Spinal Dorsal Intradural Arteriovenous Fistulae: Natural History, Imaging, and Management

In this review, we describe the pathophysiology, diagnosis, and treatment of spinal dorsal intradural arteriovenous fistulae (DI-AVFs), focusing on novel research areas. DI-AVFs compose the most common subgroup of spinal arteriovenous lesions and most commonly involve the thoracic spine, followed by lumbar and sacral segments. The pathogenesis underlying DI-AVFs is an area of emerging understanding, thought to be attributable to venous congestion and hypertension that precipitate ascending myelopathy. Patients with DI-AVFs typically present with motor, sensory, or urinary dysfunction, although a wide swath of other less common symptoms has been reported. DI-AVFs can be subdivided by spinal region, which in turn is associated with 4 distinct clinical phenotypes: craniocervical junction (CCJ), subaxial cervical, thoracic, and lumbosacral. Patients with CCJ and lumbosacral DI-AVFs have particularly interesting presentations and treatment considerations. High-value diagnostic findings on magnetic resonance imaging include flow voids, missing-piece sign, and T2-weighted intramedullary hyperintensity. However, digital subtraction angiography is the gold standard for diagnosis and localization of DI-AVFs, as well as for definitive treatment planning. Surgical disconnection of DI-AVFs is almost universally curative and frontline treatment especially for CCJ and lumbosacral DI-AVFs. Endovascular techniques evolve in promising ways, such as improved visualization, distal access, and liquid embolic techniques. The pathophysiology of DI-AVFs is better understood using newly identified radiological diagnostic markers. Despite new techniques and devices introduced in the endovascular field, surgery remains the gold-standard treatment for DI-AVFs.