Multifocal Skull Base Defects With Associated Spontaneous Cerebrospinal Fluid Leaks

OBJECTIVE: Spontaneous cerebrospinal fluid (CSF) leaks are thought to arise due to elevated intracranial pressure which is distributed across the skull base, potentially predisposing the development of multifocal CSF leaks. The aims of this study are to evaluate the characteristics of this population at presentation and surgical outcomes. STUDY DESIGN: Retrospective case series. SETTING: Tertiary referral center. PATIENTS: Patients 18 years or older with spontaneous CSF leak diagnosis were eligible for study inclusion. Multifocal spontaneous CSF leak was defined as leaks originating from more than one skull base defect at anatomically distinct subsites. INTERVENTION: None. MAIN OUTCOME MEASURE: Clinical presentation and surgical outcome. RESULTS: Two hundred ninety-three patients with diagnosis of spontaneous CSF leak were identified. Of these, 11 (3.8%) were characterized as having multifocal spontaneous CSF leaks. Mean body mass index was significantly higher in multifocal CSF leak patients (31.5 ± 8.5 vs 46.2 ± 9.9, p < 0.0001). There was also higher prevalence of women (100% vs 63.8%, p = 0.012) and African-Americans (63.6% vs 23.4%, p = 0.003). The overall success rate of CSF leak repair was 95%. Fifty percent of patients developed headaches or blurry vision after surgical repair, and two patients required ventriculoperitoneal shunting. CONCLUSIONS: Multifocal spontaneous CSF leaks are rare and occurred in 3.8% of patients with spontaneous CSF. These patients were morbidly obese and more likely to be female and African American. Surgical repair conferred excellent outcomes. However, these patients have high risk of developing symptoms suggestive of elevated intracranial pressure postoperatively and should be counseled accordingly.