Intracranial hypotension in a girl with Marfan syndrome: case report and review of the literature


A 14-year-old girl, followed in our department for Marfan syndrome, presented with postural headache for a month. Neurological examination was normal. The diagnosis of intracranial hypotension syndrome was suspected. Bilateral subdural hematomas were found on brain magnetic resonance imaging (MRI), and spinal MRI showed large lumbosacral arachnoid diverticula; no cerebrospinal fluid leaks could be found. Despite bed rest and hydration for 2 weeks, postural headache remained. Epidural blood patching was also performed. Subsequently, the patient became asymptomatic and could stand up after 1 day. Brain MRI did not find recurrent subdural hematoma after 1 month. Dural ectasia is one of the major criteria of Marfan syndrome, and it is often poorly symptomatic. Intracranial hypotension is a rare complication especially in children, and management is not standardized. In this case report, blood patching was sufficient. Further research into the diagnosis and management of spontaneous intracranial hypotension is required.

DOI: 10.1007/s00381-007-0506-3

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@article{Cheuret2007IntracranialHI, title={Intracranial hypotension in a girl with Marfan syndrome: case report and review of the literature}, author={E. Cheuret and T. Edouard and M. Mejdoubi and P. Acar and C. Pienkowski and C. Cances and S. Lebon and M. Tauber and Y. Chaix}, journal={Child's Nervous System}, year={2007}, volume={24}, pages={509-513} }