Cerebral venous drainage via the ophthalmic veins in the Sturge-Weber syndrome

Abstract

We describe a 23-year-old girl with an extremely uncommon form of cerebral venous drainage and cerebellar leptomeningeal angiomatosis as a possible variant of the Sturge-Weber syndrome. Extensive congenital port-wine stains all over the body, hypoplastic left renal and subclavian and iliac veins, cardiomegaly and ptosis and hypoplasia of the left kidney had been recognised in early childhood. She rapidly developed signs of intracranial hypertension. CT and MRI showed a right medial temporal lesion. Angiography revealed cerebellar pial angiomatosis with enlarged medullary veins and no functioning sigmoid sinuses or jugular veins. Cerebral venous drainage was via enlarged ophthalmic veins. Although the intracranial venous abnormalities were characteristic of the Sturge-Weber syndrome anomalies beyond the encephalofacial territory suggested a more complex developmental abnormality.