Arteriovenous Malformation associated with Wyburn Mason Syndrome

Wyburn Mason syndrome (aka Bonnet Dechaume Blanc syndrome) is an extremely rare congenital non hereditary disorder, associated with the development of multiple ipsilateral arteriovenous malformation (AVM) in the retinal and intracranial regions. Some of its clinical manifestations include seizure, visual impairment, hemiparesis, headaches, cranial neuropathies and hydrocephalus and the severity depends on the location, size and configuration of affected portion of central nervous system (CNS). We report a 19year old male presented with an episode of Generalized Tonic Clonic Seizure type and intermittent left sided headaches along with dilated tortuous vessels of the left eye over the optic disc. Computerized tomography (CT) brain revealed hematoma in the left ganglio-capsular bleed with extension into ipsilateral lateral ventricle and thus was conservatively managed.