Cerebrospinal fluid venous fistula causing spontaneous intracranial hypotension resulting in venous sinus thrombosis and coma

Abstract

Cerebrospinal fluid (CSF) fistulas have recently been recognized as a cause of spontaneous intracranial hypotension (SIH), predominantly presenting with headaches, especially positional headaches. Atypical presentations like tinnitus and cranial nerve symptoms have also been reported. SIH has been linked to venous sinus thrombosis; however, to our knowledge, no prior cases describe a CSF venous fistula causing SIH that leads to cerebral venous thrombosis and coma. We report a patient who developed progressive venous sinus thrombosis, leading to coma, and was found to have low intracranial pressure indicative of SIH. Invasive monitoring and imaging confirmed the low intracranial pressure, prompting a dynamic myelogram that revealed a T2/3 CSF venous fistula. The patient underwent transvenous embolization of the fistula, which resulted in the resolution of symptoms and almost immediate improvement in both venous thrombosis and intracranial hypotension. CSF venous fistulae as a cause of SIH is a recently recognized entity, with ongoing research into its treatment through transvenous embolization. Most documented cases focus on patients with headaches. This case highlights a novel presentation, emphasizing the importance of thorough diagnostic workup in patients with cerebral venous thrombosis. Early detection and treatment of this condition can lead to significant clinical improvement, including the resolution of coma.