[Sturge-Weber syndrome: glaucoma with elevated episcleral venous pressure]

The present report describes clinical findings in 13 patients with Sturge-Weber syndrome, 11 of whom had glaucoma (22-52 mm Hg). The episcleral venous pressure (EVP) was high in all glaucomatous eyes (14-23 mm Hg). Tonography showed a marked decrease in outflow facility. The observations suggest that glaucoma in Sturge-Weber syndrome is caused by a) elevated episcleral venous pressure and b) secondary damage of the trabecular meshwork with reduction of outflow facility. The cause and pathogenesis of these lesions are discussed. In nine patients a diffuse angiomatosis involving most of the choroid (1.5-4.0 mm thick), as well as the episcleral perilimbal plexus, was demonstrated by ultrasonography.