Intracranial hypotension in a boy with Marfan syndrome: a case report

M. Direk
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Abstract

Intracranial hypotension is a rare condition in children and adolescents and the management is not standardized. We report here a child with intracranial hypotension due to Marfan syndrome. A 12-year-old boy presented persistent bilateral frontal headache over two weeks. The patient had no headache when he was in a supine position and it was more severe when he was in an upright position. His physical examination revealed a characteristic marfanoid habitus with tall stature, pectus carinatum, lens dislocation, and joint hyperextensibility. Lumbar puncture was performed and cerebrospinal fluid (CSF) opening pressure was measured below 60 mmH2O. The imaging of the patient showed bilateral subdural hemorrhage, cerebellar tonsillar herniation into the foramen magnum, dural venous distention, pachymeningeal enhancement, dural ectasia, and thoracic CSF leak on the left side. According to these findings, Marfan syndrome and intracranial hypotension syndrome were diagnosed.
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马凡氏综合征男童颅内低血压1例
颅内低血压在儿童和青少年中是一种罕见的疾病,治疗不规范。我们在此报告一个因马凡氏综合征而出现颅内低血压的儿童。一个12岁的男孩表现出持续的双侧额部头痛超过两周。患者仰卧位时头痛无症状,直立位时头痛加重。体格检查显示他有典型的马氏体质,身材高大,胸突,晶状体脱位,关节过伸。行腰椎穿刺,测量脑脊液(CSF)开口压力低于60 mmH2O。患者影像学表现为双侧硬脑膜下出血,小脑扁桃体突出至枕骨大孔,硬脑膜静脉扩张,脑膜厚增强,硬脑膜扩张,左侧胸椎脑脊液漏。据此诊断为马凡氏综合征和颅内低血压综合征。
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