Investigators at the Department of Neurosurgery, Cedars-Sinai Medical Center, Los Angeles, CA, evaluated 24 children (18 girls, 6 boys) with spontaneous intracranial hypotension, seen 2001-2012. Onset of symptoms was at mean age of 14.3 years (range, 2-19 years). The majority (23 patients) presented with orthostatic headaches, mainly occipital, and one with a non-positional headache. Additional symptoms included nausea, neck pain, dizziness, and hearing abnormalities. Precipitating headache factors included lifting, dance class, handstands, and football. Spinal MRI demonstrated a CSF leak in 12 (50%) patients, usually thoracic in location, and spinal meningeal diverticula without a leak in 10 (42%). Underlying connective tissue disorders in 13 patients (54%) included Marfan syndrome in 3, Marfan-like syndrome in 4, Ehlers-Danlos syndrome in 2, and hypomelanosis of Ito in 1. Dural ectasia with multiple meningeal diverticula were found in all 3 patients with Marfan syndrome.

Treatment consisted of bed rest and hydration, epidural blood patches in 23, with permanent resolution in 9 patients (39%). Injections of fibrin glue directed at the CSF leak were successful in 2 patients (25%). Surgical treatment had a good result in 10 patients (91%), with permanent resolution of symptoms. Acetazolamide for rebound high intracranial pressure headache was required in 5 patients. Overall, outcome was good in 22 patients (92%) and poor in 2 (8%). [1]

COMMENT. Intracranial hypotension and Marfan syndrome. A PubMed search found several single case reports of spontaneous intracranial hypotension in children, two with Marfan syndrome. A 14-year-old girl followed for Marfan syndrome presented with a postural headache for a month, and MRI showed bilateral subdural hematomas. Large lumbosacral arachnoid diverticula were shown on spinal MRI. Headaches resolved after epidural blood patching. Dural ectasia is a common finding with Marfan syndrome. [2]

A 12-year-old girl with Marfan syndrome, sacral dural ectasia, and tonsillar herniation, presented with headache, unresponsive to surgical decompression of the foramen magnum. Further MR imaging demonstrated CSF leak at the level of the dural ectasia, and the headache resolved after blood patch of the leak. [3]

Skull thickening, paranasal sinus expansion, and sella turcica shrinkage are reported in a 29-year-old man with a history of VP shunt placement following traumatic brain injury at 9 years of age. MRI showed signs of chronic intracranial hypotension, and LP opening pressure was not recordable. Secondary installation of a valve to restore normal ICP is recommended. [4]