Abstract
The syndromes of multiple retinal, orbital, facial and/or cerebral vascular malformations was first described as Bonnet-Dechaume-Blanc Syndrome and shortly thereafter as Wyburn-Mason Syndrome. Better understanding of these diseases has led to the classification of Cerebrofacial Arteriovenous Metameric Syndromes (CAMS), defined by developmental neurovascular anatomy. Though rare, this spectrum of diseases has the potential to provide insight into the pathophysiology and development of sporadic vascular malformations. Modern evaluation of CAMS patients includes complete angiography of the head and neck, as identification of associated lesions is critical. Treatment may include endovascular intervention and/or surgery, but is not curative. It is instead focused on controlling symptomatic lesions and preserving function.
Original language | English |
---|---|
Title of host publication | Pediatric Vascular Neurosurgery |
Subtitle of host publication | Technical Nuances in Contemporary Pediatric Neurosurgery (Part 2) |
Publisher | Springer International Publishing |
Pages | 125-131 |
Number of pages | 7 |
ISBN (Electronic) | 9783030747497 |
ISBN (Print) | 9783030747480 |
DOIs | |
State | Published - 1 Jan 2021 |
Keywords
- Arteriovenous malformation
- Bonnet-dechaume-blanc syndrome
- Cerebrofacial arteriovenous metameric syndrome
- Wyburn-mason syndrome