A case of sturge weber syndrome
Abstract
Sturge weber syndrome is a unusual phakomatosis, multisystem disorder involving Eye, Central nervous system, Skin. Incidence is 1-9 per 100,000. Primary defect is an insult to procencephalic and mesencephalic neural crest which give rise to vascular malformation in eye, skin and meninges. This is a case presentation of a 16 year old boy with right divergent squint, episcleral hemangioma and pupil showing relative afferent pupillary defect in the right eye. Right eye intraocular pressure was 38mmHg at the first visit. Gonioscopy showed open angles. Fundus of right eye showed pale disc with dilated vessels. Left eye was normal. Imaging showed calcification in occipital lobe. Patient was treated with antiglaucoma medications. Features were suggestive of Sturge weber syndrome
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