Pathogenesis and natural history of brain arteriovenous malformation and dural arteriovenous fistula

Abstract

Neither the pathogenesis nor the natural history of brain arteriovenous malformations (BAVMs) and dural arteriovenous fistulas (DAVFs) has as yet been fully elucidated. BAVMs are generally considered to be congenital vascular disorders, while some factors after birth may still influence their genesis or growth. Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular dysplasia characterized by dilated vessels and AVMs, and is also known as a genetic disease associated with BAVMs. Three genes, endoglin, ALK-1 and SMAD-4 are associated with HHT. The natural history of BAVMs is influenced by angioarchitecture and prior hemorrhage. DAVFs are considered as an acquired lesions；however, their pathogenesis is not clear. The natural history of DAVFs is influenced by not only by the lesion’s angioarchitecture such as its cortical venous drainage, but also by the patient’s symptomatology. Furthermore DAVF can alter its angioarchitecture with proliferative changes in the arterial component and occlusive changes in venous drainage.