Epidemiology, clinical presentation, diagnostic evaluation, and prognosis of spinal arteriovenous malformations.

Spinal arteriovenous malformations (sAVM) are rare vascular pathologies whose natural history remains incompletely understood. Advances in diagnostic imaging, coupled with the evolution of endovascular and microsurgical techniques have led to the description of a number of classification schemes for these lesions. An updated method has changed AVM classification from five categories of lesion based on source and location of feeder vessels to three categories based on pathophysiology. These categories include extradural arteriovenous fistulae (AVFs), intradural AVFs, extradural-intradural AVFs, intramedullary AVMs, and conus medullaris AVM each with individual subclassifications. Treatment outcomes have been shown to differ based on classification criteria. The increased use of advanced imaging prior to surgical intervention has facilitated the treatment of AVFs. Definitive diagnosis and characterization have traditionally required digital subtraction angiography, which is now being supplemented with other forms of noninvasive imaging such as computed tomography angiography (CTA) and magnetic resonance angiography (MRA). Epidemiologically, intradural dorsal AVFs account for 80% of all sAVMs, and are characterized by low-pressure shunts located in the sleeve of the dorsal nerve root. Microsurgical treatment has been shown to be highly effective in cases of intradural dorsal AVFs, although many cases are also amenable to durable occlusion using liquid embolics. Conus medullaris AVMs, which has only been recently characterized as a separate category of sAVM, is best treated using a combination of embolization and microsurgery. Successful treatment of sAVM mandates a thorough understanding of the anatomy and classification of these lesions. The purpose of this chapter is to review and summarize the classification, natural history, and prognosis of sAVMs.