AN ART OF PORT WINE BIRTHMARK - A CASE REPORT ON TYPE II STURGE WEBER SYNDROME

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.

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Publication Details

Journal
Chulalongkorn Medical Journal
Published
2025-11-26
DOI
https://doi.org/10.56808/2673-060x.5599
Primary Topic
Vascular Malformations and Hemangiomas
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article
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article

AN ART OF PORT WINE BIRTHMARK - A CASE REPORT ON TYPE II STURGE WEBER SYNDROME

Dr DHIVYA BHARATHI, Dr.Gayathri P S, Dr ARAVIND WARRIER
Chulalongkorn Medical Journal
Vascular Malformations and Hemangiomas
article

AN ART OF PORT WINE BIRTHMARK - A CASE REPORT ON TYPE II STURGE WEBER SYNDROME

Dr DHIVYA BHARATHI, Dr.Gayathri P S, Dr ARAVIND WARRIER
article en

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.

Chulalongkorn Medical JournalVol. 69(6)
Openalex Percentile: Top 44%
Vascular Malformations and Hemangiomas
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