Bilateral Wyburn-Mason syndrome presenting as acute subarachnoid haemorrhage - a very rare congenital neurocutaneous [corrected] disorder

A 30 year old young male was admitted to our department after experiencing clinical symptoms of a subarachnoid haemorrhage. Imaging studies revealed large cerebral AVMs. Fundus examination of the left eye demonstrated a retinal racemose AVM almost completely covering the posterior pole of the eye. W...

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Bibliographic Details
Published in:Acta Neurochirurgica
Main Authors: Cortnum, S O S, Sørensen, P, Andresen, J
Format: Article in Journal/Newspaper
Language:English
Published: 2008
Subjects:
Adult
Arteriovenous Malformations/complications
Cerebral Angiography
Humans
Intracranial Arteriovenous Malformations/complications
Inuits
Male
Neurocutaneous Syndromes/complications
Ophthalmic Artery
Rare Diseases/complications
Retinal Vessels
Syndrome
Tomography
X-Ray Computed
Vision Disorders/etiology
inuits
Online Access:https://pure.au.dk/portal/da/publications/bilateral-wyburnmason-syndrome-presenting-as-acute-subarachnoid-haemorrhage--a-very-rare-congenital-neurocutaneous-corrected-disorder(a16bfae3-1e30-45eb-8105-91ea2e30be37).html
https://doi.org/10.1007/s00701-008-1604-z
Description
Summary:A 30 year old young male was admitted to our department after experiencing clinical symptoms of a subarachnoid haemorrhage. Imaging studies revealed large cerebral AVMs. Fundus examination of the left eye demonstrated a retinal racemose AVM almost completely covering the posterior pole of the eye. Wyburn-Mason syndrome is a very rare congenital neurocutaneous disorder comprising of vascular malformations of the retina, ipsilateral cerebral AVMs and occasionally lesions in the oronasopharyngeal area. Subarachnoid haemorrhage associated with Wyburn-Mason syndrome has been described in only 5 patients in the literature since 1973. The finding of retinal AVMs should warrant cerebral imaging studies including CT- or MR-angiography.

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