PMC9532261_01
Fundus photography and RE (A) and LE (B), respectively.
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Fundoscopy revealed bilateral large, yellowish lesions located in the macula, associated with significant atrophy, particularly in the LE (Figure 1).
SD-OCT of the RE (A and B) demonstrating Retinal pigmented epithelium (RPE) detachment with hyperreflective material under RPE and cystoid macular edema. SD-OCT of the LE (C) demonstrating a large area of macular atrophy, with subretinal fibrosis; both eyes exhibit anomalous enlargement of choroidal vessels.
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Optical coherence tomography (OCT) showed a flat retinal pigmented epithelium (RPE) detachment with hyper-reflective material under RPE, damage of external retinal layers, including external limiting membrane, ellipsoid zone and RPE, and cystoid macular edema on the RE (Figure 2A and B). The LE showed a large area of macular atrophy, with subretinal fibrosis (Figure 2C); both eyes exhibited an anomalous OCT pattern of the inner choroid with extensive loss of choriocapillaris.
Fundus autofluorescence (FAF) showing hypoautofluorescent central plaque in the RE (A) with slight hyperautofluorescent borders. FAF of the LE (B) reveals less intense central hypoautofluorescence due to subretinal fibrosis.
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Fundus autofluorescence (FAF) showed central hypoautofluorescence secondary to RPE and photoreceptors loss (Figure 3).  
Fluorescein angiography (FA) of the RE (A) and LE (B), showing late hyperfluorescence of the RE central lesion and a heterogeneous angiographic pattern of the LE lesion, with well-defined borders. Mid-peripheral retina seems unaffected.
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The RE FA showed an early irregular pattern with significant late hyperfluorescence due to staining (Figure 4A). On the LE, a large early hypofluorescent lesion involving all macular areas and extending to the temporal optic disc border, with well-defined edges and late internal hyperfluorescent areas, was defined (Figure 4B).
Indocyanin-green angiography of the RE (A) and LE (B). An heterogenous pattern and anomalous enlargement of choroidal vessels in the macular area is observed, particularly in the LE.
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In the indocyanine green angiography (ICGA), an heterogenous pattern with anomalous dilated choroidal vessels could be observed (Figure 5).  
Angiography-OCT of the RE showing an anomalous vascular network.
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(A and B) Macular OCT of the RE in the last visit, 6 months after systemic treatment.
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Figure 7 shows macular OCT of the RE 1 month (7A) and 6 months (7B) after systemic treatment. 