Abstract

A 37-year-old Caucasian woman presented with a blind left eye and granulomatous panuveitis in the right eye. There was a subretinal neovascular membrane with haemorrhage beneath the macula and widespread subretinal lesions that were interpreted as choroidal granulomas in the right eye. A chest x-ray that showed diffuse pulmonary fibrosis without hilar lymphadenopathy was the only contributory clinical finding. Systemic and subconjunctival steroids and photocoagulation to the right macula brought about temporary remission. The painful left eye was enucleated. Histopathological examination revealed diffuse, noncaseating granulomas in the iris and ciliary body, retina, choroid, optic nerve, sclera and scleral emissaria, and inferior oblique muscle. A preretinal membrane was studied by electron microscopy. It was composed of a collagenous matrix containing fibroblasts and fibrous astrocytes. Some vessels, surrounded by a multilaminar basement membrane, revealed many features of normal retinal vasculature. Others were lined by markedly attenuated endothelial cells with occasional "open' junctions and fenestrations.