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A Systematic Review

Kolomeyer, Anton M., MD, PhD; Brucker, Alexander J., MD

doi: 10.1097/IAE.0000000000002284

Purpose: To review the literature on persistent placoid maculopathy.

Methods: Several databases were searched for all years for “persistent placoid maculopathy.”

Results: A total of 21 unique patients were identified (most commonly Caucasian men in their 50s and 60s). Mean ± SD age at time of presentation was 58.6 ± 6.9 years of age, and follow-up time was 29.2 ± 51.9 months. Thirty-three (79%) eyes had subjective symptoms on presentation. Five (24%) patients presented with a prodrome, and four (19%) patients had vitreous cell. Mean ± SD logarithm of minimal angle of resolution presenting versus final vision was 0.48 ± 0.50 (Snellen equivalent, 20/60) versus 0.63 ± 0.52 (Snellen equivalent, 20/84). Systemic inflammation and autoimmune disease were associated with worse presenting and final vision. Persistent placoid maculopathy characteristic imaging features that aid in diagnosing this rare clinical entity include the following: 1) plaque-like hypopigmented, white/yellowish foveal/perifoveal lesions on color fundus photography; 2) early hypofluorescence and late “fill-in” or staining on fluorescein angiography; 3) persistent hypocyanescence on indocyanine green angiography; and 4) choriocapillaris hyposignal on optical coherence tomography angiography. Choroidal neovascularization was present in 50% of eyes, with 62% diagnosed at presentation. Although choroidal neovascularization is typically responsive to anti–vascular endothelial growth factor injections, it is associated with poor visual outcome. Systemic steroids are generally used as first-line agents with addition of other immunomodulatory medications if the disease is refractory or recurrent.

Conclusion: Precise disease pathophysiology of persistent placoid maculopathy has not been elucidated but most probably relates to selective choriocapillaris hypoperfusion/ischemia/vasculitis. Combination of anti–vascular endothelial growth factor injections and immunosuppressive medications may be effective in controlling disease activity and limiting development of sight-threatening complications.

Persistent placoid maculopathy is a bilateral idiopathic chorioretinopathy most commonly diagnosed in Caucasian men in the 6th–7th decade of life. In the following, we performed a comprehensive review of the 21 published cases to summarize demographics, symptoms at presentation, clinical characteristics, visual outcomes, multimodal imaging characteristics, and treatment modalities, as well as to discuss proposed disease pathophysiology.

Department of Ophthalmology, Scheie Eye Institute, Perelman School of Medicine at University of Pennsylvania, Philadelphia, Pennsylvania.

Reprint requests: Alexander J. Brucker, MD, Department of Ophthalmology, Scheie Eye Institute, University of Pennsylvania, 51 N. 39th Street, Philadelphia, PA 19104; e-mail:

None of the authors has any financial/conflicting interests to disclose.

© 2018 by Ophthalmic Communications Society, Inc.