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Chulalongkorn Medical Journal

Abstract

Sturge Weber Syndrome is a Phakomatoses group of neurocutaneous disorders. They manifest in the dermal, neural, ocular, and oral regions due to a mutation in the GNAQ gene. These lesions often are unilateral following the course of the trigeminal nerve.Here we present a case of 54-year-old woman with a history of glaucoma with a unilateral reddish-purple discoloration of the palate and a port-wine stain limited to the left side of her face. The clinical course of Sturge-Weber syndrome is quite complex and necessitates a multidisciplinary approach. In type 2 cases of Sturge-Weber syndrome, patients present with facial angiomas and glaucoma, but without any neurological involvement. There is an increased risk of hemorrhage associated with Sturge-Weber syndrome making it essential to carefully plan dental procedures to minimize any potential pre- and post-operative bleeding risks. This case report aimed to highlight the importance of recognizing similar patterns to enable prompt diagnosis.

DOI

10.56808/2673-060X.5599

First Page

1

Last Page

6

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