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

S O S Cortnum, P Sørensen, J Andresen

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Abstract

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.

Original languageEnglish
JournalActa Neurochirurgica
Volume150
Issue7
Pages (from-to)725-7
Number of pages3
ISSN0001-6268
DOIs
Publication statusPublished - Jul 2008

Keywords

  • 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

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