Wyburn-Mason syndrome

Dr Henry Knipe ◉ ◈ and Associate Professor Donna D'Souza ◉ et al.

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 craniofacial 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)

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