We report a case of unilateral, focal, pigmented paravenous retinochoroidal atrophy (PPRCA).

A 46-year-old female visited our clinic in complaint of a vague problem with her right eye identified during a general medical examination. The visual acuity (without correction) of both eyes was 1.0. Slit-lamp examination of both eyes revealed no specific signs. Fundus examination of the right eye revealed focal, bony-spicule-shaped retinochoroidal atrophy with pigmentation along the course of the superior retinal vein. A fundus autofluorescence examination revealed principally hypofluorescence with some hyperfluorescence at the margin of the atrophic retinochoroidal lesion. Optical coherence tomography revealed mixed clumping and atrophy of the retinal pigment epithelium (RPE) layer and thinning of the choriocapillaris layer. Fluorescence angiography revealed a window defect and blockage at the site of the lesion (the fluorescent material did not enter the lesion). The site of the window defect was in correlation with the atrophic RPE region. The site of the blockage at lesion also matched with the site of the regional pigment clumping. No definite leakage was observed.

To the best of our knowledge, this is the first case of unilateral focal PPRCA reported from Korea.