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Spinal vascular malformations include a heterogeneous group of pathological/anatomical entities with congenital and acquired etiologies that induce spinal cord dysfunction, primarily through intradural extramedullary drainage and enlargement of the coronal venous plexus, resulting in venous hypertension and/or a direct mass effect. These lesions are rare, comprising approximately 5% of neurovascular disorders. Clinical manifestations are pain or venous congestive myelopathy, usually in the thoracolumbar spine. This may progress to hemorrhage from vascular thrombosis and necrotizing myelopathy (Foix–Alajouanine syndrome).1 Due to the differing hemodynamics, pathophysiology, and treatment considerations, a thorough knowledge of the various types of spinal dural arteriovenous fistulas (dAVFs) and arteriovenous malformations (AVMs) is essential.
The first descriptions of the single coiled dural vessel form of spinal dAVF with intradural extramedullary venous drainage were published by Kendall and Logue in 1977.2 Type I spinal dAVFs (also known as angioma racemosum venosum) are the most common and comprise 56% of all spinal lesions in the spinal vascular malformation database at the University of Toronto.3 They occur nearly 3–4 times as frequently in men. These generally low flow lesions may be further divided into type I-A (single feeder dAVFs) and I-B (≥2 arterial feeders).4 Although they are typically supplied via radicular arteries, anterior spinal artery feeders have also been reported.5 Type II spinal AVMs (also known as angioma racemosum arteriovenosum) are characterized by a compact, glomus-type, totally intramedullary nidus. They have no gender predominance but are symptomatic in younger patients. Type III (juvenile or metameric) spinal AVMs are highly complex intramedullary lesions that frequently extend into the extramedullary, epidural, or even extraspinal compartments. Vascular …
Contributors Each author made a substantial contribution to the concept and content of this standards document.
Competing interests None.
Provenance and peer review Not commissioned; not externally peer reviewed.