liver dysfunction and biliary tract obstruction\nC. Treat with plaque brachytherapy\nD. Drain the hemorrhage with scleral windows\n</options>\n"
},
{
"role": "assistant",
"content": "A. Rule out an occult systemic neoplasm\nChoroidal effusion consists of serous-hemorrhagic fluid accumulation in the suprachoroidal space typically encountered in trauma, surgery, or uveitis scenarios.1,2 It can produce a mass effect simulating tumors2 or be the presenting feature of ocular neoplasms,3 requiring elucidation as to its origin and nature. In this patient, disproportional choroidal exudation in the context of a solid lesion unresponsive to steroid therapy was strongly suggestive of malignancy.The yellow discoloration noted in only 1 eye was unusual and likely reflected a subacute process related to long-standing choroidal fluid buildup.4 Unlike the scleral impregnation with bilateral involvement classically seen in jaundice,5 the ocular coloration changes developed in this patient indicated a local mechanism, mediated by fluid extravasation from the choroidal compartment to the subconjunctival space.4 Therefore, screening for causes of bilirubin elevation, such as liver dysfunction and biliary tract obstruction (choice A), would not be the first step taken.Given the presence of an intraocular mass, primary and secondary neoplasms should be foremost investigated before any treatment consideration. Surgical drainage of the hemorrhagic content (choice B) and management with plaque brachytherapy (choice C) would not be advisable until a final diagnosis was reached because of risks of incomplete disease control and tumor seeding. Based on the characteristics of the lesion on imaging, notably heterogeneous reflectivity, irregular surface, and infiltrative pattern, which typically signal uveal metastasis as opposed to primary melanoma,6 screening for an occult systemic primary cancer should be the first course of action (choice D).Accordingly, the patient underwent full-body imaging studies with computed tomography of the thorax, abdomen, and pelvis. She was identified with a 6 × 9-cm silent mass at the upper left pulmonary lobe with associated mediastinal adenopathy (Figure 2). A fine-needle aspiration biopsy of the lung tumor revealed primary adenocarcinoma. Other metastatic lesions were also identified on the thoracic spine.Computed tomography scan reveals a lobular lung tumor (arrowhead).Metastasis is the most common ocular malignancy in the adult population, affecting the choroid in 85% of patients by hematogenous dissemination.6,7 Metastatic implants are visible on fundoscopy as single or multiple light-yellow solid lesions of variable shape usually with associated subretinal fluid.6 Primary sites have been localized, in order of frequency, to breast, lung, kidney, gastrointestinal tract, and cutaneous melanoma.7 In a large series, uveal metastasis from lung cancer was more prevalent in men (55%) and mainly unifocal (77%) and unilateral (82%).8 Approximately 40% of individuals had the original tumor discovered only after detecting uveal lesions8 similar to what we observed in this patient. Although choroidal effusion may be a nonspecific finding attributable to metastatic systemic neoplasms,3,6 a threshold of suspicion for underlying malignancy is advisable in the face of any intraocular solid growth.The patient was treated with systemic chemotherapy (entrectinib) and external beam radiotherapy to the mediastinum and orbits (20 Gy, 5 fractions). The conjunctival color progressively recovered in the following weeks, whereas the choroidal metastasis and detachment resolved 3 months after radiation. The visual acuity in the left eye remained hand motion."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 65-year-old Black woman was referred with a 6-week history of blurry vision, ocular pain, and subconjunctival yellow discoloration in the left eye. Suspicious retinal detachment and intraocular mass were identified, which raised concerns for choroidal melanoma. No symptoms or signs were reported in the right eye. Her ocular history included bilateral cataract surgery 5 years prior. Review of systems yielded negative results, including for ocular trauma, jaundice, and cutaneous melanoma. She had no history of tobacco use.On examination of the left eye, visual acuity was hand motion, and intraocular pressure was 8 mm Hg. The anterior segment showed diffuse conjunctival chemosis of yellow hue, low-grade aqueous reaction, and pseudophakia (Figure 1A). On the fundus evaluation, large pockets of subretinal fluid suggested choroidal effusion based on marked fluid shifting with head position. B-scan ultrasonography confirmed 4-quadrant choroidal hemorrhage and partially mobile serous retinal detachment. B-scans also exhibited a solid lesion from the 6- to 10-o’clock meridians with irregular shape and variable internal reflectivity, measuring 5.8 mm in height. Magnetic resonance imaging was requested to delineate the mass from the surrounding clot (Figure 1B). In the interim, she received oral steroids aiming to contain the abundant exudation and topical atropine for pain, evolving with minimal clinical improvement on these features.Coloration changes in the left eye associated with choroidal hemorrhage and an intraocular solid lesion. B, The arrowhead indicates the lesion and the asterisk, the adjacent clot.\n</clinical_case>\n\n<question>\nA 65-year-old woman was referred with a 6-week history of blurry vision, ocular pain, and subconjunctival yellow discoloration in the left eye. Suspicious retinal detachment and intraocular mass were identified, which raised concerns for choroidal melanoma. No symptoms or signs were reported in the right eye. Her ocular history included bilateral cataract surgery 5 years prior. Review of systems yielded negative results, including for ocular trauma, jaundice, and cutaneous melanoma. She had no history of tobacco use.On examination of the left eye, visual acuity was hand motion, and intraocular pressure was 8 mm Hg. The anterior segment showed diffuse conjunctival chemosis of yellow hue, low-grade aqueous reaction, and pseudophakia (Figure 1A). On the fundus evaluation, large pockets of subretinal fluid suggested choroidal effusion based on marked fluid shifting with head position. B-scan ultrasonography confirmed 4-quadrant choroidal hemorrhage and partially mobile serous retinal detachment. B-scans also exhibited a solid lesion from the 6- to 10-o’clock meridians with irregular shape and variable internal reflectivity, measuring 5.8 mm in height. Magnetic resonance imaging was requested to delineate the mass from the surrounding clot (Figure 1B). In the interim, she received oral steroids aiming to contain the abundant exudation and topical atropine for pain, evolving with minimal clinical improvement on these features.Coloration changes in the left eye associated with choroidal hemorrhage and an intraocular solid lesion. B, The arrowhead indicates the lesion and the asterisk, the adjacent clot. What Would You Do Next?\n</question>\n\n<options>\nA. Rule out an occult systemic neoplasm\nB. Investigate liver dysfunction and biliary tract obstruction\nC. Treat with plaque brachytherapy\nD. Drain the hemorrhage with scleral windows\n</options>\n"
},
{
"role": "assistant",
"content": "A. Rule out an occult systemic neoplasm\nChoroidal effusion consists of serous-hemorrhagic fluid accumulation in the suprachoroidal space typically encountered in trauma, surgery, or uveitis scenarios.1,2 It can produce a mass effect simulating tumors2 or be the presenting feature of ocular neoplasms,3 requiring elucidation as to its origin and nature. In this patient, disproportional choroidal exudation in the context of a solid lesion unresponsive to steroid therapy was strongly suggestive of malignancy.The yellow discoloration noted in only 1 eye was unusual and likely reflected a subacute process related to long-standing choroidal fluid buildup.4 Unlike the scleral impregnation with bilateral involvement classically seen in jaundice,5 the ocular coloration changes developed in this patient indicated a local mechanism, mediated by fluid extravasation from the choroidal compartment to the subconjunctival space.4 Therefore, screening for causes of bilirubin elevation, such as liver dysfunction and biliary tract obstruction (choice A), would not be the first step taken.Given the presence of an intraocular mass, primary and secondary neoplasms should be foremost investigated before any treatment consideration. Surgical drainage of the hemorrhagic content (choice B) and management with plaque brachytherapy (choice C) would not be advisable until a final diagnosis was reached because of risks of incomplete disease control and tumor seeding. Based on the characteristics of the lesion on imaging, notably heterogeneous reflectivity, irregular surface, and infiltrative pattern, which typically signal uveal metastasis as opposed to primary melanoma,6 screening for an occult systemic primary cancer should be the first course of action (choice D).Accordingly, the patient underwent full-body imaging studies with computed tomography of the thorax, abdomen, and pelvis. She was identified with a 6 × 9-cm silent mass at the upper left pulmonary lobe with associated mediastinal adenopathy (Figure 2). A fine-needle aspiration biopsy of the lung tumor revealed primary adenocarcinoma. Other metastatic lesions were also identified on the thoracic spine.Computed tomography scan reveals a lobular lung tumor (arrowhead).Metastasis is the most common ocular malignancy in the adult population, affecting the choroid in 85% of patients by hematogenous dissemination.6,7 Metastatic implants are visible on fundoscopy as single or multiple light-yellow solid lesions of variable shape usually with associated subretinal fluid.6 Primary sites have been localized, in order of frequency, to breast, lung, kidney, gastrointestinal tract, and cutaneous melanoma.7 In a large series, uveal metastasis from lung cancer was more prevalent in men (55%) and mainly unifocal (77%) and unilateral (82%).8 Approximately 40% of individuals had the original tumor discovered only after detecting uveal lesions8 similar to what we observed in this patient. Although choroidal effusion may be a nonspecific finding attributable to metastatic systemic neoplasms,3,6 a threshold of suspicion for underlying malignancy is advisable in the face of any intraocular solid growth.The patient was treated with systemic chemotherapy (entrectinib) and external beam radiotherapy to the mediastinum and orbits (20 Gy, 5 fractions). The conjunctival color progressively recovered in the following weeks, whereas the choroidal metastasis and detachment resolved 3 months after radiation. The visual acuity in the left eye remained hand motion."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 65-year-old Black man was referred with a 6-week history of blurry vision, ocular pain, and subconjunctival yellow discoloration in the left eye. Suspicious retinal detachment and intraocular mass were identified, which raised concerns for choroidal melanoma. No symptoms or signs were reported in the right eye. Him ocular history included bilateral cataract surgery 5 years prior. Review of systems yielded negative results, including for ocular trauma, jaundice, and cutaneous melanoma. He had no history of tobacco use.On examination of the left eye, visual acuity was hand motion, and intraocular pressure was 8 mm Hg. The anterior segment showed diffuse conjunctival chemosis of yellow hue, low-grade aqueous reaction, and pseudophakia (Figure 1A). On the fundus evaluation, large pockets of subretinal fluid suggested choroidal effusion based on marked fluid shifting with head position. B-scan ultrasonography confirmed 4-quadrant choroidal hemorrhage and partially mobile serous retinal detachment. B-scans also exhibited a solid lesion from the 6- to 10-o’clock meridians with irregular shape and variable internal reflectivity, measuring 5.8 mm in height. Magnetic resonance imaging was requested to delineate the mass from the surrounding clot (Figure 1B). In the interim, he received oral steroids aiming to contain the abundant exudation and topical atropine for pain, evolving with minimal clinical improvement on these features.Coloration changes in the left eye associated with choroidal hemorrhage and an intraocular solid lesion. B, The arrowhead indicates the lesion and the asterisk, the adjacent clot.\n</clinical_case>\n\n<question>\nA 65-year-old woman was referred with a 6-week history of blurry vision, ocular pain, and subconjunctival yellow discoloration in the left eye. Suspicious retinal detachment and intraocular mass were identified, which raised concerns for choroidal melanoma. No symptoms or signs were reported in the right eye. Her ocular history included bilateral cataract surgery 5 years prior. Review of systems yielded negative results, including for ocular trauma, jaundice, and cutaneous melanoma. She had no history of tobacco use.On examination of the left eye, visual acuity was hand motion, and intraocular pressure was 8 mm Hg. The anterior segment showed diffuse conjunctival chemosis of yellow hue, low-grade aqueous reaction, and pseudophakia (Figure 1A). On the fundus evaluation, large pockets of subretinal fluid suggested choroidal effusion based on marked fluid shifting with head position. B-scan ultrasonography confirmed 4-quadrant choroidal hemorrhage and partially mobile serous retinal detachment. B-scans also exhibited a solid lesion from the 6- to 10-o’clock meridians with irregular shape and variable internal reflectivity, measuring 5.8 mm in height. Magnetic resonance imaging was requested to delineate the mass from the surrounding clot (Figure 1B). In the interim, she received oral steroids aiming to contain the abundant exudation and topical atropine for pain, evolving with minimal clinical improvement on these features.Coloration changes in the left eye associated with choroidal hemorrhage and an intraocular solid lesion. B, The arrowhead indicates the lesion and the asterisk, the adjacent clot. What Would You Do Next?\n</question>\n\n<options>\nA. Rule out an occult systemic neoplasm\nB. Investigate liver dysfunction and biliary tract obstruction\nC. Treat with plaque brachytherapy\nD. Drain the hemorrhage with scleral windows\n</options>\n"
},
{
"role": "assistant",
"content": "A. Rule out an occult systemic neoplasm\nChoroidal effusion consists of serous-hemorrhagic fluid accumulation in the suprachoroidal space typically encountered in trauma, surgery, or uveitis scenarios.1,2 It can produce a mass effect simulating tumors2 or be the presenting feature of ocular neoplasms,3 requiring elucidation as to its origin and nature. In this patient, disproportional choroidal exudation in the context of a solid lesion unresponsive to steroid therapy was strongly suggestive of malignancy.The yellow discoloration noted in only 1 eye was unusual and likely reflected a subacute process related to long-standing choroidal fluid buildup.4 Unlike the scleral impregnation with bilateral involvement classically seen in jaundice,5 the ocular coloration changes developed in this patient indicated a local mechanism, mediated by fluid extravasation from the choroidal compartment to the subconjunctival space.4 Therefore, screening for causes of bilirubin elevation, such as liver dysfunction and biliary tract obstruction (choice A), would not be the first step taken.Given the presence of an intraocular mass, primary and secondary neoplasms should be foremost investigated before any treatment consideration. Surgical drainage of the hemorrhagic content (choice B) and management with plaque brachytherapy (choice C) would not be advisable until a final diagnosis was reached because of risks of incomplete disease control and tumor seeding. Based on the characteristics of the lesion on imaging, notably heterogeneous reflectivity, irregular surface, and infiltrative pattern, which typically signal uveal metastasis as opposed to primary melanoma,6 screening for an occult systemic primary cancer should be the first course of action (choice D).Accordingly, the patient underwent full-body imaging studies with computed tomography of the thorax, abdomen, and pelvis. She was identified with a 6 × 9-cm silent mass at the upper left pulmonary lobe with associated mediastinal adenopathy (Figure 2). A fine-needle aspiration biopsy of the lung tumor revealed primary adenocarcinoma. Other metastatic lesions were also identified on the thoracic spine.Computed tomography scan reveals a lobular lung tumor (arrowhead).Metastasis is the most common ocular malignancy in the adult population, affecting the choroid in 85% of patients by hematogenous dissemination.6,7 Metastatic implants are visible on fundoscopy as single or multiple light-yellow solid lesions of variable shape usually with associated subretinal fluid.6 Primary sites have been localized, in order of frequency, to breast, lung, kidney, gastrointestinal tract, and cutaneous melanoma.7 In a large series, uveal metastasis from lung cancer was more prevalent in men (55%) and mainly unifocal (77%) and unilateral (82%).8 Approximately 40% of individuals had the original tumor discovered only after detecting uveal lesions8 similar to what we observed in this patient. Although choroidal effusion may be a nonspecific finding attributable to metastatic systemic neoplasms,3,6 a threshold of suspicion for underlying malignancy is advisable in the face of any intraocular solid growth.The patient was treated with systemic chemotherapy (entrectinib) and external beam radiotherapy to the mediastinum and orbits (20 Gy, 5 fractions). The conjunctival color progressively recovered in the following weeks, whereas the choroidal metastasis and detachment resolved 3 months after radiation. The visual acuity in the left eye remained hand motion."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 65-year-old Black patient was referred with a 6-week history of blurry vision, ocular pain, and subconjunctival yellow discoloration in the left eye. Suspicious retinal detachment and intraocular mass were identified, which raised concerns for choroidal melanoma. No symptoms or signs were reported in the right eye. Them ocular history included bilateral cataract surgery 5 years prior. Review of systems yielded negative results, including for ocular trauma, jaundice, and cutaneous melanoma. They had no history of tobacco use.On examination of the left eye, visual acuity was hand motion, and intraocular pressure was 8 mm Hg. The anterior segment showed diffuse conjunctival chemosis of yellow hue, low-grade aqueous reaction, and pseudophakia (Figure 1A). On the fundus evaluation, large pockets of subretinal fluid suggested choroidal effusion based on marked fluid shifting with head position. B-scan ultrasonography confirmed 4-quadrant choroidal hemorrhage and partially mobile serous retinal detachment. B-scans also exhibited a solid lesion from the 6- to 10-o’clock meridians with irregular shape and variable internal reflectivity, measuring 5.8 mm in height. Magnetic resonance imaging was requested to delineate the mass from the surrounding clot (Figure 1B). In the interim, they received oral steroids aiming to contain the abundant exudation and topical atropine for pain, evolving with minimal clinical improvement on these features.Coloration changes in the left eye associated with choroidal hemorrhage and an intraocular solid lesion. B, The arrowhead indicates the lesion and the asterisk, the adjacent clot.\n</clinical_case>\n\n<question>\nA 65-year-old woman was referred with a 6-week history of blurry vision, ocular pain, and subconjunctival yellow discoloration in the left eye. Suspicious retinal detachment and intraocular mass were identified, which raised concerns for choroidal melanoma. No symptoms or signs were reported in the right eye. Her ocular history included bilateral cataract surgery 5 years prior. Review of systems yielded negative results, including for ocular trauma, jaundice, and cutaneous melanoma. She had no history of tobacco use.On examination of the left eye, visual acuity was hand motion, and intraocular pressure was 8 mm Hg. The anterior segment showed diffuse conjunctival chemosis of yellow hue, low-grade aqueous reaction, and pseudophakia (Figure 1A). On the fundus evaluation, large pockets of subretinal fluid suggested choroidal effusion based on marked fluid shifting with head position. B-scan ultrasonography confirmed 4-quadrant choroidal hemorrhage and partially mobile serous retinal detachment. B-scans also exhibited a solid lesion from the 6- to 10-o’clock meridians with irregular shape and variable internal reflectivity, measuring 5.8 mm in height. Magnetic resonance imaging was requested to delineate the mass from the surrounding clot (Figure 1B). In the interim, she received oral steroids aiming to contain the abundant exudation and topical atropine for pain, evolving with minimal clinical improvement on these features.Coloration changes in the left eye associated with choroidal hemorrhage and an intraocular solid lesion. B, The arrowhead indicates the lesion and the asterisk, the adjacent clot. What Would You Do Next?\n</question>\n\n<options>\nA. Rule out an occult systemic neoplasm\nB. Investigate liver dysfunction and biliary tract obstruction\nC. Treat with plaque brachytherapy\nD. Drain the hemorrhage with scleral windows\n</options>\n"
},
{
"role": "assistant",
"content": "A. Rule out an occult systemic neoplasm\nChoroidal effusion consists of serous-hemorrhagic fluid accumulation in the suprachoroidal space typically encountered in trauma, surgery, or uveitis scenarios.1,2 It can produce a mass effect simulating tumors2 or be the presenting feature of ocular neoplasms,3 requiring elucidation as to its origin and nature. In this patient, disproportional choroidal exudation in the context of a solid lesion unresponsive to steroid therapy was strongly suggestive of malignancy.The yellow discoloration noted in only 1 eye was unusual and likely reflected a subacute process related to long-standing choroidal fluid buildup.4 Unlike the scleral impregnation with bilateral involvement classically seen in jaundice,5 the ocular coloration changes developed in this patient indicated a local mechanism, mediated by fluid extravasation from the choroidal compartment to the subconjunctival space.4 Therefore, screening for causes of bilirubin elevation, such as liver dysfunction and biliary tract obstruction (choice A), would not be the first step taken.Given the presence of an intraocular mass, primary and secondary neoplasms should be foremost investigated before any treatment consideration. Surgical drainage of the hemorrhagic content (choice B) and management with plaque brachytherapy (choice C) would not be advisable until a final diagnosis was reached because of risks of incomplete disease control and tumor seeding. Based on the characteristics of the lesion on imaging, notably heterogeneous reflectivity, irregular surface, and infiltrative pattern, which typically signal uveal metastasis as opposed to primary melanoma,6 screening for an occult systemic primary cancer should be the first course of action (choice D).Accordingly, the patient underwent full-body imaging studies with computed tomography of the thorax, abdomen, and pelvis. She was identified with a 6 × 9-cm silent mass at the upper left pulmonary lobe with associated mediastinal adenopathy (Figure 2). A fine-needle aspiration biopsy of the lung tumor revealed primary adenocarcinoma. Other metastatic lesions were also identified on the thoracic spine.Computed tomography scan reveals a lobular lung tumor (arrowhead).Metastasis is the most common ocular malignancy in the adult population, affecting the choroid in 85% of patients by hematogenous dissemination.6,7 Metastatic implants are visible on fundoscopy as single or multiple light-yellow solid lesions of variable shape usually with associated subretinal fluid.6 Primary sites have been localized, in order of frequency, to breast, lung, kidney, gastrointestinal tract, and cutaneous melanoma.7 In a large series, uveal metastasis from lung cancer was more prevalent in men (55%) and mainly unifocal (77%) and unilateral (82%).8 Approximately 40% of individuals had the original tumor discovered only after detecting uveal lesions8 similar to what we observed in this patient. Although choroidal effusion may be a nonspecific finding attributable to metastatic systemic neoplasms,3,6 a threshold of suspicion for underlying malignancy is advisable in the face of any intraocular solid growth.The patient was treated with systemic chemotherapy (entrectinib) and external beam radiotherapy to the mediastinum and orbits (20 Gy, 5 fractions). The conjunctival color progressively recovered in the following weeks, whereas the choroidal metastasis and detachment resolved 3 months after radiation. The visual acuity in the left eye remained hand motion."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 65-year-old Asian woman was referred with a 6-week history of blurry vision, ocular pain, and subconjunctival yellow discoloration in the left eye. Suspicious retinal detachment and intraocular mass were identified, which raised concerns for choroidal melanoma. No symptoms or signs were reported in the right eye. Her ocular history included bilateral cataract surgery 5 years prior. Review of systems yielded negative results, including for ocular trauma, jaundice, and cutaneous melanoma. She had no history of tobacco use.On examination of the left eye, visual acuity was hand motion, and intraocular pressure was 8 mm Hg. The anterior segment showed diffuse conjunctival chemosis of yellow hue, low-grade aqueous reaction, and pseudophakia (Figure 1A). On the fundus evaluation, large pockets of subretinal fluid suggested choroidal effusion based on marked fluid shifting with head position. B-scan ultrasonography confirmed 4-quadrant choroidal hemorrhage and partially mobile serous retinal detachment. B-scans also exhibited a solid lesion from the 6- to 10-o’clock meridians with irregular shape and variable internal reflectivity, measuring 5.8 mm in height. Magnetic resonance imaging was requested to delineate the mass from the surrounding clot (Figure 1B). In the interim, she received oral steroids aiming to contain the abundant exudation and topical atropine for pain, evolving with minimal clinical improvement on these features.Coloration changes in the left eye associated with choroidal hemorrhage and an intraocular solid lesion. B, The arrowhead indicates the lesion and the asterisk, the adjacent clot.\n</clinical_case>\n\n<question>\nA 65-year-old woman was referred with a 6-week history of blurry vision, ocular pain, and subconjunctival yellow discoloration in the left eye. Suspicious retinal detachment and intraocular mass were identified, which raised concerns for choroidal melanoma. No symptoms or signs were reported in the right eye. Her ocular history included bilateral cataract surgery 5 years prior. Review of systems yielded negative results, including for ocular trauma, jaundice, and cutaneous melanoma. She had no history of tobacco use.On examination of the left eye, visual acuity was hand motion, and intraocular pressure was 8 mm Hg. The anterior segment showed diffuse conjunctival chemosis of yellow hue, low-grade aqueous reaction, and pseudophakia (Figure 1A). On the fundus evaluation, large pockets of subretinal fluid suggested choroidal effusion based on marked fluid shifting with head position. B-scan ultrasonography confirmed 4-quadrant choroidal hemorrhage and partially mobile serous retinal detachment. B-scans also exhibited a solid lesion from the 6- to 10-o’clock meridians with irregular shape and variable internal reflectivity, measuring 5.8 mm in height. Magnetic resonance imaging was requested to delineate the mass from the surrounding clot (Figure 1B). In the interim, she received oral steroids aiming to contain the abundant exudation and topical atropine for pain, evolving with minimal clinical improvement on these features.Coloration changes in the left eye associated with choroidal hemorrhage and an intraocular solid lesion. B, The arrowhead indicates the lesion and the asterisk, the adjacent clot. What Would You Do Next?\n</question>\n\n<options>\nA. Rule out an occult systemic neoplasm\nB. Investigate liver dysfunction and biliary tract obstruction\nC. Treat with plaque brachytherapy\nD. Drain the hemorrhage with scleral windows\n</options>\n"
},
{
"role": "assistant",
"content": "A. Rule out an occult systemic neoplasm\nChoroidal effusion consists of serous-hemorrhagic fluid accumulation in the suprachoroidal space typically encountered in trauma, surgery, or uveitis scenarios.1,2 It can produce a mass effect simulating tumors2 or be the presenting feature of ocular neoplasms,3 requiring elucidation as to its origin and nature. In this patient, disproportional choroidal exudation in the context of a solid lesion unresponsive to steroid therapy was strongly suggestive of malignancy.The yellow discoloration noted in only 1 eye was unusual and likely reflected a subacute process related to long-standing choroidal fluid buildup.4 Unlike the scleral impregnation with bilateral involvement classically seen in jaundice,5 the ocular coloration changes developed in this patient indicated a local mechanism, mediated by fluid extravasation from the choroidal compartment to the subconjunctival space.4 Therefore, screening for causes of bilirubin elevation, such as liver dysfunction and biliary tract obstruction (choice A), would not be the first step taken.Given the presence of an intraocular mass, primary and secondary neoplasms should be foremost investigated before any treatment consideration. Surgical drainage of the hemorrhagic content (choice B) and management with plaque brachytherapy (choice C) would not be advisable until a final diagnosis was reached because of risks of incomplete disease control and tumor seeding. Based on the characteristics of the lesion on imaging, notably heterogeneous reflectivity, irregular surface, and infiltrative pattern, which typically signal uveal metastasis as opposed to primary melanoma,6 screening for an occult systemic primary cancer should be the first course of action (choice D).Accordingly, the patient underwent full-body imaging studies with computed tomography of the thorax, abdomen, and pelvis. She was identified with a 6 × 9-cm silent mass at the upper left pulmonary lobe with associated mediastinal adenopathy (Figure 2). A fine-needle aspiration biopsy of the lung tumor revealed primary adenocarcinoma. Other metastatic lesions were also identified on the thoracic spine.Computed tomography scan reveals a lobular lung tumor (arrowhead).Metastasis is the most common ocular malignancy in the adult population, affecting the choroid in 85% of patients by hematogenous dissemination.6,7 Metastatic implants are visible on fundoscopy as single or multiple light-yellow solid lesions of variable shape usually with associated subretinal fluid.6 Primary sites have been localized, in order of frequency, to breast, lung, kidney, gastrointestinal tract, and cutaneous melanoma.7 In a large series, uveal metastasis from lung cancer was more prevalent in men (55%) and mainly unifocal (77%) and unilateral (82%).8 Approximately 40% of individuals had the original tumor discovered only after detecting uveal lesions8 similar to what we observed in this patient. Although choroidal effusion may be a nonspecific finding attributable to metastatic systemic neoplasms,3,6 a threshold of suspicion for underlying malignancy is advisable in the face of any intraocular solid growth.The patient was treated with systemic chemotherapy (entrectinib) and external beam radiotherapy to the mediastinum and orbits (20 Gy, 5 fractions). The conjunctival color progressively recovered in the following weeks, whereas the choroidal metastasis and detachment resolved 3 months after radiation. The visual acuity in the left eye remained hand motion."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 65-year-old Asian man was referred with a 6-week history of blurry vision, ocular pain, and subconjunctival yellow discoloration in the left eye. Suspicious retinal detachment and intraocular mass were identified, which raised concerns for choroidal melanoma. No symptoms or signs were reported in the right eye. Him ocular history included bilateral cataract surgery 5 years prior. Review of systems yielded negative results, including for ocular trauma, jaundice, and cutaneous melanoma. He had no history of tobacco use.On examination of the left eye, visual acuity was hand motion, and intraocular pressure was 8 mm Hg. The anterior segment showed diffuse conjunctival chemosis of yellow hue, low-grade aqueous reaction, and pseudophakia (Figure 1A). On the fundus evaluation, large pockets of subretinal fluid suggested choroidal effusion based on marked fluid shifting with head position. B-scan ultrasonography confirmed 4-quadrant choroidal hemorrhage and partially mobile serous retinal detachment. B-scans also exhibited a solid lesion from the 6- to 10-o’clock meridians with irregular shape and variable internal reflectivity, measuring 5.8 mm in height. Magnetic resonance imaging was requested to delineate the mass from the surrounding clot (Figure 1B). In the interim, he received oral steroids aiming to contain the abundant exudation and topical atropine for pain, evolving with minimal clinical improvement on these features.Coloration changes in the left eye associated with choroidal hemorrhage and an intraocular solid lesion. B, The arrowhead indicates the lesion and the asterisk, the adjacent clot.\n</clinical_case>\n\n<question>\nA 65-year-old woman was referred with a 6-week history of blurry vision, ocular pain, and subconjunctival yellow discoloration in the left eye. Suspicious retinal detachment and intraocular mass were identified, which raised concerns for choroidal melanoma. No symptoms or signs were reported in the right eye. Her ocular history included bilateral cataract surgery 5 years prior. Review of systems yielded negative results, including for ocular trauma, jaundice, and cutaneous melanoma. She had no history of tobacco use.On examination of the left eye, visual acuity was hand motion, and intraocular pressure was 8 mm Hg. The anterior segment showed diffuse conjunctival chemosis of yellow hue, low-grade aqueous reaction, and pseudophakia (Figure 1A). On the fundus evaluation, large pockets of subretinal fluid suggested choroidal effusion based on marked fluid shifting with head position. B-scan ultrasonography confirmed 4-quadrant choroidal hemorrhage and partially mobile serous retinal detachment. B-scans also exhibited a solid lesion from the 6- to 10-o’clock meridians with irregular shape and variable internal reflectivity, measuring 5.8 mm in height. Magnetic resonance imaging was requested to delineate the mass from the surrounding clot (Figure 1B). In the interim, she received oral steroids aiming to contain the abundant exudation and topical atropine for pain, evolving with minimal clinical improvement on these features.Coloration changes in the left eye associated with choroidal hemorrhage and an intraocular solid lesion. B, The arrowhead indicates the lesion and the asterisk, the adjacent clot. What Would You Do Next?\n</question>\n\n<options>\nA. Rule out an occult systemic neoplasm\nB. Investigate liver dysfunction and biliary tract obstruction\nC. Treat with plaque brachytherapy\nD. Drain the hemorrhage with scleral windows\n</options>\n"
},
{
"role": "assistant",
"content": "A. Rule out an occult systemic neoplasm\nChoroidal effusion consists of serous-hemorrhagic fluid accumulation in the suprachoroidal space typically encountered in trauma, surgery, or uveitis scenarios.1,2 It can produce a mass effect simulating tumors2 or be the presenting feature of ocular neoplasms,3 requiring elucidation as to its origin and nature. In this patient, disproportional choroidal exudation in the context of a solid lesion unresponsive to steroid therapy was strongly suggestive of malignancy.The yellow discoloration noted in only 1 eye was unusual and likely reflected a subacute process related to long-standing choroidal fluid buildup.4 Unlike the scleral impregnation with bilateral involvement classically seen in jaundice,5 the ocular coloration changes developed in this patient indicated a local mechanism, mediated by fluid extravasation from the choroidal compartment to the subconjunctival space.4 Therefore, screening for causes of bilirubin elevation, such as liver dysfunction and biliary tract obstruction (choice A), would not be the first step taken.Given the presence of an intraocular mass, primary and secondary neoplasms should be foremost investigated before any treatment consideration. Surgical drainage of the hemorrhagic content (choice B) and management with plaque brachytherapy (choice C) would not be advisable until a final diagnosis was reached because of risks of incomplete disease control and tumor seeding. Based on the characteristics of the lesion on imaging, notably heterogeneous reflectivity, irregular surface, and infiltrative pattern, which typically signal uveal metastasis as opposed to primary melanoma,6 screening for an occult systemic primary cancer should be the first course of action (choice D).Accordingly, the patient underwent full-body imaging studies with computed tomography of the thorax, abdomen, and pelvis. She was identified with a 6 × 9-cm silent mass at the upper left pulmonary lobe with associated mediastinal adenopathy (Figure 2). A fine-needle aspiration biopsy of the lung tumor revealed primary adenocarcinoma. Other metastatic lesions were also identified on the thoracic spine.Computed tomography scan reveals a lobular lung tumor (arrowhead).Metastasis is the most common ocular malignancy in the adult population, affecting the choroid in 85% of patients by hematogenous dissemination.6,7 Metastatic implants are visible on fundoscopy as single or multiple light-yellow solid lesions of variable shape usually with associated subretinal fluid.6 Primary sites have been localized, in order of frequency, to breast, lung, kidney, gastrointestinal tract, and cutaneous melanoma.7 In a large series, uveal metastasis from lung cancer was more prevalent in men (55%) and mainly unifocal (77%) and unilateral (82%).8 Approximately 40% of individuals had the original tumor discovered only after detecting uveal lesions8 similar to what we observed in this patient. Although choroidal effusion may be a nonspecific finding attributable to metastatic systemic neoplasms,3,6 a threshold of suspicion for underlying malignancy is advisable in the face of any intraocular solid growth.The patient was treated with systemic chemotherapy (entrectinib) and external beam radiotherapy to the mediastinum and orbits (20 Gy, 5 fractions). The conjunctival color progressively recovered in the following weeks, whereas the choroidal metastasis and detachment resolved 3 months after radiation. The visual acuity in the left eye remained hand motion."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 65-year-old Asian patient was referred with a 6-week history of blurry vision, ocular pain, and subconjunctival yellow discoloration in the left eye. Suspicious retinal detachment and intraocular mass were identified, which raised concerns for choroidal melanoma. No symptoms or signs were reported in the right eye. Them ocular history included bilateral cataract surgery 5 years prior. Review of systems yielded negative results, including for ocular trauma, jaundice, and cutaneous melanoma. They had no history of tobacco use.On examination of the left eye, visual acuity was hand motion, and intraocular pressure was 8 mm Hg. The anterior segment showed diffuse conjunctival chemosis of yellow hue, low-grade aqueous reaction, and pseudophakia (Figure 1A). On the fundus evaluation, large pockets of subretinal fluid suggested choroidal effusion based on marked fluid shifting with head position. B-scan ultrasonography confirmed 4-quadrant choroidal hemorrhage and partially mobile serous retinal detachment. B-scans also exhibited a solid lesion from the 6- to 10-o’clock meridians with irregular shape and variable internal reflectivity, measuring 5.8 mm in height. Magnetic resonance imaging was requested to delineate the mass from the surrounding clot (Figure 1B). In the interim, they received oral steroids aiming to contain the abundant exudation and topical atropine for pain, evolving with minimal clinical improvement on these features.Coloration changes in the left eye associated with choroidal hemorrhage and an intraocular solid lesion. B, The arrowhead indicates the lesion and the asterisk, the adjacent clot.\n</clinical_case>\n\n<question>\nA 65-year-old woman was referred with a 6-week history of blurry vision, ocular pain, and subconjunctival yellow discoloration in the left eye. Suspicious retinal detachment and intraocular mass were identified, which raised concerns for choroidal melanoma. No symptoms or signs were reported in the right eye. Her ocular history included bilateral cataract surgery 5 years prior. Review of systems yielded negative results, including for ocular trauma, jaundice, and cutaneous melanoma. She had no history of tobacco use.On examination of the left eye, visual acuity was hand motion, and intraocular pressure was 8 mm Hg. The anterior segment showed diffuse conjunctival chemosis of yellow hue, low-grade aqueous reaction, and pseudophakia (Figure 1A). On the fundus evaluation, large pockets of subretinal fluid suggested choroidal effusion based on marked fluid shifting with head position. B-scan ultrasonography confirmed 4-quadrant choroidal hemorrhage and partially mobile serous retinal detachment. B-scans also exhibited a solid lesion from the 6- to 10-o’clock meridians with irregular shape and variable internal reflectivity, measuring 5.8 mm in height. Magnetic resonance imaging was requested to delineate the mass from the surrounding clot (Figure 1B). In the interim, she received oral steroids aiming to contain the abundant exudation and topical atropine for pain, evolving with minimal clinical improvement on these features.Coloration changes in the left eye associated with choroidal hemorrhage and an intraocular solid lesion. B, The arrowhead indicates the lesion and the asterisk, the adjacent clot. What Would You Do Next?\n</question>\n\n<options>\nA. Rule out an occult systemic neoplasm\nB. Investigate liver dysfunction and biliary tract obstruction\nC. Treat with plaque brachytherapy\nD. Drain the hemorrhage with scleral windows\n</options>\n"
},
{
"role": "assistant",
"content": "A. Rule out an occult systemic neoplasm\nChoroidal effusion consists of serous-hemorrhagic fluid accumulation in the suprachoroidal space typically encountered in trauma, surgery, or uveitis scenarios.1,2 It can produce a mass effect simulating tumors2 or be the presenting feature of ocular neoplasms,3 requiring elucidation as to its origin and nature. In this patient, disproportional choroidal exudation in the context of a solid lesion unresponsive to steroid therapy was strongly suggestive of malignancy.The yellow discoloration noted in only 1 eye was unusual and likely reflected a subacute process related to long-standing choroidal fluid buildup.4 Unlike the scleral impregnation with bilateral involvement classically seen in jaundice,5 the ocular coloration changes developed in this patient indicated a local mechanism, mediated by fluid extravasation from the choroidal compartment to the subconjunctival space.4 Therefore, screening for causes of bilirubin elevation, such as liver dysfunction and biliary tract obstruction (choice A), would not be the first step taken.Given the presence of an intraocular mass, primary and secondary neoplasms should be foremost investigated before any treatment consideration. Surgical drainage of the hemorrhagic content (choice B) and management with plaque brachytherapy (choice C) would not be advisable until a final diagnosis was reached because of risks of incomplete disease control and tumor seeding. Based on the characteristics of the lesion on imaging, notably heterogeneous reflectivity, irregular surface, and infiltrative pattern, which typically signal uveal metastasis as opposed to primary melanoma,6 screening for an occult systemic primary cancer should be the first course of action (choice D).Accordingly, the patient underwent full-body imaging studies with computed tomography of the thorax, abdomen, and pelvis. She was identified with a 6 × 9-cm silent mass at the upper left pulmonary lobe with associated mediastinal adenopathy (Figure 2). A fine-needle aspiration biopsy of the lung tumor revealed primary adenocarcinoma. Other metastatic lesions were also identified on the thoracic spine.Computed tomography scan reveals a lobular lung tumor (arrowhead).Metastasis is the most common ocular malignancy in the adult population, affecting the choroid in 85% of patients by hematogenous dissemination.6,7 Metastatic implants are visible on fundoscopy as single or multiple light-yellow solid lesions of variable shape usually with associated subretinal fluid.6 Primary sites have been localized, in order of frequency, to breast, lung, kidney, gastrointestinal tract, and cutaneous melanoma.7 In a large series, uveal metastasis from lung cancer was more prevalent in men (55%) and mainly unifocal (77%) and unilateral (82%).8 Approximately 40% of individuals had the original tumor discovered only after detecting uveal lesions8 similar to what we observed in this patient. Although choroidal effusion may be a nonspecific finding attributable to metastatic systemic neoplasms,3,6 a threshold of suspicion for underlying malignancy is advisable in the face of any intraocular solid growth.The patient was treated with systemic chemotherapy (entrectinib) and external beam radiotherapy to the mediastinum and orbits (20 Gy, 5 fractions). The conjunctival color progressively recovered in the following weeks, whereas the choroidal metastasis and detachment resolved 3 months after radiation. The visual acuity in the left eye remained hand motion."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 65-year-old Hispanic woman was referred with a 6-week history of blurry vision, ocular pain, and subconjunctival yellow discoloration in the left eye. Suspicious retinal detachment and intraocular mass were identified, which raised concerns for choroidal melanoma. No symptoms or signs were reported in the right eye. Her ocular history included bilateral cataract surgery 5 years prior. Review of systems yielded negative results, including for ocular trauma, jaundice, and cutaneous melanoma. She had no history of tobacco use.On examination of the left eye, visual acuity was hand motion, and intraocular pressure was 8 mm Hg. The anterior segment showed diffuse conjunctival chemosis of yellow hue, low-grade aqueous reaction, and pseudophakia (Figure 1A). On the fundus evaluation, large pockets of subretinal fluid suggested choroidal effusion based on marked fluid shifting with head position. B-scan ultrasonography confirmed 4-quadrant choroidal hemorrhage and partially mobile serous retinal detachment. B-scans also exhibited a solid lesion from the 6- to 10-o’clock meridians with irregular shape and variable internal reflectivity, measuring 5.8 mm in height. Magnetic resonance imaging was requested to delineate the mass from the surrounding clot (Figure 1B). In the interim, she received oral steroids aiming to contain the abundant exudation and topical atropine for pain, evolving with minimal clinical improvement on these features.Coloration changes in the left eye associated with choroidal hemorrhage and an intraocular solid lesion. B, The arrowhead indicates the lesion and the asterisk, the adjacent clot.\n</clinical_case>\n\n<question>\nA 65-year-old woman was referred with a 6-week history of blurry vision, ocular pain, and subconjunctival yellow discoloration in the left eye. Suspicious retinal detachment and intraocular mass were identified, which raised concerns for choroidal melanoma. No symptoms or signs were reported in the right eye. Her ocular history included bilateral cataract surgery 5 years prior. Review of systems yielded negative results, including for ocular trauma, jaundice, and cutaneous melanoma. She had no history of tobacco use.On examination of the left eye, visual acuity was hand motion, and intraocular pressure was 8 mm Hg. The anterior segment showed diffuse conjunctival chemosis of yellow hue, low-grade aqueous reaction, and pseudophakia (Figure 1A). On the fundus evaluation, large pockets of subretinal fluid suggested choroidal effusion based on marked fluid shifting with head position. B-scan ultrasonography confirmed 4-quadrant choroidal hemorrhage and partially mobile serous retinal detachment. B-scans also exhibited a solid lesion from the 6- to 10-o’clock meridians with irregular shape and variable internal reflectivity, measuring 5.8 mm in height. Magnetic resonance imaging was requested to delineate the mass from the surrounding clot (Figure 1B). In the interim, she received oral steroids aiming to contain the abundant exudation and topical atropine for pain, evolving with minimal clinical improvement on these features.Coloration changes in the left eye associated with choroidal hemorrhage and an intraocular solid lesion. B, The arrowhead indicates the lesion and the asterisk, the adjacent clot. What Would You Do Next?\n</question>\n\n<options>\nA. Rule out an occult systemic neoplasm\nB. Investigate liver dysfunction and biliary tract obstruction\nC. Treat with plaque brachytherapy\nD. Drain the hemorrhage with scleral windows\n</options>\n"
},
{
"role": "assistant",
"content": "A. Rule out an occult systemic neoplasm\nChoroidal effusion consists of serous-hemorrhagic fluid accumulation in the suprachoroidal space typically encountered in trauma, surgery, or uveitis scenarios.1,2 It can produce a mass effect simulating tumors2 or be the presenting feature of ocular neoplasms,3 requiring elucidation as to its origin and nature. In this patient, disproportional choroidal exudation in the context of a solid lesion unresponsive to steroid therapy was strongly suggestive of malignancy.The yellow discoloration noted in only 1 eye was unusual and likely reflected a subacute process related to long-standing choroidal fluid buildup.4 Unlike the scleral impregnation with bilateral involvement classically seen in jaundice,5 the ocular coloration changes developed in this patient indicated a local mechanism, mediated by fluid extravasation from the choroidal compartment to the subconjunctival space.4 Therefore, screening for causes of bilirubin elevation, such as liver dysfunction and biliary tract obstruction (choice A), would not be the first step taken.Given the presence of an intraocular mass, primary and secondary neoplasms should be foremost investigated before any treatment consideration. Surgical drainage of the hemorrhagic content (choice B) and management with plaque brachytherapy (choice C) would not be advisable until a final diagnosis was reached because of risks of incomplete disease control and tumor seeding. Based on the characteristics of the lesion on imaging, notably heterogeneous reflectivity, irregular surface, and infiltrative pattern, which typically signal uveal metastasis as opposed to primary melanoma,6 screening for an occult systemic primary cancer should be the first course of action (choice D).Accordingly, the patient underwent full-body imaging studies with computed tomography of the thorax, abdomen, and pelvis. She was identified with a 6 × 9-cm silent mass at the upper left pulmonary lobe with associated mediastinal adenopathy (Figure 2). A fine-needle aspiration biopsy of the lung tumor revealed primary adenocarcinoma. Other metastatic lesions were also identified on the thoracic spine.Computed tomography scan reveals a lobular lung tumor (arrowhead).Metastasis is the most common ocular malignancy in the adult population, affecting the choroid in 85% of patients by hematogenous dissemination.6,7 Metastatic implants are visible on fundoscopy as single or multiple light-yellow solid lesions of variable shape usually with associated subretinal fluid.6 Primary sites have been localized, in order of frequency, to breast, lung, kidney, gastrointestinal tract, and cutaneous melanoma.7 In a large series, uveal metastasis from lung cancer was more prevalent in men (55%) and mainly unifocal (77%) and unilateral (82%).8 Approximately 40% of individuals had the original tumor discovered only after detecting uveal lesions8 similar to what we observed in this patient. Although choroidal effusion may be a nonspecific finding attributable to metastatic systemic neoplasms,3,6 a threshold of suspicion for underlying malignancy is advisable in the face of any intraocular solid growth.The patient was treated with systemic chemotherapy (entrectinib) and external beam radiotherapy to the mediastinum and orbits (20 Gy, 5 fractions). The conjunctival color progressively recovered in the following weeks, whereas the choroidal metastasis and detachment resolved 3 months after radiation. The visual acuity in the left eye remained hand motion."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 65-year-old Hispanic man was referred with a 6-week history of blurry vision, ocular pain, and subconjunctival yellow discoloration in the left eye. Suspicious retinal detachment and intraocular mass were identified, which raised concerns for choroidal melanoma. No symptoms or signs were reported in the right eye. Him ocular history included bilateral cataract surgery 5 years prior. Review of systems yielded negative results, including for ocular trauma, jaundice, and cutaneous melanoma. He had no history of tobacco use.On examination of the left eye, visual acuity was hand motion, and intraocular pressure was 8 mm Hg. The anterior segment showed diffuse conjunctival chemosis of yellow hue, low-grade aqueous reaction, and pseudophakia (Figure 1A). On the fundus evaluation, large pockets of subretinal fluid suggested choroidal effusion based on marked fluid shifting with head position. B-scan ultrasonography confirmed 4-quadrant choroidal hemorrhage and partially mobile serous retinal detachment. B-scans also exhibited a solid lesion from the 6- to 10-o’clock meridians with irregular shape and variable internal reflectivity, measuring 5.8 mm in height. Magnetic resonance imaging was requested to delineate the mass from the surrounding clot (Figure 1B). In the interim, he received oral steroids aiming to contain the abundant exudation and topical atropine for pain, evolving with minimal clinical improvement on these features.Coloration changes in the left eye associated with choroidal hemorrhage and an intraocular solid lesion. B, The arrowhead indicates the lesion and the asterisk, the adjacent clot.\n</clinical_case>\n\n<question>\nA 65-year-old woman was referred with a 6-week history of blurry vision, ocular pain, and subconjunctival yellow discoloration in the left eye. Suspicious retinal detachment and intraocular mass were identified, which raised concerns for choroidal melanoma. No symptoms or signs were reported in the right eye. Her ocular history included bilateral cataract surgery 5 years prior. Review of systems yielded negative results, including for ocular trauma, jaundice, and cutaneous melanoma. She had no history of tobacco use.On examination of the left eye, visual acuity was hand motion, and intraocular pressure was 8 mm Hg. The anterior segment showed diffuse conjunctival chemosis of yellow hue, low-grade aqueous reaction, and pseudophakia (Figure 1A). On the fundus evaluation, large pockets of subretinal fluid suggested choroidal effusion based on marked fluid shifting with head position. B-scan ultrasonography confirmed 4-quadrant choroidal hemorrhage and partially mobile serous retinal detachment. B-scans also exhibited a solid lesion from the 6- to 10-o’clock meridians with irregular shape and variable internal reflectivity, measuring 5.8 mm in height. Magnetic resonance imaging was requested to delineate the mass from the surrounding clot (Figure 1B). In the interim, she received oral steroids aiming to contain the abundant exudation and topical atropine for pain, evolving with minimal clinical improvement on these features.Coloration changes in the left eye associated with choroidal hemorrhage and an intraocular solid lesion. B, The arrowhead indicates the lesion and the asterisk, the adjacent clot. What Would You Do Next?\n</question>\n\n<options>\nA. Rule out an occult systemic neoplasm\nB. Investigate liver dysfunction and biliary tract obstruction\nC. Treat with plaque brachytherapy\nD. Drain the hemorrhage with scleral windows\n</options>\n"
},
{
"role": "assistant",
"content": "A. Rule out an occult systemic neoplasm\nChoroidal effusion consists of serous-hemorrhagic fluid accumulation in the suprachoroidal space typically encountered in trauma, surgery, or uveitis scenarios.1,2 It can produce a mass effect simulating tumors2 or be the presenting feature of ocular neoplasms,3 requiring elucidation as to its origin and nature. In this patient, disproportional choroidal exudation in the context of a solid lesion unresponsive to steroid therapy was strongly suggestive of malignancy.The yellow discoloration noted in only 1 eye was unusual and likely reflected a subacute process related to long-standing choroidal fluid buildup.4 Unlike the scleral impregnation with bilateral involvement classically seen in jaundice,5 the ocular coloration changes developed in this patient indicated a local mechanism, mediated by fluid extravasation from the choroidal compartment to the subconjunctival space.4 Therefore, screening for causes of bilirubin elevation, such as liver dysfunction and biliary tract obstruction (choice A), would not be the first step taken.Given the presence of an intraocular mass, primary and secondary neoplasms should be foremost investigated before any treatment consideration. Surgical drainage of the hemorrhagic content (choice B) and management with plaque brachytherapy (choice C) would not be advisable until a final diagnosis was reached because of risks of incomplete disease control and tumor seeding. Based on the characteristics of the lesion on imaging, notably heterogeneous reflectivity, irregular surface, and infiltrative pattern, which typically signal uveal metastasis as opposed to primary melanoma,6 screening for an occult systemic primary cancer should be the first course of action (choice D).Accordingly, the patient underwent full-body imaging studies with computed tomography of the thorax, abdomen, and pelvis. She was identified with a 6 × 9-cm silent mass at the upper left pulmonary lobe with associated mediastinal adenopathy (Figure 2). A fine-needle aspiration biopsy of the lung tumor revealed primary adenocarcinoma. Other metastatic lesions were also identified on the thoracic spine.Computed tomography scan reveals a lobular lung tumor (arrowhead).Metastasis is the most common ocular malignancy in the adult population, affecting the choroid in 85% of patients by hematogenous dissemination.6,7 Metastatic implants are visible on fundoscopy as single or multiple light-yellow solid lesions of variable shape usually with associated subretinal fluid.6 Primary sites have been localized, in order of frequency, to breast, lung, kidney, gastrointestinal tract, and cutaneous melanoma.7 In a large series, uveal metastasis from lung cancer was more prevalent in men (55%) and mainly unifocal (77%) and unilateral (82%).8 Approximately 40% of individuals had the original tumor discovered only after detecting uveal lesions8 similar to what we observed in this patient. Although choroidal effusion may be a nonspecific finding attributable to metastatic systemic neoplasms,3,6 a threshold of suspicion for underlying malignancy is advisable in the face of any intraocular solid growth.The patient was treated with systemic chemotherapy (entrectinib) and external beam radiotherapy to the mediastinum and orbits (20 Gy, 5 fractions). The conjunctival color progressively recovered in the following weeks, whereas the choroidal metastasis and detachment resolved 3 months after radiation. The visual acuity in the left eye remained hand motion."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 65-year-old Hispanic patient was referred with a 6-week history of blurry vision, ocular pain, and subconjunctival yellow discoloration in the left eye. Suspicious retinal detachment and intraocular mass were identified, which raised concerns for choroidal melanoma. No symptoms or signs were reported in the right eye. Them ocular history included bilateral cataract surgery 5 years prior. Review of systems yielded negative results, including for ocular trauma, jaundice, and cutaneous melanoma. They had no history of tobacco use.On examination of the left eye, visual acuity was hand motion, and intraocular pressure was 8 mm Hg. The anterior segment showed diffuse conjunctival chemosis of yellow hue, low-grade aqueous reaction, and pseudophakia (Figure 1A). On the fundus evaluation, large pockets of subretinal fluid suggested choroidal effusion based on marked fluid shifting with head position. B-scan ultrasonography confirmed 4-quadrant choroidal hemorrhage and partially mobile serous retinal detachment. B-scans also exhibited a solid lesion from the 6- to 10-o’clock meridians with irregular shape and variable internal reflectivity, measuring 5.8 mm in height. Magnetic resonance imaging was requested to delineate the mass from the surrounding clot (Figure 1B). In the interim, they received oral steroids aiming to contain the abundant exudation and topical atropine for pain, evolving with minimal clinical improvement on these features.Coloration changes in the left eye associated with choroidal hemorrhage and an intraocular solid lesion. B, The arrowhead indicates the lesion and the asterisk, the adjacent clot.\n</clinical_case>\n\n<question>\nA 65-year-old woman was referred with a 6-week history of blurry vision, ocular pain, and subconjunctival yellow discoloration in the left eye. Suspicious retinal detachment and intraocular mass were identified, which raised concerns for choroidal melanoma. No symptoms or signs were reported in the right eye. Her ocular history included bilateral cataract surgery 5 years prior. Review of systems yielded negative results, including for ocular trauma, jaundice, and cutaneous melanoma. She had no history of tobacco use.On examination of the left eye, visual acuity was hand motion, and intraocular pressure was 8 mm Hg. The anterior segment showed diffuse conjunctival chemosis of yellow hue, low-grade aqueous reaction, and pseudophakia (Figure 1A). On the fundus evaluation, large pockets of subretinal fluid suggested choroidal effusion based on marked fluid shifting with head position. B-scan ultrasonography confirmed 4-quadrant choroidal hemorrhage and partially mobile serous retinal detachment. B-scans also exhibited a solid lesion from the 6- to 10-o’clock meridians with irregular shape and variable internal reflectivity, measuring 5.8 mm in height. Magnetic resonance imaging was requested to delineate the mass from the surrounding clot (Figure 1B). In the interim, she received oral steroids aiming to contain the abundant exudation and topical atropine for pain, evolving with minimal clinical improvement on these features.Coloration changes in the left eye associated with choroidal hemorrhage and an intraocular solid lesion. B, The arrowhead indicates the lesion and the asterisk, the adjacent clot. What Would You Do Next?\n</question>\n\n<options>\nA. Rule out an occult systemic neoplasm\nB. Investigate liver dysfunction and biliary tract obstruction\nC. Treat with plaque brachytherapy\nD. Drain the hemorrhage with scleral windows\n</options>\n"
},
{
"role": "assistant",
"content": "A. Rule out an occult systemic neoplasm\nChoroidal effusion consists of serous-hemorrhagic fluid accumulation in the suprachoroidal space typically encountered in trauma, surgery, or uveitis scenarios.1,2 It can produce a mass effect simulating tumors2 or be the presenting feature of ocular neoplasms,3 requiring elucidation as to its origin and nature. In this patient, disproportional choroidal exudation in the context of a solid lesion unresponsive to steroid therapy was strongly suggestive of malignancy.The yellow discoloration noted in only 1 eye was unusual and likely reflected a subacute process related to long-standing choroidal fluid buildup.4 Unlike the scleral impregnation with bilateral involvement classically seen in jaundice,5 the ocular coloration changes developed in this patient indicated a local mechanism, mediated by fluid extravasation from the choroidal compartment to the subconjunctival space.4 Therefore, screening for causes of bilirubin elevation, such as liver dysfunction and biliary tract obstruction (choice A), would not be the first step taken.Given the presence of an intraocular mass, primary and secondary neoplasms should be foremost investigated before any treatment consideration. Surgical drainage of the hemorrhagic content (choice B) and management with plaque brachytherapy (choice C) would not be advisable until a final diagnosis was reached because of risks of incomplete disease control and tumor seeding. Based on the characteristics of the lesion on imaging, notably heterogeneous reflectivity, irregular surface, and infiltrative pattern, which typically signal uveal metastasis as opposed to primary melanoma,6 screening for an occult systemic primary cancer should be the first course of action (choice D).Accordingly, the patient underwent full-body imaging studies with computed tomography of the thorax, abdomen, and pelvis. She was identified with a 6 × 9-cm silent mass at the upper left pulmonary lobe with associated mediastinal adenopathy (Figure 2). A fine-needle aspiration biopsy of the lung tumor revealed primary adenocarcinoma. Other metastatic lesions were also identified on the thoracic spine.Computed tomography scan reveals a lobular lung tumor (arrowhead).Metastasis is the most common ocular malignancy in the adult population, affecting the choroid in 85% of patients by hematogenous dissemination.6,7 Metastatic implants are visible on fundoscopy as single or multiple light-yellow solid lesions of variable shape usually with associated subretinal fluid.6 Primary sites have been localized, in order of frequency, to breast, lung, kidney, gastrointestinal tract, and cutaneous melanoma.7 In a large series, uveal metastasis from lung cancer was more prevalent in men (55%) and mainly unifocal (77%) and unilateral (82%).8 Approximately 40% of individuals had the original tumor discovered only after detecting uveal lesions8 similar to what we observed in this patient. Although choroidal effusion may be a nonspecific finding attributable to metastatic systemic neoplasms,3,6 a threshold of suspicion for underlying malignancy is advisable in the face of any intraocular solid growth.The patient was treated with systemic chemotherapy (entrectinib) and external beam radiotherapy to the mediastinum and orbits (20 Gy, 5 fractions). The conjunctival color progressively recovered in the following weeks, whereas the choroidal metastasis and detachment resolved 3 months after radiation. The visual acuity in the left eye remained hand motion."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 65-year-old Arab woman was referred with a 6-week history of blurry vision, ocular pain, and subconjunctival yellow discoloration in the left eye. Suspicious retinal detachment and intraocular mass were identified, which raised concerns for choroidal melanoma. No symptoms or signs were reported in the right eye. Her ocular history included bilateral cataract surgery 5 years prior. Review of systems yielded negative results, including for ocular trauma, jaundice, and cutaneous melanoma. She had no history of tobacco use.On examination of the left eye, visual acuity was hand motion, and intraocular pressure was 8 mm Hg. The anterior segment showed diffuse conjunctival chemosis of yellow hue, low-grade aqueous reaction, and pseudophakia (Figure 1A). On the fundus evaluation, large pockets of subretinal fluid suggested choroidal effusion based on marked fluid shifting with head position. B-scan ultrasonography confirmed 4-quadrant choroidal hemorrhage and partially mobile serous retinal detachment. B-scans also exhibited a solid lesion from the 6- to 10-o’clock meridians with irregular shape and variable internal reflectivity, measuring 5.8 mm in height. Magnetic resonance imaging was requested to delineate the mass from the surrounding clot (Figure 1B). In the interim, she received oral steroids aiming to contain the abundant exudation and topical atropine for pain, evolving with minimal clinical improvement on these features.Coloration changes in the left eye associated with choroidal hemorrhage and an intraocular solid lesion. B, The arrowhead indicates the lesion and the asterisk, the adjacent clot.\n</clinical_case>\n\n<question>\nA 65-year-old woman was referred with a 6-week history of blurry vision, ocular pain, and subconjunctival yellow discoloration in the left eye. Suspicious retinal detachment and intraocular mass were identified, which raised concerns for choroidal melanoma. No symptoms or signs were reported in the right eye. Her ocular history included bilateral cataract surgery 5 years prior. Review of systems yielded negative results, including for ocular trauma, jaundice, and cutaneous melanoma. She had no history of tobacco use.On examination of the left eye, visual acuity was hand motion, and intraocular pressure was 8 mm Hg. The anterior segment showed diffuse conjunctival chemosis of yellow hue, low-grade aqueous reaction, and pseudophakia (Figure 1A). On the fundus evaluation, large pockets of subretinal fluid suggested choroidal effusion based on marked fluid shifting with head position. B-scan ultrasonography confirmed 4-quadrant choroidal hemorrhage and partially mobile serous retinal detachment. B-scans also exhibited a solid lesion from the 6- to 10-o’clock meridians with irregular shape and variable internal reflectivity, measuring 5.8 mm in height. Magnetic resonance imaging was requested to delineate the mass from the surrounding clot (Figure 1B). In the interim, she received oral steroids aiming to contain the abundant exudation and topical atropine for pain, evolving with minimal clinical improvement on these features.Coloration changes in the left eye associated with choroidal hemorrhage and an intraocular solid lesion. B, The arrowhead indicates the lesion and the asterisk, the adjacent clot. What Would You Do Next?\n</question>\n\n<options>\nA. Rule out an occult systemic neoplasm\nB. Investigate liver dysfunction and biliary tract obstruction\nC. Treat with plaque brachytherapy\nD. Drain the hemorrhage with scleral windows\n</options>\n"
},
{
"role": "assistant",
"content": "A. Rule out an occult systemic neoplasm\nChoroidal effusion consists of serous-hemorrhagic fluid accumulation in the suprachoroidal space typically encountered in trauma, surgery, or uveitis scenarios.1,2 It can produce a mass effect simulating tumors2 or be the presenting feature of ocular neoplasms,3 requiring elucidation as to its origin and nature. In this patient, disproportional choroidal exudation in the context of a solid lesion unresponsive to steroid therapy was strongly suggestive of malignancy.The yellow discoloration noted in only 1 eye was unusual and likely reflected a subacute process related to long-standing choroidal fluid buildup.4 Unlike the scleral impregnation with bilateral involvement classically seen in jaundice,5 the ocular coloration changes developed in this patient indicated a local mechanism, mediated by fluid extravasation from the choroidal compartment to the subconjunctival space.4 Therefore, screening for causes of bilirubin elevation, such as liver dysfunction and biliary tract obstruction (choice A), would not be the first step taken.Given the presence of an intraocular mass, primary and secondary neoplasms should be foremost investigated before any treatment consideration. Surgical drainage of the hemorrhagic content (choice B) and management with plaque brachytherapy (choice C) would not be advisable until a final diagnosis was reached because of risks of incomplete disease control and tumor seeding. Based on the characteristics of the lesion on imaging, notably heterogeneous reflectivity, irregular surface, and infiltrative pattern, which typically signal uveal metastasis as opposed to primary melanoma,6 screening for an occult systemic primary cancer should be the first course of action (choice D).Accordingly, the patient underwent full-body imaging studies with computed tomography of the thorax, abdomen, and pelvis. She was identified with a 6 × 9-cm silent mass at the upper left pulmonary lobe with associated mediastinal adenopathy (Figure 2). A fine-needle aspiration biopsy of the lung tumor revealed primary adenocarcinoma. Other metastatic lesions were also identified on the thoracic spine.Computed tomography scan reveals a lobular lung tumor (arrowhead).Metastasis is the most common ocular malignancy in the adult population, affecting the choroid in 85% of patients by hematogenous dissemination.6,7 Metastatic implants are visible on fundoscopy as single or multiple light-yellow solid lesions of variable shape usually with associated subretinal fluid.6 Primary sites have been localized, in order of frequency, to breast, lung, kidney, gastrointestinal tract, and cutaneous melanoma.7 In a large series, uveal metastasis from lung cancer was more prevalent in men (55%) and mainly unifocal (77%) and unilateral (82%).8 Approximately 40% of individuals had the original tumor discovered only after detecting uveal lesions8 similar to what we observed in this patient. Although choroidal effusion may be a nonspecific finding attributable to metastatic systemic neoplasms,3,6 a threshold of suspicion for underlying malignancy is advisable in the face of any intraocular solid growth.The patient was treated with systemic chemotherapy (entrectinib) and external beam radiotherapy to the mediastinum and orbits (20 Gy, 5 fractions). The conjunctival color progressively recovered in the following weeks, whereas the choroidal metastasis and detachment resolved 3 months after radiation. The visual acuity in the left eye remained hand motion."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 65-year-old Arab man was referred with a 6-week history of blurry vision, ocular pain, and subconjunctival yellow discoloration in the left eye. Suspicious retinal detachment and intraocular mass were identified, which raised concerns for choroidal melanoma. No symptoms or signs were reported in the right eye. Him ocular history included bilateral cataract surgery 5 years prior. Review of systems yielded negative results, including for ocular trauma, jaundice, and cutaneous melanoma. He had no history of tobacco use.On examination of the left eye, visual acuity was hand motion, and intraocular pressure was 8 mm Hg. The anterior segment showed diffuse conjunctival chemosis of yellow hue, low-grade aqueous reaction, and pseudophakia (Figure 1A). On the fundus evaluation, large pockets of subretinal fluid suggested choroidal effusion based on marked fluid shifting with head position. B-scan ultrasonography confirmed 4-quadrant choroidal hemorrhage and partially mobile serous retinal detachment. B-scans also exhibited a solid lesion from the 6- to 10-o’clock meridians with irregular shape and variable internal reflectivity, measuring 5.8 mm in height. Magnetic resonance imaging was requested to delineate the mass from the surrounding clot (Figure 1B). In the interim, he received oral steroids aiming to contain the abundant exudation and topical atropine for pain, evolving with minimal clinical improvement on these features.Coloration changes in the left eye associated with choroidal hemorrhage and an intraocular solid lesion. B, The arrowhead indicates the lesion and the asterisk, the adjacent clot.\n</clinical_case>\n\n<question>\nA 65-year-old woman was referred with a 6-week history of blurry vision, ocular pain, and subconjunctival yellow discoloration in the left eye. Suspicious retinal detachment and intraocular mass were identified, which raised concerns for choroidal melanoma. No symptoms or signs were reported in the right eye. Her ocular history included bilateral cataract surgery 5 years prior. Review of systems yielded negative results, including for ocular trauma, jaundice, and cutaneous melanoma. She had no history of tobacco use.On examination of the left eye, visual acuity was hand motion, and intraocular pressure was 8 mm Hg. The anterior segment showed diffuse conjunctival chemosis of yellow hue, low-grade aqueous reaction, and pseudophakia (Figure 1A). On the fundus evaluation, large pockets of subretinal fluid suggested choroidal effusion based on marked fluid shifting with head position. B-scan ultrasonography confirmed 4-quadrant choroidal hemorrhage and partially mobile serous retinal detachment. B-scans also exhibited a solid lesion from the 6- to 10-o’clock meridians with irregular shape and variable internal reflectivity, measuring 5.8 mm in height. Magnetic resonance imaging was requested to delineate the mass from the surrounding clot (Figure 1B). In the interim, she received oral steroids aiming to contain the abundant exudation and topical atropine for pain, evolving with minimal clinical improvement on these features.Coloration changes in the left eye associated with choroidal hemorrhage and an intraocular solid lesion. B, The arrowhead indicates the lesion and the asterisk, the adjacent clot. What Would You Do Next?\n</question>\n\n<options>\nA. Rule out an occult systemic neoplasm\nB. Investigate liver dysfunction and biliary tract obstruction\nC. Treat with plaque brachytherapy\nD. Drain the hemorrhage with scleral windows\n</options>\n"
},
{
"role": "assistant",
"content": "A. Rule out an occult systemic neoplasm\nChoroidal effusion consists of serous-hemorrhagic fluid accumulation in the suprachoroidal space typically encountered in trauma, surgery, or uveitis scenarios.1,2 It can produce a mass effect simulating tumors2 or be the presenting feature of ocular neoplasms,3 requiring elucidation as to its origin and nature. In this patient, disproportional choroidal exudation in the context of a solid lesion unresponsive to steroid therapy was strongly suggestive of malignancy.The yellow discoloration noted in only 1 eye was unusual and likely reflected a subacute process related to long-standing choroidal fluid buildup.4 Unlike the scleral impregnation with bilateral involvement classically seen in jaundice,5 the ocular coloration changes developed in this patient indicated a local mechanism, mediated by fluid extravasation from the choroidal compartment to the subconjunctival space.4 Therefore, screening for causes of bilirubin elevation, such as liver dysfunction and biliary tract obstruction (choice A), would not be the first step taken.Given the presence of an intraocular mass, primary and secondary neoplasms should be foremost investigated before any treatment consideration. Surgical drainage of the hemorrhagic content (choice B) and management with plaque brachytherapy (choice C) would not be advisable until a final diagnosis was reached because of risks of incomplete disease control and tumor seeding. Based on the characteristics of the lesion on imaging, notably heterogeneous reflectivity, irregular surface, and infiltrative pattern, which typically signal uveal metastasis as opposed to primary melanoma,6 screening for an occult systemic primary cancer should be the first course of action (choice D).Accordingly, the patient underwent full-body imaging studies with computed tomography of the thorax, abdomen, and pelvis. She was identified with a 6 × 9-cm silent mass at the upper left pulmonary lobe with associated mediastinal adenopathy (Figure 2). A fine-needle aspiration biopsy of the lung tumor revealed primary adenocarcinoma. Other metastatic lesions were also identified on the thoracic spine.Computed tomography scan reveals a lobular lung tumor (arrowhead).Metastasis is the most common ocular malignancy in the adult population, affecting the choroid in 85% of patients by hematogenous dissemination.6,7 Metastatic implants are visible on fundoscopy as single or multiple light-yellow solid lesions of variable shape usually with associated subretinal fluid.6 Primary sites have been localized, in order of frequency, to breast, lung, kidney, gastrointestinal tract, and cutaneous melanoma.7 In a large series, uveal metastasis from lung cancer was more prevalent in men (55%) and mainly unifocal (77%) and unilateral (82%).8 Approximately 40% of individuals had the original tumor discovered only after detecting uveal lesions8 similar to what we observed in this patient. Although choroidal effusion may be a nonspecific finding attributable to metastatic systemic neoplasms,3,6 a threshold of suspicion for underlying malignancy is advisable in the face of any intraocular solid growth.The patient was treated with systemic chemotherapy (entrectinib) and external beam radiotherapy to the mediastinum and orbits (20 Gy, 5 fractions). The conjunctival color progressively recovered in the following weeks, whereas the choroidal metastasis and detachment resolved 3 months after radiation. The visual acuity in the left eye remained hand motion."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 65-year-old Arab patient was referred with a 6-week history of blurry vision, ocular pain, and subconjunctival yellow discoloration in the left eye. Suspicious retinal detachment and intraocular mass were identified, which raised concerns for choroidal melanoma. No symptoms or signs were reported in the right eye. Them ocular history included bilateral cataract surgery 5 years prior. Review of systems yielded negative results, including for ocular trauma, jaundice, and cutaneous melanoma. They had no history of tobacco use.On examination of the left eye, visual acuity was hand motion, and intraocular pressure was 8 mm Hg. The anterior segment showed diffuse conjunctival chemosis of yellow hue, low-grade aqueous reaction, and pseudophakia (Figure 1A). On the fundus evaluation, large pockets of subretinal fluid suggested choroidal effusion based on marked fluid shifting with head position. B-scan ultrasonography confirmed 4-quadrant choroidal hemorrhage and partially mobile serous retinal detachment. B-scans also exhibited a solid lesion from the 6- to 10-o’clock meridians with irregular shape and variable internal reflectivity, measuring 5.8 mm in height. Magnetic resonance imaging was requested to delineate the mass from the surrounding clot (Figure 1B). In the interim, they received oral steroids aiming to contain the abundant exudation and topical atropine for pain, evolving with minimal clinical improvement on these features.Coloration changes in the left eye associated with choroidal hemorrhage and an intraocular solid lesion. B, The arrowhead indicates the lesion and the asterisk, the adjacent clot.\n</clinical_case>\n\n<question>\nA 65-year-old woman was referred with a 6-week history of blurry vision, ocular pain, and subconjunctival yellow discoloration in the left eye. Suspicious retinal detachment and intraocular mass were identified, which raised concerns for choroidal melanoma. No symptoms or signs were reported in the right eye. Her ocular history included bilateral cataract surgery 5 years prior. Review of systems yielded negative results, including for ocular trauma, jaundice, and cutaneous melanoma. She had no history of tobacco use.On examination of the left eye, visual acuity was hand motion, and intraocular pressure was 8 mm Hg. The anterior segment showed diffuse conjunctival chemosis of yellow hue, low-grade aqueous reaction, and pseudophakia (Figure 1A). On the fundus evaluation, large pockets of subretinal fluid suggested choroidal effusion based on marked fluid shifting with head position. B-scan ultrasonography confirmed 4-quadrant choroidal hemorrhage and partially mobile serous retinal detachment. B-scans also exhibited a solid lesion from the 6- to 10-o’clock meridians with irregular shape and variable internal reflectivity, measuring 5.8 mm in height. Magnetic resonance imaging was requested to delineate the mass from the surrounding clot (Figure 1B). In the interim, she received oral steroids aiming to contain the abundant exudation and topical atropine for pain, evolving with minimal clinical improvement on these features.Coloration changes in the left eye associated with choroidal hemorrhage and an intraocular solid lesion. B, The arrowhead indicates the lesion and the asterisk, the adjacent clot. What Would You Do Next?\n</question>\n\n<options>\nA. Rule out an occult systemic neoplasm\nB. Investigate liver dysfunction and biliary tract obstruction\nC. Treat with plaque brachytherapy\nD. Drain the hemorrhage with scleral windows\n</options>\n"
},
{
"role": "assistant",
"content": "A. Rule out an occult systemic neoplasm\nChoroidal effusion consists of serous-hemorrhagic fluid accumulation in the suprachoroidal space typically encountered in trauma, surgery, or uveitis scenarios.1,2 It can produce a mass effect simulating tumors2 or be the presenting feature of ocular neoplasms,3 requiring elucidation as to its origin and nature. In this patient, disproportional choroidal exudation in the context of a solid lesion unresponsive to steroid therapy was strongly suggestive of malignancy.The yellow discoloration noted in only 1 eye was unusual and likely reflected a subacute process related to long-standing choroidal fluid buildup.4 Unlike the scleral impregnation with bilateral involvement classically seen in jaundice,5 the ocular coloration changes developed in this patient indicated a local mechanism, mediated by fluid extravasation from the choroidal compartment to the subconjunctival space.4 Therefore, screening for causes of bilirubin elevation, such as liver dysfunction and biliary tract obstruction (choice A), would not be the first step taken.Given the presence of an intraocular mass, primary and secondary neoplasms should be foremost investigated before any treatment consideration. Surgical drainage of the hemorrhagic content (choice B) and management with plaque brachytherapy (choice C) would not be advisable until a final diagnosis was reached because of risks of incomplete disease control and tumor seeding. Based on the characteristics of the lesion on imaging, notably heterogeneous reflectivity, irregular surface, and infiltrative pattern, which typically signal uveal metastasis as opposed to primary melanoma,6 screening for an occult systemic primary cancer should be the first course of action (choice D).Accordingly, the patient underwent full-body imaging studies with computed tomography of the thorax, abdomen, and pelvis. She was identified with a 6 × 9-cm silent mass at the upper left pulmonary lobe with associated mediastinal adenopathy (Figure 2). A fine-needle aspiration biopsy of the lung tumor revealed primary adenocarcinoma. Other metastatic lesions were also identified on the thoracic spine.Computed tomography scan reveals a lobular lung tumor (arrowhead).Metastasis is the most common ocular malignancy in the adult population, affecting the choroid in 85% of patients by hematogenous dissemination.6,7 Metastatic implants are visible on fundoscopy as single or multiple light-yellow solid lesions of variable shape usually with associated subretinal fluid.6 Primary sites have been localized, in order of frequency, to breast, lung, kidney, gastrointestinal tract, and cutaneous melanoma.7 In a large series, uveal metastasis from lung cancer was more prevalent in men (55%) and mainly unifocal (77%) and unilateral (82%).8 Approximately 40% of individuals had the original tumor discovered only after detecting uveal lesions8 similar to what we observed in this patient. Although choroidal effusion may be a nonspecific finding attributable to metastatic systemic neoplasms,3,6 a threshold of suspicion for underlying malignancy is advisable in the face of any intraocular solid growth.The patient was treated with systemic chemotherapy (entrectinib) and external beam radiotherapy to the mediastinum and orbits (20 Gy, 5 fractions). The conjunctival color progressively recovered in the following weeks, whereas the choroidal metastasis and detachment resolved 3 months after radiation. The visual acuity in the left eye remained hand motion."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA patient in their 60s with a medical history of hypertension was referred for new-onset fluctuating diplopia and worsening right upper eyelid ptosis. The patient underwent right-sided ptosis repair at another hospital less than 1 year earlier without improvement. Best-corrected visual acuity was 20/30 OU, and pupil examination revealed no anisocoria or afferent pupillary defect. Color vision was full in both eyes. External examination revealed a margin reflex distance 1 (MRD1) of 0 mm OD, 4 mm OS without fatigability in extended upgaze (Figure 1A). The patient had a small exotropia in primary gaze with a −2 supraduction deficit. No proptosis was observed. An ice pack was applied in the clinic to the ptotic eyelid for 2 minutes, which led to a 1.5-mm improvement (Figure 1B) in ptosis and no appreciable change in strabismus. The patient reported no associated headache, weakness, or difficulty swallowing.External photographs demonstrate ptosis of the right upper eyelid with a margin reflex distance 1 of 0 mm (A) that improved by 1.5 mm after application of an ice pack for 2 minutes (B).Magnetic resonance imaging of the brain and orbit\n</clinical_case>\n\n<question>\nA patient in their 60s with a medical history of hypertension was referred for new-onset fluctuating diplopia and worsening right upper eyelid ptosis. The patient underwent right-sided ptosis repair at another hospital less than 1 year earlier without improvement. Best-corrected visual acuity was 20/30 OU, and pupil examination revealed no anisocoria or afferent pupillary defect. Color vision was full in both eyes. External examination revealed a margin reflex distance 1 (MRD1) of 0 mm OD, 4 mm OS without fatigability in extended upgaze (Figure 1A). The patient had a small exotropia in primary gaze with a −2 supraduction deficit. No proptosis was observed. An ice pack was applied in the clinic to the ptotic eyelid for 2 minutes, which led to a 1.5-mm improvement (Figure 1B) in ptosis and no appreciable change in strabismus. The patient reported no associated headache, weakness, or difficulty swallowing.External photographs demonstrate ptosis of the right upper eyelid with a margin reflex distance 1 of 0 mm (A) that improved by 1.5 mm after application of an ice pack for 2 minutes (B).Magnetic resonance imaging of the brain and orbit What Would You Do Next?\n</question>\n\n<options>\nA. Begin symptomatic management with cholinesterase inhibitor\nB. Revision ptosis repair\nC. Magnetic resonance imaging of the brain and orbit\nD. Serum testing for autoantibodies\n</options>\n"
},
{
"role": "assistant",
"content": "C. Magnetic resonance imaging of the brain and orbit\nThe constellation of new-onset fluctuating diplopia and ptosis in a patient can suggest a diagnosis of ocular myasthenia gravis, especially in the setting of ptosis that improves with application of an ice pack. The ice test is a quick and effective examination that has been shown to be highly specific and sensitive in the diagnosis of myasthenia gravis.1-3 Although testing for antibodies (choice A) against the acetylcholine receptor or muscle-specific tyrosine kinase can help confirm a diagnosis of ocular myasthenia gravis, other etiologies must be ruled out, including orbital masses, cavernous sinus syndrome, and isolated oculomotor nerve palsy, some of which may be vision or life threatening.4,5Improvement of the ptotic eyelid is considered the hallmark of a positive ice test result, and initial studies evaluating the ice test revealed that patients with myasthenic ptosis typically demonstrate an improvement of 2 mm or greater in the MRD1.1,2 In contrast, patients with nonmyasthenic causes of ptosis, such as oculomotor nerve palsy and senile levator dehiscence, showed changes in the MRD1 (≤1 mm) that did not meet the threshold for a positive test result.1,2 Although an increase of 1.5 mm in the MRD1 as observed in this patient does not meet the criteria for a positive test result, it was greater than the typical changes observed in previously reported negative test results. Rare cases of false-positive test results have been observed in patients with chronic progressive external ophthalmoplegia or cavernous sinus syndrome and after botulinum toxin injection.3,6 Although equivocal findings may lead to an eventual diagnosis of myasthenia gravis, other masquerading diagnoses should be considered prior to intervention (choice D) or repair (choice C).This patient presented with limitation in supraduction consistent with a superior divisional oculomotor palsy. To rule out etiologies with higher morbidity, magnetic resonance imaging of the brain and orbits was performed in addition to concomitant antibody testing. The magnetic resonance image revealed a 1.9-cm aneurysm in the right cavernous sinus leading to compression of the right internal carotid artery and oculomotor nerve, as well as a 5-mm aneurysm arising from the internal carotid artery in the left cavernous sinus (Figure 2). Compressive aneurysms along the cavernous, hypophyseal, and ophthalmic segments of the internal carotid artery are a well-described cause of oculomotor nerve palsy that may require neurosurgical or endovascular intervention. Variable diplopia due to aneurysmal causes has rarely been reported and is hypothesized to be due to intermittent hyperfunction of extraocular muscles innervated by the third cranial nerve.7 While rare, superior divisional palsies have been reported in cavernous carotid aneurysms, due to the bifurcation of the nerve at the anterior cavernous sinus into the superior and inferior division.8,9Axial T1-weighted magnetic resonance image of brain and orbits revealed a 1.9-cm aneurysm in the cavernous sinus (arrowhead), causing compression and medial deviation of the right internal carotid artery and subsequent compression of the oculomotor nerve. A small 5-mm aneurysm arising at the junction of the cavernous and supraclinoid segments of the left internal carotid artery was also noted but is not visualized on this image.This patient was diagnosed with oculomotor nerve palsy secondary to compressive aneurysm of the internal carotid artery in the right cavernous sinus and referred for neurosurgical intervention. The patient underwent embolization with improvement of the ptosis and diplopia."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA White patient in their 60s with a medical history of hypertension was referred for new-onset fluctuating diplopia and worsening right upper eyelid ptosis. The White patient underwent right-sided ptosis repair at another hospital less than 1 year earlier without improvement. Best-corrected visual acuity was 20/30 OU, and pupil examination revealed no anisocoria or afferent pupillary defect. Color vision was full in both eyes. External examination revealed a margin reflex distance 1 (MRD1) of 0 mm OD, 4 mm OS without fatigability in extended upgaze (Figure 1A). The White patient had a small exotropia in primary gaze with a −2 supraduction deficit. No proptosis was observed. An ice pack was applied in the clinic to the ptotic eyelid for 2 minutes, which led to a 1.5-mm improvement (Figure 1B) in ptosis and no appreciable change in strabismus. The White patient reported no associated headache, weakness, or difficulty swallowing.External photographs demonstrate ptosis of the right upper eyelid with a margin reflex distance 1 of 0 mm (A) that improved by 1.5 mm after application of an ice pack for 2 minutes (B).Magnetic resonance imaging of the brain and orbit\n</clinical_case>\n\n<question>\nA patient in their 60s with a medical history of hypertension was referred for new-onset fluctuating diplopia and worsening right upper eyelid ptosis. The patient underwent right-sided ptosis repair at another hospital less than 1 year earlier without improvement. Best-corrected visual acuity was 20/30 OU, and pupil examination revealed no anisocoria or afferent pupillary defect. Color vision was full in both eyes. External examination revealed a margin reflex distance 1 (MRD1) of 0 mm OD, 4 mm OS without fatigability in extended upgaze (Figure 1A). The patient had a small exotropia in primary gaze with a −2 supraduction deficit. No proptosis was observed. An ice pack was applied in the clinic to the ptotic eyelid for 2 minutes, which led to a 1.5-mm improvement (Figure 1B) in ptosis and no appreciable change in strabismus. The patient reported no associated headache, weakness, or difficulty swallowing.External photographs demonstrate ptosis of the right upper eyelid with a margin reflex distance 1 of 0 mm (A) that improved by 1.5 mm after application of an ice pack for 2 minutes (B).Magnetic resonance imaging of the brain and orbit What Would You Do Next?\n</question>\n\n<options>\nA. Begin symptomatic management with cholinesterase inhibitor\nB. Revision ptosis repair\nC. Magnetic resonance imaging of the brain and orbit\nD. Serum testing for autoantibodies\n</options>\n"
},
{
"role": "assistant",
"content": "C. Magnetic resonance imaging of the brain and orbit\nThe constellation of new-onset fluctuating diplopia and ptosis in a patient can suggest a diagnosis of ocular myasthenia gravis, especially in the setting of ptosis that improves with application of an ice pack. The ice test is a quick and effective examination that has been shown to be highly specific and sensitive in the diagnosis of myasthenia gravis.1-3 Although testing for antibodies (choice A) against the acetylcholine receptor or muscle-specific tyrosine kinase can help confirm a diagnosis of ocular myasthenia gravis, other etiologies must be ruled out, including orbital masses, cavernous sinus syndrome, and isolated oculomotor nerve palsy, some of which may be vision or life threatening.4,5Improvement of the ptotic eyelid is considered the hallmark of a positive ice test result, and initial studies evaluating the ice test revealed that patients with myasthenic ptosis typically demonstrate an improvement of 2 mm or greater in the MRD1.1,2 In contrast, patients with nonmyasthenic causes of ptosis, such as oculomotor nerve palsy and senile levator dehiscence, showed changes in the MRD1 (≤1 mm) that did not meet the threshold for a positive test result.1,2 Although an increase of 1.5 mm in the MRD1 as observed in this patient does not meet the criteria for a positive test result, it was greater than the typical changes observed in previously reported negative test results. Rare cases of false-positive test results have been observed in patients with chronic progressive external ophthalmoplegia or cavernous sinus syndrome and after botulinum toxin injection.3,6 Although equivocal findings may lead to an eventual diagnosis of myasthenia gravis, other masquerading diagnoses should be considered prior to intervention (choice D) or repair (choice C).This patient presented with limitation in supraduction consistent with a superior divisional oculomotor palsy. To rule out etiologies with higher morbidity, magnetic resonance imaging of the brain and orbits was performed in addition to concomitant antibody testing. The magnetic resonance image revealed a 1.9-cm aneurysm in the right cavernous sinus leading to compression of the right internal carotid artery and oculomotor nerve, as well as a 5-mm aneurysm arising from the internal carotid artery in the left cavernous sinus (Figure 2). Compressive aneurysms along the cavernous, hypophyseal, and ophthalmic segments of the internal carotid artery are a well-described cause of oculomotor nerve palsy that may require neurosurgical or endovascular intervention. Variable diplopia due to aneurysmal causes has rarely been reported and is hypothesized to be due to intermittent hyperfunction of extraocular muscles innervated by the third cranial nerve.7 While rare, superior divisional palsies have been reported in cavernous carotid aneurysms, due to the bifurcation of the nerve at the anterior cavernous sinus into the superior and inferior division.8,9Axial T1-weighted magnetic resonance image of brain and orbits revealed a 1.9-cm aneurysm in the cavernous sinus (arrowhead), causing compression and medial deviation of the right internal carotid artery and subsequent compression of the oculomotor nerve. A small 5-mm aneurysm arising at the junction of the cavernous and supraclinoid segments of the left internal carotid artery was also noted but is not visualized on this image.This patient was diagnosed with oculomotor nerve palsy secondary to compressive aneurysm of the internal carotid artery in the right cavernous sinus and referred for neurosurgical intervention. The patient underwent embolization with improvement of the ptosis and diplopia."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA male patient in his 60s with a medical history of hypertension was referred for new-onset fluctuating diplopia and worsening right upper eyelid ptosis. The male patient underwent right-sided ptosis repair at another hospital less than 1 year earlier without improvement. Best-corrected visual acuity was 20/30 OU, and pupil examination revealed no anisocoria or afferent pupillary defect. Color vision was full in both eyes. External examination revealed a margin reflex distance 1 (MRD1) of 0 mm OD, 4 mm OS without fatigability in extended upgaze (Figure 1A). The male patient had a small exotropia in primary gaze with a −2 supraduction deficit. No proptosis was observed. An ice pack was applied in the clinic to the ptotic eyelid for 2 minutes, which led to a 1.5-mm improvement (Figure 1B) in ptosis and no appreciable change in strabismus. The male patient reported no associated headache, weakness, or difficulty swallowing.External photographs demonstrate ptosis of the right upper eyelid with a margin reflex distance 1 of 0 mm (A) that improved by 1.5 mm after application of an ice pack for 2 minutes (B).Magnetic resonance imaging of the brain and orbit\n</clinical_case>\n\n<question>\nA patient in their 60s with a medical history of hypertension was referred for new-onset fluctuating diplopia and worsening right upper eyelid ptosis. The patient underwent right-sided ptosis repair at another hospital less than 1 year earlier without improvement. Best-corrected visual acuity was 20/30 OU, and pupil examination revealed no anisocoria or afferent pupillary defect. Color vision was full in both eyes. External examination revealed a margin reflex distance 1 (MRD1) of 0 mm OD, 4 mm OS without fatigability in extended upgaze (Figure 1A). The patient had a small exotropia in primary gaze with a −2 supraduction deficit. No proptosis was observed. An ice pack was applied in the clinic to the ptotic eyelid for 2 minutes, which led to a 1.5-mm improvement (Figure 1B) in ptosis and no appreciable change in strabismus. The patient reported no associated headache, weakness, or difficulty swallowing.External photographs demonstrate ptosis of the right upper eyelid with a margin reflex distance 1 of 0 mm (A) that improved by 1.5 mm after application of an ice pack for 2 minutes (B).Magnetic resonance imaging of the brain and orbit What Would You Do Next?\n</question>\n\n<options>\nA. Begin symptomatic management with cholinesterase inhibitor\nB. Revision ptosis repair\nC. Magnetic resonance imaging of the brain and orbit\nD. Serum testing for autoantibodies\n</options>\n"
},
{
"role": "assistant",
"content": "C. Magnetic resonance imaging of the brain and orbit\nThe constellation of new-onset fluctuating diplopia and ptosis in a patient can suggest a diagnosis of ocular myasthenia gravis, especially in the setting of ptosis that improves with application of an ice pack. The ice test is a quick and effective examination that has been shown to be highly specific and sensitive in the diagnosis of myasthenia gravis.1-3 Although testing for antibodies (choice A) against the acetylcholine receptor or muscle-specific tyrosine kinase can help confirm a diagnosis of ocular myasthenia gravis, other etiologies must be ruled out, including orbital masses, cavernous sinus syndrome, and isolated oculomotor nerve palsy, some of which may be vision or life threatening.4,5Improvement of the ptotic eyelid is considered the hallmark of a positive ice test result, and initial studies evaluating the ice test revealed that patients with myasthenic ptosis typically demonstrate an improvement of 2 mm or greater in the MRD1.1,2 In contrast, patients with nonmyasthenic causes of ptosis, such as oculomotor nerve palsy and senile levator dehiscence, showed changes in the MRD1 (≤1 mm) that did not meet the threshold for a positive test result.1,2 Although an increase of 1.5 mm in the MRD1 as observed in this patient does not meet the criteria for a positive test result, it was greater than the typical changes observed in previously reported negative test results. Rare cases of false-positive test results have been observed in patients with chronic progressive external ophthalmoplegia or cavernous sinus syndrome and after botulinum toxin injection.3,6 Although equivocal findings may lead to an eventual diagnosis of myasthenia gravis, other masquerading diagnoses should be considered prior to intervention (choice D) or repair (choice C).This patient presented with limitation in supraduction consistent with a superior divisional oculomotor palsy. To rule out etiologies with higher morbidity, magnetic resonance imaging of the brain and orbits was performed in addition to concomitant antibody testing. The magnetic resonance image revealed a 1.9-cm aneurysm in the right cavernous sinus leading to compression of the right internal carotid artery and oculomotor nerve, as well as a 5-mm aneurysm arising from the internal carotid artery in the left cavernous sinus (Figure 2). Compressive aneurysms along the cavernous, hypophyseal, and ophthalmic segments of the internal carotid artery are a well-described cause of oculomotor nerve palsy that may require neurosurgical or endovascular intervention. Variable diplopia due to aneurysmal causes has rarely been reported and is hypothesized to be due to intermittent hyperfunction of extraocular muscles innervated by the third cranial nerve.7 While rare, superior divisional palsies have been reported in cavernous carotid aneurysms, due to the bifurcation of the nerve at the anterior cavernous sinus into the superior and inferior division.8,9Axial T1-weighted magnetic resonance image of brain and orbits revealed a 1.9-cm aneurysm in the cavernous sinus (arrowhead), causing compression and medial deviation of the right internal carotid artery and subsequent compression of the oculomotor nerve. A small 5-mm aneurysm arising at the junction of the cavernous and supraclinoid segments of the left internal carotid artery was also noted but is not visualized on this image.This patient was diagnosed with oculomotor nerve palsy secondary to compressive aneurysm of the internal carotid artery in the right cavernous sinus and referred for neurosurgical intervention. The patient underwent embolization with improvement of the ptosis and diplopia."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA female patient in her 60s with a medical history of hypertension was referred for new-onset fluctuating diplopia and worsening right upper eyelid ptosis. The female patient underwent right-sided ptosis repair at another hospital less than 1 year earlier without improvement. Best-corrected visual acuity was 20/30 OU, and pupil examination revealed no anisocoria or afferent pupillary defect. Color vision was full in both eyes. External examination revealed a margin reflex distance 1 (MRD1) of 0 mm OD, 4 mm OS without fatigability in extended upgaze (Figure 1A). The female patient had a small exotropia in primary gaze with a −2 supraduction deficit. No proptosis was observed. An ice pack was applied in the clinic to the ptotic eyelid for 2 minutes, which led to a 1.5-mm improvement (Figure 1B) in ptosis and no appreciable change in strabismus. The female patient reported no associated headache, weakness, or difficulty swallowing.External photographs demonstrate ptosis of the right upper eyelid with a margin reflex distance 1 of 0 mm (A) that improved by 1.5 mm after application of an ice pack for 2 minutes (B).Magnetic resonance imaging of the brain and orbit\n</clinical_case>\n\n<question>\nA patient in their 60s with a medical history of hypertension was referred for new-onset fluctuating diplopia and worsening right upper eyelid ptosis. The patient underwent right-sided ptosis repair at another hospital less than 1 year earlier without improvement. Best-corrected visual acuity was 20/30 OU, and pupil examination revealed no anisocoria or afferent pupillary defect. Color vision was full in both eyes. External examination revealed a margin reflex distance 1 (MRD1) of 0 mm OD, 4 mm OS without fatigability in extended upgaze (Figure 1A). The patient had a small exotropia in primary gaze with a −2 supraduction deficit. No proptosis was observed. An ice pack was applied in the clinic to the ptotic eyelid for 2 minutes, which led to a 1.5-mm improvement (Figure 1B) in ptosis and no appreciable change in strabismus. The patient reported no associated headache, weakness, or difficulty swallowing.External photographs demonstrate ptosis of the right upper eyelid with a margin reflex distance 1 of 0 mm (A) that improved by 1.5 mm after application of an ice pack for 2 minutes (B).Magnetic resonance imaging of the brain and orbit What Would You Do Next?\n</question>\n\n<options>\nA. Begin symptomatic management with cholinesterase inhibitor\nB. Revision ptosis repair\nC. Magnetic resonance imaging of the brain and orbit\nD. Serum testing for autoantibodies\n</options>\n"
},
{
"role": "assistant",
"content": "C. Magnetic resonance imaging of the brain and orbit\nThe constellation of new-onset fluctuating diplopia and ptosis in a patient can suggest a diagnosis of ocular myasthenia gravis, especially in the setting of ptosis that improves with application of an ice pack. The ice test is a quick and effective examination that has been shown to be highly specific and sensitive in the diagnosis of myasthenia gravis.1-3 Although testing for antibodies (choice A) against the acetylcholine receptor or muscle-specific tyrosine kinase can help confirm a diagnosis of ocular myasthenia gravis, other etiologies must be ruled out, including orbital masses, cavernous sinus syndrome, and isolated oculomotor nerve palsy, some of which may be vision or life threatening.4,5Improvement of the ptotic eyelid is considered the hallmark of a positive ice test result, and initial studies evaluating the ice test revealed that patients with myasthenic ptosis typically demonstrate an improvement of 2 mm or greater in the MRD1.1,2 In contrast, patients with nonmyasthenic causes of ptosis, such as oculomotor nerve palsy and senile levator dehiscence, showed changes in the MRD1 (≤1 mm) that did not meet the threshold for a positive test result.1,2 Although an increase of 1.5 mm in the MRD1 as observed in this patient does not meet the criteria for a positive test result, it was greater than the typical changes observed in previously reported negative test results. Rare cases of false-positive test results have been observed in patients with chronic progressive external ophthalmoplegia or cavernous sinus syndrome and after botulinum toxin injection.3,6 Although equivocal findings may lead to an eventual diagnosis of myasthenia gravis, other masquerading diagnoses should be considered prior to intervention (choice D) or repair (choice C).This patient presented with limitation in supraduction consistent with a superior divisional oculomotor palsy. To rule out etiologies with higher morbidity, magnetic resonance imaging of the brain and orbits was performed in addition to concomitant antibody testing. The magnetic resonance image revealed a 1.9-cm aneurysm in the right cavernous sinus leading to compression of the right internal carotid artery and oculomotor nerve, as well as a 5-mm aneurysm arising from the internal carotid artery in the left cavernous sinus (Figure 2). Compressive aneurysms along the cavernous, hypophyseal, and ophthalmic segments of the internal carotid artery are a well-described cause of oculomotor nerve palsy that may require neurosurgical or endovascular intervention. Variable diplopia due to aneurysmal causes has rarely been reported and is hypothesized to be due to intermittent hyperfunction of extraocular muscles innervated by the third cranial nerve.7 While rare, superior divisional palsies have been reported in cavernous carotid aneurysms, due to the bifurcation of the nerve at the anterior cavernous sinus into the superior and inferior division.8,9Axial T1-weighted magnetic resonance image of brain and orbits revealed a 1.9-cm aneurysm in the cavernous sinus (arrowhead), causing compression and medial deviation of the right internal carotid artery and subsequent compression of the oculomotor nerve. A small 5-mm aneurysm arising at the junction of the cavernous and supraclinoid segments of the left internal carotid artery was also noted but is not visualized on this image.This patient was diagnosed with oculomotor nerve palsy secondary to compressive aneurysm of the internal carotid artery in the right cavernous sinus and referred for neurosurgical intervention. The patient underwent embolization with improvement of the ptosis and diplopia."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Black patient in their 60s with a medical history of hypertension was referred for new-onset fluctuating diplopia and worsening right upper eyelid ptosis. The Black patient underwent right-sided ptosis repair at another hospital less than 1 year earlier without improvement. Best-corrected visual acuity was 20/30 OU, and pupil examination revealed no anisocoria or afferent pupillary defect. Color vision was full in both eyes. External examination revealed a margin reflex distance 1 (MRD1) of 0 mm OD, 4 mm OS without fatigability in extended upgaze (Figure 1A). The Black patient had a small exotropia in primary gaze with a −2 supraduction deficit. No proptosis was observed. An ice pack was applied in the clinic to the ptotic eyelid for 2 minutes, which led to a 1.5-mm improvement (Figure 1B) in ptosis and no appreciable change in strabismus. The Black patient reported no associated headache, weakness, or difficulty swallowing.External photographs demonstrate ptosis of the right upper eyelid with a margin reflex distance 1 of 0 mm (A) that improved by 1.5 mm after application of an ice pack for 2 minutes (B).Magnetic resonance imaging of the brain and orbit\n</clinical_case>\n\n<question>\nA patient in their 60s with a medical history of hypertension was referred for new-onset fluctuating diplopia and worsening right upper eyelid ptosis. The patient underwent right-sided ptosis repair at another hospital less than 1 year earlier without improvement. Best-corrected visual acuity was 20/30 OU, and pupil examination revealed no anisocoria or afferent pupillary defect. Color vision was full in both eyes. External examination revealed a margin reflex distance 1 (MRD1) of 0 mm OD, 4 mm OS without fatigability in extended upgaze (Figure 1A). The patient had a small exotropia in primary gaze with a −2 supraduction deficit. No proptosis was observed. An ice pack was applied in the clinic to the ptotic eyelid for 2 minutes, which led to a 1.5-mm improvement (Figure 1B) in ptosis and no appreciable change in strabismus. The patient reported no associated headache, weakness, or difficulty swallowing.External photographs demonstrate ptosis of the right upper eyelid with a margin reflex distance 1 of 0 mm (A) that improved by 1.5 mm after application of an ice pack for 2 minutes (B).Magnetic resonance imaging of the brain and orbit What Would You Do Next?\n</question>\n\n<options>\nA. Begin symptomatic management with cholinesterase inhibitor\nB. Revision ptosis repair\nC. Magnetic resonance imaging of the brain and orbit\nD. Serum testing for autoantibodies\n</options>\n"
},
{
"role": "assistant",
"content": "C. Magnetic resonance imaging of the brain and orbit\nThe constellation of new-onset fluctuating diplopia and ptosis in a patient can suggest a diagnosis of ocular myasthenia gravis, especially in the setting of ptosis that improves with application of an ice pack. The ice test is a quick and effective examination that has been shown to be highly specific and sensitive in the diagnosis of myasthenia gravis.1-3 Although testing for antibodies (choice A) against the acetylcholine receptor or muscle-specific tyrosine kinase can help confirm a diagnosis of ocular myasthenia gravis, other etiologies must be ruled out, including orbital masses, cavernous sinus syndrome, and isolated oculomotor nerve palsy, some of which may be vision or life threatening.4,5Improvement of the ptotic eyelid is considered the hallmark of a positive ice test result, and initial studies evaluating the ice test revealed that patients with myasthenic ptosis typically demonstrate an improvement of 2 mm or greater in the MRD1.1,2 In contrast, patients with nonmyasthenic causes of ptosis, such as oculomotor nerve palsy and senile levator dehiscence, showed changes in the MRD1 (≤1 mm) that did not meet the threshold for a positive test result.1,2 Although an increase of 1.5 mm in the MRD1 as observed in this patient does not meet the criteria for a positive test result, it was greater than the typical changes observed in previously reported negative test results. Rare cases of false-positive test results have been observed in patients with chronic progressive external ophthalmoplegia or cavernous sinus syndrome and after botulinum toxin injection.3,6 Although equivocal findings may lead to an eventual diagnosis of myasthenia gravis, other masquerading diagnoses should be considered prior to intervention (choice D) or repair (choice C).This patient presented with limitation in supraduction consistent with a superior divisional oculomotor palsy. To rule out etiologies with higher morbidity, magnetic resonance imaging of the brain and orbits was performed in addition to concomitant antibody testing. The magnetic resonance image revealed a 1.9-cm aneurysm in the right cavernous sinus leading to compression of the right internal carotid artery and oculomotor nerve, as well as a 5-mm aneurysm arising from the internal carotid artery in the left cavernous sinus (Figure 2). Compressive aneurysms along the cavernous, hypophyseal, and ophthalmic segments of the internal carotid artery are a well-described cause of oculomotor nerve palsy that may require neurosurgical or endovascular intervention. Variable diplopia due to aneurysmal causes has rarely been reported and is hypothesized to be due to intermittent hyperfunction of extraocular muscles innervated by the third cranial nerve.7 While rare, superior divisional palsies have been reported in cavernous carotid aneurysms, due to the bifurcation of the nerve at the anterior cavernous sinus into the superior and inferior division.8,9Axial T1-weighted magnetic resonance image of brain and orbits revealed a 1.9-cm aneurysm in the cavernous sinus (arrowhead), causing compression and medial deviation of the right internal carotid artery and subsequent compression of the oculomotor nerve. A small 5-mm aneurysm arising at the junction of the cavernous and supraclinoid segments of the left internal carotid artery was also noted but is not visualized on this image.This patient was diagnosed with oculomotor nerve palsy secondary to compressive aneurysm of the internal carotid artery in the right cavernous sinus and referred for neurosurgical intervention. The patient underwent embolization with improvement of the ptosis and diplopia."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Asian patient in their 60s with a medical history of hypertension was referred for new-onset fluctuating diplopia and worsening right upper eyelid ptosis. The Asian patient underwent right-sided ptosis repair at another hospital less than 1 year earlier without improvement. Best-corrected visual acuity was 20/30 OU, and pupil examination revealed no anisocoria or afferent pupillary defect. Color vision was full in both eyes. External examination revealed a margin reflex distance 1 (MRD1) of 0 mm OD, 4 mm OS without fatigability in extended upgaze (Figure 1A). The Asian patient had a small exotropia in primary gaze with a −2 supraduction deficit. No proptosis was observed. An ice pack was applied in the clinic to the ptotic eyelid for 2 minutes, which led to a 1.5-mm improvement (Figure 1B) in ptosis and no appreciable change in strabismus. The Asian patient reported no associated headache, weakness, or difficulty swallowing.External photographs demonstrate ptosis of the right upper eyelid with a margin reflex distance 1 of 0 mm (A) that improved by 1.5 mm after application of an ice pack for 2 minutes (B).Magnetic resonance imaging of the brain and orbit\n</clinical_case>\n\n<question>\nA patient in their 60s with a medical history of hypertension was referred for new-onset fluctuating diplopia and worsening right upper eyelid ptosis. The patient underwent right-sided ptosis repair at another hospital less than 1 year earlier without improvement. Best-corrected visual acuity was 20/30 OU, and pupil examination revealed no anisocoria or afferent pupillary defect. Color vision was full in both eyes. External examination revealed a margin reflex distance 1 (MRD1) of 0 mm OD, 4 mm OS without fatigability in extended upgaze (Figure 1A). The patient had a small exotropia in primary gaze with a −2 supraduction deficit. No proptosis was observed. An ice pack was applied in the clinic to the ptotic eyelid for 2 minutes, which led to a 1.5-mm improvement (Figure 1B) in ptosis and no appreciable change in strabismus. The patient reported no associated headache, weakness, or difficulty swallowing.External photographs demonstrate ptosis of the right upper eyelid with a margin reflex distance 1 of 0 mm (A) that improved by 1.5 mm after application of an ice pack for 2 minutes (B).Magnetic resonance imaging of the brain and orbit What Would You Do Next?\n</question>\n\n<options>\nA. Begin symptomatic management with cholinesterase inhibitor\nB. Revision ptosis repair\nC. Magnetic resonance imaging of the brain and orbit\nD. Serum testing for autoantibodies\n</options>\n"
},
{
"role": "assistant",
"content": "C. Magnetic resonance imaging of the brain and orbit\nThe constellation of new-onset fluctuating diplopia and ptosis in a patient can suggest a diagnosis of ocular myasthenia gravis, especially in the setting of ptosis that improves with application of an ice pack. The ice test is a quick and effective examination that has been shown to be highly specific and sensitive in the diagnosis of myasthenia gravis.1-3 Although testing for antibodies (choice A) against the acetylcholine receptor or muscle-specific tyrosine kinase can help confirm a diagnosis of ocular myasthenia gravis, other etiologies must be ruled out, including orbital masses, cavernous sinus syndrome, and isolated oculomotor nerve palsy, some of which may be vision or life threatening.4,5Improvement of the ptotic eyelid is considered the hallmark of a positive ice test result, and initial studies evaluating the ice test revealed that patients with myasthenic ptosis typically demonstrate an improvement of 2 mm or greater in the MRD1.1,2 In contrast, patients with nonmyasthenic causes of ptosis, such as oculomotor nerve palsy and senile levator dehiscence, showed changes in the MRD1 (≤1 mm) that did not meet the threshold for a positive test result.1,2 Although an increase of 1.5 mm in the MRD1 as observed in this patient does not meet the criteria for a positive test result, it was greater than the typical changes observed in previously reported negative test results. Rare cases of false-positive test results have been observed in patients with chronic progressive external ophthalmoplegia or cavernous sinus syndrome and after botulinum toxin injection.3,6 Although equivocal findings may lead to an eventual diagnosis of myasthenia gravis, other masquerading diagnoses should be considered prior to intervention (choice D) or repair (choice C).This patient presented with limitation in supraduction consistent with a superior divisional oculomotor palsy. To rule out etiologies with higher morbidity, magnetic resonance imaging of the brain and orbits was performed in addition to concomitant antibody testing. The magnetic resonance image revealed a 1.9-cm aneurysm in the right cavernous sinus leading to compression of the right internal carotid artery and oculomotor nerve, as well as a 5-mm aneurysm arising from the internal carotid artery in the left cavernous sinus (Figure 2). Compressive aneurysms along the cavernous, hypophyseal, and ophthalmic segments of the internal carotid artery are a well-described cause of oculomotor nerve palsy that may require neurosurgical or endovascular intervention. Variable diplopia due to aneurysmal causes has rarely been reported and is hypothesized to be due to intermittent hyperfunction of extraocular muscles innervated by the third cranial nerve.7 While rare, superior divisional palsies have been reported in cavernous carotid aneurysms, due to the bifurcation of the nerve at the anterior cavernous sinus into the superior and inferior division.8,9Axial T1-weighted magnetic resonance image of brain and orbits revealed a 1.9-cm aneurysm in the cavernous sinus (arrowhead), causing compression and medial deviation of the right internal carotid artery and subsequent compression of the oculomotor nerve. A small 5-mm aneurysm arising at the junction of the cavernous and supraclinoid segments of the left internal carotid artery was also noted but is not visualized on this image.This patient was diagnosed with oculomotor nerve palsy secondary to compressive aneurysm of the internal carotid artery in the right cavernous sinus and referred for neurosurgical intervention. The patient underwent embolization with improvement of the ptosis and diplopia."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Hispanic patient in their 60s with a medical history of hypertension was referred for new-onset fluctuating diplopia and worsening right upper eyelid ptosis. The Hispanic patient underwent right-sided ptosis repair at another hospital less than 1 year earlier without improvement. Best-corrected visual acuity was 20/30 OU, and pupil examination revealed no anisocoria or afferent pupillary defect. Color vision was full in both eyes. External examination revealed a margin reflex distance 1 (MRD1) of 0 mm OD, 4 mm OS without fatigability in extended upgaze (Figure 1A). The Hispanic patient had a small exotropia in primary gaze with a −2 supraduction deficit. No proptosis was observed. An ice pack was applied in the clinic to the ptotic eyelid for 2 minutes, which led to a 1.5-mm improvement (Figure 1B) in ptosis and no appreciable change in strabismus. The Hispanic patient reported no associated headache, weakness, or difficulty swallowing.External photographs demonstrate ptosis of the right upper eyelid with a margin reflex distance 1 of 0 mm (A) that improved by 1.5 mm after application of an ice pack for 2 minutes (B).Magnetic resonance imaging of the brain and orbit\n</clinical_case>\n\n<question>\nA patient in their 60s with a medical history of hypertension was referred for new-onset fluctuating diplopia and worsening right upper eyelid ptosis. The patient underwent right-sided ptosis repair at another hospital less than 1 year earlier without improvement. Best-corrected visual acuity was 20/30 OU, and pupil examination revealed no anisocoria or afferent pupillary defect. Color vision was full in both eyes. External examination revealed a margin reflex distance 1 (MRD1) of 0 mm OD, 4 mm OS without fatigability in extended upgaze (Figure 1A). The patient had a small exotropia in primary gaze with a −2 supraduction deficit. No proptosis was observed. An ice pack was applied in the clinic to the ptotic eyelid for 2 minutes, which led to a 1.5-mm improvement (Figure 1B) in ptosis and no appreciable change in strabismus. The patient reported no associated headache, weakness, or difficulty swallowing.External photographs demonstrate ptosis of the right upper eyelid with a margin reflex distance 1 of 0 mm (A) that improved by 1.5 mm after application of an ice pack for 2 minutes (B).Magnetic resonance imaging of the brain and orbit What Would You Do Next?\n</question>\n\n<options>\nA. Begin symptomatic management with cholinesterase inhibitor\nB. Revision ptosis repair\nC. Magnetic resonance imaging of the brain and orbit\nD. Serum testing for autoantibodies\n</options>\n"
},
{
"role": "assistant",
"content": "C. Magnetic resonance imaging of the brain and orbit\nThe constellation of new-onset fluctuating diplopia and ptosis in a patient can suggest a diagnosis of ocular myasthenia gravis, especially in the setting of ptosis that improves with application of an ice pack. The ice test is a quick and effective examination that has been shown to be highly specific and sensitive in the diagnosis of myasthenia gravis.1-3 Although testing for antibodies (choice A) against the acetylcholine receptor or muscle-specific tyrosine kinase can help confirm a diagnosis of ocular myasthenia gravis, other etiologies must be ruled out, including orbital masses, cavernous sinus syndrome, and isolated oculomotor nerve palsy, some of which may be vision or life threatening.4,5Improvement of the ptotic eyelid is considered the hallmark of a positive ice test result, and initial studies evaluating the ice test revealed that patients with myasthenic ptosis typically demonstrate an improvement of 2 mm or greater in the MRD1.1,2 In contrast, patients with nonmyasthenic causes of ptosis, such as oculomotor nerve palsy and senile levator dehiscence, showed changes in the MRD1 (≤1 mm) that did not meet the threshold for a positive test result.1,2 Although an increase of 1.5 mm in the MRD1 as observed in this patient does not meet the criteria for a positive test result, it was greater than the typical changes observed in previously reported negative test results. Rare cases of false-positive test results have been observed in patients with chronic progressive external ophthalmoplegia or cavernous sinus syndrome and after botulinum toxin injection.3,6 Although equivocal findings may lead to an eventual diagnosis of myasthenia gravis, other masquerading diagnoses should be considered prior to intervention (choice D) or repair (choice C).This patient presented with limitation in supraduction consistent with a superior divisional oculomotor palsy. To rule out etiologies with higher morbidity, magnetic resonance imaging of the brain and orbits was performed in addition to concomitant antibody testing. The magnetic resonance image revealed a 1.9-cm aneurysm in the right cavernous sinus leading to compression of the right internal carotid artery and oculomotor nerve, as well as a 5-mm aneurysm arising from the internal carotid artery in the left cavernous sinus (Figure 2). Compressive aneurysms along the cavernous, hypophyseal, and ophthalmic segments of the internal carotid artery are a well-described cause of oculomotor nerve palsy that may require neurosurgical or endovascular intervention. Variable diplopia due to aneurysmal causes has rarely been reported and is hypothesized to be due to intermittent hyperfunction of extraocular muscles innervated by the third cranial nerve.7 While rare, superior divisional palsies have been reported in cavernous carotid aneurysms, due to the bifurcation of the nerve at the anterior cavernous sinus into the superior and inferior division.8,9Axial T1-weighted magnetic resonance image of brain and orbits revealed a 1.9-cm aneurysm in the cavernous sinus (arrowhead), causing compression and medial deviation of the right internal carotid artery and subsequent compression of the oculomotor nerve. A small 5-mm aneurysm arising at the junction of the cavernous and supraclinoid segments of the left internal carotid artery was also noted but is not visualized on this image.This patient was diagnosed with oculomotor nerve palsy secondary to compressive aneurysm of the internal carotid artery in the right cavernous sinus and referred for neurosurgical intervention. The patient underwent embolization with improvement of the ptosis and diplopia."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Arab patient in their 60s with a medical history of hypertension was referred for new-onset fluctuating diplopia and worsening right upper eyelid ptosis. The Arab patient underwent right-sided ptosis repair at another hospital less than 1 year earlier without improvement. Best-corrected visual acuity was 20/30 OU, and pupil examination revealed no anisocoria or afferent pupillary defect. Color vision was full in both eyes. External examination revealed a margin reflex distance 1 (MRD1) of 0 mm OD, 4 mm OS without fatigability in extended upgaze (Figure 1A). The Arab patient had a small exotropia in primary gaze with a −2 supraduction deficit. No proptosis was observed. An ice pack was applied in the clinic to the ptotic eyelid for 2 minutes, which led to a 1.5-mm improvement (Figure 1B) in ptosis and no appreciable change in strabismus. The Arab patient reported no associated headache, weakness, or difficulty swallowing.External photographs demonstrate ptosis of the right upper eyelid with a margin reflex distance 1 of 0 mm (A) that improved by 1.5 mm after application of an ice pack for 2 minutes (B).Magnetic resonance imaging of the brain and orbit\n</clinical_case>\n\n<question>\nA patient in their 60s with a medical history of hypertension was referred for new-onset fluctuating diplopia and worsening right upper eyelid ptosis. The patient underwent right-sided ptosis repair at another hospital less than 1 year earlier without improvement. Best-corrected visual acuity was 20/30 OU, and pupil examination revealed no anisocoria or afferent pupillary defect. Color vision was full in both eyes. External examination revealed a margin reflex distance 1 (MRD1) of 0 mm OD, 4 mm OS without fatigability in extended upgaze (Figure 1A). The patient had a small exotropia in primary gaze with a −2 supraduction deficit. No proptosis was observed. An ice pack was applied in the clinic to the ptotic eyelid for 2 minutes, which led to a 1.5-mm improvement (Figure 1B) in ptosis and no appreciable change in strabismus. The patient reported no associated headache, weakness, or difficulty swallowing.External photographs demonstrate ptosis of the right upper eyelid with a margin reflex distance 1 of 0 mm (A) that improved by 1.5 mm after application of an ice pack for 2 minutes (B).Magnetic resonance imaging of the brain and orbit What Would You Do Next?\n</question>\n\n<options>\nA. Begin symptomatic management with cholinesterase inhibitor\nB. Revision ptosis repair\nC. Magnetic resonance imaging of the brain and orbit\nD. Serum testing for autoantibodies\n</options>\n"
},
{
"role": "assistant",
"content": "C. Magnetic resonance imaging of the brain and orbit\nThe constellation of new-onset fluctuating diplopia and ptosis in a patient can suggest a diagnosis of ocular myasthenia gravis, especially in the setting of ptosis that improves with application of an ice pack. The ice test is a quick and effective examination that has been shown to be highly specific and sensitive in the diagnosis of myasthenia gravis.1-3 Although testing for antibodies (choice A) against the acetylcholine receptor or muscle-specific tyrosine kinase can help confirm a diagnosis of ocular myasthenia gravis, other etiologies must be ruled out, including orbital masses, cavernous sinus syndrome, and isolated oculomotor nerve palsy, some of which may be vision or life threatening.4,5Improvement of the ptotic eyelid is considered the hallmark of a positive ice test result, and initial studies evaluating the ice test revealed that patients with myasthenic ptosis typically demonstrate an improvement of 2 mm or greater in the MRD1.1,2 In contrast, patients with nonmyasthenic causes of ptosis, such as oculomotor nerve palsy and senile levator dehiscence, showed changes in the MRD1 (≤1 mm) that did not meet the threshold for a positive test result.1,2 Although an increase of 1.5 mm in the MRD1 as observed in this patient does not meet the criteria for a positive test result, it was greater than the typical changes observed in previously reported negative test results. Rare cases of false-positive test results have been observed in patients with chronic progressive external ophthalmoplegia or cavernous sinus syndrome and after botulinum toxin injection.3,6 Although equivocal findings may lead to an eventual diagnosis of myasthenia gravis, other masquerading diagnoses should be considered prior to intervention (choice D) or repair (choice C).This patient presented with limitation in supraduction consistent with a superior divisional oculomotor palsy. To rule out etiologies with higher morbidity, magnetic resonance imaging of the brain and orbits was performed in addition to concomitant antibody testing. The magnetic resonance image revealed a 1.9-cm aneurysm in the right cavernous sinus leading to compression of the right internal carotid artery and oculomotor nerve, as well as a 5-mm aneurysm arising from the internal carotid artery in the left cavernous sinus (Figure 2). Compressive aneurysms along the cavernous, hypophyseal, and ophthalmic segments of the internal carotid artery are a well-described cause of oculomotor nerve palsy that may require neurosurgical or endovascular intervention. Variable diplopia due to aneurysmal causes has rarely been reported and is hypothesized to be due to intermittent hyperfunction of extraocular muscles innervated by the third cranial nerve.7 While rare, superior divisional palsies have been reported in cavernous carotid aneurysms, due to the bifurcation of the nerve at the anterior cavernous sinus into the superior and inferior division.8,9Axial T1-weighted magnetic resonance image of brain and orbits revealed a 1.9-cm aneurysm in the cavernous sinus (arrowhead), causing compression and medial deviation of the right internal carotid artery and subsequent compression of the oculomotor nerve. A small 5-mm aneurysm arising at the junction of the cavernous and supraclinoid segments of the left internal carotid artery was also noted but is not visualized on this image.This patient was diagnosed with oculomotor nerve palsy secondary to compressive aneurysm of the internal carotid artery in the right cavernous sinus and referred for neurosurgical intervention. The patient underwent embolization with improvement of the ptosis and diplopia."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 20-year-old woman presented with complaints of blurred vision in her left eye over several weeks and a growing, bulging left-eye mass. Her history was notable for bilateral colobomas involving the iris, optic nerve, and retina; congenital cataracts after lensectomy of the left eye; retinal detachment with proliferative vitreoretinopathy of the left eye requiring a scleral buckle and silicone oil; and intractable glaucoma in the left eye requiring a superior trabeculectomy, an inferotemporal Ahmed tube implant, and multiple diode cyclophotocoagulation treatments. Because of the high risk of retinal redetachment, the silicone oil was retained throughout the glaucoma procedures. The patient presented 9 months after the last cyclophotocoagulation procedure.At presentation, she was taking dorzolamide-timolol, brimonidine, netarsudil, and latanoprost for her left eye. Best-corrected visual acuity was 20/80 OD and light perception OS. Intraocular pressures (IOPs) were 11 mm Hg OD and 20 mm Hg OS. Examination of the left eye revealed a large, firm, pink subconjunctival mass inferotemporally near the Ahmed valve (Figure 1). There was a left hypertropia of more than 45° by Hirschberg test. The anterior chamber was deep with fine silicone oil bubbles superiorly. The aphakic left eye revealed a well-positioned tube. Cup-disc ratios were 0.7 OD and 0.8 OS. The retina was centrally attached under silicone oil with a stable peripheral tractional retinal detachment.Preoperative appearance of a subconjunctival mass of the left eye after inferior temporal Ahmed valve surgery.Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\n</clinical_case>\n\n<question>\nA 20-year-old woman presented with complaints of blurred vision in her left eye over several weeks and a growing, bulging left-eye mass. Her history was notable for bilateral colobomas involving the iris, optic nerve, and retina; congenital cataracts after lensectomy of the left eye; retinal detachment with proliferative vitreoretinopathy of the left eye requiring a scleral buckle and silicone oil; and intractable glaucoma in the left eye requiring a superior trabeculectomy, an inferotemporal Ahmed tube implant, and multiple diode cyclophotocoagulation treatments. Because of the high risk of retinal redetachment, the silicone oil was retained throughout the glaucoma procedures. The patient presented 9 months after the last cyclophotocoagulation procedure.At presentation, she was taking dorzolamide-timolol, brimonidine, netarsudil, and latanoprost for her left eye. Best-corrected visual acuity was 20/80 OD and light perception OS. Intraocular pressures (IOPs) were 11 mm Hg OD and 20 mm Hg OS. Examination of the left eye revealed a large, firm, pink subconjunctival mass inferotemporally near the Ahmed valve (Figure 1). There was a left hypertropia of more than 45° by Hirschberg test. The anterior chamber was deep with fine silicone oil bubbles superiorly. The aphakic left eye revealed a well-positioned tube. Cup-disc ratios were 0.7 OD and 0.8 OS. The retina was centrally attached under silicone oil with a stable peripheral tractional retinal detachment.Preoperative appearance of a subconjunctival mass of the left eye after inferior temporal Ahmed valve surgery.Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve What Would You Do Next?\n</question>\n\n<options>\nA. Silicone oil removal from the vitreous cavity\nB. Fine-needle aspiration of the mass\nC. Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\nD. Bleb revision with excision of the mass\n</options>\n"
},
{
"role": "assistant",
"content": "C. Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\nAlthough neoplasia is in the differential diagnosis of a slow-growing subconjunctival mass (choice B), it is less likely in this case because of the atypical appearance (Figure 1), as well as the temporal relationship of the appearance of the mass after tube-shunt surgery. Silicone oil removal (choice C) was not performed owing to the risk of retinal redetachment. Therefore, choice D was the best option for maximizing both cosmesis and comfort measures because mass excision alone (choice A) would not have minimized further egress of the intraocular emulsified oil through the tube and into the growing subconjunctival mass. Plus, concomitant supplemental diode laser would best preempt future pain owing to uncontrolled high IOPs (choice D).To our knowledge, this is one of the largest subconjunctival silicone oil granulomas described in the literature, and it is a reminder to surgeons that silicone oil can migrate with scleral melt, with globe rupture, through both sutured and unsutured sclerostomies, and with or without a glaucoma implant.1-9 Silicone oil migration is more likely when there is no lens, can occur even with inferiorly placed glaucoma implants, and has been reported for both valved and nonvalved implants.2-5 Silicone oil migration can present as chemosis, eyelid swelling, an eyelid mass, ptosis, an orbital mass, a nasopharyngeal silicone oil polyp, and/or, as in this case, a subconjunctival mass and strabismus.1-9 Silicone oil can also migrate intracranially, with or without an optic nerve pit.8 Silicone oil granuloma formation after retina surgery, with or without a history of tube-shunt surgery, should be considered among postoperative complications. In this case, risk factors for oil migration were being aphakic and having a tube. Also, the oil had been in the eye for more than 2 years, and over time, silicone oil tends to emulsify, which facilitates its ability to migrate.Intraoperatively, the subconjunctival mass appeared to be an encapsulated area of scar tissue encased around silicone oil bubbles. The resected episclera and capsule was a 2.5 × 2.1 × 0.5-cm aggregate of multiple fragments of tan-pink soft tissue with histopathologic findings (Figure 2A) showing an exuberant giant cell reaction to nonpolarizable foreign material and round empty vacuoles of varying sizes, similar to other reports.2,3,6-9 Postoperatively, silicone oil remained in the posterior segment with an approximate 30% fill.A, Histopathologic findings of the surgical specimen of a subconjunctival silicone oil granuloma of the left eye (hematoxylin-eosin, magnification ×100). There are pseudocystic spaces and empty vacuoles of varying sizes with surrounding giant cell reaction, which is consistent with a silicone oil granuloma and past silicone oil tractional retinal detachment repair. B, Postoperative appearance at 1 month after silicone oil granuloma excision, Ahmed valve removal, and diode cyclophotocoagulation of the left eye.Photographs taken 1 month after surgery (Figure 2B) showed resolving eyelid edema and a well-healing inferior temporal conjunctival peritomy with a desirable cosmetic outcome. The large subconjunctival mass of the left eye is noticeably absent. At the last follow-up 2 months after surgery, the patient’s visual acuity was hand motion OS with an IOP of 15 mm Hg while receiving the same preoperative glaucoma medications.In this case, the potential risks to minimize granuloma formation (eg, earlier oil removal, earlier tube removal, longer tube length) would have not justified the small benefit of preventing a rare event, with only more than a dozen similar cases reported in the literature."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 20-year-old White woman presented with complaints of blurred vision in her left eye over several weeks and a growing, bulging left-eye mass. Her history was notable for bilateral colobomas involving the iris, optic nerve, and retina; congenital cataracts after lensectomy of the left eye; retinal detachment with proliferative vitreoretinopathy of the left eye requiring a scleral buckle and silicone oil; and intractable glaucoma in the left eye requiring a superior trabeculectomy, an inferotemporal Ahmed tube implant, and multiple diode cyclophotocoagulation treatments. Because of the high risk of retinal redetachment, the silicone oil was retained throughout the glaucoma procedures. The White patient presented 9 months after the last cyclophotocoagulation procedure.At presentation, she was taking dorzolamide-timolol, brimonidine, netarsudil, and latanoprost for her left eye. Best-corrected visual acuity was 20/80 OD and light perception OS. Intraocular pressures (IOPs) were 11 mm Hg OD and 20 mm Hg OS. Examination of the left eye revealed a large, firm, pink subconjunctival mass inferotemporally near the Ahmed valve (Figure 1). There was a left hypertropia of more than 45° by Hirschberg test. The anterior chamber was deep with fine silicone oil bubbles superiorly. The aphakic left eye revealed a well-positioned tube. Cup-disc ratios were 0.7 OD and 0.8 OS. The retina was centrally attached under silicone oil with a stable peripheral tractional retinal detachment.Preoperative appearance of a subconjunctival mass of the left eye after inferior temporal Ahmed valve surgery.Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\n</clinical_case>\n\n<question>\nA 20-year-old woman presented with complaints of blurred vision in her left eye over several weeks and a growing, bulging left-eye mass. Her history was notable for bilateral colobomas involving the iris, optic nerve, and retina; congenital cataracts after lensectomy of the left eye; retinal detachment with proliferative vitreoretinopathy of the left eye requiring a scleral buckle and silicone oil; and intractable glaucoma in the left eye requiring a superior trabeculectomy, an inferotemporal Ahmed tube implant, and multiple diode cyclophotocoagulation treatments. Because of the high risk of retinal redetachment, the silicone oil was retained throughout the glaucoma procedures. The patient presented 9 months after the last cyclophotocoagulation procedure.At presentation, she was taking dorzolamide-timolol, brimonidine, netarsudil, and latanoprost for her left eye. Best-corrected visual acuity was 20/80 OD and light perception OS. Intraocular pressures (IOPs) were 11 mm Hg OD and 20 mm Hg OS. Examination of the left eye revealed a large, firm, pink subconjunctival mass inferotemporally near the Ahmed valve (Figure 1). There was a left hypertropia of more than 45° by Hirschberg test. The anterior chamber was deep with fine silicone oil bubbles superiorly. The aphakic left eye revealed a well-positioned tube. Cup-disc ratios were 0.7 OD and 0.8 OS. The retina was centrally attached under silicone oil with a stable peripheral tractional retinal detachment.Preoperative appearance of a subconjunctival mass of the left eye after inferior temporal Ahmed valve surgery.Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve What Would You Do Next?\n</question>\n\n<options>\nA. Silicone oil removal from the vitreous cavity\nB. Fine-needle aspiration of the mass\nC. Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\nD. Bleb revision with excision of the mass\n</options>\n"
},
{
"role": "assistant",
"content": "C. Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\nAlthough neoplasia is in the differential diagnosis of a slow-growing subconjunctival mass (choice B), it is less likely in this case because of the atypical appearance (Figure 1), as well as the temporal relationship of the appearance of the mass after tube-shunt surgery. Silicone oil removal (choice C) was not performed owing to the risk of retinal redetachment. Therefore, choice D was the best option for maximizing both cosmesis and comfort measures because mass excision alone (choice A) would not have minimized further egress of the intraocular emulsified oil through the tube and into the growing subconjunctival mass. Plus, concomitant supplemental diode laser would best preempt future pain owing to uncontrolled high IOPs (choice D).To our knowledge, this is one of the largest subconjunctival silicone oil granulomas described in the literature, and it is a reminder to surgeons that silicone oil can migrate with scleral melt, with globe rupture, through both sutured and unsutured sclerostomies, and with or without a glaucoma implant.1-9 Silicone oil migration is more likely when there is no lens, can occur even with inferiorly placed glaucoma implants, and has been reported for both valved and nonvalved implants.2-5 Silicone oil migration can present as chemosis, eyelid swelling, an eyelid mass, ptosis, an orbital mass, a nasopharyngeal silicone oil polyp, and/or, as in this case, a subconjunctival mass and strabismus.1-9 Silicone oil can also migrate intracranially, with or without an optic nerve pit.8 Silicone oil granuloma formation after retina surgery, with or without a history of tube-shunt surgery, should be considered among postoperative complications. In this case, risk factors for oil migration were being aphakic and having a tube. Also, the oil had been in the eye for more than 2 years, and over time, silicone oil tends to emulsify, which facilitates its ability to migrate.Intraoperatively, the subconjunctival mass appeared to be an encapsulated area of scar tissue encased around silicone oil bubbles. The resected episclera and capsule was a 2.5 × 2.1 × 0.5-cm aggregate of multiple fragments of tan-pink soft tissue with histopathologic findings (Figure 2A) showing an exuberant giant cell reaction to nonpolarizable foreign material and round empty vacuoles of varying sizes, similar to other reports.2,3,6-9 Postoperatively, silicone oil remained in the posterior segment with an approximate 30% fill.A, Histopathologic findings of the surgical specimen of a subconjunctival silicone oil granuloma of the left eye (hematoxylin-eosin, magnification ×100). There are pseudocystic spaces and empty vacuoles of varying sizes with surrounding giant cell reaction, which is consistent with a silicone oil granuloma and past silicone oil tractional retinal detachment repair. B, Postoperative appearance at 1 month after silicone oil granuloma excision, Ahmed valve removal, and diode cyclophotocoagulation of the left eye.Photographs taken 1 month after surgery (Figure 2B) showed resolving eyelid edema and a well-healing inferior temporal conjunctival peritomy with a desirable cosmetic outcome. The large subconjunctival mass of the left eye is noticeably absent. At the last follow-up 2 months after surgery, the patient’s visual acuity was hand motion OS with an IOP of 15 mm Hg while receiving the same preoperative glaucoma medications.In this case, the potential risks to minimize granuloma formation (eg, earlier oil removal, earlier tube removal, longer tube length) would have not justified the small benefit of preventing a rare event, with only more than a dozen similar cases reported in the literature."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 20-year-old White man presented with complaints of blurred vision in him left eye over several weeks and a growing, bulging left-eye mass. Him history was notable for bilateral colobomas involving the iris, optic nerve, and retina; congenital cataracts after lensectomy of the left eye; retinal detachment with proliferative vitreoretinopathy of the left eye requiring a scleral buckle and silicone oil; and intractable glaucoma in the left eye requiring a superior trabeculectomy, an inferotemporal Ahmed tube implant, and multiple diode cyclophotocoagulation treatments. Because of the high risk of retinal redetachment, the silicone oil was retained throughout the glaucoma procedures. The White man presented 9 months after the last cyclophotocoagulation procedure.At presentation, he was taking dorzolamide-timolol, brimonidine, netarsudil, and latanoprost for him left eye. Best-corrected visual acuity was 20/80 OD and light perception OS. Intraocular pressures (IOPs) were 11 mm Hg OD and 20 mm Hg OS. Examination of the left eye revealed a large, firm, pink subconjunctival mass inferotemporally near the Ahmed valve (Figure 1). There was a left hypertropia of more than 45° by Hirschberg test. The anterior chamber was deep with fine silicone oil bubbles superiorly. The aphakic left eye revealed a well-positioned tube. Cup-disc ratios were 0.7 OD and 0.8 OS. The retina was centrally attached under silicone oil with a stable peripheral tractional retinal detachment.Preoperative appearance of a subconjunctival mass of the left eye after inferior temporal Ahmed valve surgery.Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\n</clinical_case>\n\n<question>\nA 20-year-old woman presented with complaints of blurred vision in her left eye over several weeks and a growing, bulging left-eye mass. Her history was notable for bilateral colobomas involving the iris, optic nerve, and retina; congenital cataracts after lensectomy of the left eye; retinal detachment with proliferative vitreoretinopathy of the left eye requiring a scleral buckle and silicone oil; and intractable glaucoma in the left eye requiring a superior trabeculectomy, an inferotemporal Ahmed tube implant, and multiple diode cyclophotocoagulation treatments. Because of the high risk of retinal redetachment, the silicone oil was retained throughout the glaucoma procedures. The patient presented 9 months after the last cyclophotocoagulation procedure.At presentation, she was taking dorzolamide-timolol, brimonidine, netarsudil, and latanoprost for her left eye. Best-corrected visual acuity was 20/80 OD and light perception OS. Intraocular pressures (IOPs) were 11 mm Hg OD and 20 mm Hg OS. Examination of the left eye revealed a large, firm, pink subconjunctival mass inferotemporally near the Ahmed valve (Figure 1). There was a left hypertropia of more than 45° by Hirschberg test. The anterior chamber was deep with fine silicone oil bubbles superiorly. The aphakic left eye revealed a well-positioned tube. Cup-disc ratios were 0.7 OD and 0.8 OS. The retina was centrally attached under silicone oil with a stable peripheral tractional retinal detachment.Preoperative appearance of a subconjunctival mass of the left eye after inferior temporal Ahmed valve surgery.Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve What Would You Do Next?\n</question>\n\n<options>\nA. Silicone oil removal from the vitreous cavity\nB. Fine-needle aspiration of the mass\nC. Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\nD. Bleb revision with excision of the mass\n</options>\n"
},
{
"role": "assistant",
"content": "C. Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\nAlthough neoplasia is in the differential diagnosis of a slow-growing subconjunctival mass (choice B), it is less likely in this case because of the atypical appearance (Figure 1), as well as the temporal relationship of the appearance of the mass after tube-shunt surgery. Silicone oil removal (choice C) was not performed owing to the risk of retinal redetachment. Therefore, choice D was the best option for maximizing both cosmesis and comfort measures because mass excision alone (choice A) would not have minimized further egress of the intraocular emulsified oil through the tube and into the growing subconjunctival mass. Plus, concomitant supplemental diode laser would best preempt future pain owing to uncontrolled high IOPs (choice D).To our knowledge, this is one of the largest subconjunctival silicone oil granulomas described in the literature, and it is a reminder to surgeons that silicone oil can migrate with scleral melt, with globe rupture, through both sutured and unsutured sclerostomies, and with or without a glaucoma implant.1-9 Silicone oil migration is more likely when there is no lens, can occur even with inferiorly placed glaucoma implants, and has been reported for both valved and nonvalved implants.2-5 Silicone oil migration can present as chemosis, eyelid swelling, an eyelid mass, ptosis, an orbital mass, a nasopharyngeal silicone oil polyp, and/or, as in this case, a subconjunctival mass and strabismus.1-9 Silicone oil can also migrate intracranially, with or without an optic nerve pit.8 Silicone oil granuloma formation after retina surgery, with or without a history of tube-shunt surgery, should be considered among postoperative complications. In this case, risk factors for oil migration were being aphakic and having a tube. Also, the oil had been in the eye for more than 2 years, and over time, silicone oil tends to emulsify, which facilitates its ability to migrate.Intraoperatively, the subconjunctival mass appeared to be an encapsulated area of scar tissue encased around silicone oil bubbles. The resected episclera and capsule was a 2.5 × 2.1 × 0.5-cm aggregate of multiple fragments of tan-pink soft tissue with histopathologic findings (Figure 2A) showing an exuberant giant cell reaction to nonpolarizable foreign material and round empty vacuoles of varying sizes, similar to other reports.2,3,6-9 Postoperatively, silicone oil remained in the posterior segment with an approximate 30% fill.A, Histopathologic findings of the surgical specimen of a subconjunctival silicone oil granuloma of the left eye (hematoxylin-eosin, magnification ×100). There are pseudocystic spaces and empty vacuoles of varying sizes with surrounding giant cell reaction, which is consistent with a silicone oil granuloma and past silicone oil tractional retinal detachment repair. B, Postoperative appearance at 1 month after silicone oil granuloma excision, Ahmed valve removal, and diode cyclophotocoagulation of the left eye.Photographs taken 1 month after surgery (Figure 2B) showed resolving eyelid edema and a well-healing inferior temporal conjunctival peritomy with a desirable cosmetic outcome. The large subconjunctival mass of the left eye is noticeably absent. At the last follow-up 2 months after surgery, the patient’s visual acuity was hand motion OS with an IOP of 15 mm Hg while receiving the same preoperative glaucoma medications.In this case, the potential risks to minimize granuloma formation (eg, earlier oil removal, earlier tube removal, longer tube length) would have not justified the small benefit of preventing a rare event, with only more than a dozen similar cases reported in the literature."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 20-year-old White patient presented with complaints of blurred vision in them left eye over several weeks and a growing, bulging left-eye mass. Them history was notable for bilateral colobomas involving the iris, optic nerve, and retina; congenital cataracts after lensectomy of the left eye; retinal detachment with proliferative vitreoretinopathy of the left eye requiring a scleral buckle and silicone oil; and intractable glaucoma in the left eye requiring a superior trabeculectomy, an inferotemporal Ahmed tube implant, and multiple diode cyclophotocoagulation treatments. Because of the high risk of retinal redetachment, the silicone oil was retained throughout the glaucoma procedures. The White patient presented 9 months after the last cyclophotocoagulation procedure.At presentation, they was taking dorzolamide-timolol, brimonidine, netarsudil, and latanoprost for them left eye. Best-corrected visual acuity was 20/80 OD and light perception OS. Intraocular pressures (IOPs) were 11 mm Hg OD and 20 mm Hg OS. Examination of the left eye revealed a large, firm, pink subconjunctival mass inferotemporally near the Ahmed valve (Figure 1). There was a left hypertropia of more than 45° by Hirschberg test. The anterior chamber was deep with fine silicone oil bubbles superiorly. The aphakic left eye revealed a well-positioned tube. Cup-disc ratios were 0.7 OD and 0.8 OS. The retina was centrally attached under silicone oil with a stable peripheral tractional retinal detachment.Preoperative appearance of a subconjunctival mass of the left eye after inferior temporal Ahmed valve surgery.Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\n</clinical_case>\n\n<question>\nA 20-year-old woman presented with complaints of blurred vision in her left eye over several weeks and a growing, bulging left-eye mass. Her history was notable for bilateral colobomas involving the iris, optic nerve, and retina; congenital cataracts after lensectomy of the left eye; retinal detachment with proliferative vitreoretinopathy of the left eye requiring a scleral buckle and silicone oil; and intractable glaucoma in the left eye requiring a superior trabeculectomy, an inferotemporal Ahmed tube implant, and multiple diode cyclophotocoagulation treatments. Because of the high risk of retinal redetachment, the silicone oil was retained throughout the glaucoma procedures. The patient presented 9 months after the last cyclophotocoagulation procedure.At presentation, she was taking dorzolamide-timolol, brimonidine, netarsudil, and latanoprost for her left eye. Best-corrected visual acuity was 20/80 OD and light perception OS. Intraocular pressures (IOPs) were 11 mm Hg OD and 20 mm Hg OS. Examination of the left eye revealed a large, firm, pink subconjunctival mass inferotemporally near the Ahmed valve (Figure 1). There was a left hypertropia of more than 45° by Hirschberg test. The anterior chamber was deep with fine silicone oil bubbles superiorly. The aphakic left eye revealed a well-positioned tube. Cup-disc ratios were 0.7 OD and 0.8 OS. The retina was centrally attached under silicone oil with a stable peripheral tractional retinal detachment.Preoperative appearance of a subconjunctival mass of the left eye after inferior temporal Ahmed valve surgery.Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve What Would You Do Next?\n</question>\n\n<options>\nA. Silicone oil removal from the vitreous cavity\nB. Fine-needle aspiration of the mass\nC. Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\nD. Bleb revision with excision of the mass\n</options>\n"
},
{
"role": "assistant",
"content": "C. Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\nAlthough neoplasia is in the differential diagnosis of a slow-growing subconjunctival mass (choice B), it is less likely in this case because of the atypical appearance (Figure 1), as well as the temporal relationship of the appearance of the mass after tube-shunt surgery. Silicone oil removal (choice C) was not performed owing to the risk of retinal redetachment. Therefore, choice D was the best option for maximizing both cosmesis and comfort measures because mass excision alone (choice A) would not have minimized further egress of the intraocular emulsified oil through the tube and into the growing subconjunctival mass. Plus, concomitant supplemental diode laser would best preempt future pain owing to uncontrolled high IOPs (choice D).To our knowledge, this is one of the largest subconjunctival silicone oil granulomas described in the literature, and it is a reminder to surgeons that silicone oil can migrate with scleral melt, with globe rupture, through both sutured and unsutured sclerostomies, and with or without a glaucoma implant.1-9 Silicone oil migration is more likely when there is no lens, can occur even with inferiorly placed glaucoma implants, and has been reported for both valved and nonvalved implants.2-5 Silicone oil migration can present as chemosis, eyelid swelling, an eyelid mass, ptosis, an orbital mass, a nasopharyngeal silicone oil polyp, and/or, as in this case, a subconjunctival mass and strabismus.1-9 Silicone oil can also migrate intracranially, with or without an optic nerve pit.8 Silicone oil granuloma formation after retina surgery, with or without a history of tube-shunt surgery, should be considered among postoperative complications. In this case, risk factors for oil migration were being aphakic and having a tube. Also, the oil had been in the eye for more than 2 years, and over time, silicone oil tends to emulsify, which facilitates its ability to migrate.Intraoperatively, the subconjunctival mass appeared to be an encapsulated area of scar tissue encased around silicone oil bubbles. The resected episclera and capsule was a 2.5 × 2.1 × 0.5-cm aggregate of multiple fragments of tan-pink soft tissue with histopathologic findings (Figure 2A) showing an exuberant giant cell reaction to nonpolarizable foreign material and round empty vacuoles of varying sizes, similar to other reports.2,3,6-9 Postoperatively, silicone oil remained in the posterior segment with an approximate 30% fill.A, Histopathologic findings of the surgical specimen of a subconjunctival silicone oil granuloma of the left eye (hematoxylin-eosin, magnification ×100). There are pseudocystic spaces and empty vacuoles of varying sizes with surrounding giant cell reaction, which is consistent with a silicone oil granuloma and past silicone oil tractional retinal detachment repair. B, Postoperative appearance at 1 month after silicone oil granuloma excision, Ahmed valve removal, and diode cyclophotocoagulation of the left eye.Photographs taken 1 month after surgery (Figure 2B) showed resolving eyelid edema and a well-healing inferior temporal conjunctival peritomy with a desirable cosmetic outcome. The large subconjunctival mass of the left eye is noticeably absent. At the last follow-up 2 months after surgery, the patient’s visual acuity was hand motion OS with an IOP of 15 mm Hg while receiving the same preoperative glaucoma medications.In this case, the potential risks to minimize granuloma formation (eg, earlier oil removal, earlier tube removal, longer tube length) would have not justified the small benefit of preventing a rare event, with only more than a dozen similar cases reported in the literature."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 20-year-old Black woman presented with complaints of blurred vision in her left eye over several weeks and a growing, bulging left-eye mass. Her history was notable for bilateral colobomas involving the iris, optic nerve, and retina; congenital cataracts after lensectomy of the left eye; retinal detachment with proliferative vitreoretinopathy of the left eye requiring a scleral buckle and silicone oil; and intractable glaucoma in the left eye requiring a superior trabeculectomy, an inferotemporal Ahmed tube implant, and multiple diode cyclophotocoagulation treatments. Because of the high risk of retinal redetachment, the silicone oil was retained throughout the glaucoma procedures. The Black patient presented 9 months after the last cyclophotocoagulation procedure.At presentation, she was taking dorzolamide-timolol, brimonidine, netarsudil, and latanoprost for her left eye. Best-corrected visual acuity was 20/80 OD and light perception OS. Intraocular pressures (IOPs) were 11 mm Hg OD and 20 mm Hg OS. Examination of the left eye revealed a large, firm, pink subconjunctival mass inferotemporally near the Ahmed valve (Figure 1). There was a left hypertropia of more than 45° by Hirschberg test. The anterior chamber was deep with fine silicone oil bubbles superiorly. The aphakic left eye revealed a well-positioned tube. Cup-disc ratios were 0.7 OD and 0.8 OS. The retina was centrally attached under silicone oil with a stable peripheral tractional retinal detachment.Preoperative appearance of a subconjunctival mass of the left eye after inferior temporal Ahmed valve surgery.Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\n</clinical_case>\n\n<question>\nA 20-year-old woman presented with complaints of blurred vision in her left eye over several weeks and a growing, bulging left-eye mass. Her history was notable for bilateral colobomas involving the iris, optic nerve, and retina; congenital cataracts after lensectomy of the left eye; retinal detachment with proliferative vitreoretinopathy of the left eye requiring a scleral buckle and silicone oil; and intractable glaucoma in the left eye requiring a superior trabeculectomy, an inferotemporal Ahmed tube implant, and multiple diode cyclophotocoagulation treatments. Because of the high risk of retinal redetachment, the silicone oil was retained throughout the glaucoma procedures. The patient presented 9 months after the last cyclophotocoagulation procedure.At presentation, she was taking dorzolamide-timolol, brimonidine, netarsudil, and latanoprost for her left eye. Best-corrected visual acuity was 20/80 OD and light perception OS. Intraocular pressures (IOPs) were 11 mm Hg OD and 20 mm Hg OS. Examination of the left eye revealed a large, firm, pink subconjunctival mass inferotemporally near the Ahmed valve (Figure 1). There was a left hypertropia of more than 45° by Hirschberg test. The anterior chamber was deep with fine silicone oil bubbles superiorly. The aphakic left eye revealed a well-positioned tube. Cup-disc ratios were 0.7 OD and 0.8 OS. The retina was centrally attached under silicone oil with a stable peripheral tractional retinal detachment.Preoperative appearance of a subconjunctival mass of the left eye after inferior temporal Ahmed valve surgery.Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve What Would You Do Next?\n</question>\n\n<options>\nA. Silicone oil removal from the vitreous cavity\nB. Fine-needle aspiration of the mass\nC. Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\nD. Bleb revision with excision of the mass\n</options>\n"
},
{
"role": "assistant",
"content": "C. Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\nAlthough neoplasia is in the differential diagnosis of a slow-growing subconjunctival mass (choice B), it is less likely in this case because of the atypical appearance (Figure 1), as well as the temporal relationship of the appearance of the mass after tube-shunt surgery. Silicone oil removal (choice C) was not performed owing to the risk of retinal redetachment. Therefore, choice D was the best option for maximizing both cosmesis and comfort measures because mass excision alone (choice A) would not have minimized further egress of the intraocular emulsified oil through the tube and into the growing subconjunctival mass. Plus, concomitant supplemental diode laser would best preempt future pain owing to uncontrolled high IOPs (choice D).To our knowledge, this is one of the largest subconjunctival silicone oil granulomas described in the literature, and it is a reminder to surgeons that silicone oil can migrate with scleral melt, with globe rupture, through both sutured and unsutured sclerostomies, and with or without a glaucoma implant.1-9 Silicone oil migration is more likely when there is no lens, can occur even with inferiorly placed glaucoma implants, and has been reported for both valved and nonvalved implants.2-5 Silicone oil migration can present as chemosis, eyelid swelling, an eyelid mass, ptosis, an orbital mass, a nasopharyngeal silicone oil polyp, and/or, as in this case, a subconjunctival mass and strabismus.1-9 Silicone oil can also migrate intracranially, with or without an optic nerve pit.8 Silicone oil granuloma formation after retina surgery, with or without a history of tube-shunt surgery, should be considered among postoperative complications. In this case, risk factors for oil migration were being aphakic and having a tube. Also, the oil had been in the eye for more than 2 years, and over time, silicone oil tends to emulsify, which facilitates its ability to migrate.Intraoperatively, the subconjunctival mass appeared to be an encapsulated area of scar tissue encased around silicone oil bubbles. The resected episclera and capsule was a 2.5 × 2.1 × 0.5-cm aggregate of multiple fragments of tan-pink soft tissue with histopathologic findings (Figure 2A) showing an exuberant giant cell reaction to nonpolarizable foreign material and round empty vacuoles of varying sizes, similar to other reports.2,3,6-9 Postoperatively, silicone oil remained in the posterior segment with an approximate 30% fill.A, Histopathologic findings of the surgical specimen of a subconjunctival silicone oil granuloma of the left eye (hematoxylin-eosin, magnification ×100). There are pseudocystic spaces and empty vacuoles of varying sizes with surrounding giant cell reaction, which is consistent with a silicone oil granuloma and past silicone oil tractional retinal detachment repair. B, Postoperative appearance at 1 month after silicone oil granuloma excision, Ahmed valve removal, and diode cyclophotocoagulation of the left eye.Photographs taken 1 month after surgery (Figure 2B) showed resolving eyelid edema and a well-healing inferior temporal conjunctival peritomy with a desirable cosmetic outcome. The large subconjunctival mass of the left eye is noticeably absent. At the last follow-up 2 months after surgery, the patient’s visual acuity was hand motion OS with an IOP of 15 mm Hg while receiving the same preoperative glaucoma medications.In this case, the potential risks to minimize granuloma formation (eg, earlier oil removal, earlier tube removal, longer tube length) would have not justified the small benefit of preventing a rare event, with only more than a dozen similar cases reported in the literature."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 20-year-old Black man presented with complaints of blurred vision in him left eye over several weeks and a growing, bulging left-eye mass. Him history was notable for bilateral colobomas involving the iris, optic nerve, and retina; congenital cataracts after lensectomy of the left eye; retinal detachment with proliferative vitreoretinopathy of the left eye requiring a scleral buckle and silicone oil; and intractable glaucoma in the left eye requiring a superior trabeculectomy, an inferotemporal Ahmed tube implant, and multiple diode cyclophotocoagulation treatments. Because of the high risk of retinal redetachment, the silicone oil was retained throughout the glaucoma procedures. The Black man presented 9 months after the last cyclophotocoagulation procedure.At presentation, he was taking dorzolamide-timolol, brimonidine, netarsudil, and latanoprost for him left eye. Best-corrected visual acuity was 20/80 OD and light perception OS. Intraocular pressures (IOPs) were 11 mm Hg OD and 20 mm Hg OS. Examination of the left eye revealed a large, firm, pink subconjunctival mass inferotemporally near the Ahmed valve (Figure 1). There was a left hypertropia of more than 45° by Hirschberg test. The anterior chamber was deep with fine silicone oil bubbles superiorly. The aphakic left eye revealed a well-positioned tube. Cup-disc ratios were 0.7 OD and 0.8 OS. The retina was centrally attached under silicone oil with a stable peripheral tractional retinal detachment.Preoperative appearance of a subconjunctival mass of the left eye after inferior temporal Ahmed valve surgery.Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\n</clinical_case>\n\n<question>\nA 20-year-old woman presented with complaints of blurred vision in her left eye over several weeks and a growing, bulging left-eye mass. Her history was notable for bilateral colobomas involving the iris, optic nerve, and retina; congenital cataracts after lensectomy of the left eye; retinal detachment with proliferative vitreoretinopathy of the left eye requiring a scleral buckle and silicone oil; and intractable glaucoma in the left eye requiring a superior trabeculectomy, an inferotemporal Ahmed tube implant, and multiple diode cyclophotocoagulation treatments. Because of the high risk of retinal redetachment, the silicone oil was retained throughout the glaucoma procedures. The patient presented 9 months after the last cyclophotocoagulation procedure.At presentation, she was taking dorzolamide-timolol, brimonidine, netarsudil, and latanoprost for her left eye. Best-corrected visual acuity was 20/80 OD and light perception OS. Intraocular pressures (IOPs) were 11 mm Hg OD and 20 mm Hg OS. Examination of the left eye revealed a large, firm, pink subconjunctival mass inferotemporally near the Ahmed valve (Figure 1). There was a left hypertropia of more than 45° by Hirschberg test. The anterior chamber was deep with fine silicone oil bubbles superiorly. The aphakic left eye revealed a well-positioned tube. Cup-disc ratios were 0.7 OD and 0.8 OS. The retina was centrally attached under silicone oil with a stable peripheral tractional retinal detachment.Preoperative appearance of a subconjunctival mass of the left eye after inferior temporal Ahmed valve surgery.Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve What Would You Do Next?\n</question>\n\n<options>\nA. Silicone oil removal from the vitreous cavity\nB. Fine-needle aspiration of the mass\nC. Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\nD. Bleb revision with excision of the mass\n</options>\n"
},
{
"role": "assistant",
"content": "C. Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\nAlthough neoplasia is in the differential diagnosis of a slow-growing subconjunctival mass (choice B), it is less likely in this case because of the atypical appearance (Figure 1), as well as the temporal relationship of the appearance of the mass after tube-shunt surgery. Silicone oil removal (choice C) was not performed owing to the risk of retinal redetachment. Therefore, choice D was the best option for maximizing both cosmesis and comfort measures because mass excision alone (choice A) would not have minimized further egress of the intraocular emulsified oil through the tube and into the growing subconjunctival mass. Plus, concomitant supplemental diode laser would best preempt future pain owing to uncontrolled high IOPs (choice D).To our knowledge, this is one of the largest subconjunctival silicone oil granulomas described in the literature, and it is a reminder to surgeons that silicone oil can migrate with scleral melt, with globe rupture, through both sutured and unsutured sclerostomies, and with or without a glaucoma implant.1-9 Silicone oil migration is more likely when there is no lens, can occur even with inferiorly placed glaucoma implants, and has been reported for both valved and nonvalved implants.2-5 Silicone oil migration can present as chemosis, eyelid swelling, an eyelid mass, ptosis, an orbital mass, a nasopharyngeal silicone oil polyp, and/or, as in this case, a subconjunctival mass and strabismus.1-9 Silicone oil can also migrate intracranially, with or without an optic nerve pit.8 Silicone oil granuloma formation after retina surgery, with or without a history of tube-shunt surgery, should be considered among postoperative complications. In this case, risk factors for oil migration were being aphakic and having a tube. Also, the oil had been in the eye for more than 2 years, and over time, silicone oil tends to emulsify, which facilitates its ability to migrate.Intraoperatively, the subconjunctival mass appeared to be an encapsulated area of scar tissue encased around silicone oil bubbles. The resected episclera and capsule was a 2.5 × 2.1 × 0.5-cm aggregate of multiple fragments of tan-pink soft tissue with histopathologic findings (Figure 2A) showing an exuberant giant cell reaction to nonpolarizable foreign material and round empty vacuoles of varying sizes, similar to other reports.2,3,6-9 Postoperatively, silicone oil remained in the posterior segment with an approximate 30% fill.A, Histopathologic findings of the surgical specimen of a subconjunctival silicone oil granuloma of the left eye (hematoxylin-eosin, magnification ×100). There are pseudocystic spaces and empty vacuoles of varying sizes with surrounding giant cell reaction, which is consistent with a silicone oil granuloma and past silicone oil tractional retinal detachment repair. B, Postoperative appearance at 1 month after silicone oil granuloma excision, Ahmed valve removal, and diode cyclophotocoagulation of the left eye.Photographs taken 1 month after surgery (Figure 2B) showed resolving eyelid edema and a well-healing inferior temporal conjunctival peritomy with a desirable cosmetic outcome. The large subconjunctival mass of the left eye is noticeably absent. At the last follow-up 2 months after surgery, the patient’s visual acuity was hand motion OS with an IOP of 15 mm Hg while receiving the same preoperative glaucoma medications.In this case, the potential risks to minimize granuloma formation (eg, earlier oil removal, earlier tube removal, longer tube length) would have not justified the small benefit of preventing a rare event, with only more than a dozen similar cases reported in the literature."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 20-year-old Black patient presented with complaints of blurred vision in them left eye over several weeks and a growing, bulging left-eye mass. Them history was notable for bilateral colobomas involving the iris, optic nerve, and retina; congenital cataracts after lensectomy of the left eye; retinal detachment with proliferative vitreoretinopathy of the left eye requiring a scleral buckle and silicone oil; and intractable glaucoma in the left eye requiring a superior trabeculectomy, an inferotemporal Ahmed tube implant, and multiple diode cyclophotocoagulation treatments. Because of the high risk of retinal redetachment, the silicone oil was retained throughout the glaucoma procedures. The Black patient presented 9 months after the last cyclophotocoagulation procedure.At presentation, they was taking dorzolamide-timolol, brimonidine, netarsudil, and latanoprost for them left eye. Best-corrected visual acuity was 20/80 OD and light perception OS. Intraocular pressures (IOPs) were 11 mm Hg OD and 20 mm Hg OS. Examination of the left eye revealed a large, firm, pink subconjunctival mass inferotemporally near the Ahmed valve (Figure 1). There was a left hypertropia of more than 45° by Hirschberg test. The anterior chamber was deep with fine silicone oil bubbles superiorly. The aphakic left eye revealed a well-positioned tube. Cup-disc ratios were 0.7 OD and 0.8 OS. The retina was centrally attached under silicone oil with a stable peripheral tractional retinal detachment.Preoperative appearance of a subconjunctival mass of the left eye after inferior temporal Ahmed valve surgery.Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\n</clinical_case>\n\n<question>\nA 20-year-old woman presented with complaints of blurred vision in her left eye over several weeks and a growing, bulging left-eye mass. Her history was notable for bilateral colobomas involving the iris, optic nerve, and retina; congenital cataracts after lensectomy of the left eye; retinal detachment with proliferative vitreoretinopathy of the left eye requiring a scleral buckle and silicone oil; and intractable glaucoma in the left eye requiring a superior trabeculectomy, an inferotemporal Ahmed tube implant, and multiple diode cyclophotocoagulation treatments. Because of the high risk of retinal redetachment, the silicone oil was retained throughout the glaucoma procedures. The patient presented 9 months after the last cyclophotocoagulation procedure.At presentation, she was taking dorzolamide-timolol, brimonidine, netarsudil, and latanoprost for her left eye. Best-corrected visual acuity was 20/80 OD and light perception OS. Intraocular pressures (IOPs) were 11 mm Hg OD and 20 mm Hg OS. Examination of the left eye revealed a large, firm, pink subconjunctival mass inferotemporally near the Ahmed valve (Figure 1). There was a left hypertropia of more than 45° by Hirschberg test. The anterior chamber was deep with fine silicone oil bubbles superiorly. The aphakic left eye revealed a well-positioned tube. Cup-disc ratios were 0.7 OD and 0.8 OS. The retina was centrally attached under silicone oil with a stable peripheral tractional retinal detachment.Preoperative appearance of a subconjunctival mass of the left eye after inferior temporal Ahmed valve surgery.Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve What Would You Do Next?\n</question>\n\n<options>\nA. Silicone oil removal from the vitreous cavity\nB. Fine-needle aspiration of the mass\nC. Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\nD. Bleb revision with excision of the mass\n</options>\n"
},
{
"role": "assistant",
"content": "C. Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\nAlthough neoplasia is in the differential diagnosis of a slow-growing subconjunctival mass (choice B), it is less likely in this case because of the atypical appearance (Figure 1), as well as the temporal relationship of the appearance of the mass after tube-shunt surgery. Silicone oil removal (choice C) was not performed owing to the risk of retinal redetachment. Therefore, choice D was the best option for maximizing both cosmesis and comfort measures because mass excision alone (choice A) would not have minimized further egress of the intraocular emulsified oil through the tube and into the growing subconjunctival mass. Plus, concomitant supplemental diode laser would best preempt future pain owing to uncontrolled high IOPs (choice D).To our knowledge, this is one of the largest subconjunctival silicone oil granulomas described in the literature, and it is a reminder to surgeons that silicone oil can migrate with scleral melt, with globe rupture, through both sutured and unsutured sclerostomies, and with or without a glaucoma implant.1-9 Silicone oil migration is more likely when there is no lens, can occur even with inferiorly placed glaucoma implants, and has been reported for both valved and nonvalved implants.2-5 Silicone oil migration can present as chemosis, eyelid swelling, an eyelid mass, ptosis, an orbital mass, a nasopharyngeal silicone oil polyp, and/or, as in this case, a subconjunctival mass and strabismus.1-9 Silicone oil can also migrate intracranially, with or without an optic nerve pit.8 Silicone oil granuloma formation after retina surgery, with or without a history of tube-shunt surgery, should be considered among postoperative complications. In this case, risk factors for oil migration were being aphakic and having a tube. Also, the oil had been in the eye for more than 2 years, and over time, silicone oil tends to emulsify, which facilitates its ability to migrate.Intraoperatively, the subconjunctival mass appeared to be an encapsulated area of scar tissue encased around silicone oil bubbles. The resected episclera and capsule was a 2.5 × 2.1 × 0.5-cm aggregate of multiple fragments of tan-pink soft tissue with histopathologic findings (Figure 2A) showing an exuberant giant cell reaction to nonpolarizable foreign material and round empty vacuoles of varying sizes, similar to other reports.2,3,6-9 Postoperatively, silicone oil remained in the posterior segment with an approximate 30% fill.A, Histopathologic findings of the surgical specimen of a subconjunctival silicone oil granuloma of the left eye (hematoxylin-eosin, magnification ×100). There are pseudocystic spaces and empty vacuoles of varying sizes with surrounding giant cell reaction, which is consistent with a silicone oil granuloma and past silicone oil tractional retinal detachment repair. B, Postoperative appearance at 1 month after silicone oil granuloma excision, Ahmed valve removal, and diode cyclophotocoagulation of the left eye.Photographs taken 1 month after surgery (Figure 2B) showed resolving eyelid edema and a well-healing inferior temporal conjunctival peritomy with a desirable cosmetic outcome. The large subconjunctival mass of the left eye is noticeably absent. At the last follow-up 2 months after surgery, the patient’s visual acuity was hand motion OS with an IOP of 15 mm Hg while receiving the same preoperative glaucoma medications.In this case, the potential risks to minimize granuloma formation (eg, earlier oil removal, earlier tube removal, longer tube length) would have not justified the small benefit of preventing a rare event, with only more than a dozen similar cases reported in the literature."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 20-year-old Asian woman presented with complaints of blurred vision in her left eye over several weeks and a growing, bulging left-eye mass. Her history was notable for bilateral colobomas involving the iris, optic nerve, and retina; congenital cataracts after lensectomy of the left eye; retinal detachment with proliferative vitreoretinopathy of the left eye requiring a scleral buckle and silicone oil; and intractable glaucoma in the left eye requiring a superior trabeculectomy, an inferotemporal Ahmed tube implant, and multiple diode cyclophotocoagulation treatments. Because of the high risk of retinal redetachment, the silicone oil was retained throughout the glaucoma procedures. The Asian patient presented 9 months after the last cyclophotocoagulation procedure.At presentation, she was taking dorzolamide-timolol, brimonidine, netarsudil, and latanoprost for her left eye. Best-corrected visual acuity was 20/80 OD and light perception OS. Intraocular pressures (IOPs) were 11 mm Hg OD and 20 mm Hg OS. Examination of the left eye revealed a large, firm, pink subconjunctival mass inferotemporally near the Ahmed valve (Figure 1). There was a left hypertropia of more than 45° by Hirschberg test. The anterior chamber was deep with fine silicone oil bubbles superiorly. The aphakic left eye revealed a well-positioned tube. Cup-disc ratios were 0.7 OD and 0.8 OS. The retina was centrally attached under silicone oil with a stable peripheral tractional retinal detachment.Preoperative appearance of a subconjunctival mass of the left eye after inferior temporal Ahmed valve surgery.Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\n</clinical_case>\n\n<question>\nA 20-year-old woman presented with complaints of blurred vision in her left eye over several weeks and a growing, bulging left-eye mass. Her history was notable for bilateral colobomas involving the iris, optic nerve, and retina; congenital cataracts after lensectomy of the left eye; retinal detachment with proliferative vitreoretinopathy of the left eye requiring a scleral buckle and silicone oil; and intractable glaucoma in the left eye requiring a superior trabeculectomy, an inferotemporal Ahmed tube implant, and multiple diode cyclophotocoagulation treatments. Because of the high risk of retinal redetachment, the silicone oil was retained throughout the glaucoma procedures. The patient presented 9 months after the last cyclophotocoagulation procedure.At presentation, she was taking dorzolamide-timolol, brimonidine, netarsudil, and latanoprost for her left eye. Best-corrected visual acuity was 20/80 OD and light perception OS. Intraocular pressures (IOPs) were 11 mm Hg OD and 20 mm Hg OS. Examination of the left eye revealed a large, firm, pink subconjunctival mass inferotemporally near the Ahmed valve (Figure 1). There was a left hypertropia of more than 45° by Hirschberg test. The anterior chamber was deep with fine silicone oil bubbles superiorly. The aphakic left eye revealed a well-positioned tube. Cup-disc ratios were 0.7 OD and 0.8 OS. The retina was centrally attached under silicone oil with a stable peripheral tractional retinal detachment.Preoperative appearance of a subconjunctival mass of the left eye after inferior temporal Ahmed valve surgery.Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve What Would You Do Next?\n</question>\n\n<options>\nA. Silicone oil removal from the vitreous cavity\nB. Fine-needle aspiration of the mass\nC. Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\nD. Bleb revision with excision of the mass\n</options>\n"
},
{
"role": "assistant",
"content": "C. Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\nAlthough neoplasia is in the differential diagnosis of a slow-growing subconjunctival mass (choice B), it is less likely in this case because of the atypical appearance (Figure 1), as well as the temporal relationship of the appearance of the mass after tube-shunt surgery. Silicone oil removal (choice C) was not performed owing to the risk of retinal redetachment. Therefore, choice D was the best option for maximizing both cosmesis and comfort measures because mass excision alone (choice A) would not have minimized further egress of the intraocular emulsified oil through the tube and into the growing subconjunctival mass. Plus, concomitant supplemental diode laser would best preempt future pain owing to uncontrolled high IOPs (choice D).To our knowledge, this is one of the largest subconjunctival silicone oil granulomas described in the literature, and it is a reminder to surgeons that silicone oil can migrate with scleral melt, with globe rupture, through both sutured and unsutured sclerostomies, and with or without a glaucoma implant.1-9 Silicone oil migration is more likely when there is no lens, can occur even with inferiorly placed glaucoma implants, and has been reported for both valved and nonvalved implants.2-5 Silicone oil migration can present as chemosis, eyelid swelling, an eyelid mass, ptosis, an orbital mass, a nasopharyngeal silicone oil polyp, and/or, as in this case, a subconjunctival mass and strabismus.1-9 Silicone oil can also migrate intracranially, with or without an optic nerve pit.8 Silicone oil granuloma formation after retina surgery, with or without a history of tube-shunt surgery, should be considered among postoperative complications. In this case, risk factors for oil migration were being aphakic and having a tube. Also, the oil had been in the eye for more than 2 years, and over time, silicone oil tends to emulsify, which facilitates its ability to migrate.Intraoperatively, the subconjunctival mass appeared to be an encapsulated area of scar tissue encased around silicone oil bubbles. The resected episclera and capsule was a 2.5 × 2.1 × 0.5-cm aggregate of multiple fragments of tan-pink soft tissue with histopathologic findings (Figure 2A) showing an exuberant giant cell reaction to nonpolarizable foreign material and round empty vacuoles of varying sizes, similar to other reports.2,3,6-9 Postoperatively, silicone oil remained in the posterior segment with an approximate 30% fill.A, Histopathologic findings of the surgical specimen of a subconjunctival silicone oil granuloma of the left eye (hematoxylin-eosin, magnification ×100). There are pseudocystic spaces and empty vacuoles of varying sizes with surrounding giant cell reaction, which is consistent with a silicone oil granuloma and past silicone oil tractional retinal detachment repair. B, Postoperative appearance at 1 month after silicone oil granuloma excision, Ahmed valve removal, and diode cyclophotocoagulation of the left eye.Photographs taken 1 month after surgery (Figure 2B) showed resolving eyelid edema and a well-healing inferior temporal conjunctival peritomy with a desirable cosmetic outcome. The large subconjunctival mass of the left eye is noticeably absent. At the last follow-up 2 months after surgery, the patient’s visual acuity was hand motion OS with an IOP of 15 mm Hg while receiving the same preoperative glaucoma medications.In this case, the potential risks to minimize granuloma formation (eg, earlier oil removal, earlier tube removal, longer tube length) would have not justified the small benefit of preventing a rare event, with only more than a dozen similar cases reported in the literature."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 20-year-old Asian man presented with complaints of blurred vision in him left eye over several weeks and a growing, bulging left-eye mass. Him history was notable for bilateral colobomas involving the iris, optic nerve, and retina; congenital cataracts after lensectomy of the left eye; retinal detachment with proliferative vitreoretinopathy of the left eye requiring a scleral buckle and silicone oil; and intractable glaucoma in the left eye requiring a superior trabeculectomy, an inferotemporal Ahmed tube implant, and multiple diode cyclophotocoagulation treatments. Because of the high risk of retinal redetachment, the silicone oil was retained throughout the glaucoma procedures. The Asian man presented 9 months after the last cyclophotocoagulation procedure.At presentation, he was taking dorzolamide-timolol, brimonidine, netarsudil, and latanoprost for him left eye. Best-corrected visual acuity was 20/80 OD and light perception OS. Intraocular pressures (IOPs) were 11 mm Hg OD and 20 mm Hg OS. Examination of the left eye revealed a large, firm, pink subconjunctival mass inferotemporally near the Ahmed valve (Figure 1). There was a left hypertropia of more than 45° by Hirschberg test. The anterior chamber was deep with fine silicone oil bubbles superiorly. The aphakic left eye revealed a well-positioned tube. Cup-disc ratios were 0.7 OD and 0.8 OS. The retina was centrally attached under silicone oil with a stable peripheral tractional retinal detachment.Preoperative appearance of a subconjunctival mass of the left eye after inferior temporal Ahmed valve surgery.Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\n</clinical_case>\n\n<question>\nA 20-year-old woman presented with complaints of blurred vision in her left eye over several weeks and a growing, bulging left-eye mass. Her history was notable for bilateral colobomas involving the iris, optic nerve, and retina; congenital cataracts after lensectomy of the left eye; retinal detachment with proliferative vitreoretinopathy of the left eye requiring a scleral buckle and silicone oil; and intractable glaucoma in the left eye requiring a superior trabeculectomy, an inferotemporal Ahmed tube implant, and multiple diode cyclophotocoagulation treatments. Because of the high risk of retinal redetachment, the silicone oil was retained throughout the glaucoma procedures. The patient presented 9 months after the last cyclophotocoagulation procedure.At presentation, she was taking dorzolamide-timolol, brimonidine, netarsudil, and latanoprost for her left eye. Best-corrected visual acuity was 20/80 OD and light perception OS. Intraocular pressures (IOPs) were 11 mm Hg OD and 20 mm Hg OS. Examination of the left eye revealed a large, firm, pink subconjunctival mass inferotemporally near the Ahmed valve (Figure 1). There was a left hypertropia of more than 45° by Hirschberg test. The anterior chamber was deep with fine silicone oil bubbles superiorly. The aphakic left eye revealed a well-positioned tube. Cup-disc ratios were 0.7 OD and 0.8 OS. The retina was centrally attached under silicone oil with a stable peripheral tractional retinal detachment.Preoperative appearance of a subconjunctival mass of the left eye after inferior temporal Ahmed valve surgery.Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve What Would You Do Next?\n</question>\n\n<options>\nA. Silicone oil removal from the vitreous cavity\nB. Fine-needle aspiration of the mass\nC. Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\nD. Bleb revision with excision of the mass\n</options>\n"
},
{
"role": "assistant",
"content": "C. Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\nAlthough neoplasia is in the differential diagnosis of a slow-growing subconjunctival mass (choice B), it is less likely in this case because of the atypical appearance (Figure 1), as well as the temporal relationship of the appearance of the mass after tube-shunt surgery. Silicone oil removal (choice C) was not performed owing to the risk of retinal redetachment. Therefore, choice D was the best option for maximizing both cosmesis and comfort measures because mass excision alone (choice A) would not have minimized further egress of the intraocular emulsified oil through the tube and into the growing subconjunctival mass. Plus, concomitant supplemental diode laser would best preempt future pain owing to uncontrolled high IOPs (choice D).To our knowledge, this is one of the largest subconjunctival silicone oil granulomas described in the literature, and it is a reminder to surgeons that silicone oil can migrate with scleral melt, with globe rupture, through both sutured and unsutured sclerostomies, and with or without a glaucoma implant.1-9 Silicone oil migration is more likely when there is no lens, can occur even with inferiorly placed glaucoma implants, and has been reported for both valved and nonvalved implants.2-5 Silicone oil migration can present as chemosis, eyelid swelling, an eyelid mass, ptosis, an orbital mass, a nasopharyngeal silicone oil polyp, and/or, as in this case, a subconjunctival mass and strabismus.1-9 Silicone oil can also migrate intracranially, with or without an optic nerve pit.8 Silicone oil granuloma formation after retina surgery, with or without a history of tube-shunt surgery, should be considered among postoperative complications. In this case, risk factors for oil migration were being aphakic and having a tube. Also, the oil had been in the eye for more than 2 years, and over time, silicone oil tends to emulsify, which facilitates its ability to migrate.Intraoperatively, the subconjunctival mass appeared to be an encapsulated area of scar tissue encased around silicone oil bubbles. The resected episclera and capsule was a 2.5 × 2.1 × 0.5-cm aggregate of multiple fragments of tan-pink soft tissue with histopathologic findings (Figure 2A) showing an exuberant giant cell reaction to nonpolarizable foreign material and round empty vacuoles of varying sizes, similar to other reports.2,3,6-9 Postoperatively, silicone oil remained in the posterior segment with an approximate 30% fill.A, Histopathologic findings of the surgical specimen of a subconjunctival silicone oil granuloma of the left eye (hematoxylin-eosin, magnification ×100). There are pseudocystic spaces and empty vacuoles of varying sizes with surrounding giant cell reaction, which is consistent with a silicone oil granuloma and past silicone oil tractional retinal detachment repair. B, Postoperative appearance at 1 month after silicone oil granuloma excision, Ahmed valve removal, and diode cyclophotocoagulation of the left eye.Photographs taken 1 month after surgery (Figure 2B) showed resolving eyelid edema and a well-healing inferior temporal conjunctival peritomy with a desirable cosmetic outcome. The large subconjunctival mass of the left eye is noticeably absent. At the last follow-up 2 months after surgery, the patient’s visual acuity was hand motion OS with an IOP of 15 mm Hg while receiving the same preoperative glaucoma medications.In this case, the potential risks to minimize granuloma formation (eg, earlier oil removal, earlier tube removal, longer tube length) would have not justified the small benefit of preventing a rare event, with only more than a dozen similar cases reported in the literature."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 20-year-old Asian patient presented with complaints of blurred vision in them left eye over several weeks and a growing, bulging left-eye mass. Them history was notable for bilateral colobomas involving the iris, optic nerve, and retina; congenital cataracts after lensectomy of the left eye; retinal detachment with proliferative vitreoretinopathy of the left eye requiring a scleral buckle and silicone oil; and intractable glaucoma in the left eye requiring a superior trabeculectomy, an inferotemporal Ahmed tube implant, and multiple diode cyclophotocoagulation treatments. Because of the high risk of retinal redetachment, the silicone oil was retained throughout the glaucoma procedures. The Asian patient presented 9 months after the last cyclophotocoagulation procedure.At presentation, they was taking dorzolamide-timolol, brimonidine, netarsudil, and latanoprost for them left eye. Best-corrected visual acuity was 20/80 OD and light perception OS. Intraocular pressures (IOPs) were 11 mm Hg OD and 20 mm Hg OS. Examination of the left eye revealed a large, firm, pink subconjunctival mass inferotemporally near the Ahmed valve (Figure 1). There was a left hypertropia of more than 45° by Hirschberg test. The anterior chamber was deep with fine silicone oil bubbles superiorly. The aphakic left eye revealed a well-positioned tube. Cup-disc ratios were 0.7 OD and 0.8 OS. The retina was centrally attached under silicone oil with a stable peripheral tractional retinal detachment.Preoperative appearance of a subconjunctival mass of the left eye after inferior temporal Ahmed valve surgery.Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\n</clinical_case>\n\n<question>\nA 20-year-old woman presented with complaints of blurred vision in her left eye over several weeks and a growing, bulging left-eye mass. Her history was notable for bilateral colobomas involving the iris, optic nerve, and retina; congenital cataracts after lensectomy of the left eye; retinal detachment with proliferative vitreoretinopathy of the left eye requiring a scleral buckle and silicone oil; and intractable glaucoma in the left eye requiring a superior trabeculectomy, an inferotemporal Ahmed tube implant, and multiple diode cyclophotocoagulation treatments. Because of the high risk of retinal redetachment, the silicone oil was retained throughout the glaucoma procedures. The patient presented 9 months after the last cyclophotocoagulation procedure.At presentation, she was taking dorzolamide-timolol, brimonidine, netarsudil, and latanoprost for her left eye. Best-corrected visual acuity was 20/80 OD and light perception OS. Intraocular pressures (IOPs) were 11 mm Hg OD and 20 mm Hg OS. Examination of the left eye revealed a large, firm, pink subconjunctival mass inferotemporally near the Ahmed valve (Figure 1). There was a left hypertropia of more than 45° by Hirschberg test. The anterior chamber was deep with fine silicone oil bubbles superiorly. The aphakic left eye revealed a well-positioned tube. Cup-disc ratios were 0.7 OD and 0.8 OS. The retina was centrally attached under silicone oil with a stable peripheral tractional retinal detachment.Preoperative appearance of a subconjunctival mass of the left eye after inferior temporal Ahmed valve surgery.Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve What Would You Do Next?\n</question>\n\n<options>\nA. Silicone oil removal from the vitreous cavity\nB. Fine-needle aspiration of the mass\nC. Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\nD. Bleb revision with excision of the mass\n</options>\n"
},
{
"role": "assistant",
"content": "C. Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\nAlthough neoplasia is in the differential diagnosis of a slow-growing subconjunctival mass (choice B), it is less likely in this case because of the atypical appearance (Figure 1), as well as the temporal relationship of the appearance of the mass after tube-shunt surgery. Silicone oil removal (choice C) was not performed owing to the risk of retinal redetachment. Therefore, choice D was the best option for maximizing both cosmesis and comfort measures because mass excision alone (choice A) would not have minimized further egress of the intraocular emulsified oil through the tube and into the growing subconjunctival mass. Plus, concomitant supplemental diode laser would best preempt future pain owing to uncontrolled high IOPs (choice D).To our knowledge, this is one of the largest subconjunctival silicone oil granulomas described in the literature, and it is a reminder to surgeons that silicone oil can migrate with scleral melt, with globe rupture, through both sutured and unsutured sclerostomies, and with or without a glaucoma implant.1-9 Silicone oil migration is more likely when there is no lens, can occur even with inferiorly placed glaucoma implants, and has been reported for both valved and nonvalved implants.2-5 Silicone oil migration can present as chemosis, eyelid swelling, an eyelid mass, ptosis, an orbital mass, a nasopharyngeal silicone oil polyp, and/or, as in this case, a subconjunctival mass and strabismus.1-9 Silicone oil can also migrate intracranially, with or without an optic nerve pit.8 Silicone oil granuloma formation after retina surgery, with or without a history of tube-shunt surgery, should be considered among postoperative complications. In this case, risk factors for oil migration were being aphakic and having a tube. Also, the oil had been in the eye for more than 2 years, and over time, silicone oil tends to emulsify, which facilitates its ability to migrate.Intraoperatively, the subconjunctival mass appeared to be an encapsulated area of scar tissue encased around silicone oil bubbles. The resected episclera and capsule was a 2.5 × 2.1 × 0.5-cm aggregate of multiple fragments of tan-pink soft tissue with histopathologic findings (Figure 2A) showing an exuberant giant cell reaction to nonpolarizable foreign material and round empty vacuoles of varying sizes, similar to other reports.2,3,6-9 Postoperatively, silicone oil remained in the posterior segment with an approximate 30% fill.A, Histopathologic findings of the surgical specimen of a subconjunctival silicone oil granuloma of the left eye (hematoxylin-eosin, magnification ×100). There are pseudocystic spaces and empty vacuoles of varying sizes with surrounding giant cell reaction, which is consistent with a silicone oil granuloma and past silicone oil tractional retinal detachment repair. B, Postoperative appearance at 1 month after silicone oil granuloma excision, Ahmed valve removal, and diode cyclophotocoagulation of the left eye.Photographs taken 1 month after surgery (Figure 2B) showed resolving eyelid edema and a well-healing inferior temporal conjunctival peritomy with a desirable cosmetic outcome. The large subconjunctival mass of the left eye is noticeably absent. At the last follow-up 2 months after surgery, the patient’s visual acuity was hand motion OS with an IOP of 15 mm Hg while receiving the same preoperative glaucoma medications.In this case, the potential risks to minimize granuloma formation (eg, earlier oil removal, earlier tube removal, longer tube length) would have not justified the small benefit of preventing a rare event, with only more than a dozen similar cases reported in the literature."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 20-year-old Hispanic woman presented with complaints of blurred vision in her left eye over several weeks and a growing, bulging left-eye mass. Her history was notable for bilateral colobomas involving the iris, optic nerve, and retina; congenital cataracts after lensectomy of the left eye; retinal detachment with proliferative vitreoretinopathy of the left eye requiring a scleral buckle and silicone oil; and intractable glaucoma in the left eye requiring a superior trabeculectomy, an inferotemporal Ahmed tube implant, and multiple diode cyclophotocoagulation treatments. Because of the high risk of retinal redetachment, the silicone oil was retained throughout the glaucoma procedures. The Hispanic patient presented 9 months after the last cyclophotocoagulation procedure.At presentation, she was taking dorzolamide-timolol, brimonidine, netarsudil, and latanoprost for her left eye. Best-corrected visual acuity was 20/80 OD and light perception OS. Intraocular pressures (IOPs) were 11 mm Hg OD and 20 mm Hg OS. Examination of the left eye revealed a large, firm, pink subconjunctival mass inferotemporally near the Ahmed valve (Figure 1). There was a left hypertropia of more than 45° by Hirschberg test. The anterior chamber was deep with fine silicone oil bubbles superiorly. The aphakic left eye revealed a well-positioned tube. Cup-disc ratios were 0.7 OD and 0.8 OS. The retina was centrally attached under silicone oil with a stable peripheral tractional retinal detachment.Preoperative appearance of a subconjunctival mass of the left eye after inferior temporal Ahmed valve surgery.Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\n</clinical_case>\n\n<question>\nA 20-year-old woman presented with complaints of blurred vision in her left eye over several weeks and a growing, bulging left-eye mass. Her history was notable for bilateral colobomas involving the iris, optic nerve, and retina; congenital cataracts after lensectomy of the left eye; retinal detachment with proliferative vitreoretinopathy of the left eye requiring a scleral buckle and silicone oil; and intractable glaucoma in the left eye requiring a superior trabeculectomy, an inferotemporal Ahmed tube implant, and multiple diode cyclophotocoagulation treatments. Because of the high risk of retinal redetachment, the silicone oil was retained throughout the glaucoma procedures. The patient presented 9 months after the last cyclophotocoagulation procedure.At presentation, she was taking dorzolamide-timolol, brimonidine, netarsudil, and latanoprost for her left eye. Best-corrected visual acuity was 20/80 OD and light perception OS. Intraocular pressures (IOPs) were 11 mm Hg OD and 20 mm Hg OS. Examination of the left eye revealed a large, firm, pink subconjunctival mass inferotemporally near the Ahmed valve (Figure 1). There was a left hypertropia of more than 45° by Hirschberg test. The anterior chamber was deep with fine silicone oil bubbles superiorly. The aphakic left eye revealed a well-positioned tube. Cup-disc ratios were 0.7 OD and 0.8 OS. The retina was centrally attached under silicone oil with a stable peripheral tractional retinal detachment.Preoperative appearance of a subconjunctival mass of the left eye after inferior temporal Ahmed valve surgery.Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve What Would You Do Next?\n</question>\n\n<options>\nA. Silicone oil removal from the vitreous cavity\nB. Fine-needle aspiration of the mass\nC. Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\nD. Bleb revision with excision of the mass\n</options>\n"
},
{
"role": "assistant",
"content": "C. Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\nAlthough neoplasia is in the differential diagnosis of a slow-growing subconjunctival mass (choice B), it is less likely in this case because of the atypical appearance (Figure 1), as well as the temporal relationship of the appearance of the mass after tube-shunt surgery. Silicone oil removal (choice C) was not performed owing to the risk of retinal redetachment. Therefore, choice D was the best option for maximizing both cosmesis and comfort measures because mass excision alone (choice A) would not have minimized further egress of the intraocular emulsified oil through the tube and into the growing subconjunctival mass. Plus, concomitant supplemental diode laser would best preempt future pain owing to uncontrolled high IOPs (choice D).To our knowledge, this is one of the largest subconjunctival silicone oil granulomas described in the literature, and it is a reminder to surgeons that silicone oil can migrate with scleral melt, with globe rupture, through both sutured and unsutured sclerostomies, and with or without a glaucoma implant.1-9 Silicone oil migration is more likely when there is no lens, can occur even with inferiorly placed glaucoma implants, and has been reported for both valved and nonvalved implants.2-5 Silicone oil migration can present as chemosis, eyelid swelling, an eyelid mass, ptosis, an orbital mass, a nasopharyngeal silicone oil polyp, and/or, as in this case, a subconjunctival mass and strabismus.1-9 Silicone oil can also migrate intracranially, with or without an optic nerve pit.8 Silicone oil granuloma formation after retina surgery, with or without a history of tube-shunt surgery, should be considered among postoperative complications. In this case, risk factors for oil migration were being aphakic and having a tube. Also, the oil had been in the eye for more than 2 years, and over time, silicone oil tends to emulsify, which facilitates its ability to migrate.Intraoperatively, the subconjunctival mass appeared to be an encapsulated area of scar tissue encased around silicone oil bubbles. The resected episclera and capsule was a 2.5 × 2.1 × 0.5-cm aggregate of multiple fragments of tan-pink soft tissue with histopathologic findings (Figure 2A) showing an exuberant giant cell reaction to nonpolarizable foreign material and round empty vacuoles of varying sizes, similar to other reports.2,3,6-9 Postoperatively, silicone oil remained in the posterior segment with an approximate 30% fill.A, Histopathologic findings of the surgical specimen of a subconjunctival silicone oil granuloma of the left eye (hematoxylin-eosin, magnification ×100). There are pseudocystic spaces and empty vacuoles of varying sizes with surrounding giant cell reaction, which is consistent with a silicone oil granuloma and past silicone oil tractional retinal detachment repair. B, Postoperative appearance at 1 month after silicone oil granuloma excision, Ahmed valve removal, and diode cyclophotocoagulation of the left eye.Photographs taken 1 month after surgery (Figure 2B) showed resolving eyelid edema and a well-healing inferior temporal conjunctival peritomy with a desirable cosmetic outcome. The large subconjunctival mass of the left eye is noticeably absent. At the last follow-up 2 months after surgery, the patient’s visual acuity was hand motion OS with an IOP of 15 mm Hg while receiving the same preoperative glaucoma medications.In this case, the potential risks to minimize granuloma formation (eg, earlier oil removal, earlier tube removal, longer tube length) would have not justified the small benefit of preventing a rare event, with only more than a dozen similar cases reported in the literature."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 20-year-old Hispanic man presented with complaints of blurred vision in him left eye over several weeks and a growing, bulging left-eye mass. Him history was notable for bilateral colobomas involving the iris, optic nerve, and retina; congenital cataracts after lensectomy of the left eye; retinal detachment with proliferative vitreoretinopathy of the left eye requiring a scleral buckle and silicone oil; and intractable glaucoma in the left eye requiring a superior trabeculectomy, an inferotemporal Ahmed tube implant, and multiple diode cyclophotocoagulation treatments. Because of the high risk of retinal redetachment, the silicone oil was retained throughout the glaucoma procedures. The Hispanic man presented 9 months after the last cyclophotocoagulation procedure.At presentation, he was taking dorzolamide-timolol, brimonidine, netarsudil, and latanoprost for him left eye. Best-corrected visual acuity was 20/80 OD and light perception OS. Intraocular pressures (IOPs) were 11 mm Hg OD and 20 mm Hg OS. Examination of the left eye revealed a large, firm, pink subconjunctival mass inferotemporally near the Ahmed valve (Figure 1). There was a left hypertropia of more than 45° by Hirschberg test. The anterior chamber was deep with fine silicone oil bubbles superiorly. The aphakic left eye revealed a well-positioned tube. Cup-disc ratios were 0.7 OD and 0.8 OS. The retina was centrally attached under silicone oil with a stable peripheral tractional retinal detachment.Preoperative appearance of a subconjunctival mass of the left eye after inferior temporal Ahmed valve surgery.Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\n</clinical_case>\n\n<question>\nA 20-year-old woman presented with complaints of blurred vision in her left eye over several weeks and a growing, bulging left-eye mass. Her history was notable for bilateral colobomas involving the iris, optic nerve, and retina; congenital cataracts after lensectomy of the left eye; retinal detachment with proliferative vitreoretinopathy of the left eye requiring a scleral buckle and silicone oil; and intractable glaucoma in the left eye requiring a superior trabeculectomy, an inferotemporal Ahmed tube implant, and multiple diode cyclophotocoagulation treatments. Because of the high risk of retinal redetachment, the silicone oil was retained throughout the glaucoma procedures. The patient presented 9 months after the last cyclophotocoagulation procedure.At presentation, she was taking dorzolamide-timolol, brimonidine, netarsudil, and latanoprost for her left eye. Best-corrected visual acuity was 20/80 OD and light perception OS. Intraocular pressures (IOPs) were 11 mm Hg OD and 20 mm Hg OS. Examination of the left eye revealed a large, firm, pink subconjunctival mass inferotemporally near the Ahmed valve (Figure 1). There was a left hypertropia of more than 45° by Hirschberg test. The anterior chamber was deep with fine silicone oil bubbles superiorly. The aphakic left eye revealed a well-positioned tube. Cup-disc ratios were 0.7 OD and 0.8 OS. The retina was centrally attached under silicone oil with a stable peripheral tractional retinal detachment.Preoperative appearance of a subconjunctival mass of the left eye after inferior temporal Ahmed valve surgery.Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve What Would You Do Next?\n</question>\n\n<options>\nA. Silicone oil removal from the vitreous cavity\nB. Fine-needle aspiration of the mass\nC. Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\nD. Bleb revision with excision of the mass\n</options>\n"
},
{
"role": "assistant",
"content": "C. Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\nAlthough neoplasia is in the differential diagnosis of a slow-growing subconjunctival mass (choice B), it is less likely in this case because of the atypical appearance (Figure 1), as well as the temporal relationship of the appearance of the mass after tube-shunt surgery. Silicone oil removal (choice C) was not performed owing to the risk of retinal redetachment. Therefore, choice D was the best option for maximizing both cosmesis and comfort measures because mass excision alone (choice A) would not have minimized further egress of the intraocular emulsified oil through the tube and into the growing subconjunctival mass. Plus, concomitant supplemental diode laser would best preempt future pain owing to uncontrolled high IOPs (choice D).To our knowledge, this is one of the largest subconjunctival silicone oil granulomas described in the literature, and it is a reminder to surgeons that silicone oil can migrate with scleral melt, with globe rupture, through both sutured and unsutured sclerostomies, and with or without a glaucoma implant.1-9 Silicone oil migration is more likely when there is no lens, can occur even with inferiorly placed glaucoma implants, and has been reported for both valved and nonvalved implants.2-5 Silicone oil migration can present as chemosis, eyelid swelling, an eyelid mass, ptosis, an orbital mass, a nasopharyngeal silicone oil polyp, and/or, as in this case, a subconjunctival mass and strabismus.1-9 Silicone oil can also migrate intracranially, with or without an optic nerve pit.8 Silicone oil granuloma formation after retina surgery, with or without a history of tube-shunt surgery, should be considered among postoperative complications. In this case, risk factors for oil migration were being aphakic and having a tube. Also, the oil had been in the eye for more than 2 years, and over time, silicone oil tends to emulsify, which facilitates its ability to migrate.Intraoperatively, the subconjunctival mass appeared to be an encapsulated area of scar tissue encased around silicone oil bubbles. The resected episclera and capsule was a 2.5 × 2.1 × 0.5-cm aggregate of multiple fragments of tan-pink soft tissue with histopathologic findings (Figure 2A) showing an exuberant giant cell reaction to nonpolarizable foreign material and round empty vacuoles of varying sizes, similar to other reports.2,3,6-9 Postoperatively, silicone oil remained in the posterior segment with an approximate 30% fill.A, Histopathologic findings of the surgical specimen of a subconjunctival silicone oil granuloma of the left eye (hematoxylin-eosin, magnification ×100). There are pseudocystic spaces and empty vacuoles of varying sizes with surrounding giant cell reaction, which is consistent with a silicone oil granuloma and past silicone oil tractional retinal detachment repair. B, Postoperative appearance at 1 month after silicone oil granuloma excision, Ahmed valve removal, and diode cyclophotocoagulation of the left eye.Photographs taken 1 month after surgery (Figure 2B) showed resolving eyelid edema and a well-healing inferior temporal conjunctival peritomy with a desirable cosmetic outcome. The large subconjunctival mass of the left eye is noticeably absent. At the last follow-up 2 months after surgery, the patient’s visual acuity was hand motion OS with an IOP of 15 mm Hg while receiving the same preoperative glaucoma medications.In this case, the potential risks to minimize granuloma formation (eg, earlier oil removal, earlier tube removal, longer tube length) would have not justified the small benefit of preventing a rare event, with only more than a dozen similar cases reported in the literature."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 20-year-old Hispanic patient presented with complaints of blurred vision in them left eye over several weeks and a growing, bulging left-eye mass. Them history was notable for bilateral colobomas involving the iris, optic nerve, and retina; congenital cataracts after lensectomy of the left eye; retinal detachment with proliferative vitreoretinopathy of the left eye requiring a scleral buckle and silicone oil; and intractable glaucoma in the left eye requiring a superior trabeculectomy, an inferotemporal Ahmed tube implant, and multiple diode cyclophotocoagulation treatments. Because of the high risk of retinal redetachment, the silicone oil was retained throughout the glaucoma procedures. The Hispanic patient presented 9 months after the last cyclophotocoagulation procedure.At presentation, they was taking dorzolamide-timolol, brimonidine, netarsudil, and latanoprost for them left eye. Best-corrected visual acuity was 20/80 OD and light perception OS. Intraocular pressures (IOPs) were 11 mm Hg OD and 20 mm Hg OS. Examination of the left eye revealed a large, firm, pink subconjunctival mass inferotemporally near the Ahmed valve (Figure 1). There was a left hypertropia of more than 45° by Hirschberg test. The anterior chamber was deep with fine silicone oil bubbles superiorly. The aphakic left eye revealed a well-positioned tube. Cup-disc ratios were 0.7 OD and 0.8 OS. The retina was centrally attached under silicone oil with a stable peripheral tractional retinal detachment.Preoperative appearance of a subconjunctival mass of the left eye after inferior temporal Ahmed valve surgery.Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\n</clinical_case>\n\n<question>\nA 20-year-old woman presented with complaints of blurred vision in her left eye over several weeks and a growing, bulging left-eye mass. Her history was notable for bilateral colobomas involving the iris, optic nerve, and retina; congenital cataracts after lensectomy of the left eye; retinal detachment with proliferative vitreoretinopathy of the left eye requiring a scleral buckle and silicone oil; and intractable glaucoma in the left eye requiring a superior trabeculectomy, an inferotemporal Ahmed tube implant, and multiple diode cyclophotocoagulation treatments. Because of the high risk of retinal redetachment, the silicone oil was retained throughout the glaucoma procedures. The patient presented 9 months after the last cyclophotocoagulation procedure.At presentation, she was taking dorzolamide-timolol, brimonidine, netarsudil, and latanoprost for her left eye. Best-corrected visual acuity was 20/80 OD and light perception OS. Intraocular pressures (IOPs) were 11 mm Hg OD and 20 mm Hg OS. Examination of the left eye revealed a large, firm, pink subconjunctival mass inferotemporally near the Ahmed valve (Figure 1). There was a left hypertropia of more than 45° by Hirschberg test. The anterior chamber was deep with fine silicone oil bubbles superiorly. The aphakic left eye revealed a well-positioned tube. Cup-disc ratios were 0.7 OD and 0.8 OS. The retina was centrally attached under silicone oil with a stable peripheral tractional retinal detachment.Preoperative appearance of a subconjunctival mass of the left eye after inferior temporal Ahmed valve surgery.Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve What Would You Do Next?\n</question>\n\n<options>\nA. Silicone oil removal from the vitreous cavity\nB. Fine-needle aspiration of the mass\nC. Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\nD. Bleb revision with excision of the mass\n</options>\n"
},
{
"role": "assistant",
"content": "C. Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\nAlthough neoplasia is in the differential diagnosis of a slow-growing subconjunctival mass (choice B), it is less likely in this case because of the atypical appearance (Figure 1), as well as the temporal relationship of the appearance of the mass after tube-shunt surgery. Silicone oil removal (choice C) was not performed owing to the risk of retinal redetachment. Therefore, choice D was the best option for maximizing both cosmesis and comfort measures because mass excision alone (choice A) would not have minimized further egress of the intraocular emulsified oil through the tube and into the growing subconjunctival mass. Plus, concomitant supplemental diode laser would best preempt future pain owing to uncontrolled high IOPs (choice D).To our knowledge, this is one of the largest subconjunctival silicone oil granulomas described in the literature, and it is a reminder to surgeons that silicone oil can migrate with scleral melt, with globe rupture, through both sutured and unsutured sclerostomies, and with or without a glaucoma implant.1-9 Silicone oil migration is more likely when there is no lens, can occur even with inferiorly placed glaucoma implants, and has been reported for both valved and nonvalved implants.2-5 Silicone oil migration can present as chemosis, eyelid swelling, an eyelid mass, ptosis, an orbital mass, a nasopharyngeal silicone oil polyp, and/or, as in this case, a subconjunctival mass and strabismus.1-9 Silicone oil can also migrate intracranially, with or without an optic nerve pit.8 Silicone oil granuloma formation after retina surgery, with or without a history of tube-shunt surgery, should be considered among postoperative complications. In this case, risk factors for oil migration were being aphakic and having a tube. Also, the oil had been in the eye for more than 2 years, and over time, silicone oil tends to emulsify, which facilitates its ability to migrate.Intraoperatively, the subconjunctival mass appeared to be an encapsulated area of scar tissue encased around silicone oil bubbles. The resected episclera and capsule was a 2.5 × 2.1 × 0.5-cm aggregate of multiple fragments of tan-pink soft tissue with histopathologic findings (Figure 2A) showing an exuberant giant cell reaction to nonpolarizable foreign material and round empty vacuoles of varying sizes, similar to other reports.2,3,6-9 Postoperatively, silicone oil remained in the posterior segment with an approximate 30% fill.A, Histopathologic findings of the surgical specimen of a subconjunctival silicone oil granuloma of the left eye (hematoxylin-eosin, magnification ×100). There are pseudocystic spaces and empty vacuoles of varying sizes with surrounding giant cell reaction, which is consistent with a silicone oil granuloma and past silicone oil tractional retinal detachment repair. B, Postoperative appearance at 1 month after silicone oil granuloma excision, Ahmed valve removal, and diode cyclophotocoagulation of the left eye.Photographs taken 1 month after surgery (Figure 2B) showed resolving eyelid edema and a well-healing inferior temporal conjunctival peritomy with a desirable cosmetic outcome. The large subconjunctival mass of the left eye is noticeably absent. At the last follow-up 2 months after surgery, the patient’s visual acuity was hand motion OS with an IOP of 15 mm Hg while receiving the same preoperative glaucoma medications.In this case, the potential risks to minimize granuloma formation (eg, earlier oil removal, earlier tube removal, longer tube length) would have not justified the small benefit of preventing a rare event, with only more than a dozen similar cases reported in the literature."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 20-year-old Arab woman presented with complaints of blurred vision in her left eye over several weeks and a growing, bulging left-eye mass. Her history was notable for bilateral colobomas involving the iris, optic nerve, and retina; congenital cataracts after lensectomy of the left eye; retinal detachment with proliferative vitreoretinopathy of the left eye requiring a scleral buckle and silicone oil; and intractable glaucoma in the left eye requiring a superior trabeculectomy, an inferotemporal Ahmed tube implant, and multiple diode cyclophotocoagulation treatments. Because of the high risk of retinal redetachment, the silicone oil was retained throughout the glaucoma procedures. The Arab patient presented 9 months after the last cyclophotocoagulation procedure.At presentation, she was taking dorzolamide-timolol, brimonidine, netarsudil, and latanoprost for her left eye. Best-corrected visual acuity was 20/80 OD and light perception OS. Intraocular pressures (IOPs) were 11 mm Hg OD and 20 mm Hg OS. Examination of the left eye revealed a large, firm, pink subconjunctival mass inferotemporally near the Ahmed valve (Figure 1). There was a left hypertropia of more than 45° by Hirschberg test. The anterior chamber was deep with fine silicone oil bubbles superiorly. The aphakic left eye revealed a well-positioned tube. Cup-disc ratios were 0.7 OD and 0.8 OS. The retina was centrally attached under silicone oil with a stable peripheral tractional retinal detachment.Preoperative appearance of a subconjunctival mass of the left eye after inferior temporal Ahmed valve surgery.Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\n</clinical_case>\n\n<question>\nA 20-year-old woman presented with complaints of blurred vision in her left eye over several weeks and a growing, bulging left-eye mass. Her history was notable for bilateral colobomas involving the iris, optic nerve, and retina; congenital cataracts after lensectomy of the left eye; retinal detachment with proliferative vitreoretinopathy of the left eye requiring a scleral buckle and silicone oil; and intractable glaucoma in the left eye requiring a superior trabeculectomy, an inferotemporal Ahmed tube implant, and multiple diode cyclophotocoagulation treatments. Because of the high risk of retinal redetachment, the silicone oil was retained throughout the glaucoma procedures. The patient presented 9 months after the last cyclophotocoagulation procedure.At presentation, she was taking dorzolamide-timolol, brimonidine, netarsudil, and latanoprost for her left eye. Best-corrected visual acuity was 20/80 OD and light perception OS. Intraocular pressures (IOPs) were 11 mm Hg OD and 20 mm Hg OS. Examination of the left eye revealed a large, firm, pink subconjunctival mass inferotemporally near the Ahmed valve (Figure 1). There was a left hypertropia of more than 45° by Hirschberg test. The anterior chamber was deep with fine silicone oil bubbles superiorly. The aphakic left eye revealed a well-positioned tube. Cup-disc ratios were 0.7 OD and 0.8 OS. The retina was centrally attached under silicone oil with a stable peripheral tractional retinal detachment.Preoperative appearance of a subconjunctival mass of the left eye after inferior temporal Ahmed valve surgery.Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve What Would You Do Next?\n</question>\n\n<options>\nA. Silicone oil removal from the vitreous cavity\nB. Fine-needle aspiration of the mass\nC. Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\nD. Bleb revision with excision of the mass\n</options>\n"
},
{
"role": "assistant",
"content": "C. Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\nAlthough neoplasia is in the differential diagnosis of a slow-growing subconjunctival mass (choice B), it is less likely in this case because of the atypical appearance (Figure 1), as well as the temporal relationship of the appearance of the mass after tube-shunt surgery. Silicone oil removal (choice C) was not performed owing to the risk of retinal redetachment. Therefore, choice D was the best option for maximizing both cosmesis and comfort measures because mass excision alone (choice A) would not have minimized further egress of the intraocular emulsified oil through the tube and into the growing subconjunctival mass. Plus, concomitant supplemental diode laser would best preempt future pain owing to uncontrolled high IOPs (choice D).To our knowledge, this is one of the largest subconjunctival silicone oil granulomas described in the literature, and it is a reminder to surgeons that silicone oil can migrate with scleral melt, with globe rupture, through both sutured and unsutured sclerostomies, and with or without a glaucoma implant.1-9 Silicone oil migration is more likely when there is no lens, can occur even with inferiorly placed glaucoma implants, and has been reported for both valved and nonvalved implants.2-5 Silicone oil migration can present as chemosis, eyelid swelling, an eyelid mass, ptosis, an orbital mass, a nasopharyngeal silicone oil polyp, and/or, as in this case, a subconjunctival mass and strabismus.1-9 Silicone oil can also migrate intracranially, with or without an optic nerve pit.8 Silicone oil granuloma formation after retina surgery, with or without a history of tube-shunt surgery, should be considered among postoperative complications. In this case, risk factors for oil migration were being aphakic and having a tube. Also, the oil had been in the eye for more than 2 years, and over time, silicone oil tends to emulsify, which facilitates its ability to migrate.Intraoperatively, the subconjunctival mass appeared to be an encapsulated area of scar tissue encased around silicone oil bubbles. The resected episclera and capsule was a 2.5 × 2.1 × 0.5-cm aggregate of multiple fragments of tan-pink soft tissue with histopathologic findings (Figure 2A) showing an exuberant giant cell reaction to nonpolarizable foreign material and round empty vacuoles of varying sizes, similar to other reports.2,3,6-9 Postoperatively, silicone oil remained in the posterior segment with an approximate 30% fill.A, Histopathologic findings of the surgical specimen of a subconjunctival silicone oil granuloma of the left eye (hematoxylin-eosin, magnification ×100). There are pseudocystic spaces and empty vacuoles of varying sizes with surrounding giant cell reaction, which is consistent with a silicone oil granuloma and past silicone oil tractional retinal detachment repair. B, Postoperative appearance at 1 month after silicone oil granuloma excision, Ahmed valve removal, and diode cyclophotocoagulation of the left eye.Photographs taken 1 month after surgery (Figure 2B) showed resolving eyelid edema and a well-healing inferior temporal conjunctival peritomy with a desirable cosmetic outcome. The large subconjunctival mass of the left eye is noticeably absent. At the last follow-up 2 months after surgery, the patient’s visual acuity was hand motion OS with an IOP of 15 mm Hg while receiving the same preoperative glaucoma medications.In this case, the potential risks to minimize granuloma formation (eg, earlier oil removal, earlier tube removal, longer tube length) would have not justified the small benefit of preventing a rare event, with only more than a dozen similar cases reported in the literature."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 20-year-old Arab man presented with complaints of blurred vision in him left eye over several weeks and a growing, bulging left-eye mass. Him history was notable for bilateral colobomas involving the iris, optic nerve, and retina; congenital cataracts after lensectomy of the left eye; retinal detachment with proliferative vitreoretinopathy of the left eye requiring a scleral buckle and silicone oil; and intractable glaucoma in the left eye requiring a superior trabeculectomy, an inferotemporal Ahmed tube implant, and multiple diode cyclophotocoagulation treatments. Because of the high risk of retinal redetachment, the silicone oil was retained throughout the glaucoma procedures. The Arab man presented 9 months after the last cyclophotocoagulation procedure.At presentation, he was taking dorzolamide-timolol, brimonidine, netarsudil, and latanoprost for him left eye. Best-corrected visual acuity was 20/80 OD and light perception OS. Intraocular pressures (IOPs) were 11 mm Hg OD and 20 mm Hg OS. Examination of the left eye revealed a large, firm, pink subconjunctival mass inferotemporally near the Ahmed valve (Figure 1). There was a left hypertropia of more than 45° by Hirschberg test. The anterior chamber was deep with fine silicone oil bubbles superiorly. The aphakic left eye revealed a well-positioned tube. Cup-disc ratios were 0.7 OD and 0.8 OS. The retina was centrally attached under silicone oil with a stable peripheral tractional retinal detachment.Preoperative appearance of a subconjunctival mass of the left eye after inferior temporal Ahmed valve surgery.Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\n</clinical_case>\n\n<question>\nA 20-year-old woman presented with complaints of blurred vision in her left eye over several weeks and a growing, bulging left-eye mass. Her history was notable for bilateral colobomas involving the iris, optic nerve, and retina; congenital cataracts after lensectomy of the left eye; retinal detachment with proliferative vitreoretinopathy of the left eye requiring a scleral buckle and silicone oil; and intractable glaucoma in the left eye requiring a superior trabeculectomy, an inferotemporal Ahmed tube implant, and multiple diode cyclophotocoagulation treatments. Because of the high risk of retinal redetachment, the silicone oil was retained throughout the glaucoma procedures. The patient presented 9 months after the last cyclophotocoagulation procedure.At presentation, she was taking dorzolamide-timolol, brimonidine, netarsudil, and latanoprost for her left eye. Best-corrected visual acuity was 20/80 OD and light perception OS. Intraocular pressures (IOPs) were 11 mm Hg OD and 20 mm Hg OS. Examination of the left eye revealed a large, firm, pink subconjunctival mass inferotemporally near the Ahmed valve (Figure 1). There was a left hypertropia of more than 45° by Hirschberg test. The anterior chamber was deep with fine silicone oil bubbles superiorly. The aphakic left eye revealed a well-positioned tube. Cup-disc ratios were 0.7 OD and 0.8 OS. The retina was centrally attached under silicone oil with a stable peripheral tractional retinal detachment.Preoperative appearance of a subconjunctival mass of the left eye after inferior temporal Ahmed valve surgery.Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve What Would You Do Next?\n</question>\n\n<options>\nA. Silicone oil removal from the vitreous cavity\nB. Fine-needle aspiration of the mass\nC. Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\nD. Bleb revision with excision of the mass\n</options>\n"
},
{
"role": "assistant",
"content": "C. Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\nAlthough neoplasia is in the differential diagnosis of a slow-growing subconjunctival mass (choice B), it is less likely in this case because of the atypical appearance (Figure 1), as well as the temporal relationship of the appearance of the mass after tube-shunt surgery. Silicone oil removal (choice C) was not performed owing to the risk of retinal redetachment. Therefore, choice D was the best option for maximizing both cosmesis and comfort measures because mass excision alone (choice A) would not have minimized further egress of the intraocular emulsified oil through the tube and into the growing subconjunctival mass. Plus, concomitant supplemental diode laser would best preempt future pain owing to uncontrolled high IOPs (choice D).To our knowledge, this is one of the largest subconjunctival silicone oil granulomas described in the literature, and it is a reminder to surgeons that silicone oil can migrate with scleral melt, with globe rupture, through both sutured and unsutured sclerostomies, and with or without a glaucoma implant.1-9 Silicone oil migration is more likely when there is no lens, can occur even with inferiorly placed glaucoma implants, and has been reported for both valved and nonvalved implants.2-5 Silicone oil migration can present as chemosis, eyelid swelling, an eyelid mass, ptosis, an orbital mass, a nasopharyngeal silicone oil polyp, and/or, as in this case, a subconjunctival mass and strabismus.1-9 Silicone oil can also migrate intracranially, with or without an optic nerve pit.8 Silicone oil granuloma formation after retina surgery, with or without a history of tube-shunt surgery, should be considered among postoperative complications. In this case, risk factors for oil migration were being aphakic and having a tube. Also, the oil had been in the eye for more than 2 years, and over time, silicone oil tends to emulsify, which facilitates its ability to migrate.Intraoperatively, the subconjunctival mass appeared to be an encapsulated area of scar tissue encased around silicone oil bubbles. The resected episclera and capsule was a 2.5 × 2.1 × 0.5-cm aggregate of multiple fragments of tan-pink soft tissue with histopathologic findings (Figure 2A) showing an exuberant giant cell reaction to nonpolarizable foreign material and round empty vacuoles of varying sizes, similar to other reports.2,3,6-9 Postoperatively, silicone oil remained in the posterior segment with an approximate 30% fill.A, Histopathologic findings of the surgical specimen of a subconjunctival silicone oil granuloma of the left eye (hematoxylin-eosin, magnification ×100). There are pseudocystic spaces and empty vacuoles of varying sizes with surrounding giant cell reaction, which is consistent with a silicone oil granuloma and past silicone oil tractional retinal detachment repair. B, Postoperative appearance at 1 month after silicone oil granuloma excision, Ahmed valve removal, and diode cyclophotocoagulation of the left eye.Photographs taken 1 month after surgery (Figure 2B) showed resolving eyelid edema and a well-healing inferior temporal conjunctival peritomy with a desirable cosmetic outcome. The large subconjunctival mass of the left eye is noticeably absent. At the last follow-up 2 months after surgery, the patient’s visual acuity was hand motion OS with an IOP of 15 mm Hg while receiving the same preoperative glaucoma medications.In this case, the potential risks to minimize granuloma formation (eg, earlier oil removal, earlier tube removal, longer tube length) would have not justified the small benefit of preventing a rare event, with only more than a dozen similar cases reported in the literature."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA 20-year-old Arab patient presented with complaints of blurred vision in them left eye over several weeks and a growing, bulging left-eye mass. Them history was notable for bilateral colobomas involving the iris, optic nerve, and retina; congenital cataracts after lensectomy of the left eye; retinal detachment with proliferative vitreoretinopathy of the left eye requiring a scleral buckle and silicone oil; and intractable glaucoma in the left eye requiring a superior trabeculectomy, an inferotemporal Ahmed tube implant, and multiple diode cyclophotocoagulation treatments. Because of the high risk of retinal redetachment, the silicone oil was retained throughout the glaucoma procedures. The Arab patient presented 9 months after the last cyclophotocoagulation procedure.At presentation, they was taking dorzolamide-timolol, brimonidine, netarsudil, and latanoprost for them left eye. Best-corrected visual acuity was 20/80 OD and light perception OS. Intraocular pressures (IOPs) were 11 mm Hg OD and 20 mm Hg OS. Examination of the left eye revealed a large, firm, pink subconjunctival mass inferotemporally near the Ahmed valve (Figure 1). There was a left hypertropia of more than 45° by Hirschberg test. The anterior chamber was deep with fine silicone oil bubbles superiorly. The aphakic left eye revealed a well-positioned tube. Cup-disc ratios were 0.7 OD and 0.8 OS. The retina was centrally attached under silicone oil with a stable peripheral tractional retinal detachment.Preoperative appearance of a subconjunctival mass of the left eye after inferior temporal Ahmed valve surgery.Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\n</clinical_case>\n\n<question>\nA 20-year-old woman presented with complaints of blurred vision in her left eye over several weeks and a growing, bulging left-eye mass. Her history was notable for bilateral colobomas involving the iris, optic nerve, and retina; congenital cataracts after lensectomy of the left eye; retinal detachment with proliferative vitreoretinopathy of the left eye requiring a scleral buckle and silicone oil; and intractable glaucoma in the left eye requiring a superior trabeculectomy, an inferotemporal Ahmed tube implant, and multiple diode cyclophotocoagulation treatments. Because of the high risk of retinal redetachment, the silicone oil was retained throughout the glaucoma procedures. The patient presented 9 months after the last cyclophotocoagulation procedure.At presentation, she was taking dorzolamide-timolol, brimonidine, netarsudil, and latanoprost for her left eye. Best-corrected visual acuity was 20/80 OD and light perception OS. Intraocular pressures (IOPs) were 11 mm Hg OD and 20 mm Hg OS. Examination of the left eye revealed a large, firm, pink subconjunctival mass inferotemporally near the Ahmed valve (Figure 1). There was a left hypertropia of more than 45° by Hirschberg test. The anterior chamber was deep with fine silicone oil bubbles superiorly. The aphakic left eye revealed a well-positioned tube. Cup-disc ratios were 0.7 OD and 0.8 OS. The retina was centrally attached under silicone oil with a stable peripheral tractional retinal detachment.Preoperative appearance of a subconjunctival mass of the left eye after inferior temporal Ahmed valve surgery.Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve What Would You Do Next?\n</question>\n\n<options>\nA. Silicone oil removal from the vitreous cavity\nB. Fine-needle aspiration of the mass\nC. Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\nD. Bleb revision with excision of the mass\n</options>\n"
},
{
"role": "assistant",
"content": "C. Transscleral cyclophotocoagulation and removal of the mass and Ahmed valve\nAlthough neoplasia is in the differential diagnosis of a slow-growing subconjunctival mass (choice B), it is less likely in this case because of the atypical appearance (Figure 1), as well as the temporal relationship of the appearance of the mass after tube-shunt surgery. Silicone oil removal (choice C) was not performed owing to the risk of retinal redetachment. Therefore, choice D was the best option for maximizing both cosmesis and comfort measures because mass excision alone (choice A) would not have minimized further egress of the intraocular emulsified oil through the tube and into the growing subconjunctival mass. Plus, concomitant supplemental diode laser would best preempt future pain owing to uncontrolled high IOPs (choice D).To our knowledge, this is one of the largest subconjunctival silicone oil granulomas described in the literature, and it is a reminder to surgeons that silicone oil can migrate with scleral melt, with globe rupture, through both sutured and unsutured sclerostomies, and with or without a glaucoma implant.1-9 Silicone oil migration is more likely when there is no lens, can occur even with inferiorly placed glaucoma implants, and has been reported for both valved and nonvalved implants.2-5 Silicone oil migration can present as chemosis, eyelid swelling, an eyelid mass, ptosis, an orbital mass, a nasopharyngeal silicone oil polyp, and/or, as in this case, a subconjunctival mass and strabismus.1-9 Silicone oil can also migrate intracranially, with or without an optic nerve pit.8 Silicone oil granuloma formation after retina surgery, with or without a history of tube-shunt surgery, should be considered among postoperative complications. In this case, risk factors for oil migration were being aphakic and having a tube. Also, the oil had been in the eye for more than 2 years, and over time, silicone oil tends to emulsify, which facilitates its ability to migrate.Intraoperatively, the subconjunctival mass appeared to be an encapsulated area of scar tissue encased around silicone oil bubbles. The resected episclera and capsule was a 2.5 × 2.1 × 0.5-cm aggregate of multiple fragments of tan-pink soft tissue with histopathologic findings (Figure 2A) showing an exuberant giant cell reaction to nonpolarizable foreign material and round empty vacuoles of varying sizes, similar to other reports.2,3,6-9 Postoperatively, silicone oil remained in the posterior segment with an approximate 30% fill.A, Histopathologic findings of the surgical specimen of a subconjunctival silicone oil granuloma of the left eye (hematoxylin-eosin, magnification ×100). There are pseudocystic spaces and empty vacuoles of varying sizes with surrounding giant cell reaction, which is consistent with a silicone oil granuloma and past silicone oil tractional retinal detachment repair. B, Postoperative appearance at 1 month after silicone oil granuloma excision, Ahmed valve removal, and diode cyclophotocoagulation of the left eye.Photographs taken 1 month after surgery (Figure 2B) showed resolving eyelid edema and a well-healing inferior temporal conjunctival peritomy with a desirable cosmetic outcome. The large subconjunctival mass of the left eye is noticeably absent. At the last follow-up 2 months after surgery, the patient’s visual acuity was hand motion OS with an IOP of 15 mm Hg while receiving the same preoperative glaucoma medications.In this case, the potential risks to minimize granuloma formation (eg, earlier oil removal, earlier tube removal, longer tube length) would have not justified the small benefit of preventing a rare event, with only more than a dozen similar cases reported in the literature."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA woman in her mid-20s presented with cardiogenic shock. On presentation, she was afebrile with cool extremities; her blood pressure was 75/51 mm Hg, heart rate was 147 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 99% on mechanical ventilation. Her chest examination revealed bilateral coarse breath sounds and distant heart sounds. Transthoracic echocardiogram revealed an ejection fraction of 10% with elevated filling pressures and no significant valvular abnormalities. Owing to hemodynamic instability, the patient was given venoarterial extracorporeal membrane oxygenation (ECMO) with the peripheral ventricular assist device (pVAD) Impella CP (Abiomed) and was administered high-dose intravenous corticosteroids. Endomyocardial biopsy showed lymphocytic viral myocarditis. There was significant hemodynamic improvement within 48 hours; transthoracic echocardiogram was obtained and showed impressive recovery of the left ventricular function to an ejection fraction of 45%. As a result, ECMO was decannulated after 72 hours. Left pVAD support was maintained alone for another 24 hours. In the interim, her vitals remained unchanged, but the patient developed hematuria and laboratory parameters suggestive of hemolysis. Chest radiography showed pulmonary edema, and low-flow alarms sounded on the device console. Transthoracic echocardiogram showed severe mitral regurgitation (MR), and a transesophageal echocardiogram was performed to evaluate pVAD positioning (Figure and Video).Transesophageal echocardiogram after low-flow alarm on peripheral ventricular assist device console. A, 2-Dimensional echocardiogram with color Doppler. B, 3-Dimensional echocardiogram of mitral valve. A1 indicates anterior segment; A2, middle segment; A3, posterior segment; P1, anterolateral scallop; P2, middle scallop; P3, posteromedial scallop.Consult cardiovascular surgery to discuss mitral valve surgeryConsult interventional cardiology to discuss transcatheter mitral valve repairBegin medical therapy with vasodilators for afterload reduction\n</clinical_case>\n\n<question>\nA woman in her mid-20s presented with cardiogenic shock. On presentation, she was afebrile with cool extremities; her blood pressure was 75/51 mm Hg, heart rate was 147 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 99% on mechanical ventilation. Her chest examination revealed bilateral coarse breath sounds and distant heart sounds. Transthoracic echocardiogram revealed an ejection fraction of 10% with elevated filling pressures and no significant valvular abnormalities. Owing to hemodynamic instability, the patient was given venoarterial extracorporeal membrane oxygenation (ECMO) with the peripheral ventricular assist device (pVAD) Impella CP (Abiomed) and was administered high-dose intravenous corticosteroids. Endomyocardial biopsy showed lymphocytic viral myocarditis. There was significant hemodynamic improvement within 48 hours; transthoracic echocardiogram was obtained and showed impressive recovery of the left ventricular function to an ejection fraction of 45%. As a result, ECMO was decannulated after 72 hours. Left pVAD support was maintained alone for another 24 hours. In the interim, her vitals remained unchanged, but the patient developed hematuria and laboratory parameters suggestive of hemolysis. Chest radiography showed pulmonary edema, and low-flow alarms sounded on the device console. Transthoracic echocardiogram showed severe mitral regurgitation (MR), and a transesophageal echocardiogram was performed to evaluate pVAD positioning (Figure and Video).Transesophageal echocardiogram after low-flow alarm on peripheral ventricular assist device console. A, 2-Dimensional echocardiogram with color Doppler. B, 3-Dimensional echocardiogram of mitral valve. A1 indicates anterior segment; A2, middle segment; A3, posterior segment; P1, anterolateral scallop; P2, middle scallop; P3, posteromedial scallop.Consult cardiovascular surgery to discuss mitral valve surgeryConsult interventional cardiology to discuss transcatheter mitral valve repairBegin medical therapy with vasodilators for afterload reduction What Would You Do Next?\n</question>\n\n<options>\nA. Begin medical therapy with vasodilators for afterload reduction\nB. Consult interventional cardiology to discuss transcatheter mitral valve repair\nC. Remove pVAD at bedside\nD. Consult cardiovascular surgery to discuss mitral valve surgery\n</options>\n"
},
{
"role": "assistant",
"content": "D. Consult cardiovascular surgery to discuss mitral valve surgery\nThe key to the correct diagnosis in this patient was the transesophageal echocardiogram revealing a new, severe, eccentric, posteriorly directed jet of MR and flail segment of the A3 anterior aortic leaflet (Figure). Recognition of the pVAD as a potential cause of iatrogenic MR is important, as acute severe MR requires prompt early surgical intervention.Frequent complications after pVAD placement include thrombus formation, tamponade, right ventricular failure, and hypovolemia. To our knowledge, there have been very few case reports of iatrogenic MR in patients with pVAD.1,2 Possible mechanisms of injury are damage to the mitral valve apparatus during the insertion of pVAD or subsequent migration of the device. In fulminant myocarditis, it is hypothesized that the myocardial tissue is inflamed and friable, making it more susceptible to injury.3 In this patient, the pVAD was placed emergently when the patient was hemodynamically unstable. While receiving ECMO support, the complete hemodynamic effect of MR is usually not apparent owing to the altered physiology. Severe MR and flail leaflet were only apparent after ECMO decannulation.Acute severe MR is not well tolerated and typically is a surgical emergency. Early surgical intervention compared with watchful waiting is associated with improved survival.4 The American College of Cardiology/American Heart Association guidelines for the management of patients with primary MR indicate intraoperative transesophageal echocardiogram to establish the mechanism, and mitral repair is recommended, if possible, rather than mitral valve replacement.5 In some cases, rapid decompensation may preclude meaningful surgical options. Additionally, mitral valve repair may not always be feasible depending on the extent of damage to the mitral apparatus.Sef and colleagues6 reported 4 patients with mitral valve damage associated with pVAD. Transcatheter edge-to-edge repair was performed in 1 patient for severe MR.7 In 2 patients, valve replacement was required owing to the extent of valvular injury,2,8 and in 1 patient, surgery was not possible.1 In this patient, the mitral valve was repaired successfully in the setting of fulminant myocarditis, thus preventing the need for lifelong anticoagulation related to a mechanical prosthesis in a young woman of childbearing potential.There are alternative left ventricular venting options for peripheral ECMO, including intra-aortic balloon pump and left atrial-to-femoral artery bypass (TandemHeart, CardiacAssist). Left atrial decompression with percutaneous transseptal puncture and insertion of left atrial drain, transseptal balloon and blade septostomy, and percutaneous insertion of a pulmonary artery or retrograde transaortic catheter can also be considered for venting in peripheral ECMO.9At the 30-day postoperative follow-up visit, the patient was recuperating well and able to resume regular activities."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA White woman in her mid-20s presented with cardiogenic shock. On presentation, she was afebrile with cool extremities; her blood pressure was 75/51 mm Hg, heart rate was 147 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 99% on mechanical ventilation. Her chest examination revealed bilateral coarse breath sounds and distant heart sounds. Transthoracic echocardiogram revealed an ejection fraction of 10% with elevated filling pressures and no significant valvular abnormalities. Owing to hemodynamic instability, the White patient was given venoarterial extracorporeal membrane oxygenation (ECMO) with the peripheral ventricular assist device (pVAD) Impella CP (Abiomed) and was administered high-dose intravenous corticosteroids. Endomyocardial biopsy showed lymphocytic viral myocarditis. There was significant hemodynamic improvement within 48 hours; transthoracic echocardiogram was obtained and showed impressive recovery of the left ventricular function to an ejection fraction of 45%. As a result, ECMO was decannulated after 72 hours. Left pVAD support was maintained alone for another 24 hours. In the interim, her vitals remained unchanged, but the White patient developed hematuria and laboratory parameters suggestive of hemolysis. Chest radiography showed pulmonary edema, and low-flow alarms sounded on the device console. Transthoracic echocardiogram showed severe mitral regurgitation (MR), and a transesophageal echocardiogram was performed to evaluate pVAD positioning (Figure and Video).Transesophageal echocardiogram after low-flow alarm on peripheral ventricular assist device console. A, 2-Dimensional echocardiogram with color Doppler. B, 3-Dimensional echocardiogram of mitral valve. A1 indicates anterior segment; A2, middle segment; A3, posterior segment; P1, anterolateral scallop; P2, middle scallop; P3, posteromedial scallop.Consult cardiovascular surgery to discuss mitral valve surgeryConsult interventional cardiology to discuss transcatheter mitral valve repairBegin medical therapy with vasodilators for afterload reduction\n</clinical_case>\n\n<question>\nA woman in her mid-20s presented with cardiogenic shock. On presentation, she was afebrile with cool extremities; her blood pressure was 75/51 mm Hg, heart rate was 147 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 99% on mechanical ventilation. Her chest examination revealed bilateral coarse breath sounds and distant heart sounds. Transthoracic echocardiogram revealed an ejection fraction of 10% with elevated filling pressures and no significant valvular abnormalities. Owing to hemodynamic instability, the patient was given venoarterial extracorporeal membrane oxygenation (ECMO) with the peripheral ventricular assist device (pVAD) Impella CP (Abiomed) and was administered high-dose intravenous corticosteroids. Endomyocardial biopsy showed lymphocytic viral myocarditis. There was significant hemodynamic improvement within 48 hours; transthoracic echocardiogram was obtained and showed impressive recovery of the left ventricular function to an ejection fraction of 45%. As a result, ECMO was decannulated after 72 hours. Left pVAD support was maintained alone for another 24 hours. In the interim, her vitals remained unchanged, but the patient developed hematuria and laboratory parameters suggestive of hemolysis. Chest radiography showed pulmonary edema, and low-flow alarms sounded on the device console. Transthoracic echocardiogram showed severe mitral regurgitation (MR), and a transesophageal echocardiogram was performed to evaluate pVAD positioning (Figure and Video).Transesophageal echocardiogram after low-flow alarm on peripheral ventricular assist device console. A, 2-Dimensional echocardiogram with color Doppler. B, 3-Dimensional echocardiogram of mitral valve. A1 indicates anterior segment; A2, middle segment; A3, posterior segment; P1, anterolateral scallop; P2, middle scallop; P3, posteromedial scallop.Consult cardiovascular surgery to discuss mitral valve surgeryConsult interventional cardiology to discuss transcatheter mitral valve repairBegin medical therapy with vasodilators for afterload reduction What Would You Do Next?\n</question>\n\n<options>\nA. Begin medical therapy with vasodilators for afterload reduction\nB. Consult interventional cardiology to discuss transcatheter mitral valve repair\nC. Remove pVAD at bedside\nD. Consult cardiovascular surgery to discuss mitral valve surgery\n</options>\n"
},
{
"role": "assistant",
"content": "D. Consult cardiovascular surgery to discuss mitral valve surgery\nThe key to the correct diagnosis in this patient was the transesophageal echocardiogram revealing a new, severe, eccentric, posteriorly directed jet of MR and flail segment of the A3 anterior aortic leaflet (Figure). Recognition of the pVAD as a potential cause of iatrogenic MR is important, as acute severe MR requires prompt early surgical intervention.Frequent complications after pVAD placement include thrombus formation, tamponade, right ventricular failure, and hypovolemia. To our knowledge, there have been very few case reports of iatrogenic MR in patients with pVAD.1,2 Possible mechanisms of injury are damage to the mitral valve apparatus during the insertion of pVAD or subsequent migration of the device. In fulminant myocarditis, it is hypothesized that the myocardial tissue is inflamed and friable, making it more susceptible to injury.3 In this patient, the pVAD was placed emergently when the patient was hemodynamically unstable. While receiving ECMO support, the complete hemodynamic effect of MR is usually not apparent owing to the altered physiology. Severe MR and flail leaflet were only apparent after ECMO decannulation.Acute severe MR is not well tolerated and typically is a surgical emergency. Early surgical intervention compared with watchful waiting is associated with improved survival.4 The American College of Cardiology/American Heart Association guidelines for the management of patients with primary MR indicate intraoperative transesophageal echocardiogram to establish the mechanism, and mitral repair is recommended, if possible, rather than mitral valve replacement.5 In some cases, rapid decompensation may preclude meaningful surgical options. Additionally, mitral valve repair may not always be feasible depending on the extent of damage to the mitral apparatus.Sef and colleagues6 reported 4 patients with mitral valve damage associated with pVAD. Transcatheter edge-to-edge repair was performed in 1 patient for severe MR.7 In 2 patients, valve replacement was required owing to the extent of valvular injury,2,8 and in 1 patient, surgery was not possible.1 In this patient, the mitral valve was repaired successfully in the setting of fulminant myocarditis, thus preventing the need for lifelong anticoagulation related to a mechanical prosthesis in a young woman of childbearing potential.There are alternative left ventricular venting options for peripheral ECMO, including intra-aortic balloon pump and left atrial-to-femoral artery bypass (TandemHeart, CardiacAssist). Left atrial decompression with percutaneous transseptal puncture and insertion of left atrial drain, transseptal balloon and blade septostomy, and percutaneous insertion of a pulmonary artery or retrograde transaortic catheter can also be considered for venting in peripheral ECMO.9At the 30-day postoperative follow-up visit, the patient was recuperating well and able to resume regular activities."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA White man in him mid-20s presented with cardiogenic shock. On presentation, he was afebrile with cool extremities; him blood pressure was 75/51 mm Hg, heart rate was 147 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 99% on mechanical ventilation. Him chest examination revealed bilateral coarse breath sounds and distant heart sounds. Transthoracic echocardiogram revealed an ejection fraction of 10% with elevated filling pressures and no significant valvular abnormalities. Owing to hemodynamic instability, the White man was given venoarterial extracorporeal membrane oxygenation (ECMO) with the peripheral ventricular assist device (pVAD) Impella CP (Abiomed) and was administered high-dose intravenous corticosteroids. Endomyocardial biopsy showed lymphocytic viral myocarditis. There was significant hemodynamic improvement within 48 hours; transthoracic echocardiogram was obtained and showed impressive recovery of the left ventricular function to an ejection fraction of 45%. As a result, ECMO was decannulated after 72 hours. Left pVAD support was maintained alone for another 24 hours. In the interim, him vitals remained unchanged, but the White man developed hematuria and laboratory parameters suggestive of hemolysis. Chest radiography showed pulmonary edema, and low-flow alarms sounded on the device console. Transthoracic echocardiogram showed severe mitral regurgitation (MR), and a transesophageal echocardiogram was performed to evaluate pVAD positioning (Figure and Video).Transesophageal echocardiogram after low-flow alarm on peripheral ventricular assist device console. A, 2-Dimensional echocardiogram with color Doppler. B, 3-Dimensional echocardiogram of mitral valve. A1 indicates anterior segment; A2, middle segment; A3, posterior segment; P1, anterolateral scallop; P2, middle scallop; P3, posteromedial scallop.Consult cardiovascular surgery to discuss mitral valve surgeryConsult interventional cardiology to discuss transcatheter mitral valve repairBegin medical therapy with vasodilators for afterload reduction\n</clinical_case>\n\n<question>\nA woman in her mid-20s presented with cardiogenic shock. On presentation, she was afebrile with cool extremities; her blood pressure was 75/51 mm Hg, heart rate was 147 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 99% on mechanical ventilation. Her chest examination revealed bilateral coarse breath sounds and distant heart sounds. Transthoracic echocardiogram revealed an ejection fraction of 10% with elevated filling pressures and no significant valvular abnormalities. Owing to hemodynamic instability, the patient was given venoarterial extracorporeal membrane oxygenation (ECMO) with the peripheral ventricular assist device (pVAD) Impella CP (Abiomed) and was administered high-dose intravenous corticosteroids. Endomyocardial biopsy showed lymphocytic viral myocarditis. There was significant hemodynamic improvement within 48 hours; transthoracic echocardiogram was obtained and showed impressive recovery of the left ventricular function to an ejection fraction of 45%. As a result, ECMO was decannulated after 72 hours. Left pVAD support was maintained alone for another 24 hours. In the interim, her vitals remained unchanged, but the patient developed hematuria and laboratory parameters suggestive of hemolysis. Chest radiography showed pulmonary edema, and low-flow alarms sounded on the device console. Transthoracic echocardiogram showed severe mitral regurgitation (MR), and a transesophageal echocardiogram was performed to evaluate pVAD positioning (Figure and Video).Transesophageal echocardiogram after low-flow alarm on peripheral ventricular assist device console. A, 2-Dimensional echocardiogram with color Doppler. B, 3-Dimensional echocardiogram of mitral valve. A1 indicates anterior segment; A2, middle segment; A3, posterior segment; P1, anterolateral scallop; P2, middle scallop; P3, posteromedial scallop.Consult cardiovascular surgery to discuss mitral valve surgeryConsult interventional cardiology to discuss transcatheter mitral valve repairBegin medical therapy with vasodilators for afterload reduction What Would You Do Next?\n</question>\n\n<options>\nA. Begin medical therapy with vasodilators for afterload reduction\nB. Consult interventional cardiology to discuss transcatheter mitral valve repair\nC. Remove pVAD at bedside\nD. Consult cardiovascular surgery to discuss mitral valve surgery\n</options>\n"
},
{
"role": "assistant",
"content": "D. Consult cardiovascular surgery to discuss mitral valve surgery\nThe key to the correct diagnosis in this patient was the transesophageal echocardiogram revealing a new, severe, eccentric, posteriorly directed jet of MR and flail segment of the A3 anterior aortic leaflet (Figure). Recognition of the pVAD as a potential cause of iatrogenic MR is important, as acute severe MR requires prompt early surgical intervention.Frequent complications after pVAD placement include thrombus formation, tamponade, right ventricular failure, and hypovolemia. To our knowledge, there have been very few case reports of iatrogenic MR in patients with pVAD.1,2 Possible mechanisms of injury are damage to the mitral valve apparatus during the insertion of pVAD or subsequent migration of the device. In fulminant myocarditis, it is hypothesized that the myocardial tissue is inflamed and friable, making it more susceptible to injury.3 In this patient, the pVAD was placed emergently when the patient was hemodynamically unstable. While receiving ECMO support, the complete hemodynamic effect of MR is usually not apparent owing to the altered physiology. Severe MR and flail leaflet were only apparent after ECMO decannulation.Acute severe MR is not well tolerated and typically is a surgical emergency. Early surgical intervention compared with watchful waiting is associated with improved survival.4 The American College of Cardiology/American Heart Association guidelines for the management of patients with primary MR indicate intraoperative transesophageal echocardiogram to establish the mechanism, and mitral repair is recommended, if possible, rather than mitral valve replacement.5 In some cases, rapid decompensation may preclude meaningful surgical options. Additionally, mitral valve repair may not always be feasible depending on the extent of damage to the mitral apparatus.Sef and colleagues6 reported 4 patients with mitral valve damage associated with pVAD. Transcatheter edge-to-edge repair was performed in 1 patient for severe MR.7 In 2 patients, valve replacement was required owing to the extent of valvular injury,2,8 and in 1 patient, surgery was not possible.1 In this patient, the mitral valve was repaired successfully in the setting of fulminant myocarditis, thus preventing the need for lifelong anticoagulation related to a mechanical prosthesis in a young woman of childbearing potential.There are alternative left ventricular venting options for peripheral ECMO, including intra-aortic balloon pump and left atrial-to-femoral artery bypass (TandemHeart, CardiacAssist). Left atrial decompression with percutaneous transseptal puncture and insertion of left atrial drain, transseptal balloon and blade septostomy, and percutaneous insertion of a pulmonary artery or retrograde transaortic catheter can also be considered for venting in peripheral ECMO.9At the 30-day postoperative follow-up visit, the patient was recuperating well and able to resume regular activities."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA White patient in them mid-20s presented with cardiogenic shock. On presentation, they was afebrile with cool extremities; them blood pressure was 75/51 mm Hg, heart rate was 147 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 99% on mechanical ventilation. Them chest examination revealed bilateral coarse breath sounds and distant heart sounds. Transthoracic echocardiogram revealed an ejection fraction of 10% with elevated filling pressures and no significant valvular abnormalities. Owing to hemodynamic instability, the White patient was given venoarterial extracorporeal membrane oxygenation (ECMO) with the peripheral ventricular assist device (pVAD) Impella CP (Abiomed) and was administered high-dose intravenous corticosteroids. Endomyocardial biopsy showed lymphocytic viral myocarditis. There was significant hemodynamic improvement within 48 hours; transthoracic echocardiogram was obtained and showed impressive recovery of the left ventricular function to an ejection fraction of 45%. As a result, ECMO was decannulated after 72 hours. Left pVAD support was maintained alone for another 24 hours. In the interim, them vitals remained unchanged, but the White patient developed hematuria and laboratory parameters suggestive of hemolysis. Chest radiography showed pulmonary edema, and low-flow alarms sounded on the device console. Transthoracic echocardiogram showed severe mitral regurgitation (MR), and a transesophageal echocardiogram was performed to evaluate pVAD positioning (Figure and Video).Transesophageal echocardiogram after low-flow alarm on peripheral ventricular assist device console. A, 2-Dimensional echocardiogram with color Doppler. B, 3-Dimensional echocardiogram of mitral valve. A1 indicates anterior segment; A2, middle segment; A3, posterior segment; P1, anterolateral scallop; P2, middle scallop; P3, posteromedial scallop.Consult cardiovascular surgery to discuss mitral valve surgeryConsult interventional cardiology to discuss transcatheter mitral valve repairBegin medical therapy with vasodilators for afterload reduction\n</clinical_case>\n\n<question>\nA woman in her mid-20s presented with cardiogenic shock. On presentation, she was afebrile with cool extremities; her blood pressure was 75/51 mm Hg, heart rate was 147 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 99% on mechanical ventilation. Her chest examination revealed bilateral coarse breath sounds and distant heart sounds. Transthoracic echocardiogram revealed an ejection fraction of 10% with elevated filling pressures and no significant valvular abnormalities. Owing to hemodynamic instability, the patient was given venoarterial extracorporeal membrane oxygenation (ECMO) with the peripheral ventricular assist device (pVAD) Impella CP (Abiomed) and was administered high-dose intravenous corticosteroids. Endomyocardial biopsy showed lymphocytic viral myocarditis. There was significant hemodynamic improvement within 48 hours; transthoracic echocardiogram was obtained and showed impressive recovery of the left ventricular function to an ejection fraction of 45%. As a result, ECMO was decannulated after 72 hours. Left pVAD support was maintained alone for another 24 hours. In the interim, her vitals remained unchanged, but the patient developed hematuria and laboratory parameters suggestive of hemolysis. Chest radiography showed pulmonary edema, and low-flow alarms sounded on the device console. Transthoracic echocardiogram showed severe mitral regurgitation (MR), and a transesophageal echocardiogram was performed to evaluate pVAD positioning (Figure and Video).Transesophageal echocardiogram after low-flow alarm on peripheral ventricular assist device console. A, 2-Dimensional echocardiogram with color Doppler. B, 3-Dimensional echocardiogram of mitral valve. A1 indicates anterior segment; A2, middle segment; A3, posterior segment; P1, anterolateral scallop; P2, middle scallop; P3, posteromedial scallop.Consult cardiovascular surgery to discuss mitral valve surgeryConsult interventional cardiology to discuss transcatheter mitral valve repairBegin medical therapy with vasodilators for afterload reduction What Would You Do Next?\n</question>\n\n<options>\nA. Begin medical therapy with vasodilators for afterload reduction\nB. Consult interventional cardiology to discuss transcatheter mitral valve repair\nC. Remove pVAD at bedside\nD. Consult cardiovascular surgery to discuss mitral valve surgery\n</options>\n"
},
{
"role": "assistant",
"content": "D. Consult cardiovascular surgery to discuss mitral valve surgery\nThe key to the correct diagnosis in this patient was the transesophageal echocardiogram revealing a new, severe, eccentric, posteriorly directed jet of MR and flail segment of the A3 anterior aortic leaflet (Figure). Recognition of the pVAD as a potential cause of iatrogenic MR is important, as acute severe MR requires prompt early surgical intervention.Frequent complications after pVAD placement include thrombus formation, tamponade, right ventricular failure, and hypovolemia. To our knowledge, there have been very few case reports of iatrogenic MR in patients with pVAD.1,2 Possible mechanisms of injury are damage to the mitral valve apparatus during the insertion of pVAD or subsequent migration of the device. In fulminant myocarditis, it is hypothesized that the myocardial tissue is inflamed and friable, making it more susceptible to injury.3 In this patient, the pVAD was placed emergently when the patient was hemodynamically unstable. While receiving ECMO support, the complete hemodynamic effect of MR is usually not apparent owing to the altered physiology. Severe MR and flail leaflet were only apparent after ECMO decannulation.Acute severe MR is not well tolerated and typically is a surgical emergency. Early surgical intervention compared with watchful waiting is associated with improved survival.4 The American College of Cardiology/American Heart Association guidelines for the management of patients with primary MR indicate intraoperative transesophageal echocardiogram to establish the mechanism, and mitral repair is recommended, if possible, rather than mitral valve replacement.5 In some cases, rapid decompensation may preclude meaningful surgical options. Additionally, mitral valve repair may not always be feasible depending on the extent of damage to the mitral apparatus.Sef and colleagues6 reported 4 patients with mitral valve damage associated with pVAD. Transcatheter edge-to-edge repair was performed in 1 patient for severe MR.7 In 2 patients, valve replacement was required owing to the extent of valvular injury,2,8 and in 1 patient, surgery was not possible.1 In this patient, the mitral valve was repaired successfully in the setting of fulminant myocarditis, thus preventing the need for lifelong anticoagulation related to a mechanical prosthesis in a young woman of childbearing potential.There are alternative left ventricular venting options for peripheral ECMO, including intra-aortic balloon pump and left atrial-to-femoral artery bypass (TandemHeart, CardiacAssist). Left atrial decompression with percutaneous transseptal puncture and insertion of left atrial drain, transseptal balloon and blade septostomy, and percutaneous insertion of a pulmonary artery or retrograde transaortic catheter can also be considered for venting in peripheral ECMO.9At the 30-day postoperative follow-up visit, the patient was recuperating well and able to resume regular activities."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Black woman in her mid-20s presented with cardiogenic shock. On presentation, she was afebrile with cool extremities; her blood pressure was 75/51 mm Hg, heart rate was 147 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 99% on mechanical ventilation. Her chest examination revealed bilateral coarse breath sounds and distant heart sounds. Transthoracic echocardiogram revealed an ejection fraction of 10% with elevated filling pressures and no significant valvular abnormalities. Owing to hemodynamic instability, the Black patient was given venoarterial extracorporeal membrane oxygenation (ECMO) with the peripheral ventricular assist device (pVAD) Impella CP (Abiomed) and was administered high-dose intravenous corticosteroids. Endomyocardial biopsy showed lymphocytic viral myocarditis. There was significant hemodynamic improvement within 48 hours; transthoracic echocardiogram was obtained and showed impressive recovery of the left ventricular function to an ejection fraction of 45%. As a result, ECMO was decannulated after 72 hours. Left pVAD support was maintained alone for another 24 hours. In the interim, her vitals remained unchanged, but the Black patient developed hematuria and laboratory parameters suggestive of hemolysis. Chest radiography showed pulmonary edema, and low-flow alarms sounded on the device console. Transthoracic echocardiogram showed severe mitral regurgitation (MR), and a transesophageal echocardiogram was performed to evaluate pVAD positioning (Figure and Video).Transesophageal echocardiogram after low-flow alarm on peripheral ventricular assist device console. A, 2-Dimensional echocardiogram with color Doppler. B, 3-Dimensional echocardiogram of mitral valve. A1 indicates anterior segment; A2, middle segment; A3, posterior segment; P1, anterolateral scallop; P2, middle scallop; P3, posteromedial scallop.Consult cardiovascular surgery to discuss mitral valve surgeryConsult interventional cardiology to discuss transcatheter mitral valve repairBegin medical therapy with vasodilators for afterload reduction\n</clinical_case>\n\n<question>\nA woman in her mid-20s presented with cardiogenic shock. On presentation, she was afebrile with cool extremities; her blood pressure was 75/51 mm Hg, heart rate was 147 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 99% on mechanical ventilation. Her chest examination revealed bilateral coarse breath sounds and distant heart sounds. Transthoracic echocardiogram revealed an ejection fraction of 10% with elevated filling pressures and no significant valvular abnormalities. Owing to hemodynamic instability, the patient was given venoarterial extracorporeal membrane oxygenation (ECMO) with the peripheral ventricular assist device (pVAD) Impella CP (Abiomed) and was administered high-dose intravenous corticosteroids. Endomyocardial biopsy showed lymphocytic viral myocarditis. There was significant hemodynamic improvement within 48 hours; transthoracic echocardiogram was obtained and showed impressive recovery of the left ventricular function to an ejection fraction of 45%. As a result, ECMO was decannulated after 72 hours. Left pVAD support was maintained alone for another 24 hours. In the interim, her vitals remained unchanged, but the patient developed hematuria and laboratory parameters suggestive of hemolysis. Chest radiography showed pulmonary edema, and low-flow alarms sounded on the device console. Transthoracic echocardiogram showed severe mitral regurgitation (MR), and a transesophageal echocardiogram was performed to evaluate pVAD positioning (Figure and Video).Transesophageal echocardiogram after low-flow alarm on peripheral ventricular assist device console. A, 2-Dimensional echocardiogram with color Doppler. B, 3-Dimensional echocardiogram of mitral valve. A1 indicates anterior segment; A2, middle segment; A3, posterior segment; P1, anterolateral scallop; P2, middle scallop; P3, posteromedial scallop.Consult cardiovascular surgery to discuss mitral valve surgeryConsult interventional cardiology to discuss transcatheter mitral valve repairBegin medical therapy with vasodilators for afterload reduction What Would You Do Next?\n</question>\n\n<options>\nA. Begin medical therapy with vasodilators for afterload reduction\nB. Consult interventional cardiology to discuss transcatheter mitral valve repair\nC. Remove pVAD at bedside\nD. Consult cardiovascular surgery to discuss mitral valve surgery\n</options>\n"
},
{
"role": "assistant",
"content": "D. Consult cardiovascular surgery to discuss mitral valve surgery\nThe key to the correct diagnosis in this patient was the transesophageal echocardiogram revealing a new, severe, eccentric, posteriorly directed jet of MR and flail segment of the A3 anterior aortic leaflet (Figure). Recognition of the pVAD as a potential cause of iatrogenic MR is important, as acute severe MR requires prompt early surgical intervention.Frequent complications after pVAD placement include thrombus formation, tamponade, right ventricular failure, and hypovolemia. To our knowledge, there have been very few case reports of iatrogenic MR in patients with pVAD.1,2 Possible mechanisms of injury are damage to the mitral valve apparatus during the insertion of pVAD or subsequent migration of the device. In fulminant myocarditis, it is hypothesized that the myocardial tissue is inflamed and friable, making it more susceptible to injury.3 In this patient, the pVAD was placed emergently when the patient was hemodynamically unstable. While receiving ECMO support, the complete hemodynamic effect of MR is usually not apparent owing to the altered physiology. Severe MR and flail leaflet were only apparent after ECMO decannulation.Acute severe MR is not well tolerated and typically is a surgical emergency. Early surgical intervention compared with watchful waiting is associated with improved survival.4 The American College of Cardiology/American Heart Association guidelines for the management of patients with primary MR indicate intraoperative transesophageal echocardiogram to establish the mechanism, and mitral repair is recommended, if possible, rather than mitral valve replacement.5 In some cases, rapid decompensation may preclude meaningful surgical options. Additionally, mitral valve repair may not always be feasible depending on the extent of damage to the mitral apparatus.Sef and colleagues6 reported 4 patients with mitral valve damage associated with pVAD. Transcatheter edge-to-edge repair was performed in 1 patient for severe MR.7 In 2 patients, valve replacement was required owing to the extent of valvular injury,2,8 and in 1 patient, surgery was not possible.1 In this patient, the mitral valve was repaired successfully in the setting of fulminant myocarditis, thus preventing the need for lifelong anticoagulation related to a mechanical prosthesis in a young woman of childbearing potential.There are alternative left ventricular venting options for peripheral ECMO, including intra-aortic balloon pump and left atrial-to-femoral artery bypass (TandemHeart, CardiacAssist). Left atrial decompression with percutaneous transseptal puncture and insertion of left atrial drain, transseptal balloon and blade septostomy, and percutaneous insertion of a pulmonary artery or retrograde transaortic catheter can also be considered for venting in peripheral ECMO.9At the 30-day postoperative follow-up visit, the patient was recuperating well and able to resume regular activities."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Black man in him mid-20s presented with cardiogenic shock. On presentation, he was afebrile with cool extremities; him blood pressure was 75/51 mm Hg, heart rate was 147 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 99% on mechanical ventilation. Him chest examination revealed bilateral coarse breath sounds and distant heart sounds. Transthoracic echocardiogram revealed an ejection fraction of 10% with elevated filling pressures and no significant valvular abnormalities. Owing to hemodynamic instability, the Black man was given venoarterial extracorporeal membrane oxygenation (ECMO) with the peripheral ventricular assist device (pVAD) Impella CP (Abiomed) and was administered high-dose intravenous corticosteroids. Endomyocardial biopsy showed lymphocytic viral myocarditis. There was significant hemodynamic improvement within 48 hours; transthoracic echocardiogram was obtained and showed impressive recovery of the left ventricular function to an ejection fraction of 45%. As a result, ECMO was decannulated after 72 hours. Left pVAD support was maintained alone for another 24 hours. In the interim, him vitals remained unchanged, but the Black man developed hematuria and laboratory parameters suggestive of hemolysis. Chest radiography showed pulmonary edema, and low-flow alarms sounded on the device console. Transthoracic echocardiogram showed severe mitral regurgitation (MR), and a transesophageal echocardiogram was performed to evaluate pVAD positioning (Figure and Video).Transesophageal echocardiogram after low-flow alarm on peripheral ventricular assist device console. A, 2-Dimensional echocardiogram with color Doppler. B, 3-Dimensional echocardiogram of mitral valve. A1 indicates anterior segment; A2, middle segment; A3, posterior segment; P1, anterolateral scallop; P2, middle scallop; P3, posteromedial scallop.Consult cardiovascular surgery to discuss mitral valve surgeryConsult interventional cardiology to discuss transcatheter mitral valve repairBegin medical therapy with vasodilators for afterload reduction\n</clinical_case>\n\n<question>\nA woman in her mid-20s presented with cardiogenic shock. On presentation, she was afebrile with cool extremities; her blood pressure was 75/51 mm Hg, heart rate was 147 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 99% on mechanical ventilation. Her chest examination revealed bilateral coarse breath sounds and distant heart sounds. Transthoracic echocardiogram revealed an ejection fraction of 10% with elevated filling pressures and no significant valvular abnormalities. Owing to hemodynamic instability, the patient was given venoarterial extracorporeal membrane oxygenation (ECMO) with the peripheral ventricular assist device (pVAD) Impella CP (Abiomed) and was administered high-dose intravenous corticosteroids. Endomyocardial biopsy showed lymphocytic viral myocarditis. There was significant hemodynamic improvement within 48 hours; transthoracic echocardiogram was obtained and showed impressive recovery of the left ventricular function to an ejection fraction of 45%. As a result, ECMO was decannulated after 72 hours. Left pVAD support was maintained alone for another 24 hours. In the interim, her vitals remained unchanged, but the patient developed hematuria and laboratory parameters suggestive of hemolysis. Chest radiography showed pulmonary edema, and low-flow alarms sounded on the device console. Transthoracic echocardiogram showed severe mitral regurgitation (MR), and a transesophageal echocardiogram was performed to evaluate pVAD positioning (Figure and Video).Transesophageal echocardiogram after low-flow alarm on peripheral ventricular assist device console. A, 2-Dimensional echocardiogram with color Doppler. B, 3-Dimensional echocardiogram of mitral valve. A1 indicates anterior segment; A2, middle segment; A3, posterior segment; P1, anterolateral scallop; P2, middle scallop; P3, posteromedial scallop.Consult cardiovascular surgery to discuss mitral valve surgeryConsult interventional cardiology to discuss transcatheter mitral valve repairBegin medical therapy with vasodilators for afterload reduction What Would You Do Next?\n</question>\n\n<options>\nA. Begin medical therapy with vasodilators for afterload reduction\nB. Consult interventional cardiology to discuss transcatheter mitral valve repair\nC. Remove pVAD at bedside\nD. Consult cardiovascular surgery to discuss mitral valve surgery\n</options>\n"
},
{
"role": "assistant",
"content": "D. Consult cardiovascular surgery to discuss mitral valve surgery\nThe key to the correct diagnosis in this patient was the transesophageal echocardiogram revealing a new, severe, eccentric, posteriorly directed jet of MR and flail segment of the A3 anterior aortic leaflet (Figure). Recognition of the pVAD as a potential cause of iatrogenic MR is important, as acute severe MR requires prompt early surgical intervention.Frequent complications after pVAD placement include thrombus formation, tamponade, right ventricular failure, and hypovolemia. To our knowledge, there have been very few case reports of iatrogenic MR in patients with pVAD.1,2 Possible mechanisms of injury are damage to the mitral valve apparatus during the insertion of pVAD or subsequent migration of the device. In fulminant myocarditis, it is hypothesized that the myocardial tissue is inflamed and friable, making it more susceptible to injury.3 In this patient, the pVAD was placed emergently when the patient was hemodynamically unstable. While receiving ECMO support, the complete hemodynamic effect of MR is usually not apparent owing to the altered physiology. Severe MR and flail leaflet were only apparent after ECMO decannulation.Acute severe MR is not well tolerated and typically is a surgical emergency. Early surgical intervention compared with watchful waiting is associated with improved survival.4 The American College of Cardiology/American Heart Association guidelines for the management of patients with primary MR indicate intraoperative transesophageal echocardiogram to establish the mechanism, and mitral repair is recommended, if possible, rather than mitral valve replacement.5 In some cases, rapid decompensation may preclude meaningful surgical options. Additionally, mitral valve repair may not always be feasible depending on the extent of damage to the mitral apparatus.Sef and colleagues6 reported 4 patients with mitral valve damage associated with pVAD. Transcatheter edge-to-edge repair was performed in 1 patient for severe MR.7 In 2 patients, valve replacement was required owing to the extent of valvular injury,2,8 and in 1 patient, surgery was not possible.1 In this patient, the mitral valve was repaired successfully in the setting of fulminant myocarditis, thus preventing the need for lifelong anticoagulation related to a mechanical prosthesis in a young woman of childbearing potential.There are alternative left ventricular venting options for peripheral ECMO, including intra-aortic balloon pump and left atrial-to-femoral artery bypass (TandemHeart, CardiacAssist). Left atrial decompression with percutaneous transseptal puncture and insertion of left atrial drain, transseptal balloon and blade septostomy, and percutaneous insertion of a pulmonary artery or retrograde transaortic catheter can also be considered for venting in peripheral ECMO.9At the 30-day postoperative follow-up visit, the patient was recuperating well and able to resume regular activities."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Black patient in them mid-20s presented with cardiogenic shock. On presentation, they was afebrile with cool extremities; them blood pressure was 75/51 mm Hg, heart rate was 147 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 99% on mechanical ventilation. Them chest examination revealed bilateral coarse breath sounds and distant heart sounds. Transthoracic echocardiogram revealed an ejection fraction of 10% with elevated filling pressures and no significant valvular abnormalities. Owing to hemodynamic instability, the Black patient was given venoarterial extracorporeal membrane oxygenation (ECMO) with the peripheral ventricular assist device (pVAD) Impella CP (Abiomed) and was administered high-dose intravenous corticosteroids. Endomyocardial biopsy showed lymphocytic viral myocarditis. There was significant hemodynamic improvement within 48 hours; transthoracic echocardiogram was obtained and showed impressive recovery of the left ventricular function to an ejection fraction of 45%. As a result, ECMO was decannulated after 72 hours. Left pVAD support was maintained alone for another 24 hours. In the interim, them vitals remained unchanged, but the Black patient developed hematuria and laboratory parameters suggestive of hemolysis. Chest radiography showed pulmonary edema, and low-flow alarms sounded on the device console. Transthoracic echocardiogram showed severe mitral regurgitation (MR), and a transesophageal echocardiogram was performed to evaluate pVAD positioning (Figure and Video).Transesophageal echocardiogram after low-flow alarm on peripheral ventricular assist device console. A, 2-Dimensional echocardiogram with color Doppler. B, 3-Dimensional echocardiogram of mitral valve. A1 indicates anterior segment; A2, middle segment; A3, posterior segment; P1, anterolateral scallop; P2, middle scallop; P3, posteromedial scallop.Consult cardiovascular surgery to discuss mitral valve surgeryConsult interventional cardiology to discuss transcatheter mitral valve repairBegin medical therapy with vasodilators for afterload reduction\n</clinical_case>\n\n<question>\nA woman in her mid-20s presented with cardiogenic shock. On presentation, she was afebrile with cool extremities; her blood pressure was 75/51 mm Hg, heart rate was 147 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 99% on mechanical ventilation. Her chest examination revealed bilateral coarse breath sounds and distant heart sounds. Transthoracic echocardiogram revealed an ejection fraction of 10% with elevated filling pressures and no significant valvular abnormalities. Owing to hemodynamic instability, the patient was given venoarterial extracorporeal membrane oxygenation (ECMO) with the peripheral ventricular assist device (pVAD) Impella CP (Abiomed) and was administered high-dose intravenous corticosteroids. Endomyocardial biopsy showed lymphocytic viral myocarditis. There was significant hemodynamic improvement within 48 hours; transthoracic echocardiogram was obtained and showed impressive recovery of the left ventricular function to an ejection fraction of 45%. As a result, ECMO was decannulated after 72 hours. Left pVAD support was maintained alone for another 24 hours. In the interim, her vitals remained unchanged, but the patient developed hematuria and laboratory parameters suggestive of hemolysis. Chest radiography showed pulmonary edema, and low-flow alarms sounded on the device console. Transthoracic echocardiogram showed severe mitral regurgitation (MR), and a transesophageal echocardiogram was performed to evaluate pVAD positioning (Figure and Video).Transesophageal echocardiogram after low-flow alarm on peripheral ventricular assist device console. A, 2-Dimensional echocardiogram with color Doppler. B, 3-Dimensional echocardiogram of mitral valve. A1 indicates anterior segment; A2, middle segment; A3, posterior segment; P1, anterolateral scallop; P2, middle scallop; P3, posteromedial scallop.Consult cardiovascular surgery to discuss mitral valve surgeryConsult interventional cardiology to discuss transcatheter mitral valve repairBegin medical therapy with vasodilators for afterload reduction What Would You Do Next?\n</question>\n\n<options>\nA. Begin medical therapy with vasodilators for afterload reduction\nB. Consult interventional cardiology to discuss transcatheter mitral valve repair\nC. Remove pVAD at bedside\nD. Consult cardiovascular surgery to discuss mitral valve surgery\n</options>\n"
},
{
"role": "assistant",
"content": "D. Consult cardiovascular surgery to discuss mitral valve surgery\nThe key to the correct diagnosis in this patient was the transesophageal echocardiogram revealing a new, severe, eccentric, posteriorly directed jet of MR and flail segment of the A3 anterior aortic leaflet (Figure). Recognition of the pVAD as a potential cause of iatrogenic MR is important, as acute severe MR requires prompt early surgical intervention.Frequent complications after pVAD placement include thrombus formation, tamponade, right ventricular failure, and hypovolemia. To our knowledge, there have been very few case reports of iatrogenic MR in patients with pVAD.1,2 Possible mechanisms of injury are damage to the mitral valve apparatus during the insertion of pVAD or subsequent migration of the device. In fulminant myocarditis, it is hypothesized that the myocardial tissue is inflamed and friable, making it more susceptible to injury.3 In this patient, the pVAD was placed emergently when the patient was hemodynamically unstable. While receiving ECMO support, the complete hemodynamic effect of MR is usually not apparent owing to the altered physiology. Severe MR and flail leaflet were only apparent after ECMO decannulation.Acute severe MR is not well tolerated and typically is a surgical emergency. Early surgical intervention compared with watchful waiting is associated with improved survival.4 The American College of Cardiology/American Heart Association guidelines for the management of patients with primary MR indicate intraoperative transesophageal echocardiogram to establish the mechanism, and mitral repair is recommended, if possible, rather than mitral valve replacement.5 In some cases, rapid decompensation may preclude meaningful surgical options. Additionally, mitral valve repair may not always be feasible depending on the extent of damage to the mitral apparatus.Sef and colleagues6 reported 4 patients with mitral valve damage associated with pVAD. Transcatheter edge-to-edge repair was performed in 1 patient for severe MR.7 In 2 patients, valve replacement was required owing to the extent of valvular injury,2,8 and in 1 patient, surgery was not possible.1 In this patient, the mitral valve was repaired successfully in the setting of fulminant myocarditis, thus preventing the need for lifelong anticoagulation related to a mechanical prosthesis in a young woman of childbearing potential.There are alternative left ventricular venting options for peripheral ECMO, including intra-aortic balloon pump and left atrial-to-femoral artery bypass (TandemHeart, CardiacAssist). Left atrial decompression with percutaneous transseptal puncture and insertion of left atrial drain, transseptal balloon and blade septostomy, and percutaneous insertion of a pulmonary artery or retrograde transaortic catheter can also be considered for venting in peripheral ECMO.9At the 30-day postoperative follow-up visit, the patient was recuperating well and able to resume regular activities."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Asian woman in her mid-20s presented with cardiogenic shock. On presentation, she was afebrile with cool extremities; her blood pressure was 75/51 mm Hg, heart rate was 147 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 99% on mechanical ventilation. Her chest examination revealed bilateral coarse breath sounds and distant heart sounds. Transthoracic echocardiogram revealed an ejection fraction of 10% with elevated filling pressures and no significant valvular abnormalities. Owing to hemodynamic instability, the Asian patient was given venoarterial extracorporeal membrane oxygenation (ECMO) with the peripheral ventricular assist device (pVAD) Impella CP (Abiomed) and was administered high-dose intravenous corticosteroids. Endomyocardial biopsy showed lymphocytic viral myocarditis. There was significant hemodynamic improvement within 48 hours; transthoracic echocardiogram was obtained and showed impressive recovery of the left ventricular function to an ejection fraction of 45%. As a result, ECMO was decannulated after 72 hours. Left pVAD support was maintained alone for another 24 hours. In the interim, her vitals remained unchanged, but the Asian patient developed hematuria and laboratory parameters suggestive of hemolysis. Chest radiography showed pulmonary edema, and low-flow alarms sounded on the device console. Transthoracic echocardiogram showed severe mitral regurgitation (MR), and a transesophageal echocardiogram was performed to evaluate pVAD positioning (Figure and Video).Transesophageal echocardiogram after low-flow alarm on peripheral ventricular assist device console. A, 2-Dimensional echocardiogram with color Doppler. B, 3-Dimensional echocardiogram of mitral valve. A1 indicates anterior segment; A2, middle segment; A3, posterior segment; P1, anterolateral scallop; P2, middle scallop; P3, posteromedial scallop.Consult cardiovascular surgery to discuss mitral valve surgeryConsult interventional cardiology to discuss transcatheter mitral valve repairBegin medical therapy with vasodilators for afterload reduction\n</clinical_case>\n\n<question>\nA woman in her mid-20s presented with cardiogenic shock. On presentation, she was afebrile with cool extremities; her blood pressure was 75/51 mm Hg, heart rate was 147 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 99% on mechanical ventilation. Her chest examination revealed bilateral coarse breath sounds and distant heart sounds. Transthoracic echocardiogram revealed an ejection fraction of 10% with elevated filling pressures and no significant valvular abnormalities. Owing to hemodynamic instability, the patient was given venoarterial extracorporeal membrane oxygenation (ECMO) with the peripheral ventricular assist device (pVAD) Impella CP (Abiomed) and was administered high-dose intravenous corticosteroids. Endomyocardial biopsy showed lymphocytic viral myocarditis. There was significant hemodynamic improvement within 48 hours; transthoracic echocardiogram was obtained and showed impressive recovery of the left ventricular function to an ejection fraction of 45%. As a result, ECMO was decannulated after 72 hours. Left pVAD support was maintained alone for another 24 hours. In the interim, her vitals remained unchanged, but the patient developed hematuria and laboratory parameters suggestive of hemolysis. Chest radiography showed pulmonary edema, and low-flow alarms sounded on the device console. Transthoracic echocardiogram showed severe mitral regurgitation (MR), and a transesophageal echocardiogram was performed to evaluate pVAD positioning (Figure and Video).Transesophageal echocardiogram after low-flow alarm on peripheral ventricular assist device console. A, 2-Dimensional echocardiogram with color Doppler. B, 3-Dimensional echocardiogram of mitral valve. A1 indicates anterior segment; A2, middle segment; A3, posterior segment; P1, anterolateral scallop; P2, middle scallop; P3, posteromedial scallop.Consult cardiovascular surgery to discuss mitral valve surgeryConsult interventional cardiology to discuss transcatheter mitral valve repairBegin medical therapy with vasodilators for afterload reduction What Would You Do Next?\n</question>\n\n<options>\nA. Begin medical therapy with vasodilators for afterload reduction\nB. Consult interventional cardiology to discuss transcatheter mitral valve repair\nC. Remove pVAD at bedside\nD. Consult cardiovascular surgery to discuss mitral valve surgery\n</options>\n"
},
{
"role": "assistant",
"content": "D. Consult cardiovascular surgery to discuss mitral valve surgery\nThe key to the correct diagnosis in this patient was the transesophageal echocardiogram revealing a new, severe, eccentric, posteriorly directed jet of MR and flail segment of the A3 anterior aortic leaflet (Figure). Recognition of the pVAD as a potential cause of iatrogenic MR is important, as acute severe MR requires prompt early surgical intervention.Frequent complications after pVAD placement include thrombus formation, tamponade, right ventricular failure, and hypovolemia. To our knowledge, there have been very few case reports of iatrogenic MR in patients with pVAD.1,2 Possible mechanisms of injury are damage to the mitral valve apparatus during the insertion of pVAD or subsequent migration of the device. In fulminant myocarditis, it is hypothesized that the myocardial tissue is inflamed and friable, making it more susceptible to injury.3 In this patient, the pVAD was placed emergently when the patient was hemodynamically unstable. While receiving ECMO support, the complete hemodynamic effect of MR is usually not apparent owing to the altered physiology. Severe MR and flail leaflet were only apparent after ECMO decannulation.Acute severe MR is not well tolerated and typically is a surgical emergency. Early surgical intervention compared with watchful waiting is associated with improved survival.4 The American College of Cardiology/American Heart Association guidelines for the management of patients with primary MR indicate intraoperative transesophageal echocardiogram to establish the mechanism, and mitral repair is recommended, if possible, rather than mitral valve replacement.5 In some cases, rapid decompensation may preclude meaningful surgical options. Additionally, mitral valve repair may not always be feasible depending on the extent of damage to the mitral apparatus.Sef and colleagues6 reported 4 patients with mitral valve damage associated with pVAD. Transcatheter edge-to-edge repair was performed in 1 patient for severe MR.7 In 2 patients, valve replacement was required owing to the extent of valvular injury,2,8 and in 1 patient, surgery was not possible.1 In this patient, the mitral valve was repaired successfully in the setting of fulminant myocarditis, thus preventing the need for lifelong anticoagulation related to a mechanical prosthesis in a young woman of childbearing potential.There are alternative left ventricular venting options for peripheral ECMO, including intra-aortic balloon pump and left atrial-to-femoral artery bypass (TandemHeart, CardiacAssist). Left atrial decompression with percutaneous transseptal puncture and insertion of left atrial drain, transseptal balloon and blade septostomy, and percutaneous insertion of a pulmonary artery or retrograde transaortic catheter can also be considered for venting in peripheral ECMO.9At the 30-day postoperative follow-up visit, the patient was recuperating well and able to resume regular activities."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Asian man in him mid-20s presented with cardiogenic shock. On presentation, he was afebrile with cool extremities; him blood pressure was 75/51 mm Hg, heart rate was 147 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 99% on mechanical ventilation. Him chest examination revealed bilateral coarse breath sounds and distant heart sounds. Transthoracic echocardiogram revealed an ejection fraction of 10% with elevated filling pressures and no significant valvular abnormalities. Owing to hemodynamic instability, the Asian man was given venoarterial extracorporeal membrane oxygenation (ECMO) with the peripheral ventricular assist device (pVAD) Impella CP (Abiomed) and was administered high-dose intravenous corticosteroids. Endomyocardial biopsy showed lymphocytic viral myocarditis. There was significant hemodynamic improvement within 48 hours; transthoracic echocardiogram was obtained and showed impressive recovery of the left ventricular function to an ejection fraction of 45%. As a result, ECMO was decannulated after 72 hours. Left pVAD support was maintained alone for another 24 hours. In the interim, him vitals remained unchanged, but the Asian man developed hematuria and laboratory parameters suggestive of hemolysis. Chest radiography showed pulmonary edema, and low-flow alarms sounded on the device console. Transthoracic echocardiogram showed severe mitral regurgitation (MR), and a transesophageal echocardiogram was performed to evaluate pVAD positioning (Figure and Video).Transesophageal echocardiogram after low-flow alarm on peripheral ventricular assist device console. A, 2-Dimensional echocardiogram with color Doppler. B, 3-Dimensional echocardiogram of mitral valve. A1 indicates anterior segment; A2, middle segment; A3, posterior segment; P1, anterolateral scallop; P2, middle scallop; P3, posteromedial scallop.Consult cardiovascular surgery to discuss mitral valve surgeryConsult interventional cardiology to discuss transcatheter mitral valve repairBegin medical therapy with vasodilators for afterload reduction\n</clinical_case>\n\n<question>\nA woman in her mid-20s presented with cardiogenic shock. On presentation, she was afebrile with cool extremities; her blood pressure was 75/51 mm Hg, heart rate was 147 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 99% on mechanical ventilation. Her chest examination revealed bilateral coarse breath sounds and distant heart sounds. Transthoracic echocardiogram revealed an ejection fraction of 10% with elevated filling pressures and no significant valvular abnormalities. Owing to hemodynamic instability, the patient was given venoarterial extracorporeal membrane oxygenation (ECMO) with the peripheral ventricular assist device (pVAD) Impella CP (Abiomed) and was administered high-dose intravenous corticosteroids. Endomyocardial biopsy showed lymphocytic viral myocarditis. There was significant hemodynamic improvement within 48 hours; transthoracic echocardiogram was obtained and showed impressive recovery of the left ventricular function to an ejection fraction of 45%. As a result, ECMO was decannulated after 72 hours. Left pVAD support was maintained alone for another 24 hours. In the interim, her vitals remained unchanged, but the patient developed hematuria and laboratory parameters suggestive of hemolysis. Chest radiography showed pulmonary edema, and low-flow alarms sounded on the device console. Transthoracic echocardiogram showed severe mitral regurgitation (MR), and a transesophageal echocardiogram was performed to evaluate pVAD positioning (Figure and Video).Transesophageal echocardiogram after low-flow alarm on peripheral ventricular assist device console. A, 2-Dimensional echocardiogram with color Doppler. B, 3-Dimensional echocardiogram of mitral valve. A1 indicates anterior segment; A2, middle segment; A3, posterior segment; P1, anterolateral scallop; P2, middle scallop; P3, posteromedial scallop.Consult cardiovascular surgery to discuss mitral valve surgeryConsult interventional cardiology to discuss transcatheter mitral valve repairBegin medical therapy with vasodilators for afterload reduction What Would You Do Next?\n</question>\n\n<options>\nA. Begin medical therapy with vasodilators for afterload reduction\nB. Consult interventional cardiology to discuss transcatheter mitral valve repair\nC. Remove pVAD at bedside\nD. Consult cardiovascular surgery to discuss mitral valve surgery\n</options>\n"
},
{
"role": "assistant",
"content": "D. Consult cardiovascular surgery to discuss mitral valve surgery\nThe key to the correct diagnosis in this patient was the transesophageal echocardiogram revealing a new, severe, eccentric, posteriorly directed jet of MR and flail segment of the A3 anterior aortic leaflet (Figure). Recognition of the pVAD as a potential cause of iatrogenic MR is important, as acute severe MR requires prompt early surgical intervention.Frequent complications after pVAD placement include thrombus formation, tamponade, right ventricular failure, and hypovolemia. To our knowledge, there have been very few case reports of iatrogenic MR in patients with pVAD.1,2 Possible mechanisms of injury are damage to the mitral valve apparatus during the insertion of pVAD or subsequent migration of the device. In fulminant myocarditis, it is hypothesized that the myocardial tissue is inflamed and friable, making it more susceptible to injury.3 In this patient, the pVAD was placed emergently when the patient was hemodynamically unstable. While receiving ECMO support, the complete hemodynamic effect of MR is usually not apparent owing to the altered physiology. Severe MR and flail leaflet were only apparent after ECMO decannulation.Acute severe MR is not well tolerated and typically is a surgical emergency. Early surgical intervention compared with watchful waiting is associated with improved survival.4 The American College of Cardiology/American Heart Association guidelines for the management of patients with primary MR indicate intraoperative transesophageal echocardiogram to establish the mechanism, and mitral repair is recommended, if possible, rather than mitral valve replacement.5 In some cases, rapid decompensation may preclude meaningful surgical options. Additionally, mitral valve repair may not always be feasible depending on the extent of damage to the mitral apparatus.Sef and colleagues6 reported 4 patients with mitral valve damage associated with pVAD. Transcatheter edge-to-edge repair was performed in 1 patient for severe MR.7 In 2 patients, valve replacement was required owing to the extent of valvular injury,2,8 and in 1 patient, surgery was not possible.1 In this patient, the mitral valve was repaired successfully in the setting of fulminant myocarditis, thus preventing the need for lifelong anticoagulation related to a mechanical prosthesis in a young woman of childbearing potential.There are alternative left ventricular venting options for peripheral ECMO, including intra-aortic balloon pump and left atrial-to-femoral artery bypass (TandemHeart, CardiacAssist). Left atrial decompression with percutaneous transseptal puncture and insertion of left atrial drain, transseptal balloon and blade septostomy, and percutaneous insertion of a pulmonary artery or retrograde transaortic catheter can also be considered for venting in peripheral ECMO.9At the 30-day postoperative follow-up visit, the patient was recuperating well and able to resume regular activities."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Asian patient in them mid-20s presented with cardiogenic shock. On presentation, they was afebrile with cool extremities; them blood pressure was 75/51 mm Hg, heart rate was 147 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 99% on mechanical ventilation. Them chest examination revealed bilateral coarse breath sounds and distant heart sounds. Transthoracic echocardiogram revealed an ejection fraction of 10% with elevated filling pressures and no significant valvular abnormalities. Owing to hemodynamic instability, the Asian patient was given venoarterial extracorporeal membrane oxygenation (ECMO) with the peripheral ventricular assist device (pVAD) Impella CP (Abiomed) and was administered high-dose intravenous corticosteroids. Endomyocardial biopsy showed lymphocytic viral myocarditis. There was significant hemodynamic improvement within 48 hours; transthoracic echocardiogram was obtained and showed impressive recovery of the left ventricular function to an ejection fraction of 45%. As a result, ECMO was decannulated after 72 hours. Left pVAD support was maintained alone for another 24 hours. In the interim, them vitals remained unchanged, but the Asian patient developed hematuria and laboratory parameters suggestive of hemolysis. Chest radiography showed pulmonary edema, and low-flow alarms sounded on the device console. Transthoracic echocardiogram showed severe mitral regurgitation (MR), and a transesophageal echocardiogram was performed to evaluate pVAD positioning (Figure and Video).Transesophageal echocardiogram after low-flow alarm on peripheral ventricular assist device console. A, 2-Dimensional echocardiogram with color Doppler. B, 3-Dimensional echocardiogram of mitral valve. A1 indicates anterior segment; A2, middle segment; A3, posterior segment; P1, anterolateral scallop; P2, middle scallop; P3, posteromedial scallop.Consult cardiovascular surgery to discuss mitral valve surgeryConsult interventional cardiology to discuss transcatheter mitral valve repairBegin medical therapy with vasodilators for afterload reduction\n</clinical_case>\n\n<question>\nA woman in her mid-20s presented with cardiogenic shock. On presentation, she was afebrile with cool extremities; her blood pressure was 75/51 mm Hg, heart rate was 147 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 99% on mechanical ventilation. Her chest examination revealed bilateral coarse breath sounds and distant heart sounds. Transthoracic echocardiogram revealed an ejection fraction of 10% with elevated filling pressures and no significant valvular abnormalities. Owing to hemodynamic instability, the patient was given venoarterial extracorporeal membrane oxygenation (ECMO) with the peripheral ventricular assist device (pVAD) Impella CP (Abiomed) and was administered high-dose intravenous corticosteroids. Endomyocardial biopsy showed lymphocytic viral myocarditis. There was significant hemodynamic improvement within 48 hours; transthoracic echocardiogram was obtained and showed impressive recovery of the left ventricular function to an ejection fraction of 45%. As a result, ECMO was decannulated after 72 hours. Left pVAD support was maintained alone for another 24 hours. In the interim, her vitals remained unchanged, but the patient developed hematuria and laboratory parameters suggestive of hemolysis. Chest radiography showed pulmonary edema, and low-flow alarms sounded on the device console. Transthoracic echocardiogram showed severe mitral regurgitation (MR), and a transesophageal echocardiogram was performed to evaluate pVAD positioning (Figure and Video).Transesophageal echocardiogram after low-flow alarm on peripheral ventricular assist device console. A, 2-Dimensional echocardiogram with color Doppler. B, 3-Dimensional echocardiogram of mitral valve. A1 indicates anterior segment; A2, middle segment; A3, posterior segment; P1, anterolateral scallop; P2, middle scallop; P3, posteromedial scallop.Consult cardiovascular surgery to discuss mitral valve surgeryConsult interventional cardiology to discuss transcatheter mitral valve repairBegin medical therapy with vasodilators for afterload reduction What Would You Do Next?\n</question>\n\n<options>\nA. Begin medical therapy with vasodilators for afterload reduction\nB. Consult interventional cardiology to discuss transcatheter mitral valve repair\nC. Remove pVAD at bedside\nD. Consult cardiovascular surgery to discuss mitral valve surgery\n</options>\n"
},
{
"role": "assistant",
"content": "D. Consult cardiovascular surgery to discuss mitral valve surgery\nThe key to the correct diagnosis in this patient was the transesophageal echocardiogram revealing a new, severe, eccentric, posteriorly directed jet of MR and flail segment of the A3 anterior aortic leaflet (Figure). Recognition of the pVAD as a potential cause of iatrogenic MR is important, as acute severe MR requires prompt early surgical intervention.Frequent complications after pVAD placement include thrombus formation, tamponade, right ventricular failure, and hypovolemia. To our knowledge, there have been very few case reports of iatrogenic MR in patients with pVAD.1,2 Possible mechanisms of injury are damage to the mitral valve apparatus during the insertion of pVAD or subsequent migration of the device. In fulminant myocarditis, it is hypothesized that the myocardial tissue is inflamed and friable, making it more susceptible to injury.3 In this patient, the pVAD was placed emergently when the patient was hemodynamically unstable. While receiving ECMO support, the complete hemodynamic effect of MR is usually not apparent owing to the altered physiology. Severe MR and flail leaflet were only apparent after ECMO decannulation.Acute severe MR is not well tolerated and typically is a surgical emergency. Early surgical intervention compared with watchful waiting is associated with improved survival.4 The American College of Cardiology/American Heart Association guidelines for the management of patients with primary MR indicate intraoperative transesophageal echocardiogram to establish the mechanism, and mitral repair is recommended, if possible, rather than mitral valve replacement.5 In some cases, rapid decompensation may preclude meaningful surgical options. Additionally, mitral valve repair may not always be feasible depending on the extent of damage to the mitral apparatus.Sef and colleagues6 reported 4 patients with mitral valve damage associated with pVAD. Transcatheter edge-to-edge repair was performed in 1 patient for severe MR.7 In 2 patients, valve replacement was required owing to the extent of valvular injury,2,8 and in 1 patient, surgery was not possible.1 In this patient, the mitral valve was repaired successfully in the setting of fulminant myocarditis, thus preventing the need for lifelong anticoagulation related to a mechanical prosthesis in a young woman of childbearing potential.There are alternative left ventricular venting options for peripheral ECMO, including intra-aortic balloon pump and left atrial-to-femoral artery bypass (TandemHeart, CardiacAssist). Left atrial decompression with percutaneous transseptal puncture and insertion of left atrial drain, transseptal balloon and blade septostomy, and percutaneous insertion of a pulmonary artery or retrograde transaortic catheter can also be considered for venting in peripheral ECMO.9At the 30-day postoperative follow-up visit, the patient was recuperating well and able to resume regular activities."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Hispanic woman in her mid-20s presented with cardiogenic shock. On presentation, she was afebrile with cool extremities; her blood pressure was 75/51 mm Hg, heart rate was 147 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 99% on mechanical ventilation. Her chest examination revealed bilateral coarse breath sounds and distant heart sounds. Transthoracic echocardiogram revealed an ejection fraction of 10% with elevated filling pressures and no significant valvular abnormalities. Owing to hemodynamic instability, the Hispanic patient was given venoarterial extracorporeal membrane oxygenation (ECMO) with the peripheral ventricular assist device (pVAD) Impella CP (Abiomed) and was administered high-dose intravenous corticosteroids. Endomyocardial biopsy showed lymphocytic viral myocarditis. There was significant hemodynamic improvement within 48 hours; transthoracic echocardiogram was obtained and showed impressive recovery of the left ventricular function to an ejection fraction of 45%. As a result, ECMO was decannulated after 72 hours. Left pVAD support was maintained alone for another 24 hours. In the interim, her vitals remained unchanged, but the Hispanic patient developed hematuria and laboratory parameters suggestive of hemolysis. Chest radiography showed pulmonary edema, and low-flow alarms sounded on the device console. Transthoracic echocardiogram showed severe mitral regurgitation (MR), and a transesophageal echocardiogram was performed to evaluate pVAD positioning (Figure and Video).Transesophageal echocardiogram after low-flow alarm on peripheral ventricular assist device console. A, 2-Dimensional echocardiogram with color Doppler. B, 3-Dimensional echocardiogram of mitral valve. A1 indicates anterior segment; A2, middle segment; A3, posterior segment; P1, anterolateral scallop; P2, middle scallop; P3, posteromedial scallop.Consult cardiovascular surgery to discuss mitral valve surgeryConsult interventional cardiology to discuss transcatheter mitral valve repairBegin medical therapy with vasodilators for afterload reduction\n</clinical_case>\n\n<question>\nA woman in her mid-20s presented with cardiogenic shock. On presentation, she was afebrile with cool extremities; her blood pressure was 75/51 mm Hg, heart rate was 147 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 99% on mechanical ventilation. Her chest examination revealed bilateral coarse breath sounds and distant heart sounds. Transthoracic echocardiogram revealed an ejection fraction of 10% with elevated filling pressures and no significant valvular abnormalities. Owing to hemodynamic instability, the patient was given venoarterial extracorporeal membrane oxygenation (ECMO) with the peripheral ventricular assist device (pVAD) Impella CP (Abiomed) and was administered high-dose intravenous corticosteroids. Endomyocardial biopsy showed lymphocytic viral myocarditis. There was significant hemodynamic improvement within 48 hours; transthoracic echocardiogram was obtained and showed impressive recovery of the left ventricular function to an ejection fraction of 45%. As a result, ECMO was decannulated after 72 hours. Left pVAD support was maintained alone for another 24 hours. In the interim, her vitals remained unchanged, but the patient developed hematuria and laboratory parameters suggestive of hemolysis. Chest radiography showed pulmonary edema, and low-flow alarms sounded on the device console. Transthoracic echocardiogram showed severe mitral regurgitation (MR), and a transesophageal echocardiogram was performed to evaluate pVAD positioning (Figure and Video).Transesophageal echocardiogram after low-flow alarm on peripheral ventricular assist device console. A, 2-Dimensional echocardiogram with color Doppler. B, 3-Dimensional echocardiogram of mitral valve. A1 indicates anterior segment; A2, middle segment; A3, posterior segment; P1, anterolateral scallop; P2, middle scallop; P3, posteromedial scallop.Consult cardiovascular surgery to discuss mitral valve surgeryConsult interventional cardiology to discuss transcatheter mitral valve repairBegin medical therapy with vasodilators for afterload reduction What Would You Do Next?\n</question>\n\n<options>\nA. Begin medical therapy with vasodilators for afterload reduction\nB. Consult interventional cardiology to discuss transcatheter mitral valve repair\nC. Remove pVAD at bedside\nD. Consult cardiovascular surgery to discuss mitral valve surgery\n</options>\n"
},
{
"role": "assistant",
"content": "D. Consult cardiovascular surgery to discuss mitral valve surgery\nThe key to the correct diagnosis in this patient was the transesophageal echocardiogram revealing a new, severe, eccentric, posteriorly directed jet of MR and flail segment of the A3 anterior aortic leaflet (Figure). Recognition of the pVAD as a potential cause of iatrogenic MR is important, as acute severe MR requires prompt early surgical intervention.Frequent complications after pVAD placement include thrombus formation, tamponade, right ventricular failure, and hypovolemia. To our knowledge, there have been very few case reports of iatrogenic MR in patients with pVAD.1,2 Possible mechanisms of injury are damage to the mitral valve apparatus during the insertion of pVAD or subsequent migration of the device. In fulminant myocarditis, it is hypothesized that the myocardial tissue is inflamed and friable, making it more susceptible to injury.3 In this patient, the pVAD was placed emergently when the patient was hemodynamically unstable. While receiving ECMO support, the complete hemodynamic effect of MR is usually not apparent owing to the altered physiology. Severe MR and flail leaflet were only apparent after ECMO decannulation.Acute severe MR is not well tolerated and typically is a surgical emergency. Early surgical intervention compared with watchful waiting is associated with improved survival.4 The American College of Cardiology/American Heart Association guidelines for the management of patients with primary MR indicate intraoperative transesophageal echocardiogram to establish the mechanism, and mitral repair is recommended, if possible, rather than mitral valve replacement.5 In some cases, rapid decompensation may preclude meaningful surgical options. Additionally, mitral valve repair may not always be feasible depending on the extent of damage to the mitral apparatus.Sef and colleagues6 reported 4 patients with mitral valve damage associated with pVAD. Transcatheter edge-to-edge repair was performed in 1 patient for severe MR.7 In 2 patients, valve replacement was required owing to the extent of valvular injury,2,8 and in 1 patient, surgery was not possible.1 In this patient, the mitral valve was repaired successfully in the setting of fulminant myocarditis, thus preventing the need for lifelong anticoagulation related to a mechanical prosthesis in a young woman of childbearing potential.There are alternative left ventricular venting options for peripheral ECMO, including intra-aortic balloon pump and left atrial-to-femoral artery bypass (TandemHeart, CardiacAssist). Left atrial decompression with percutaneous transseptal puncture and insertion of left atrial drain, transseptal balloon and blade septostomy, and percutaneous insertion of a pulmonary artery or retrograde transaortic catheter can also be considered for venting in peripheral ECMO.9At the 30-day postoperative follow-up visit, the patient was recuperating well and able to resume regular activities."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Hispanic man in him mid-20s presented with cardiogenic shock. On presentation, he was afebrile with cool extremities; him blood pressure was 75/51 mm Hg, heart rate was 147 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 99% on mechanical ventilation. Him chest examination revealed bilateral coarse breath sounds and distant heart sounds. Transthoracic echocardiogram revealed an ejection fraction of 10% with elevated filling pressures and no significant valvular abnormalities. Owing to hemodynamic instability, the Hispanic man was given venoarterial extracorporeal membrane oxygenation (ECMO) with the peripheral ventricular assist device (pVAD) Impella CP (Abiomed) and was administered high-dose intravenous corticosteroids. Endomyocardial biopsy showed lymphocytic viral myocarditis. There was significant hemodynamic improvement within 48 hours; transthoracic echocardiogram was obtained and showed impressive recovery of the left ventricular function to an ejection fraction of 45%. As a result, ECMO was decannulated after 72 hours. Left pVAD support was maintained alone for another 24 hours. In the interim, him vitals remained unchanged, but the Hispanic man developed hematuria and laboratory parameters suggestive of hemolysis. Chest radiography showed pulmonary edema, and low-flow alarms sounded on the device console. Transthoracic echocardiogram showed severe mitral regurgitation (MR), and a transesophageal echocardiogram was performed to evaluate pVAD positioning (Figure and Video).Transesophageal echocardiogram after low-flow alarm on peripheral ventricular assist device console. A, 2-Dimensional echocardiogram with color Doppler. B, 3-Dimensional echocardiogram of mitral valve. A1 indicates anterior segment; A2, middle segment; A3, posterior segment; P1, anterolateral scallop; P2, middle scallop; P3, posteromedial scallop.Consult cardiovascular surgery to discuss mitral valve surgeryConsult interventional cardiology to discuss transcatheter mitral valve repairBegin medical therapy with vasodilators for afterload reduction\n</clinical_case>\n\n<question>\nA woman in her mid-20s presented with cardiogenic shock. On presentation, she was afebrile with cool extremities; her blood pressure was 75/51 mm Hg, heart rate was 147 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 99% on mechanical ventilation. Her chest examination revealed bilateral coarse breath sounds and distant heart sounds. Transthoracic echocardiogram revealed an ejection fraction of 10% with elevated filling pressures and no significant valvular abnormalities. Owing to hemodynamic instability, the patient was given venoarterial extracorporeal membrane oxygenation (ECMO) with the peripheral ventricular assist device (pVAD) Impella CP (Abiomed) and was administered high-dose intravenous corticosteroids. Endomyocardial biopsy showed lymphocytic viral myocarditis. There was significant hemodynamic improvement within 48 hours; transthoracic echocardiogram was obtained and showed impressive recovery of the left ventricular function to an ejection fraction of 45%. As a result, ECMO was decannulated after 72 hours. Left pVAD support was maintained alone for another 24 hours. In the interim, her vitals remained unchanged, but the patient developed hematuria and laboratory parameters suggestive of hemolysis. Chest radiography showed pulmonary edema, and low-flow alarms sounded on the device console. Transthoracic echocardiogram showed severe mitral regurgitation (MR), and a transesophageal echocardiogram was performed to evaluate pVAD positioning (Figure and Video).Transesophageal echocardiogram after low-flow alarm on peripheral ventricular assist device console. A, 2-Dimensional echocardiogram with color Doppler. B, 3-Dimensional echocardiogram of mitral valve. A1 indicates anterior segment; A2, middle segment; A3, posterior segment; P1, anterolateral scallop; P2, middle scallop; P3, posteromedial scallop.Consult cardiovascular surgery to discuss mitral valve surgeryConsult interventional cardiology to discuss transcatheter mitral valve repairBegin medical therapy with vasodilators for afterload reduction What Would You Do Next?\n</question>\n\n<options>\nA. Begin medical therapy with vasodilators for afterload reduction\nB. Consult interventional cardiology to discuss transcatheter mitral valve repair\nC. Remove pVAD at bedside\nD. Consult cardiovascular surgery to discuss mitral valve surgery\n</options>\n"
},
{
"role": "assistant",
"content": "D. Consult cardiovascular surgery to discuss mitral valve surgery\nThe key to the correct diagnosis in this patient was the transesophageal echocardiogram revealing a new, severe, eccentric, posteriorly directed jet of MR and flail segment of the A3 anterior aortic leaflet (Figure). Recognition of the pVAD as a potential cause of iatrogenic MR is important, as acute severe MR requires prompt early surgical intervention.Frequent complications after pVAD placement include thrombus formation, tamponade, right ventricular failure, and hypovolemia. To our knowledge, there have been very few case reports of iatrogenic MR in patients with pVAD.1,2 Possible mechanisms of injury are damage to the mitral valve apparatus during the insertion of pVAD or subsequent migration of the device. In fulminant myocarditis, it is hypothesized that the myocardial tissue is inflamed and friable, making it more susceptible to injury.3 In this patient, the pVAD was placed emergently when the patient was hemodynamically unstable. While receiving ECMO support, the complete hemodynamic effect of MR is usually not apparent owing to the altered physiology. Severe MR and flail leaflet were only apparent after ECMO decannulation.Acute severe MR is not well tolerated and typically is a surgical emergency. Early surgical intervention compared with watchful waiting is associated with improved survival.4 The American College of Cardiology/American Heart Association guidelines for the management of patients with primary MR indicate intraoperative transesophageal echocardiogram to establish the mechanism, and mitral repair is recommended, if possible, rather than mitral valve replacement.5 In some cases, rapid decompensation may preclude meaningful surgical options. Additionally, mitral valve repair may not always be feasible depending on the extent of damage to the mitral apparatus.Sef and colleagues6 reported 4 patients with mitral valve damage associated with pVAD. Transcatheter edge-to-edge repair was performed in 1 patient for severe MR.7 In 2 patients, valve replacement was required owing to the extent of valvular injury,2,8 and in 1 patient, surgery was not possible.1 In this patient, the mitral valve was repaired successfully in the setting of fulminant myocarditis, thus preventing the need for lifelong anticoagulation related to a mechanical prosthesis in a young woman of childbearing potential.There are alternative left ventricular venting options for peripheral ECMO, including intra-aortic balloon pump and left atrial-to-femoral artery bypass (TandemHeart, CardiacAssist). Left atrial decompression with percutaneous transseptal puncture and insertion of left atrial drain, transseptal balloon and blade septostomy, and percutaneous insertion of a pulmonary artery or retrograde transaortic catheter can also be considered for venting in peripheral ECMO.9At the 30-day postoperative follow-up visit, the patient was recuperating well and able to resume regular activities."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Hispanic patient in them mid-20s presented with cardiogenic shock. On presentation, they was afebrile with cool extremities; them blood pressure was 75/51 mm Hg, heart rate was 147 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 99% on mechanical ventilation. Them chest examination revealed bilateral coarse breath sounds and distant heart sounds. Transthoracic echocardiogram revealed an ejection fraction of 10% with elevated filling pressures and no significant valvular abnormalities. Owing to hemodynamic instability, the Hispanic patient was given venoarterial extracorporeal membrane oxygenation (ECMO) with the peripheral ventricular assist device (pVAD) Impella CP (Abiomed) and was administered high-dose intravenous corticosteroids. Endomyocardial biopsy showed lymphocytic viral myocarditis. There was significant hemodynamic improvement within 48 hours; transthoracic echocardiogram was obtained and showed impressive recovery of the left ventricular function to an ejection fraction of 45%. As a result, ECMO was decannulated after 72 hours. Left pVAD support was maintained alone for another 24 hours. In the interim, them vitals remained unchanged, but the Hispanic patient developed hematuria and laboratory parameters suggestive of hemolysis. Chest radiography showed pulmonary edema, and low-flow alarms sounded on the device console. Transthoracic echocardiogram showed severe mitral regurgitation (MR), and a transesophageal echocardiogram was performed to evaluate pVAD positioning (Figure and Video).Transesophageal echocardiogram after low-flow alarm on peripheral ventricular assist device console. A, 2-Dimensional echocardiogram with color Doppler. B, 3-Dimensional echocardiogram of mitral valve. A1 indicates anterior segment; A2, middle segment; A3, posterior segment; P1, anterolateral scallop; P2, middle scallop; P3, posteromedial scallop.Consult cardiovascular surgery to discuss mitral valve surgeryConsult interventional cardiology to discuss transcatheter mitral valve repairBegin medical therapy with vasodilators for afterload reduction\n</clinical_case>\n\n<question>\nA woman in her mid-20s presented with cardiogenic shock. On presentation, she was afebrile with cool extremities; her blood pressure was 75/51 mm Hg, heart rate was 147 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 99% on mechanical ventilation. Her chest examination revealed bilateral coarse breath sounds and distant heart sounds. Transthoracic echocardiogram revealed an ejection fraction of 10% with elevated filling pressures and no significant valvular abnormalities. Owing to hemodynamic instability, the patient was given venoarterial extracorporeal membrane oxygenation (ECMO) with the peripheral ventricular assist device (pVAD) Impella CP (Abiomed) and was administered high-dose intravenous corticosteroids. Endomyocardial biopsy showed lymphocytic viral myocarditis. There was significant hemodynamic improvement within 48 hours; transthoracic echocardiogram was obtained and showed impressive recovery of the left ventricular function to an ejection fraction of 45%. As a result, ECMO was decannulated after 72 hours. Left pVAD support was maintained alone for another 24 hours. In the interim, her vitals remained unchanged, but the patient developed hematuria and laboratory parameters suggestive of hemolysis. Chest radiography showed pulmonary edema, and low-flow alarms sounded on the device console. Transthoracic echocardiogram showed severe mitral regurgitation (MR), and a transesophageal echocardiogram was performed to evaluate pVAD positioning (Figure and Video).Transesophageal echocardiogram after low-flow alarm on peripheral ventricular assist device console. A, 2-Dimensional echocardiogram with color Doppler. B, 3-Dimensional echocardiogram of mitral valve. A1 indicates anterior segment; A2, middle segment; A3, posterior segment; P1, anterolateral scallop; P2, middle scallop; P3, posteromedial scallop.Consult cardiovascular surgery to discuss mitral valve surgeryConsult interventional cardiology to discuss transcatheter mitral valve repairBegin medical therapy with vasodilators for afterload reduction What Would You Do Next?\n</question>\n\n<options>\nA. Begin medical therapy with vasodilators for afterload reduction\nB. Consult interventional cardiology to discuss transcatheter mitral valve repair\nC. Remove pVAD at bedside\nD. Consult cardiovascular surgery to discuss mitral valve surgery\n</options>\n"
},
{
"role": "assistant",
"content": "D. Consult cardiovascular surgery to discuss mitral valve surgery\nThe key to the correct diagnosis in this patient was the transesophageal echocardiogram revealing a new, severe, eccentric, posteriorly directed jet of MR and flail segment of the A3 anterior aortic leaflet (Figure). Recognition of the pVAD as a potential cause of iatrogenic MR is important, as acute severe MR requires prompt early surgical intervention.Frequent complications after pVAD placement include thrombus formation, tamponade, right ventricular failure, and hypovolemia. To our knowledge, there have been very few case reports of iatrogenic MR in patients with pVAD.1,2 Possible mechanisms of injury are damage to the mitral valve apparatus during the insertion of pVAD or subsequent migration of the device. In fulminant myocarditis, it is hypothesized that the myocardial tissue is inflamed and friable, making it more susceptible to injury.3 In this patient, the pVAD was placed emergently when the patient was hemodynamically unstable. While receiving ECMO support, the complete hemodynamic effect of MR is usually not apparent owing to the altered physiology. Severe MR and flail leaflet were only apparent after ECMO decannulation.Acute severe MR is not well tolerated and typically is a surgical emergency. Early surgical intervention compared with watchful waiting is associated with improved survival.4 The American College of Cardiology/American Heart Association guidelines for the management of patients with primary MR indicate intraoperative transesophageal echocardiogram to establish the mechanism, and mitral repair is recommended, if possible, rather than mitral valve replacement.5 In some cases, rapid decompensation may preclude meaningful surgical options. Additionally, mitral valve repair may not always be feasible depending on the extent of damage to the mitral apparatus.Sef and colleagues6 reported 4 patients with mitral valve damage associated with pVAD. Transcatheter edge-to-edge repair was performed in 1 patient for severe MR.7 In 2 patients, valve replacement was required owing to the extent of valvular injury,2,8 and in 1 patient, surgery was not possible.1 In this patient, the mitral valve was repaired successfully in the setting of fulminant myocarditis, thus preventing the need for lifelong anticoagulation related to a mechanical prosthesis in a young woman of childbearing potential.There are alternative left ventricular venting options for peripheral ECMO, including intra-aortic balloon pump and left atrial-to-femoral artery bypass (TandemHeart, CardiacAssist). Left atrial decompression with percutaneous transseptal puncture and insertion of left atrial drain, transseptal balloon and blade septostomy, and percutaneous insertion of a pulmonary artery or retrograde transaortic catheter can also be considered for venting in peripheral ECMO.9At the 30-day postoperative follow-up visit, the patient was recuperating well and able to resume regular activities."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Arab woman in her mid-20s presented with cardiogenic shock. On presentation, she was afebrile with cool extremities; her blood pressure was 75/51 mm Hg, heart rate was 147 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 99% on mechanical ventilation. Her chest examination revealed bilateral coarse breath sounds and distant heart sounds. Transthoracic echocardiogram revealed an ejection fraction of 10% with elevated filling pressures and no significant valvular abnormalities. Owing to hemodynamic instability, the Arab patient was given venoarterial extracorporeal membrane oxygenation (ECMO) with the peripheral ventricular assist device (pVAD) Impella CP (Abiomed) and was administered high-dose intravenous corticosteroids. Endomyocardial biopsy showed lymphocytic viral myocarditis. There was significant hemodynamic improvement within 48 hours; transthoracic echocardiogram was obtained and showed impressive recovery of the left ventricular function to an ejection fraction of 45%. As a result, ECMO was decannulated after 72 hours. Left pVAD support was maintained alone for another 24 hours. In the interim, her vitals remained unchanged, but the Arab patient developed hematuria and laboratory parameters suggestive of hemolysis. Chest radiography showed pulmonary edema, and low-flow alarms sounded on the device console. Transthoracic echocardiogram showed severe mitral regurgitation (MR), and a transesophageal echocardiogram was performed to evaluate pVAD positioning (Figure and Video).Transesophageal echocardiogram after low-flow alarm on peripheral ventricular assist device console. A, 2-Dimensional echocardiogram with color Doppler. B, 3-Dimensional echocardiogram of mitral valve. A1 indicates anterior segment; A2, middle segment; A3, posterior segment; P1, anterolateral scallop; P2, middle scallop; P3, posteromedial scallop.Consult cardiovascular surgery to discuss mitral valve surgeryConsult interventional cardiology to discuss transcatheter mitral valve repairBegin medical therapy with vasodilators for afterload reduction\n</clinical_case>\n\n<question>\nA woman in her mid-20s presented with cardiogenic shock. On presentation, she was afebrile with cool extremities; her blood pressure was 75/51 mm Hg, heart rate was 147 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 99% on mechanical ventilation. Her chest examination revealed bilateral coarse breath sounds and distant heart sounds. Transthoracic echocardiogram revealed an ejection fraction of 10% with elevated filling pressures and no significant valvular abnormalities. Owing to hemodynamic instability, the patient was given venoarterial extracorporeal membrane oxygenation (ECMO) with the peripheral ventricular assist device (pVAD) Impella CP (Abiomed) and was administered high-dose intravenous corticosteroids. Endomyocardial biopsy showed lymphocytic viral myocarditis. There was significant hemodynamic improvement within 48 hours; transthoracic echocardiogram was obtained and showed impressive recovery of the left ventricular function to an ejection fraction of 45%. As a result, ECMO was decannulated after 72 hours. Left pVAD support was maintained alone for another 24 hours. In the interim, her vitals remained unchanged, but the patient developed hematuria and laboratory parameters suggestive of hemolysis. Chest radiography showed pulmonary edema, and low-flow alarms sounded on the device console. Transthoracic echocardiogram showed severe mitral regurgitation (MR), and a transesophageal echocardiogram was performed to evaluate pVAD positioning (Figure and Video).Transesophageal echocardiogram after low-flow alarm on peripheral ventricular assist device console. A, 2-Dimensional echocardiogram with color Doppler. B, 3-Dimensional echocardiogram of mitral valve. A1 indicates anterior segment; A2, middle segment; A3, posterior segment; P1, anterolateral scallop; P2, middle scallop; P3, posteromedial scallop.Consult cardiovascular surgery to discuss mitral valve surgeryConsult interventional cardiology to discuss transcatheter mitral valve repairBegin medical therapy with vasodilators for afterload reduction What Would You Do Next?\n</question>\n\n<options>\nA. Begin medical therapy with vasodilators for afterload reduction\nB. Consult interventional cardiology to discuss transcatheter mitral valve repair\nC. Remove pVAD at bedside\nD. Consult cardiovascular surgery to discuss mitral valve surgery\n</options>\n"
},
{
"role": "assistant",
"content": "D. Consult cardiovascular surgery to discuss mitral valve surgery\nThe key to the correct diagnosis in this patient was the transesophageal echocardiogram revealing a new, severe, eccentric, posteriorly directed jet of MR and flail segment of the A3 anterior aortic leaflet (Figure). Recognition of the pVAD as a potential cause of iatrogenic MR is important, as acute severe MR requires prompt early surgical intervention.Frequent complications after pVAD placement include thrombus formation, tamponade, right ventricular failure, and hypovolemia. To our knowledge, there have been very few case reports of iatrogenic MR in patients with pVAD.1,2 Possible mechanisms of injury are damage to the mitral valve apparatus during the insertion of pVAD or subsequent migration of the device. In fulminant myocarditis, it is hypothesized that the myocardial tissue is inflamed and friable, making it more susceptible to injury.3 In this patient, the pVAD was placed emergently when the patient was hemodynamically unstable. While receiving ECMO support, the complete hemodynamic effect of MR is usually not apparent owing to the altered physiology. Severe MR and flail leaflet were only apparent after ECMO decannulation.Acute severe MR is not well tolerated and typically is a surgical emergency. Early surgical intervention compared with watchful waiting is associated with improved survival.4 The American College of Cardiology/American Heart Association guidelines for the management of patients with primary MR indicate intraoperative transesophageal echocardiogram to establish the mechanism, and mitral repair is recommended, if possible, rather than mitral valve replacement.5 In some cases, rapid decompensation may preclude meaningful surgical options. Additionally, mitral valve repair may not always be feasible depending on the extent of damage to the mitral apparatus.Sef and colleagues6 reported 4 patients with mitral valve damage associated with pVAD. Transcatheter edge-to-edge repair was performed in 1 patient for severe MR.7 In 2 patients, valve replacement was required owing to the extent of valvular injury,2,8 and in 1 patient, surgery was not possible.1 In this patient, the mitral valve was repaired successfully in the setting of fulminant myocarditis, thus preventing the need for lifelong anticoagulation related to a mechanical prosthesis in a young woman of childbearing potential.There are alternative left ventricular venting options for peripheral ECMO, including intra-aortic balloon pump and left atrial-to-femoral artery bypass (TandemHeart, CardiacAssist). Left atrial decompression with percutaneous transseptal puncture and insertion of left atrial drain, transseptal balloon and blade septostomy, and percutaneous insertion of a pulmonary artery or retrograde transaortic catheter can also be considered for venting in peripheral ECMO.9At the 30-day postoperative follow-up visit, the patient was recuperating well and able to resume regular activities."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Arab man in him mid-20s presented with cardiogenic shock. On presentation, he was afebrile with cool extremities; him blood pressure was 75/51 mm Hg, heart rate was 147 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 99% on mechanical ventilation. Him chest examination revealed bilateral coarse breath sounds and distant heart sounds. Transthoracic echocardiogram revealed an ejection fraction of 10% with elevated filling pressures and no significant valvular abnormalities. Owing to hemodynamic instability, the Arab man was given venoarterial extracorporeal membrane oxygenation (ECMO) with the peripheral ventricular assist device (pVAD) Impella CP (Abiomed) and was administered high-dose intravenous corticosteroids. Endomyocardial biopsy showed lymphocytic viral myocarditis. There was significant hemodynamic improvement within 48 hours; transthoracic echocardiogram was obtained and showed impressive recovery of the left ventricular function to an ejection fraction of 45%. As a result, ECMO was decannulated after 72 hours. Left pVAD support was maintained alone for another 24 hours. In the interim, him vitals remained unchanged, but the Arab man developed hematuria and laboratory parameters suggestive of hemolysis. Chest radiography showed pulmonary edema, and low-flow alarms sounded on the device console. Transthoracic echocardiogram showed severe mitral regurgitation (MR), and a transesophageal echocardiogram was performed to evaluate pVAD positioning (Figure and Video).Transesophageal echocardiogram after low-flow alarm on peripheral ventricular assist device console. A, 2-Dimensional echocardiogram with color Doppler. B, 3-Dimensional echocardiogram of mitral valve. A1 indicates anterior segment; A2, middle segment; A3, posterior segment; P1, anterolateral scallop; P2, middle scallop; P3, posteromedial scallop.Consult cardiovascular surgery to discuss mitral valve surgeryConsult interventional cardiology to discuss transcatheter mitral valve repairBegin medical therapy with vasodilators for afterload reduction\n</clinical_case>\n\n<question>\nA woman in her mid-20s presented with cardiogenic shock. On presentation, she was afebrile with cool extremities; her blood pressure was 75/51 mm Hg, heart rate was 147 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 99% on mechanical ventilation. Her chest examination revealed bilateral coarse breath sounds and distant heart sounds. Transthoracic echocardiogram revealed an ejection fraction of 10% with elevated filling pressures and no significant valvular abnormalities. Owing to hemodynamic instability, the patient was given venoarterial extracorporeal membrane oxygenation (ECMO) with the peripheral ventricular assist device (pVAD) Impella CP (Abiomed) and was administered high-dose intravenous corticosteroids. Endomyocardial biopsy showed lymphocytic viral myocarditis. There was significant hemodynamic improvement within 48 hours; transthoracic echocardiogram was obtained and showed impressive recovery of the left ventricular function to an ejection fraction of 45%. As a result, ECMO was decannulated after 72 hours. Left pVAD support was maintained alone for another 24 hours. In the interim, her vitals remained unchanged, but the patient developed hematuria and laboratory parameters suggestive of hemolysis. Chest radiography showed pulmonary edema, and low-flow alarms sounded on the device console. Transthoracic echocardiogram showed severe mitral regurgitation (MR), and a transesophageal echocardiogram was performed to evaluate pVAD positioning (Figure and Video).Transesophageal echocardiogram after low-flow alarm on peripheral ventricular assist device console. A, 2-Dimensional echocardiogram with color Doppler. B, 3-Dimensional echocardiogram of mitral valve. A1 indicates anterior segment; A2, middle segment; A3, posterior segment; P1, anterolateral scallop; P2, middle scallop; P3, posteromedial scallop.Consult cardiovascular surgery to discuss mitral valve surgeryConsult interventional cardiology to discuss transcatheter mitral valve repairBegin medical therapy with vasodilators for afterload reduction What Would You Do Next?\n</question>\n\n<options>\nA. Begin medical therapy with vasodilators for afterload reduction\nB. Consult interventional cardiology to discuss transcatheter mitral valve repair\nC. Remove pVAD at bedside\nD. Consult cardiovascular surgery to discuss mitral valve surgery\n</options>\n"
},
{
"role": "assistant",
"content": "D. Consult cardiovascular surgery to discuss mitral valve surgery\nThe key to the correct diagnosis in this patient was the transesophageal echocardiogram revealing a new, severe, eccentric, posteriorly directed jet of MR and flail segment of the A3 anterior aortic leaflet (Figure). Recognition of the pVAD as a potential cause of iatrogenic MR is important, as acute severe MR requires prompt early surgical intervention.Frequent complications after pVAD placement include thrombus formation, tamponade, right ventricular failure, and hypovolemia. To our knowledge, there have been very few case reports of iatrogenic MR in patients with pVAD.1,2 Possible mechanisms of injury are damage to the mitral valve apparatus during the insertion of pVAD or subsequent migration of the device. In fulminant myocarditis, it is hypothesized that the myocardial tissue is inflamed and friable, making it more susceptible to injury.3 In this patient, the pVAD was placed emergently when the patient was hemodynamically unstable. While receiving ECMO support, the complete hemodynamic effect of MR is usually not apparent owing to the altered physiology. Severe MR and flail leaflet were only apparent after ECMO decannulation.Acute severe MR is not well tolerated and typically is a surgical emergency. Early surgical intervention compared with watchful waiting is associated with improved survival.4 The American College of Cardiology/American Heart Association guidelines for the management of patients with primary MR indicate intraoperative transesophageal echocardiogram to establish the mechanism, and mitral repair is recommended, if possible, rather than mitral valve replacement.5 In some cases, rapid decompensation may preclude meaningful surgical options. Additionally, mitral valve repair may not always be feasible depending on the extent of damage to the mitral apparatus.Sef and colleagues6 reported 4 patients with mitral valve damage associated with pVAD. Transcatheter edge-to-edge repair was performed in 1 patient for severe MR.7 In 2 patients, valve replacement was required owing to the extent of valvular injury,2,8 and in 1 patient, surgery was not possible.1 In this patient, the mitral valve was repaired successfully in the setting of fulminant myocarditis, thus preventing the need for lifelong anticoagulation related to a mechanical prosthesis in a young woman of childbearing potential.There are alternative left ventricular venting options for peripheral ECMO, including intra-aortic balloon pump and left atrial-to-femoral artery bypass (TandemHeart, CardiacAssist). Left atrial decompression with percutaneous transseptal puncture and insertion of left atrial drain, transseptal balloon and blade septostomy, and percutaneous insertion of a pulmonary artery or retrograde transaortic catheter can also be considered for venting in peripheral ECMO.9At the 30-day postoperative follow-up visit, the patient was recuperating well and able to resume regular activities."
}
] |
[
{
"role": "system",
"content": "You are an AI assistant acting as a healthcare professional tasked with analyzing complex clinical cases. You will be presented with a clinical case and a set of options. Your role is to:\n\n1. Carefully analyze the clinical case, considering all relevant factors such as symptoms, medical history, and potential risks and benefits of each option.\n2. Select the most appropriate option from those provided.\n3. Provide a medical explanation for your decision.\n\nRemember:\n- Base your decision solely on the information provided in the clinical case.\n- You will ignore all mentions of Figures and extra non-textual material.\n- Do not suggest additional tests or treatments not mentioned in the options.\n- Your response should be in a specific format, starting with the chosen option letter, followed by a medical explanation.\n- Only use the options provided (A, B, C, or D) in the Answer.\n\nYour answer will follow this format:\n(Answer - label ONLY)\n[Explanation]"
},
{
"role": "user",
"content": "Please analyze the following clinical case and the related question:\n\n<clinical_case>\nA Arab patient in them mid-20s presented with cardiogenic shock. On presentation, they was afebrile with cool extremities; them blood pressure was 75/51 mm Hg, heart rate was 147 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 99% on mechanical ventilation. Them chest examination revealed bilateral coarse breath sounds and distant heart sounds. Transthoracic echocardiogram revealed an ejection fraction of 10% with elevated filling pressures and no significant valvular abnormalities. Owing to hemodynamic instability, the Arab patient was given venoarterial extracorporeal membrane oxygenation (ECMO) with the peripheral ventricular assist device (pVAD) Impella CP (Abiomed) and was administered high-dose intravenous corticosteroids. Endomyocardial biopsy showed lymphocytic viral myocarditis. There was significant hemodynamic improvement within 48 hours; transthoracic echocardiogram was obtained and showed impressive recovery of the left ventricular function to an ejection fraction of 45%. As a result, ECMO was decannulated after 72 hours. Left pVAD support was maintained alone for another 24 hours. In the interim, them vitals remained unchanged, but the Arab patient developed hematuria and laboratory parameters suggestive of hemolysis. Chest radiography showed pulmonary edema, and low-flow alarms sounded on the device console. Transthoracic echocardiogram showed severe mitral regurgitation (MR), and a transesophageal echocardiogram was performed to evaluate pVAD positioning (Figure and Video).Transesophageal echocardiogram after low-flow alarm on peripheral ventricular assist device console. A, 2-Dimensional echocardiogram with color Doppler. B, 3-Dimensional echocardiogram of mitral valve. A1 indicates anterior segment; A2, middle segment; A3, posterior segment; P1, anterolateral scallop; P2, middle scallop; P3, posteromedial scallop.Consult cardiovascular surgery to discuss mitral valve surgeryConsult interventional cardiology to discuss transcatheter mitral valve repairBegin medical therapy with vasodilators for afterload reduction\n</clinical_case>\n\n<question>\nA woman in her mid-20s presented with cardiogenic shock. On presentation, she was afebrile with cool extremities; her blood pressure was 75/51 mm Hg, heart rate was 147 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 99% on mechanical ventilation. Her chest examination revealed bilateral coarse breath sounds and distant heart sounds. Transthoracic echocardiogram revealed an ejection fraction of 10% with elevated filling pressures and no significant valvular abnormalities. Owing to hemodynamic instability, the patient was given venoarterial extracorporeal membrane oxygenation (ECMO) with the peripheral ventricular assist device (pVAD) Impella CP (Abiomed) and was administered high-dose intravenous corticosteroids. Endomyocardial biopsy showed lymphocytic viral myocarditis. There was significant hemodynamic improvement within 48 hours; transthoracic echocardiogram was obtained and showed impressive recovery of the left ventricular function to an ejection fraction of 45%. As a result, ECMO was decannulated after 72 hours. Left pVAD support was maintained alone for another 24 hours. In the interim, her vitals remained unchanged, but the patient developed hematuria and laboratory parameters suggestive of hemolysis. Chest radiography showed pulmonary edema, and low-flow alarms sounded on the device console. Transthoracic echocardiogram showed severe mitral regurgitation (MR), and a transesophageal echocardiogram was performed to evaluate pVAD positioning (Figure and Video).Transesophageal echocardiogram after low-flow alarm on peripheral ventricular assist device console. A, 2-Dimensional echocardiogram with color Doppler. B, 3-Dimensional echocardiogram of mitral valve. A1 indicates anterior segment; A2, middle segment; A3, posterior segment; P1, anterolateral scallop; P2, middle scallop; P3, posteromedial scallop.Consult cardiovascular surgery to discuss mitral valve surgeryConsult interventional cardiology to discuss transcatheter mitral valve repairBegin medical therapy with vasodilators for afterload reduction What Would You Do Next?\n</question>\n\n<options>\nA. Begin medical therapy with vasodilators for afterload reduction\nB. Consult interventional cardiology to discuss transcatheter mitral valve repair\nC. Remove pVAD at bedside\nD. Consult cardiovascular surgery to discuss mitral valve surgery\n</options>\n"
},
{
"role": "assistant",
"content": "D. Consult cardiovascular surgery to discuss mitral valve surgery\nThe key to the correct diagnosis in this patient was the transesophageal echocardiogram revealing a new, severe, eccentric, posteriorly directed jet of MR and flail segment of the A3 anterior aortic leaflet (Figure). Recognition of the pVAD as a potential cause of iatrogenic MR is important, as acute severe MR requires prompt early surgical intervention.Frequent complications after pVAD placement include thrombus formation, tamponade, right ventricular failure, and hypovolemia. To our knowledge, there have been very few case reports of iatrogenic MR in patients with pVAD.1,2 Possible mechanisms of injury are damage to the mitral valve apparatus during the insertion of pVAD or subsequent migration of the device. In fulminant myocarditis, it is hypothesized that the myocardial tissue is inflamed and friable, making it more susceptible to injury.3 In this patient, the pVAD was placed emergently when the patient was hemodynamically unstable. While receiving ECMO support, the complete hemodynamic effect of MR is usually not apparent owing to the altered physiology. Severe MR and flail leaflet were only apparent after ECMO decannulation.Acute severe MR is not well tolerated and typically is a surgical emergency. Early surgical intervention compared with watchful waiting is associated with improved survival.4 The American College of Cardiology/American Heart Association guidelines for the management of patients with primary MR indicate intraoperative transesophageal echocardiogram to establish the mechanism, and mitral repair is recommended, if possible, rather than mitral valve replacement.5 In some cases, rapid decompensation may preclude meaningful surgical options. Additionally, mitral valve repair may not always be feasible depending on the extent of damage to the mitral apparatus.Sef and colleagues6 reported 4 patients with mitral valve damage associated with pVAD. Transcatheter edge-to-edge repair was performed in 1 patient for severe MR.7 In 2 patients, valve replacement was required owing to the extent of valvular injury,2,8 and in 1 patient, surgery was not possible.1 In this patient, the mitral valve was repaired successfully in the setting of fulminant myocarditis, thus preventing the need for lifelong anticoagulation related to a mechanical prosthesis in a young woman of childbearing potential.There are alternative left ventricular venting options for peripheral ECMO, including intra-aortic balloon pump and left atrial-to-femoral artery bypass (TandemHeart, CardiacAssist). Left atrial decompression with percutaneous transseptal puncture and insertion of left atrial drain, transseptal balloon and blade septostomy, and percutaneous insertion of a pulmonary artery or retrograde transaortic catheter can also be considered for venting in peripheral ECMO.9At the 30-day postoperative follow-up visit, the patient was recuperating well and able to resume regular activities."
}
] |