Cerebrospinal Fluid-Venous Fistulas: Pathophysiology, Diagnosis, and Management

The purpose of this review is to provide a practical framework for understanding, diagnosing, and managing cerebrospinal fluid-venous fistulas (CVFs), a major cause of spontaneous intracranial hypotension (SIH). First, we summarize current concepts of CSF physiology, including glymphatic pathways, and discuss leading hypotheses for why CVFs occur, including potential roles of pressure gradients and underlying intracranial hypertension in select patients. We then outline a stepwise diagnostic approach and detail contemporary myelographic techniques used to localize CVFs: lateral decubitus digital subtraction and CT myelography, with emphasis on acquisition strategies, interpretive 'pearls and pitfalls', common mimics, reasons for false-negative studies, and escalation pathways when suspicion remains high. Finally, we review and discuss contemporary literature regarding management options across the spectrum of disease severity including conservative measures, epidural blood patching and targeted percutaneous therapies, surgical disconnection, and endovascular treatment. Regarding the latter, we discuss anatomical considerations, access and targeting strategies, expected outcomes, complications, and radioprotection considerations. We then summarize post-treatment issues such as rebound intracranial hypertension and conclude with open questions and future directions to guide research and standardization in this rapidly evolving field.