Sturge -Weber Syndrome - Three Classic variants

Abstract

Sturge-Weber syndrome (SWS), also known as encephalotrigeminal angiomatosis, a sporadic, non-familial, congenital disorder consists of congenital hamartomatous malformations that may affect the eye, skin and central nervous system at different times. Sturge-Weber syndrome is classified as 1) Complete trisymptomatic: - when all three organ systems i.e. eye, skin and CNS are involved 2) Incomplete bisymptomatic:- when the involvement is either oculocutaneous or neurocutaneous, and 3) Incomplete monosymptomatic: when there is only neural or cutaneous involvement. Failure of proper vascular development is believed to be the most likely cause of this condition. The malformed blood vessels or hemangiomas may lead to port-wine stain, epilepsy and glaucoma depending on its location. Three classic variants with typical findings are discussed.

Keywords

• Sturge -Weber syndrome
• phakomatoses
• port-wine stain
• glaucoma
• intracranial calcification