Sturge Weber syndrome: A case report

• Autor(a) principal: Godge, Pournima
• Data de Publicação: 2011
• Outros Autores: Sharma, Shubhra, Yadav, Monica, Patil, Pallavi, Kulkarni, Sandeep
• Tipo de documento: Artigo
• Idioma: eng
• Título da fonte: Revista odonto ciência (Online)
• Texto Completo: https://revistaseletronicas.pucrs.br/ojs/index.php/fo/article/view/9580
• topic: Sturge-Weber Syndrome; Portwine stain; encephalotrigeminal syndrome; oral mucosa; Síndrome de Sturge-Weber; angiomatose encefalotrigeminal; mucosa bucal

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.