Sturge Weber syndrome: A case report

Pournima Godge, Shubhra Sharma, Monica Yadav, Pallavi Patil, Sandeep Kulkarni


Purpose: The Sturge-Weber syndrome (SWS) is a rare congenital disorder that occurs sporadically and features lesions of both skin and nervous system. We report a case of 7 year-old girl with Sturge-Weber syndrome and discuss its clinicopathological features, differential diagnosis and also emphasize the importance of its diagnosis in the clinical oral practice.
Case Description: The classic pathognomonic manifestations include angioma of the leptomeninges extending to cerebral cortex with ipsilateral angiomatous lesions, unilateral facial nevus affecting a division of trigeminal nerve, hemiparesis, intracranial calcification, mental retardation and refractory epilepsy. The most apparent indication of SWS is a facial birthmark or “Port Wine Stain” present at birth and typically involving at least one upper eyelid and the forehead. Management of a patient with Sturge-Weber Syndrome may be challenging due to risk of hemorrhage.
Conclusion: The dentists should focus on comprehensive therapy, starting with behavior management and stress on preventive measures.


Sturge-Weber Syndrome; Portwine stain; encephalotrigeminal syndrome; oral mucosa

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e-ISSN: 1980-6523

ISSN-L: 0102-9460


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