Progression of gyrate atrophy of the choroid and retina

Abstract

Fundus fluorescein angiograms of 21 gyrate atrophy patients were evaluated. In 16 of 21 patients between 2 to 13 angiograms were taken during the follow-up which extended from 1.6 to 13.4 years. In 5 of 21 patients the fluorescein angiography was done once. Optic disc drusen occurred in 3 patients. Optic disc atrophy was not observed. Some attenuation occurred in the retinal arteries in the far-advanced stages. Up to the midretinal venous phase 5 of 13 patients had a markedly increased visibility of the larger choroidal vessels in areas of ophthalmoscopically intact retina. Pigment epithelial window defects presented in 17 of 21 patients. In 4, they were seen to enlarge into patches of chorioretinal atrophy during this long-term follow-up. Contrary to the general notion, the macula was affected frequently, i.e. in 9 of 21 patients; In 6 the pigment epithelial window defects presented in the macula, one had bilateral cystoid macular oedema, and in 2 patients the patches of chorioretinal atrophy were central.