Spontaneous Intracranial Hypotension and CSF-Venous Fistulae: from Diagnostic Imaging to Interventional Management

Abstract

Over the past decade, major advances have transformed the understanding and management of cerebrospinal fluid (CSF) leaks leading to spontaneous intracranial hypotension (SIH). Among the most impactful developments has been the identification and characterization of CSF—venous fistulae (CVF), a distinct and still underrecognized cause of SIH. This discovery has prompted a paradigm shift in both diagnostic and therapeutic strategies. Novel imaging techniques—including lateral decubitus CT myelography and digital subtraction myelography—have markedly improved detection rates, while targeted interventions such as transvenous embolization have emerged as safe and effective treatment options. In this review, we first outline the general pathophysiology and imaging principles related to SIH and CSF leaks, followed by a focused and comprehensive analysis of the current evidence managing CVFs. We discuss their diagnostic challenges, therapeutic approaches, and evolving clinical implications, aiming to provide a thorough update for diagnostic and interventional neuroradiologists involved in the care of patients with SIH.