Vascular Malformations of the Central Nervous System

Abstract

This article reviews vascular malformations of the central nervous system. These may be arteriovenous, venous, capillary, or cavernous. Arteriovenous malformations form in utero or shortly thereafter and carry an annual risk of hemorrhage of 2% to 4%. They are associated with Wyburn-Mason and Rendu-Osler-Weber syndromes, but the vast majority are sporadic. Treatment may be surgical, endovascular, and/or radiosurgical. Cavernous malformations may be sporadic or familial and associated with abnormalities of chromosome 7q, 7p, or 3q. They may be associated with mutations in the gene for KRIT1. The natural history is believed to be hemorrhage at a rate of 1% to 4% per year. Treatment, if indicated, is surgical excision. Venous malformations are congenital variations in venous anatomy that generally do not hemorrhage and do not require treatment. Capillary telangiectasias are for the most part incidental autopsy findings. Spinal vascular malformations also are reviewed.