This healthy 55YO female presented with 2 years of mild bilateral vision loss. Family history was negative. Vision was 20/20 OD and 20/40 OS.
Color photography shows bilateral paravenous chorioretinal scarring with intraretinal pigment migration.
Learning Points:
Pigmented paravenous chorioretinal atrophy (PPCRA) is a rare condition characterized by paravenous pigment clumps with peripapillary and radial zones of retinal pigment epithelial (RPE) atrophy (Lee et al, AJO 2021;224:120-132). It is bilateral, often asymmetric, with various causes, including genetic and pseudo-PPCRA (inflammatory, infectious, or unknown). Patients may be asymptomatic or present with symptoms such as nyctalopia, while central visual acuity is often relatively preserved in the absence of macular involvement. The underlying pathophysiology remains incompletely understood, although primary choroidal and RPE abnormalities have been proposed to contribute to the characteristic retinal changes.
