case_images: PMC10726563_01
case_images: Figure 1. Colour fundus photographs (top row): overall increased translucence of the RPE with visible choroidal vasculature. Area of atrophy in the inferior macula of the right eye is more extensive than in the left eye, where the location is nasal. Also, in the right eye the atrophy is closer to the foveola as compared with the left side. Very few microaneurysms/dot haemorrhages in the macula of both eyes, better visible under magnification (bottom right, arrows).. Blue light autofulorescence image (bottom row; right eye: composite of 2 images of the macula and optic disc) clearly shows the extent of the retinal atrophy and reveals the true extent of the retinal pathology with characteristic "salt and pepper" mottled hyper- and hypoautofluoresence beyond vascular arcades and in nasal peripapillary area.
case_images: OC-13-23-g-001.jpg
case_images: PMC10726563_01_OC-13-23-g-001.jpg
case_images: F1
case_images: The foveola was spared in both eyes, but in the right eye atrophic changes encroached inferior foveal area (Figure 1 (Fig. 1)). Fundus autofluorescence (FAF) (Figure 1 (Fig. 1)) revealed hypo-autofluoresence (AF) corresponding to AT and mottled hypo- and hyper-AF in the macular and peripapillary area.
case_images: Visual fields: Humphrey 10-2 showing defects consistent with atrophy
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case_images: F2
case_images: HVF (Figure 2 (Fig. 2)) confirmed reduced sensitivity corresponding to the area of AT.
case_images: OCT scans . Right eye: 1-3 show slices from different levels: 1. foveolar: normal retinal profile, no cystoid macular oedema, but intraretinal hyperreflective foci visible as well as reduced thickness of outer retina and granular irregularity at its level (rectangular frames); 2. superior part of the atrophy: two atrophic areas separated with preserved retina. Extent of RPE atrophy (solid lines) is larger than outer retinal loss (dashed line); 3. central part of the atrophy: loss of RPE and outer retinal layers. Outer retinal tubulation marked with an arrow.. Left eye: 4. nasal area of atrophy with visible hyporeflective wedge (arrowhead).. On images 3 and 4 subretinal hyperreflective material is marked with asterisks. Also increased transmission to the choroid is present on the images along atrophic regions.
case_images: OC-13-23-g-003.jpg
case_images: PMC10726563_01_OC-13-23-g-003.jpg
case_images: F3
case_images: On OCT (Figure 3 (Fig. 3)) we noted loss of retinal pigment epithelium (RPE) and outer retinal layers (ORL) (photoreceptor-ellipsoid, external limiting membrane and outer nuclear layer), smaller in size compared to RPE loss, in the area of atrophy, with concomitant increased signal transmission to the choroid.
case_images: OCT-A. Superficial plexus: rarefaction in the perifoveal region visible as a darker area surrounding the fovea, in the right eye also in the inferior fovea and in the left eye in nasal macula (red circle) in the location of atrophy.. Deep plexus: transmission (right eye) and projection (left eye) artifacts in the area of atrophy (blue circle).. Choriocapillaris: thinning with increased transmission form the deep choroid in the area of atrophy (green circle) as well as outside (orange circle).. RE=right eye; LE=left eye
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case_images: F4
case_images: OCT-A (Figure 4 (Fig. 4)) showed minimally enlarged foveal avascular zone and thinning of deep plexus and choriocapillaris in the area of atrophic changes.
case_images: Electrophysiology results. a: delay of the P100 wave for small check size; b: full field ERG: low amplitude scotopic B waves; c: 30 Hz flicker: an increased implicit time/latency; d: single cone flash: increase latency and reduced amplitude; e: multifocal ERG: reduced responses, with no foveal peak on the right and only a small one on the left
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case_images: PMC10726563_01_OC-13-23-g-005.jpg
case_images: F5
case_images: Electrophysiology findings confirmed abnormalities associated with central macular dysfunction (Figure 5 (Fig. 5)).
case_images: Ganglion cell and internal plexiform layer change during the observation period showing thinning in both eyes
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case_images: PMC10726563_01_OC-13-23-g-006.jpg
case_images: F6
case_images: OCT retinal ganglion cell (RGC) analysis showed loss of combined RGC and internal plexifom layer (IPL) thickness in both eyes (Figure 6 (Fig. 6)).