PMC3527992_01
A funduscopic examination reveals chorioretinal atrophy along the paravenous area in both eyes and supranasal retinal areas left eye
without macular involvement. Note also a marked bone spicule pigment clumping together with the atrophy of the left eye (A, B). Fundus
autofluorescence examination reveals geographic hypofluorescence (C, D; white arrows) along the paravenous and supranasal retinal areas.
Hyperfluorescence surrounding the hypofluorescence in the peripheral paravenous distribution (C, D; yellow arrows). These arrows are
corresponding to those shown in fundus examination.
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F1
A marked bone spicule pigment clumping together with the atrophy was noted left eye (Fig.1A,B). Linear hyperfluorescence was noted, which comparatively surrounded the hypofluorescence in the peripheral paravenous distribution (Fig. 1C,D).
Fluorescein angiography shows a window defect (A, B) and indocyanine green angiography shows hypofluorescence (C, D)
corresponding to the atrophy.
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F2
FA and IA showed a window defect (Fig. 2A,B), and hypofluorescence (Fig.2C,D), respectively, which exclusively corresponds to the atrophic area along the retinal vein area and the optic disc both eyes.
Blue area of the color map reveals thinning of the retina on optical coherence tomography (OCT) in macular map mode (A, B).
Cross scans in the atrophic areas revealed thinning of not only RPE layer and the outer retinal layer, but also inner retinal layer (C, D).
Enhanced depth imaging OCT scans demonstrated that choroidal thickness was thin (arrowheads).
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F3
Spectral domain OCT (RS-3000 advance ; NIDEK, Gamagori, Japan) displayed thinning of the retina in macular map and cross scans in the atrophic areas revealed thinning of not only RPE layer and the outer retinal layer, but also inner retinal layer (Fig. 3A,B). Enhanced depth imaging OCT scans demonstrated that choroidal thickness was thin in the areas (Fig. 3C-F).
An electroretinogram demonstrated that right eye was
subnormal, while reduced a and b waves response was noted in left
eye together with more extensive atrophy.
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F4
An electroretinogram (ERG) demonstrated that right eye was subnormal, while reduced a and b waves response was noted in left eye together (Fig. 4) with more extensive atrophy.