Persistent Placoid Maculopathy. A New Clinical Entity

Purpose: To describe a previously unreported clinical entity superficially resembling macular serpiginous choroiditis but with a distinct presentation and clinical course. Design: Retrospective observational case series. Participants: Six patients, 50 to 68 years old, exhibiting this entity who were seen at 5 different centers from 1984 to 2006. Methods: Review of medical records. Main Outcome Measures: Best-corrected visual acuity (VA) and clinical and angiographic findings. Results: The lesions in our patients are similar to those of acute macular serpiginous choroiditis. Our patients had well-delineated whitish plaquelike lesions involving the macula and sparing the peripapillary areas of both eyes. In contrast to serpiginous choroiditis, VA remained good despite early involvement of the fovea until complications related to choroidal neovascularization or pigmentary mottling developed. Angiographic characteristics and the clinical course were also atypical. Fluorescein angiography revealed well-defined early hypofluorescent areas that partially filled in in the late phase. Indocyanine green angiography showed the hypofluorescence to be persistent. Unlike serpiginous choroiditis, the white macular lesions faded over a period of months to years, whereas the characteristic angiographic findings often persisted longer. Choroidal neovascularization developed in 11 of 12 eyes, with subsequent conversion to disciform macular scars in 9 of 12 eyes. Unlike serpiginous choroiditis, none of the eyes showed chorioretinal scar formation unless related to choroidal neovascularization. Conclusion: Persistent placoid maculopathy has features resembling macular serpiginous choroiditis but differs in its clinical course and effect on VA. It appears to be a new entity. The majority of eyes develop choroidal neovascularization that results in loss of central vision.