Wyburn-Mason syndrome

Dr Henry Knipe and Dr 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 naevus
  • visual pathway and orbital arteriovenous malformations (AVMs)
  • intracranial arteriovenous malformations (AVMs)

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

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
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Article information

rID: 2296
Section: Syndromes
Tag: cases
Synonyms or Alternate Spellings:
  • Bonnet-Dechaume-Blanc syndrome
  • Wyburn Mason syndrome

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