title: Primary spontaneous cerebrospinal fluid leaks and idiopathic intracranial hypertension.
abstract: BACKGROUND
  Idiopathic intracranial hypertension (IIH) is increasingly recognized as a cause of spontaneous cerebrospinal fluid (CSF) leak in the otolarnygological and neurosurgical literature. The diagnosis of IIH in patients with spontaneous CSF leaks typically is made a few weeks after surgical repair of the leak when symptoms and signs of elevated intracranial pressure (ICP) appear.
  
  
  METHODS
  Case reports and literature review. Two young obese women developed spontaneous CSF rhinorrhea related to an empty sella in one and a cribriform plate encephalocele in the other. Both patients underwent surgical repair of the CSF leak. A few weeks later, they developed chronic headaches and bilateral papilledema. Lumbar punctures showed elevated CSF opening pressures with normal CSF contents, with temporary improvement of headaches. A man with a 3-year history of untreated IIH developed spontaneous CSF rhinorrhea. He experienced improvement of his headaches and papilledema after a CSF shunting procedure, and the rhinorrhea resolved after endoscopic repair of the leak.
  
  
  RESULTS
  These cases and the literature review confirm a definite association between IIH and spontaneous CSF leak based on: 1) similar demographics; 2) increased ICP in some patients with spontaneous CSF leak after leak repair; 3) higher rate of leak recurrence in patients with raised ICP; 4) patients with intracranial hypertension secondary to tumors may develop CSF leak, confirming that raised ICP from other causes than IIH can cause CSF leak.
  
  
  CONCLUSIONS
  CSF leak occasionally may keep IIH patients symptom-free; however, classic symptoms and signs of intracranial hypertension may develop after a CSF leak is repaired, exposing these patients to a high risk of recurrence of the leak unless an ICP-lowering intervention is performed.
authors.affiliation: Departments of Ophthalmology (MAP, OYB, BBB, NJN, VB), Neurology (BBB, NJN, VB), and Neurological Surgery (NJN), Emory University, Atlanta, Georgia.
authors.forename: Mario A
authors.initials: MA
authors.lastname: Pérez
authors.forename: Omer Y
authors.initials: OY
authors.lastname: Bialer
authors.forename: Beau B
authors.initials: BB
authors.lastname: Bruce
authors.forename: Nancy J
authors.initials: NJ
authors.lastname: Newman
authors.forename: Valérie
authors.initials: V
authors.lastname: Biousse
mesh_terms: D000328:Adult; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology; D065634:Cerebrospinal Fluid Leak; D002559:Cerebrospinal Fluid Rhinorrhea / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D005260:Female; D006801:Humans; D019586:Intracranial Hypertension / Q000150:complications* / Q000175:diagnosis; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009765:Obesity; D011859:Radiography; D015898:Tomography Scanners, X-Ray Computed
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