Introduction

Sturge–Weber syndrome (SWS), is a sporadic, congenital, frequently progressive, neurological disorder classically characterized by the association of a congenital facial capillary angioma with leptomeningeal angiomatosis. Epilepsy, mental retardation, and focal neurological deficits are the major neurologic abnormalities (Alexander & Norman, 1960; Alexander, 1972; Gomez & Bebin, 1987). The motor attacks include, in addition to epileptic seizures, transient hemiparesis or hemiplegia, often leaving a definite neurological deficit. Other features of the syndrome include hemiatrophy, homonymous hemianopia, glaucoma, dental abnormalities, and skeletal lesions (Roach & Bodensteiner, 1999).

Vascular abnormalities

Although the cutaneous capillary angioma is the hallmark of the SWS, most children born with facial port-wine stains do not have the syndrome. The SWS facial angioma is classically found on the forehead and upper eyelid. For a patient with any facial port-wine stain, the risk of having the SWS is only about 8%. It increases to 25% when half of the face, including the ophthalmic division of the trigeminal nerve, is involved (Morelli, 1999). Intracranial leptomeningeal angiomatosis is almost always ipsilateral to the cutaneous nevus. Exceptions to the rule are not infrequent and the correlation between the extent and location of the naevus and that of the pial angioma is poor (Alexander, 1972; Gomez & Bebin, 1987; Pascual-Castroviejo et al., 1993). Some children have bilateral nevus with unilateral pial involvement or vice versa. Angiomas may extend beyond the face and involve the neck, trunk or limbs on one or both sides (Uram & Zubillaga, 1982). In a recent series of 20 surgically treated SWS patients, six presented with extensive cutaneous angiomatosis, which was bilateral in three (Arzimanoglou et al., 2000).