Posterior polar annular choroidal dystrophy associated with
Posterior polar annular choroidal dystrophy (PPACD) is an uncommon disease characterized by retinal pigment epithelium and choriocapillaris atrophy in an annular pattern surrounding the optic nerve and temporal vascular arcades sparing the fovea. There are very few reports about this entity; however, there are no reports with macular edema association.

The following case has been reported in the journal Clinical Case Reports. A 65‐year‐old woman presented with a 3‐year history of nyctalopia. On referral, visual acuity was 20/25 in the right eye and 20/20 in the left eye. Anterior segment examination for both eyes was normal. Bilateral fundus examination revealed retinal pigment epithelium (RPE) and choriocapillaris atrophy in an annular pattern along the optic nerve and the temporal vascular arcades, sparing the central macula and peripheral retina.

Fundus autofluorescence imaging displayed a well‐defined annular pattern of hypoautofluorescence concentric to the parafoveal area, corresponding to the region of atrophy. Retinal fluorescein angiography (FA) demonstrates an annular hypofluorescence defect due to the lack of filling of both retinal and choroidal vasculatures corresponding to the atrophy region.

Spectral‐domain optical coherence tomography (SD‐OCT) showed cystoid macular edema with outer retinal atrophy sparing subfoveal region. Electroretinography revealed a depressed cone response with a normal rod response. Due to the clinical imaging and electrophysiological studies, diagnosis of posterior polar annular choroidal dystrophy (PPACD) was made.

Key clinical message:-
Posterior polar annular choroidal dystrophy (PPACD) is an uncommon retinal dystrophy causing nyctalopia. PPACD has been characteristically described as a foveal sparing dystrophy. This is the first case with cystoid macular edema association.

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