Intracranial hypotension syndrome (IHS) due to cerebrospinal fluid (CSF) leaks, causing orthostatic headache and other neurological disorders, has been more often described in adult females with Marfan syndrome (MS), or Ehlers-Danlos syndrome 1-6. The prevalence of IHS is 1:5,000 to 1:10,000 population, with a non-consensual female predominance, at mean age of 42 years 2,4-6, and presenting connective tissue disease 1-6. Worthy of note, dural ectasias commonly found in MS patients, may favor the CSF leaks, and images of neuroaxis by computed tomography or magnetic resonance imaging (MRI) of brain and spine (from cervical to lumbosacral levels) can confirm this diagnosis 1-6. The higher risk of SIH development in Ehlers-Danlos or MS may be explained by an enhanced compliance of the spinal canal, besides the more fragile structure of the dura 2 . The main types of CSF leaks are: 1) ventral – by dural tear due to calcified disc herniation or an osteophyte; 2) – by leaking meningeal diverticula; and 3) by CSF-venous fistula 2 . The management of these leaks is more often conservative and by non-targeted epidural blood patches; and the targeted treatment includes patching, embolization, or surgery 1-6.
