CEREBRAL VENOUS DRAINAGE VIA THE OPHTHALMIC VEINS IN THE STURGE-WEBERSYNDROME

We describe a 23-year-old girl with an extremely uncommon form of cere bral venous drainage and cerebellar leptomeningeal angiomatosis as a p ossible variant of the Sturge-Weber syndrome. Extensive congenital por t-wine stains all over the body, hypoplastic left renal and subclavian and iliac veins, cardiomegaly and ptosis and hypoplasia of the left k idney had been recognised in early childhood. She rapidly developed si gns of intracranial hypertension. CT and MRI showed a right medial tem poral lesion. Angiography revealed cerebellar pial angiomatosis with e nlarged medullary veins and no functioning sigmoid sinuses or jugular veins. Cerebral venous drainage was via enlarged ophthalmic veins. Alt hough the intracranial venous abnormalities were characteristic of the Sturge-Weber syndrome anomalies beyond the encephalofacial territory suggested a more complex developmental abnormality.