Association of Benign Intracranial Hypertension and Spontaneous Encephalocele With Cerebrospinal Fluid Leak

Objective: To determine the incidence of intracranial hypertension in patients with spontaneous encephalocele with cerebrospinal fluid (CSF) leak. Study Design: Retrospective case review. Setting: Tertiary care neurotology practice. Patients: Patients presenting between 2008 and 2011 with spontaneous encephalocele and CSF leak in the temporal bone. Intervention(s): Lumbar puncture with opening pressure measurement after encephalocele repair. Main Outcome Measures: Patient age, sex, postoperative course, body mass index, and postoperative intracranial pressure. Results: Of the 26 patients identified with spontaneous encephalocele with CSF leak, 9 patients had postoperative lumbar puncture data. Of those 9, 89% were female subjects, and 11% were male, with a mean age of 57 and a mean BMI of 41 kg/m(2) (morbidly obese). The mean opening pressure was 24.5 cm H2O. Approximately 33% had normal intracranial pressure mean, 15 cm H2O; range, 10-17 cm H2O); 67% had elevated intracranial pressure (mean, 29 cm H2O; range, 23.5Y40 cm H2O). The incidence of BIH in the general population is 0.001%. Of the 6 with intracranial hypertension, 3 (50%) were placed on acetazolamide for fundoscopic findings, postoperative headache, and/or visual changes. Mean time to LP after repair of encephalocele was 13 months (range, 4 days to 75 months). Conclusion: This study shows that benign intracranial hypertension is prevalent in a significant number of patients presenting with spontaneous encephalocele with CSF otorrhea at a rate much higher than is found in the general population. This finding has direct clinical implications and suggests that all patients with spontaneous encephalocele/CSF leak warrant evaluation for benign intracranial hypertension.