PURPOSE OF REVIEW: To provide an update on recent developments in the understanding, diagnosis, and treatment of CSF-venous fistula (CVF). RECENT FINDING: CVF is a recently recognized cause of spontaneous intracranial hypotension (SIH), an important secondary headache, in which an aberrant connection is formed between the spinal subarachnoid space and an adjacent spinal epidural vein permitting unregulated loss of CSF into the circulatory system. CVFs often occur without a concurrent epidural fluid collection; therefore, CVF should be considered as a potential etiology for patients with SIH symptomatology but without an identifiable CSF leak. Imaging plays a critical role in the detection and localization of CVFs, with a number of imaging techniques and provocative maneuvers described in the literature to facilitate their localization for targeted and definitive treatment. Increasing awareness and improving the localization of CVFs can allow for improved outcomes in the SIH patient population. Future prospective studies are needed to determine the diagnostic performance of currently available imaging techniques as well as their ability to inform workup and guide treatment decisions.
