Clinical Course and Angioarchitecture of Cerebrofacial Arteriovenous Metameric Syndromes

Abstract

Bonnet-Dechaume-Blanc or Wyburn-Mason disease has been considered a rare condition and mysterious disease for the last century. It was recently introduced as a spectrum of disease expression of cerebrofacial vascular structures related by the same neural crest origin, named “Cerebrofacial Arteriovenous Malformations Syndrome” (CAMS) by JJ Bhattacharya. We illustrate the special observations of reported cases in the literature and our three demonstrative cases of CAMS in terms of natural history including progressive appearance of the AVMs, clinical presentation, angioarchitecture, clinical risk of retinal AVMs, AVMs along the optic pathways, intracranial AVMs and proper investigation in the suspicious case.