Arteriovenous malformations of the brain

Prad Selvan MD (Dr. Selvan of the SUNY Downstate Medical Center has no relevant financial relationships to disclose.)
Steven R Levine MD, editor. (Dr. Levine of the SUNY Health Science Center at Brooklyn has received honorariums from Genentech for service on a scientific advisory committee and a research grant from Genentech as a principal investigator.)
Originally released June 9, 2014; last updated August 11, 2015; expires August 11, 2018

This article includes discussion of arteriovenous malformations of the brain, brain arteriovenous malformations, cerebral arteriovenous malformations, cAVMs, and CNS arteriovenous malformations. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Cerebral arteriovenous malformations are the second most common cause of nontraumatic subarachnoid hemorrhage. These lesions can present with a variety of neurologic complaints, including headaches, seizures, and progressive neurologic deficits. Short-term morbidity and mortality associated with arteriovenous malformations are low, but patients do poorly in the long term. Morphologic and clinical characteristics of arteriovenous malformations can help clinicians estimate the risk of hemorrhage, which can assist in the decision to intervene. Recently, the first large-scale randomized clinical trial comparing medical and interventional management of unruptured brain arteriovenous malformations, the AURBA trial, was published. In this article, the author discusses the clinical manifestations, pathophysiology, diagnosis, prognosis, and management of cerebral arteriovenous malformations.

Historical note and terminology

Vascular malformations of the central nervous system (CNS) have been described in the literature dating back to the 19th century. With advances in angiography, imaging modalities, and microsurgical techniques, the understanding of and treatment options for these lesions have since grown. Modern categorization and nomenclature of CNS vascular malformations was established by McCormick in 1966. This system used histopathologic features to classify each lesion as a venous angioma, cavernous malformation, capillary telangiectasia, or arteriovenous malformation. The Spetzler-Martin grading system for brain arteriovenous malformations used major factors influencing the risk of surgical resection and hemorrhage to score the arteriovenous malformations (Spetzler and Martin 1986). It is the most commonly used grading system that helps clinicians make treatment decisions and offers a standardized way of classifying arteriovenous malformations for literature and research.

Table 1. Spetzler-Martin Grading Scale for Arteriovenous Malformations

Characteristic

Number of points assigned

Size of arteriovenous malformation
Small (<3 cm)
Medium (3 to 6 cm)
Large (>6 cm)

1 point
2 points
3 points

Location
Non-eloquent site
Eloquent site

0 points
1 point

Pattern of venous drainage
Superficial only
Deep only

0 points
1 point

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