• Waleed Brinjikji, Patrick Nicholson, Christopher A Hilditch, Timo Krings, Vitor Pereira, and Ronit Agid.
• Division of Neuroradiology, Joint Department of Medical Imaging, Toronto Western Hospital, UHN, University of Toronto, Toronto, Ontario, Canada. brinjikji.waleed@mayo.edu.
• Neuroradiology. 2020 Apr 1; 62 (4): 417-425.

AbstractCerebrofacial venous metameric syndrome (CVMS) is a complex craniofacial vascular malformation disorder in which patients have a constellation of venous vascular malformations affecting soft tissues, bone, dura, and neural structures including the eye and brain. It is hypothesized that a somatic mutation responsible for the venous abnormalities occurred prior to migration of the neural crest cells, and because of this, facial, osseous, and cerebral involvement typically follows a segmental or "metameric" distribution. The most commonly recognized form of CVMS is Sturge-Weber syndrome. However, a wide spectrum of CVMS phenotypical presentations exist with various metameric distributions of slow-flow vascular lesions including facial venous vascular malformations, developmental venous anomalies, venous angiomas, cavernous malformations (cavernomas), dural sinus malformations, and maybe even vascular tumors such as cavernous hemangiomas. Awareness of the various manifestations as described herewith is important for treatment and screening purposes.

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