ABSTRACT

Arteriovenous malformation (AVM) is a congenital vascular malformation in which an anatomic defect results in shunting of arterial blood to the venous system to varying degrees. The incidence and prevalence of cerebrospinal AVMs were reported in a systematic review. The vast majority of AVMs alone as independent lesions, but infrequently occur with a venous malformation and/or lymphatic malformations, making its diagnosis and management more difficult. There has been significant progress to explain the growth tendency of extratruncular AVM lesions based on gene mutations occurring in tissues. The majority of AVM lesions develop progressively over time, particularly in the brain, lungs, and liver, and are eventually diagnosed in patients when they are adults. The endothelial cell turnover rate has been found to be significantly greater in AVMs than in normal blood vessels. The “sporadic” type of AVMs without documented genetic mutations may be explained by underlying genetic abnormalities in the future.