Gd-DOTA-enhanced MR imaging in two cases of Sturge-Weber syndrome.

Sturge-Weber syndrome is characterized by the association of a trigeminal port-wine facial angioma with a pial venular angioma, usually ipsilateral to the facial nevus. However, a bilateral pial angioma may coexist with a unilateral facial nevus (1 ), and pial angioma may be present without any facial nevus (2] . The pial angioma is responsible for partial seizures, hemiplegia, and mental retardation. In typical cases, CT shows cortical calcifications and localized or diffuse hemiatrophy. Such findings were found, respectively , in 86% and 71% of our 14 cases in a companion study (3]. Direct demonstration of the pial angioma is difficult or impossible either by CT or angiography. Cortical enhancement after contrast injection is seen in the brain cortex and is thought to be due to blood-brain barrier breakdown resulting from prolonged episodes of seizures (3 , 4) rather than to the angioma itself. Moreover, CT can be normal, especially in infants. Direct visualization of the pial angioma is of paramount importance in order to (1) ascertain the diagnosis when CT is normal or when the cutaneous angioma is lacking, (2) look for a bilateral pial angioma, and (3) identify precisely the extension of the angioma when surgical resection is contemplated . We report two cases of proved Sturge-Weber syndrome in which the pial angioma was visualized by Gd-DOTA (Dotarem, Guerbet Laboratories, France).