Abstract

RODOFILE, Clarisa; GREES, Susana A; VALLE, Lidia E  and  MARTINO, Gabriel. Sturge-Weber syndrome: Report of a case with poor dermatological manifestations. Arch. argent. pediatr. [online]. 2011, vol.109, n.2, pp.42-45. ISSN 0325-0075.

Sturge-Weber syndrome (SSW) is a congenital neurocutaneous disorder, which presents a port wine vascular malformation that covers the territory of the trigeminal nerve, neurological manifestations (ipsilateral leptomeningeal involvement, seizures and mental retardation) and ophthalmic signs (choroidal vascular malformation, glaucoma). There is no evidence to indicate that this is an inherited disease. Our patient had a small vascular malformation in the frontal and right upper eyelid, significant neurological involvement, and no ocular involvement. In SSW, not always the magnitude of the skin lesion is directly related to the commitment of the central nervous system, which can cause serious consequences on child's health and quality of life, as noted in our case. We emphasize the importance of being awere of this neurocutaneous syndrome given the importance of early screening, additional studies, interconsultations and the necessary interdisciplinary approach from the time of diagnosis.

Keywords : Vascular malformation; Seizures.

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