Bonnet-dechaume-blanc syndrome Q85.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Neuro-Retino Angiomatosis Syndrome; Wyburn-Mason Syndrome

History
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Bonnet, Dechaume and Blanc, 1937; Wyburn-Mason, 1943

Definition
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Very rare congenital angiodysplasia syndrome: Combination of a naevus flammeus lateralis in the facial region (inconstant) with malformations of the retinal vessels (aneurysms, tendral angiomas) and the midbrain and brainstem vessels (neurological symptoms).

Therapy
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Treatment of the skin symptoms, if necessary with laser therapy. Cooperation with neurologists, if necessary ophthalmologists.

Literature
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  1. Bonnet P, Dechaume J, Blanc E (1937) L'anévrysme cirsoïde de la rétine (anévrysme recémeux): ses relations avec l'anéurysme cirsoïde de la face et avec l'anévrysme cirsoïde du cerveau. J Med Lyon 18: 165-178
  2. Wyburn-Mason R (1943) Arteriovenous malformation of the mid-brain and retina, facial naevi, and mental changes. Brain 66: 163-203

Outgoing links (2)

Asymmetrical nevus flammeus; Laser;

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020