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
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Roger Wyburn-Mason (1911-1983), British neurologist at the National Hospital of Neurology and Neurosurgery (Queen Square), London
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Paul Bonnet (1894-1959), French ophthalmologist
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Jean Dechaume (1896-1968), French neurosurgeon
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Eugene Blanc (1888-1961), French neurosurgeon
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- 1. Wyburn-Mason, R Arterioevenous aneurysm of the mid-brain and retina facial naevi and mental changes, Brain 1943, 66, pp12-15
- 2. Patel U, Gupta SC. Wyburn-Mason syndrome. A case report and review of the literature. Neuroradiology. 1990;31 (6): 544-6. - Pubmed citation
- 3. Lee AW, Chen CS, Gailloud P et-al. Wyburn-Mason syndrome associated with thyroid arteriovenous malformation: a first case report. AJNR Am J Neuroradiol. 28 (6): 1153-4. doi:10.3174/ajnr.A0512 - Pubmed citation
- 4. Geibprasert S, Pongpech S, Jiarakongmun P et-al. Radiologic assessment of brain arteriovenous malformations: what clinicians need to know. Radiographics. 2010;30 (2): 483-501. doi:10.1148/rg.302095728 - Pubmed citation
- 5. Bonnet P, Dechaume J, Blanc E. L'aneurysme cirsoide de la retine (aneurysme vasemeaux). J Med Lyon 1937;18:165-178
- 6. So J, So HR, So. Wyburn-Mason Syndrome. (2020) . doi: - Pubmed
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