PMC6533446_01
Colour fundus montage of the right (A) and left eye (B) shows a greyish opacification of the peripapillary retina; with more details of the posterior pole (C, D). FAF imaging reveals a speckled hyper-autofluorescence denoting an active disease phase and a speckled hypo-autofluorescence indicating RPE atrophy (E, F). OCT reveals a zonal loss of the outer retinal layers with a disruption of the photoreceptors (arrows; G, H).
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F1
Fundus examination revealed a circumscribed, flat, peripapillary, deep retinal lesion with a greyish, marginal opacification or demarcation line in both eyes; right larger than left (Figure 1 (Fig. 1)).  The delineating line was predominantly hyper-autofluorescent in the left eye and hypo-autofluorescent in the right eye corresponding to the stage of the disease; hypo-autofluorescence corresponding to atrophic stage, hyper-autofluorescent corresponding to active stage (Figure 1 (Fig. 1)). In zone 3, OCT showed a zonal loss of the outer retinal layers with a disruption of the photoreceptors, the ellipsoid line (formerly known as the inner and outer segment junction) and the interdigitation line (formerly known as cone outer segment tips, COST line) in both eyes with minimal sub-retinal fluid (SRF) in the left eye (Figure 1 (Fig. 1)). Multifocal electroretinogram (mfERG) showed a loss of foveal peak in the right eye and a reduced foveal peak in the left eye with normal parafoveal and perifoveal ring response in both eyes (Figure 1 (Fig. 1)).
At 3-month follow-up, fundus reveals persistent AZOOR lesions with a marginal opacification in both eyes; right (A) larger than left (B). FAF imaging reveals a progressive hypo-autofluorescence at the margins (C, D). OCT scan of the right (E) and left (F) eye shows no change.
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F2
Humphrey's visual field (HVF; 30-2) showed enlargement of blind spot in both eyes; right larger than left (Figure 2 (Fig. 2)). Autofluorescence revealed the demarcation line assuming an incomplete or interrupted pattern with disease progression (Figure 2 (Fig. 2)). OCT showed a zonal loss of the outer retinal layers with a disruption of the photoreceptors, the ellipsoid and cone outer segment (COST) in both eyes (Figure 2 (Fig. 2)).
At baseline, HVF showing enlargement of blind spot with few temporal focal field defects in the right (A, B) and left eye (C, D). At 3-month follow-up, HVF shows a marginal reduction of scotoma in the right (E, F) and left (G, H) eye.
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F3
Fundus examination revealed a circumscribed, flat, peripapillary, deep retinal lesion with a greyish, marginal opacification in both eyes; right larger than left (Figure 3 (Fig. 3)). Humphrey's visual field (HVF; 30-2) showed a slight reduction of scotoma size in the left eye, and stable in the right eye (Figure 3 (Fig. 3)).