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Category: Volume 08 Issue 06 June 2020
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Title: Sturge-Weber Syndrome a Rare Neurocutaneous Syndrome Presenting As Open Angle Glaucoma: A Care Report

Authors: Nishant Acharya, Priyanka Sharma

 DOI: https://dx.doi.org/10.18535/jmscr/v8i6.86

Abstract

Sturge-Weber syndrome (SWS) is a rare congenital neurocutaneous syndrome is characterized by unilateral facial cutaneous vascular malformations affecting the eye and skin in association with ipsilateral leptomeningeal angiomatosis. The incidence of SWS is ~ 1:50,000 infants, with no predilection for the sex. Glaucoma in SWS patients shows a bimodal peak of age development: an early-onset (congenital) form affecting ~ 60% of patients and a late onset form during childhood and adolescence (40% of cases). Association of glaucoma with SWS is unilateral, most frequently as open-angle glaucoma leading to progressive visual field loss. Our case illustrated the need of regular follow up and early intervention in the patients with cutaneous port wine stain (PWS) in the distribution of trigeminal nerve to prevent loss of vision due to glaucoma.

Keywords: Struge weber syndrome, Port wine strain, Glaucoma, Leptomeningeal angiomatosis

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Corresponding Author

Priyanka Sharma

Department of OBG, Bhandari Hospital Hamirpur (H.P), India