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Mohamed Gendy, Amani Fawzi, Robert Wendel, Dante Pieramici, Joel Miller, David Brown, Lee Jampol; Multimodal imaging in Persistent Placoid Maculopathy. Invest. Ophthalmol. Vis. Sci. 2013;54(15):5852.
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To describe the acute and long term retinal changes in Persistent Placoid Maculopathy (PPM) on Spectral Domain Optical Coherence Tomography (SD-OCT), indocyanine green angiography (ICG-A), Fluorescein angiography (FA) and fundus autofluorescence (FAF).
A retrospective review of medical records of twelve eyes of six patients, aged 50 to 60 years, diagnosed with PPM at four different centers.
All patients presented within one week of subjective symptoms. All patients presented with bilateral macular lesions except one patient, who initially presented with unilateral lesions. Six eyes had extra-macular lesions in the course of follow-up. On SD-OCT, the acute placoid lesions showed hyperreflectivity in the outer nuclear layer (ONL), disruption of the external limiting membrane (ELM) and inner segments with a sliver of hyporeflectivity at the level of the outer segments in some cases. Over the course of follow-up, most lesions showed complete restoration of the outer retinal architecture, while some showed partial restoration or progression to atrophy. On FA, all placoid lesions were hypofluorescent in early frames and hyperfluorescent in late frames. ICG-A showed sharply delineated dense early hypofluorescent lesions, which persisted on late ICG-A in all patients. Hypofluorescent lesions faded completely or partially after resolution of the placoid lesions on SD-OCT and clinical examination. Three different patterns were observed on FAF. Acute placoid lesions showed hyperautofluorescence, which became hypo-autofluorescence as the disease progressed to atrophy. Acute lesions were hypo-autofluorescent in one patient. Meanwhile, in one case, lesions were not seen on FAF initially, and showed minimal hyperautofluorescence later when lesions were atrophied. Choroidal neovascularization (CNV) developed in one patient who was treated with photodynamic therapy, intravitreal Bevacizumab and sub-Tenon’s capsule Triamcinolone acetonide injections. Two patients received treatment with oral immunosuppresssion. The mean follow-up time was 25 (range, 2-92) weeks.
On SD-OCT, acute retinal changes in PPM involve the ONL, ELM and inner segments. Retinal pigment epithelium (RPE) and choroid are involved in severely affected cases. Choroidal hypoflurescence is secondary to a blockage effect, hypoperfusion or mixed mechanisms. Variable extent of RPE involvement in this condition is reflected in highly variable FAF findings.
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