Wyburn-Mason syndrome

Last revised by Rohit Sharma on 15 Nov 2023

Wyburn-Mason syndrome (also known as Bonnet-Dechaume-Blanc syndrome) is a rare, nonhereditary neurocutaneous disorder that typically presents with unilateral vascular malformations that primarily involve the brain, orbits and facial structures. It is currently classified as a cerebrofacial arteriovenous metameric syndrome (CAMS) 3,4

Pathology

Features include:

  • facial vascular nevus

  • visual pathway and orbital arteriovenous malformations (AVMs), e.g. racemose angioma

  • intracranial arteriovenous malformations (AVMs) commonly in mid-brain 6

Cases of bilateral malformations have been described and there is an association with subarachnoid hemorrhage.

History and etymology

  • Roger Wyburn-Mason (1911-1983), British neurologist at the National Hospital of Neurology and Neurosurgery (Queen Square), London

  • Paul Bonnet (1894-1959), French ophthalmologist

  • Jean Dechaume (1896-1968), French neurosurgeon

  • Eugene Blanc (1888-1961), French neurosurgeon

ADVERTISEMENT: Supporters see fewer/no ads