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

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.