• Written By: Michael Vaphiades, DO
    Neuro-Ophthalmology/Orbit

    The authors describe spontaneous cerebrospinal fluid (CSF) leakage as a result of chronic increased intracranial pressure (ICP) in four patients with idiopathic intracranial hypertension (IIH). Three of them had rhinorrhea, and one had otorrhea. They conclude that CSF leak is a rare complication in IIH, rhinorrhea can be a presenting sign, and both rhinorrhea and otorrhea can be a late sign of the disease.

    Although rhinorrhea previously has been described in IIH patients, the authors believe this to be the first report of otorrhea in these patients.

    Three of the patients had been diagnosed with IIH several years earlier and had been noncompliant with their medical treatment. In one patient, CSF rhinorrhea was the presenting symptom of IIH.

    The authors say that because most patients do not realize the relationship between a clear fluid discharge from the nose or ear and their IIH, they usually do not provide this information. They say that initial patient evaluation when serous discharge from the ear or nose is considered to be CSF includes analysis of the fluid discharge for the presence of beta-2 transferrin. This is a carbohydrate-free isoform of transferrin produced by cerebral neuro-transaminase from beta-1 transferrin by desialization, and is present only in CSF, perilymph and vitreous of the eye.

    They note that maxillofacial and brain neuroimaging with CT or MRI is essential, with careful searching for a defect of the skull base. Optimal therapy includes lowering of ICP, if possible before repair of the site of CSF leakage, and surgical repair of the bony defect.