Sturge Weber syndrome: A case report
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...
| Autores: | , , , , |
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| Formato: | artículo |
| Estado: | Versión publicada |
| Fecha de publicación: | 2011 |
| País: | Brasil |
| Recursos: | Pontifícia Universidade Católica do Rio Grande do Sul (PUCRS) |
| Repositorio: | Revista odonto ciência (Online) |
| Idioma: | inglés |
| OAI Identifier: | oai:ojs.revistaseletronicas.pucrs.br:article/9580 |
| Acesso em linha: | https://revistaseletronicas.pucrs.br/fo/article/view/9580 |
| Access Level: | acceso abierto |
| Palavra-chave: | Sturge-Weber Syndrome Portwine stain encephalotrigeminal syndrome oral mucosa Síndrome de Sturge-Weber angiomatose encefalotrigeminal mucosa bucal |
| Resumo: | 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. |
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