Central pigmentary retinal dystrophy and its angiographic classification.

Twenty-one cases of central pigmentary retinal dystrophy were compiled and their clinical features were described. On the basis of fluorescein fundus angiography, these cases were classified into three types according to the changes in the choriocapillaris. In Type 1, the choriocapillaris showed annular or bow-shaped atrophy around the vascular arcade in the posterior pole of the fundus, but little change in the foveal region. Type 2 exhibited round or irregularly shaped atrophy of the choriocapillaris in the center of the posterior pole of the fundus. Type 3 had no atrophy of the choriocapillaris and showed only hyperfluorescence in the posterior pole of the fundus, due to damage of the retinal pigment epithelium. These types differed from each other not only from the morphological point of view, but also in other clinical findings that included age of onset, progression of visual impairment, pattern of color vision defects or recordability of the electroretinogram. It was discussed that central pigmentary retinal dystrophy consists of heterogenous conditions.