Chapter 2 - The natural history of cerebral arteriovenous malformations

Abstract

Cerebral arteriovenous malformations (AVMs) are composed of a complex tangle of abnormal arteries and veins and are a significant source of cerebral hemorrhage and consequent morbidity and mortality in young adults, representing a diagnostic and therapeutic challenge. Current natural-history studies of cerebral AVMs report overall annual rates of 1% and 3% for the risk of epilepsy and hemorrhage, respectively. Unruptured AVMs have an annual hemorrhage rate of 2.2% while ruptured lesions have an annual hemorrhage rate of 4.5%. These hemorrhage rates are can change over time, particularly for hemorrhagic lesions, with the rebleed rate ranging from 6% to 15.8% in the first year after rupture across several studies. Besides hemorrhage, other significant risk factors for AVM hemorrhage include deep location, deep venous drainage, associated aneurysms, and pregnancy. Other factors include patient age, sex, and small AVM size, which are not currently considered significant risk factors for AVM hemorrhage. In addition to hemorrhage risk and seizure risk, the natural history of an AVM also encompasses the daily psychologic burden that a patient must endure knowing that he or she possesses an untreated AVM. This chapter reviews the epidemiology, clinical features, and natural history of cerebral AVMs.