English

Abstract

INTRODUCTION: Sturge-Weber syndrome (SWS) (MIM ID 185300) (ORPHA 3205) (ICD 10 code Q85.8) or encephalotrigeminalangiomatosis belongs to group of disorders collectively called asphakomatoses (“mother-spot” disease). This rare congenital neurocutaneous syndrome is characterized by unilateral facial cutaneous vascular malformations affecting the eye and skin in association with ipsilateralleptomeningealangiomatosis.[1,2] In 1860, Schirmer first identified this syndrome, and Sturge in 1879 described it in detail; later Frederick Parkes Weber in 1992 demonstrated intracranial calcification.[1,2] The prevalence of the diseases 1 : 50,000 live birth sand equally affects males and females with no racial predilection.[2]