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Subject | Fundus appearance | FAF | SD-OCT | Goldmann perimetry |
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1 | Peripheral bone spicules, retinal vessel attenuation, optic disc pallor, generalized hypopigmented dotting and peripheral pavingstone-like lesions | n/a | n/a | n/a |
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2 | Peripheral isolated bone spicules, retinal vessel attenuation, optic disc pallor, generalized hypopigmented dotting and peripheral pavingstone-like lesions, circumscribed atrophy with diffuse pigmentation | Isolated hypo-AF with a nummular pattern in the periphery. Peripheral and macular hypo/hyper-AF mottling. Peripapillary hypo-AF. Central macular circumscribed hypo-AF, with a central hyper-AF dot | Epiretinal membrane. Widespread loss of outer retinal layers and RPE irregularities. Prominent foveal atrophy. Large perifoveal ORT (OD), perifoveal ORT (OS). Hyper-reflective deposits above the RPE | V4e: OU minimal central island of vision |
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3 | Peripheral bone spicules, retinal vessel attenuation, optic disc pallor, generalized hypopigmented dotting and peripheral pavingstone-like lesions, circumscribed atrophy with diffuse pigmentation | Hypo-AF with a nummular pattern in the periphery. Peripheral and macular hypo/hyper-AF mottling. Peripapillary hypo-AF. Circumscribed central macular hypo-AF, surrounded by a hyper-AF ring, and a central hyper-AF dot | Epiretinal membrane. Widespread loss of outer retinal layers, thinning of inner retinal layers and RPE irregularities. Large foveal ORT (OU). Hyper-reflective deposits above the RPE | V4e: OU minimal central island of vision |
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4 | Macular hypopigmented and hyperpigmented lesions | n/a | n/a | n/a |
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5 | n/a | n/a | n/a | n/a |
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6 | Peripheral bone spicules, retinal vessel attenuation, optic disc pallor, generalized hypopigmented dotting and peripheral pavingstone-like lesions, macular clumped pigmentation | Extensive hypo-AF with a nummular pattern in peripheral, peripapillary and macular areas. Hypo/hyper-AF mottling involving the periphery and macula. Circumscribed central macular hypo-AF | Epiretinal membrane. Widespread loss of outer retinal layers, thinning of inner retinal layers and RPE irregularities. Foveal atrophy. Hyper-reflective deposits above the RPE | n/a |
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7 | Tessellated appearance, hypopigmented peripheral dotting | Peripheral hypo-AF mottling. Hyper-AF macular ring surrounding a normal-AF area with a central hyper-AF dot | Irregularities of outer retinal layers. Peripheral EZ disruption | V4e: OU normal I4e: OD increased peripheral relative scotoma, peripapillary scotoma; OS normal |
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8 | Peripapillary atrophy, tessellated appearance, hypopigmented peripheral dotting | Peripheral hypo-AF mottling. Hyper-AF in the central macular area with a diffuse central hyper-AF dot | Widespread loss or outer retinal layers with parafoveal ELM preservation, and foveal EZ preservation | V4e: OD central island of vision, preservation of nasal-peripheral area; OS temporal constriction I4e: OD undetectable; OS central island of vision |
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9 | Hypopigmented generalized dotting, macular clumped pigmentation | Peripheral and macular hypo/hyper-AF mottling. Peripapillary hypo-AF with adjacent diffuse nasal hyper-AF. Diffuse central macular hypo-AF, surrounded by a hyper-AF ring and with a central hyper-AF dot | Epiretinal membrane. Widespread loss of outer retinal layers and RPE irregularities. Hyper-reflective deposits above the RPE | V4e: OD superior constriction; OS concentric constriction I4e: OD minimal central island of vision; OS undetectable |
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10 | Peripheral isolated bone spicules, retinal vessel attenuation, generalized hypopigmented dotting, macular circumscribed atrophy with diffuse pigmentation | Perivascular hypo-AF mottling. Hyper-AF macular ring surrounding a circumscribed central macular hypo-AF area. OD with a foveal hyper-AF dot | Widespread loss or outer retinal layers with foveal ELM and EZ preservation. Hyper-reflective deposits above the RPE | V4e: OD minimal central island of vision; OS central island of vision |
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11 | Hypopigmented dotting in the retinal equator, vessel attenuation and macular clumped pigmentation | Peripheral hyper-AF mottling, and equatorial hypo-AF. Temporal peripapillary hyper-AF. Diffuse central macular hypo-AF, with a central hyper-AF dot | Epiretinal membrane. Widespread loss of outer retinal layers, thinning of inner retinal layers with some foveal preservation. Foveal atrophy. RPE irregularities. Hyper-reflective deposits above the RPE | V4e: OU annular scotoma |
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12 | Peripheral bone spicules, retinal vessel attenuation, optic disc pallor, generalized hypopigmented dotting and peripheral pavingstone-like lesions | Isolated peripheral nummular hypo-AF. Peripheral and macular hypo/hyper-AF mottling. Peripapillary hypo-AF. Circumscribed central macular hypo-AF. OD with a central hyper-AF dot | Epiretinal membrane. Widespread loss of outer retinal layers, thinning of inner retinal layers and RPE irregularities. Large foveal ORT (OD), perifoveal ORT (OS). Hyper-reflective deposits above the RPE | n/a |
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13 | Peripheral bone spicules, retinal vessel attenuation, optic disc pallor, macular clumped pigmentation | Peripheral and macular hypo/hyper-AF mottling. Peripapillary hypo-AF, hyper-AF between the peripapillary area and the fovea. Diffuse central macular hypo-AF, and a central hyper-AF dot | Epiretinal membrane. Widespread loss of outer retinal layers, thinning of inner retinal layers with some foveal preservation. RPE irregularities. Hyper-reflective deposits above the RPE | n/a |
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14 | Peripheral isolated bone spicules, retinal vessel attenuation, generalized hypopigmented dotting, optic disc pallor, circumscribed macular atrophy | Perivascular hypo-AF mottling. Hyper-AF macular ring surrounding a circumscribed central macular hypo-AF area | Epiretinal membrane. Widespread loss of outer retinal layers. Hyper-reflective deposits above the RPE | n/a |
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