Posterior polar annular choroidal dystrophy association with cystoid macular edema

Abstract

Posterior polar annular choroidal dystrophy (PPACD) is an uncommon retinal dystrophy causing nyctalopia. PPACD has been characteristically described as a foveal sparing dystrophy. We report the first case with cystoid macular edema association.

Keywords: choroidal dystrophies; cystoid macular edema; posterior polar annular choroidal dystrophy; retinal pigment epithelium atrophy.

Figure 1

A, Fundus photographs show bilateral RPE and choroidal atrophy around the optic nerve and temporal vascular arcades with foveal sparing. B, Fundus autofluorescence displays bilateral annular hypoautofluorescence corresponding to the areas of RPE atrophy. C, Fluorescein angiography demonstrates an annular hypofluorescence defect due to the lack of filling of both retinal and choroidal vasculatures, which correspond to chorioretinal atrophy. D, SD‐OCT illustrates intraretinal cystoid spaces on the inner nuclear layer as well as parafoveal atrophy of choriocapillaris and outer retinal layers (arrows)