Sturge Weber syndrome: a case report / Síndrome de Sturge Weber: um relato de caso

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.

OBJETIVO: A síndrome de Sturge-Weber (SWS) é uma rara doença congênita que ocorre esporadicamente e apresenta lesões de pele e do sistema nervoso. Este artigo é um relato de caso de uma menina de 7 anos com SWS para discutir suas características clinicopatológicas, diagnóstico diferencial e enfatizar a importância do seu diagnóstico na prática clínica odontológica. DESCRIÇÃO DO CASO: As manifestações clássicas patognomônicas incluem angioma das leptomeninges que se estendem para o córtex cerebral com lesões angiomatosas ipsilaterais, nevo facial unilateral, que afetam uma divisão do nervo trigêmeo, hemiparesia, calcificação intracraniana, retardo mental e epilepsia refratária. A indicação mais evidente de SWS é uma marca de nascença facial ou "mancha vinho do porto" presente no nascimento e tipicamente envolvendo pelo menos uma pálpebra superior e a testa. O controle clínico de um paciente com SWS pode ser desafiador devido ao risco de hemorragia. CONCLUSÃO: Os dentistas devem adotar uma abordagem terapêutica abrangente, começando com o controle de comportamento e de estresse com medidas preventivas.

Treatment of the Portwine Stain with Dermabrasion and Dermal Overgraft: A Clinical Experience

Portwine stains(PWSs) are congenital vascular malformations, commonly found in the face, caused by progressively ectactic postcapillary venules of the superficial vascular plexus. They are reported to occur in 0.3% of newborn, and depending on the location and size, they may lead to psychosocial complications. Recently, with the advent of the lasers, ruby, dye, argon and CO2 lasers have been used for the treatment of PWSs. However, the results are variable and unpredictable. We present a case of congenital hemifacial PWS treating with dermabrasion and overgraft with very thin split-thickness skin rather than laser therapy. We achieved good result by using this method.