ArticleVascular Malformations of the Central Nervous SystemMacdonald, R. Loch; Stoodley, Marcus; Weir, BryceAuthor Information Section of Neurosurgery, Department of Surgery, Pritzker School of Medicine and the University of Chicago Medical Center, Chicago, Illinois Supported by grants from the National Institutes of Health to Dr. Macdonald (K08 NS01831) and Dr. Weir (NS25946) and grants from the Brain Research Institute. Dr. Macdonald is supported by an American College of Surgeons Faculty Fellowship and a Young Clinician Investigator Award from the American Association of Neurological Surgeons. Address correspondence and reprint requests to: R. Loch Macdonald, MD, Section of Neurosurgery, MC3026, University of Chicago Medical Center, 5841 S. Maryland Ave., Chicago, IL 60637. E-mail: [email protected] Neurosurgery Quarterly: December 2001 - Volume 11 - Issue 4 - p 231-247 Buy 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. © 2001 Lippincott Williams & Wilkins, Inc.