Sturge Weber syndrome: a case report

Abstract

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.

Sí­ndrome de Sturge-Weber: A importância do cuidado integral na assistência ao adolescente portador

“…As complicações oculares da síndrome se manifestam, como glaucoma e malformações vasculares da conjuntiva, episclera, coróide e retina (14) . O acompanhamento anual para o glaucoma, por meio de fundoscopia e tonometria, é recomendado para todos os pacientes com Síndrome de Sturge-Weber (10) .…”

Extraoral and oral signs in two patients with Sturge-Weber syndrome - Case Report and Review of the Literature

“…The most apparent extraoral 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 (1, 5,9).…”

“…Port-wine stains can be treated by dermabrasion, tattooing, flash lamp pulsed tunable dye laser therapy, which can result in partial or complete clearing of the port-wine stains. A high dose of hydrocortisone given orally in infancy results in regression of well-localised small lesions (5).…”

“…It consists of congenital hamartomatous malformations that may affect the eye, skin and central nervous system at different times (11). The syndrome is presented as angioma of the leptomeninges extending to the cerebral cortex with ipsilateral angiomatous lesions, unilateral facial nevus affecting a division of the trigeminal nerve, hemiparesis, intracranial calcification, mental retardation, glaucoma and refractory epilepsy (4,5).…”

Periodontal Management of Sturge-Weber Syndrome

“…Inspite of being rare, it is the most frequent disease among the neurocutaneous syndromes specifically with vascular predominance [2]. It is sporadic neurocutaneous disease characterized by focal port-wine stain, ocular abnormalities (glaucoma), and choroidal hemangioma and leptomeningeal angioma most often involving occipital and parietal lobes [3].…”