Bilateral Ocular and Facial with Unilateral Intracranial Involvement In Sturge Weber Syndrome: A Case Report and Review of Literature

Abstract

The Sturge-Weber syndrome (SWS) is a rare congenital disorder involving the eye in the form of glaucoma and choroidal hemangioma, facial skin in the form of port wine stain, usually in the distribution of ophthalmic division of the trigeminal nerve, and the brain in the form of leptomeningeal angiomas. We report a rare case of 6 year-old girl with Sturge-Weber syndrome with bilateral port wine stain involving the ophthalmic and maxillary division of trigeminal nerve, unilateral intracranial involvement, bilateral glaucoma and abnormal retinal vasculature, without the presence of choroidal hemangioma.