PMC9719283_01
Color fundus photography and autofluorescence showing the extent of macular dystrophy. (A) Right eye, demonstrating pale optic discs with extensive deep atrophy of the central macula, epithelial pigment hyperplasia, and other areas of multifocal atrophy. (B) Left eye, depicts macular atrophy. (C) Right eye, central area of hypoautofluorescence at the macula. Diffuse outward centrifugal extension from the center to the periphery of the macula and at the nasal side of the optic disc, with sparing of peripapillary area. Very few hyperfluorescent flecks near the outer border of the affected area. (D) Hypoautoflourescent areas at the central macula and nasal to the optic disc. Outward centrifugal extension from the center to the periphery of the macula and at the nasal side of the optic disc. Very few hyperfluorescent flecks near the outer border of the affected area.
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As depicted in Figure 1A and B, the patient had pale optic discs with extensive deep atrophy of the central macula, epithelial pigment hyperplasia, and other areas of multifocal atrophy in the right eye. In the patient's fundus autofluorescence (FAF) imaging, there was evidence of central hypoautofluorescence of the macula, with diffuse outward centrifugal extension from its center to its periphery (depicted in Figure 1C and D).  Pattern standard deviations were +3.69 dB and +4.69 dB in the right and left eyes, respectively (Supplementary Figures 1 and 2).
Macular optical coherence tomography demonstrating macular dystrophy with retinal pigment epithelium atrophy. (A) Right eye. (B) Left eye.
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Figures 2A and B depict Macular OCT imaging.  Pattern standard deviations were +3.69 dB and +4.69 dB in the right and left eyes, respectively (Supplementary Figures 1 and 2).