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Vascular malformations of the central nervous system

Robert J Singer, MD
Christopher S Ogilvy, MD
Guy Rordorf, MD
Section Editor
Jose Biller, MD, FACP, FAAN, FAHA
Deputy Editor
Janet L Wilterdink, MD


Cerebral vascular malformations occur in 0.1 to 4.0 percent of the general population [1,2]. Four general subtypes of congenital malformations have been described:

  • Developmental venous anomalies
  • Capillary telangiectasias
  • Cavernous malformations
  • Arteriovenous malformations

Developmental venous anomalies are most common in autopsy series, with an incidence of 2 percent [3,4]. This is followed by arteriovenous malformations (1 percent), capillary malformations (telangiectasias, 0.7 percent), and cavernous malformations (0.4 percent). Developmental venous anomalies and capillary telangiectasia are usually benign, while cavernous malformations and arteriovenous malformations have a greater tendency toward neurologic sequelae.

This topic will review our understanding of the natural history and treatment of these three lesions, which continues to evolve with our burgeoning imaging capabilities and clinical experience.

Cerebral and spinal cord arteriovenous malformations are discussed separately. Carotid cavernous fistulas are also discussed separately. (See "Brain arteriovenous malformations" and "Disorders affecting the spinal cord", section on 'Vascular malformations' and "Carotid-cavernous fistulas".)

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Literature review current through: Nov 2017. | This topic last updated: May 03, 2012.
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