Blood-retinal barrier function in patients with cone or cone-rod dystrophy.

We assessed blood-retinal barrier function by vitreous fluorophotometry in 24 patients with either cone or cone-rod dystrophy who were segregated into three subgroups. Compared with a normal population, the patients demonstrated increased vitreous fluorescence (breakdown of the blood-retinal barrier) that positively correlated with peripheral pigmentary changes and an appreciable reduction in electroretinographic scotopic b-wave amplitude. A unique subgroup of three women with supernormal electroretinographic scotopic b-wave amplitudes to a high-intensity stimulus had an abnormal increase in permeability of the blood-retinal barrier even without peripheral pigmentary fundus changes.