Refractory Cerebrospinal Fluid Rhinorrhea Secondary to Occult Superior Vena Cava Syndrome and Benign Intracranial Hypertension: Diagnosis and Management

Abstract

Objective: This study is designed to describe the association between benign intracranial hypertension (BIH) and spontaneous cerebrospinal fluid (CSF) rhinorrhea and address the effect of extracranial venous flow dynamics on intracranial pressure (ICP). Methods: We present a 58-year-old woman with refractory spontaneous CSF rhinorrhea who was later found to have superior vena cava syndrome. The patient had undergone two prior transnasal endoscopic repair attempts. In retrospect, a preoperative magnetic resonance venogram (MRV) suggested very prolonged cerebral transit time, despite otherwise normal intracranial venous anatomy. Results: The CSF leak was repaired through a bifrontal craniotomy. The intraoperative and postoperative course was complicated due to the patient's significant comorbidities. She ultimately made a good recovery and has not had any further CSF rhinorrhea in more than 2 years of follow-up. Conclusions: Refractory, spontaneous CSF leak must prompt aggressive investigation for multiple causes of elevated ICP. A cerebral transit time can be obtained from scout imaging when a magnetic resonance angiogram or MRV is performed, and this may disclose elevated ICP if it is prolonged. If endoscopic transnasal repair fails, craniotomy and direct suture repair and autologous tissue reinforcement of the skull base may prove successful and durable, even if BIH persists.