PMC6114516_01
Case 1. a, b Color fundus photos at presentation. a The right fundus was found to be normal; b the left fundus revealed a well-delineated area of retinal pigment epithelium atrophy in the inferonasal macula with foveal sparing. c, e Early and late phase fluorescein angiography images of the right eye were normal. d, f In the left eye, there was early diffuse hyperfluorescence with a hypofluorescent border. In late phase, there was late staining of a patch in the center of the lesion. g, h Humphrey visual field 24-2 revealed a full visual field in the right eye and a superior paracentral scotoma corresponding to the lesion in the left eye
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Fig1
Fundus examination of the LE revealed an area of RPE atrophy in the inferonasal macula with foveal sparing (Fig. 1).
Case 1. Fundus autofluorescence (FAF) at presentation (a), day 10 (b), week 3 (c), month 2 (d), and month 5 (e). At presentation (a), there was a trizonal FAF pattern. There was an area of diffuse hypoFAF (yellow star) surrounded by a large area of speckled FAF (green asterisk). These areas were circumscribed by a continuous hyper-FAF demarcation line (blue arrow) outside of which the retina appeared normal (red asterisk). In b-d, there was FAF evidence of lesion expansion in areas encircled by the green dashed line. Of note, in 3C, there is no demarcation line around the area of new expansion, possibly indicating a very recent change. Between month 2 and month 5, there was minimal evidence of lesion change
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Fig2
FAF in the left eye revealed a trizonal pattern and a demarcating hyper-FAF line between the involved and uninvolved retina (Fig. 2); these findings were consistent with AZOOR. At this time, visual acuity had reduced to 20/60, and two perilesional haemorrhages were observed along with a hypo-FAF zonal lesion expansion which included the fovea (Fig. 2).
PMC6114516_02
Case 2. Multimodal retinal imaging at presentation. a, b Fundus examination revealed bilateral peripapillary atrophy with drusen-like material and retinal swelling at the macula of the left eye (LE). c, d Fundus autofluorescence (FAF) showed bilateral zonal peripapillary hypoFAF areas surrounded by a granular patchy hyper-FAF border. In the LE (d), a zonal defect with similar characteristics was detected superior to the fovea. e, f Fluorescein angiography (FA) showed bilateral peripapillary hyperfluorescence. f FA of the LE showed dye leakage from a juxtafoveal type 2 choroidal neovascularizarition
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Fig4
Fundus examination revealed bilateral peripapillary atrophy and retinal swelling at the macula of the LE (Fig. 4).
Case 2. Fundus autofluorescence (FAF) at presentation (a, b), 1 month after 1 anti-VEGF injection (c, d) and 1 month after 3 anti-VEGF injections (e, f). c, d After the first injection, bilateral zonal progression was noted. FAF showed the typical trizonal pattern consisting of an hypo-FAF area (yellow stars) surrounded by a large area of speckled FAF (green asterisks), circumscribed by a continuous annular hyper-FAF demarcation line (blue arrows) at the junction between the involved and uninvolved retina (red asterisks). c In the right eye (RE) FAF showed enlargement of the peripapillary zonal lesion and new hyperFAF zonal lesions appeared temporal to the fovea. d In the left eye (LE) FAF showed progression of the zonal macular lesion with foveal involvement. e, f After the third injection FAF showed bilateral progression of the zonal lesions with confluence of the lesions in the RE (e) and foveal sparing. In the LE (f), the hyper-FAF annular outer border lesion was no longer detectable
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Fig5
Four 4 weeks after the injection, BCVA was stable; in the RE the peripapillary lesion extended and new zonal lesions were visible temporal to the fovea (Fig. 5); in the LE the peripapillary and the macular defect extended and merged appearing as a single, large zonal defect involving the fovea. FAF in both eyes revealed a trizonal pattern and a demarcating hyper-FAF line between the involved and uninvolved retina (Fig. 5); these findings were consistent with AZOOR. One month after the third injection, further progression of the zonal lesions was observed in both eyes (Fig. 5).
Case 2. Spectral-domain optical coherence tomography (SD-OCT) scan after 3 anti-VEGF injections in the left eye. a, b SD-OCT scan of right eye (a) and left eye (b) revealed photoreceptor dysfunction by showing disruption of the ellipsoid zone (white arrows) with thinning of the outer nuclear layer (white asterisks). b SD-OCT scan of the left eye showed presence of well-defined hyperreflective material consistent with retinal scar
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Fig6
SD-OCT scan showed complete resolution of the subretinal fluid with persistent well-defined subretinal hyperreflective material (Fig. 6).
PMC6114516_03
Case 3. Wide field color image and wide field fundus autofluorescence (FAF) at presentation. a, b Wide field color image showed multiple, well-demarcated zonal areas of outer retinal atrophy that appeared slightly depigmented, with an increased visualization of the major underlying choroidal vessels. a In the right eye, there was one main zonal area in the posterior pole and two additional areas in the nasal and the supero-temporal mid-periphery. b In the left eye three zonal areas were located at the posterior pole and the temporal mid-periphery. c, d FAF showed hypo-FAF with granular hyper-FAF signal in correspondence of these depigmented areas. A leading border of hyper-FAF was evident at the junction between the involved and uninvolved retina
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Fig7
Dilated fundus examination showed multiple, well-demarcated zonal areas of outer retinal atrophy at the posterior pole and at the mid periphery of both eyes (Fig. 7).
Case 3. Near infrared reflectance (NIR) imaging acquired simultaneously with spectral domain optical coherence tomography (SD-OCT) at presentation and during treatment of the right eye. a At presentation SD-OCT scan at the level of the zonal lesion showed focal interruption of the ellipsoid zone (yellow arrowhead). b After 2 months NIR imaging showed progression of the zonal lesion (white arrow) and SD-OCT scan showed increased retinal thickness and ill-defined hyperreflective material consistent with a type 2 choroidal neovascularization. c, d During treatment NIR imaging showed zonal progression and SD-OCT scan showed consolidation of the hyperreflective material
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Fig8
AZOOR. After 2 months, a progression of the zonal lesions was found in both eyes; in the RE, the progression occurred around the disc and towards the fovea and development of a subfoveal type 2 CNV was noted (Fig. 8). During treatment there was zonal progression at the right macula (Fig. 8).
PMC6114516_04
Case 4. a, b Color fundus photograph (CFP) of AZOOR 10 years after photodynamic therapy in the right eye. a CFP of the right eye showed extensive macular scarring. b CFP of the left eye showed peripapillary depigmentation and drusen like material. c Fundus autofluorescence (FAF) in correspondence of these areas showed a mixed hypo-FAF and granular hyper-FAF signal with a leading hyper-FAF border at the junction between the involved and uninvolved retina (white arrow). d After one year of follow-up FAF of the left eye revealed minimal progression of the peripapillary zonal lesion in the left eye (yellow arrow)
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Fig9
From 2005 to 2015, a progressive enlargement of the scar was noted in the RE (Fig. 9). These zonal lesions increased in size during follow-up (Fig. 9) and remained stable thereafter.