PMC7113613_01
A, B) Retinal pigment epithelium mottling around the macular and para-macular area in fundus photo. C, D) Dark patches at the macular region in fundus autofluorescence (FAF). E, F) Hyperfluorescence observed at macula and surrounding macular region in fundus fluorescein angiography (FFA)
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F1
Fundus photo (FF 450Plus with Visupac, Zeiss, USA) showed RPE mottling around the macular and para-macular area (Figure 1 A, B (Fig. 1)). Fundus autofluorescence (FAF) (FF 450Plus with Visupac, Zeiss, USA) revealed discrete dark patches at the macula in both eyes corresponding to the area of RPE mottling suggestive of RPE atrophy (Figure 1 C, D (Fig. 1)). Fundus fluorescein angiography (FFA) (FF 450Plus with Visupac, Zeiss, USA) showed hyperfluorescence at the macula and the surrounding macular region in both eyes (Figure 1 E, F (Fig. 1)).
Central and paracentral field defects in both eyes in 30-2 Humphery visual fields (HVF)
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F2
The 30-2 Humphery visual fields (HVF) (HFAII 750, Carl Zeiss, Germany) showed central and paracentral field defects in both eyes (Figure 2 (Fig. 2)).
Reduced foveal, parafoveal and perifoveal responses noted in multifocal electroretinogram (MFERG)
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F3
Multifocal electroretinogram (MfERG) (Veris FMS II, Electro-Diagnostic Imaging, CA, USA) showed reduced N1 and P1 amplitudes in foveal, parafoveal, and perifoveal ring responses (Figure 3 (Fig. 3)).
Spectral domain optical coherence tomography (SD-OCT) showed complete loss of the ellipsoid layer and external limiting membrane, although inner retinal layers were intact.
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F4
The inner retinal layers were unaffected (Figure 4 (Fig. 4)).