Anton M Kolomeyer1, Alexander J Brucker. 1. Department of Ophthalmology, Scheie Eye Institute, Perelman School of Medicine at University of Pennsylvania, Philadelphia, Pennsylvania.
Abstract
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.
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.