Sturge Weber syndrome with absent facial nevus and fronal leptomeningeal angioma :A rare dilemma

Abstract

Sturge Weber syndrome (SWS) is a rare , sporadic , neurocutaneous disorder characterized by cutaneous facial angioma ,leptomeningeal angiomas and glaucoma. The incidence of SWS without facial nevus is not known, however it is rare with only few patients reported in the literature. The occipital lobe is characteristically involved in SWS .Other lobes are rarely involved especially the frontal lobe .The association between SWS without facial nevus and frontal lobe angiomas is extremely rare . We report here two cases of SWS with absent facial nevus and frontal lobe involvement .The first case has a unique location of the leptomeningeal angioma involving solely the frontal lobe, and the second with a late onset appearance of the leptomeningeal angioma without calcification . Our cases highlight the diagnostic and therapeutic challenges that pediatricians might face in patients with absent facial nevus with unusual angioma location .