PMC3704547_01
Fundus findings on initial examination.. Notes: (A and B) Fundus photographs: Choroidal atrophy around the optic papillae and angioid streaks radiating from around the optic discs are evident in both eyes. (A) Atrophic changes to the macular region are seen in the right eye, and (B) subretinal hemorrhage within the vascular arcade is apparent in the left; (C-F) Fluorescein angiograms 2 weeks after the onset: (C) Tissue staining and window defect are present in the macular region of the right eye. (D) The early phase image (25 seconds after injection) of the left eye reveals signs of blocking due to subretinal hemorrhage, and hyperfluorescent spots (arrow) due to early leakage superior to the fovea. The images of (E) the middle phase (80 seconds after injection) and (F) the late phase (10 minutes after injection) show increasing hyperfluorescence due to leakage, suggestive of extrafoveal choroidal neovascularization; (G and H) Optical coherence tomography: (G) Thinning of the macula is seen in the right eye, but (H) the left eye shows no obvious abnormality in the neighborhood of the fovea.
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AS radiating from around the optic discs in both eyes. Atrophic changes to the macular region were evident in the right eye, and a subretinal hemorrhage was apparent around the macular region in the left eye (Figure 1A and B). The middle phase FA image of the right eye showed tissue staining and a window defect in the macular region (Figure 1C). The early phase FA image of the left eye revealed signs of blocking due to subretinal hemorrhage and hyperfluorescent spots due to early leakage superior to the fovea (Figure 1D). Additionally, the FA images of the middle (Figure 1E) and late (Figure 1F) phases showed increasing hyperfluorescence due to the leakage, suggestive of extrafoveal CNV. Spectral-domain optical coherence tomography (OCT) (Cirrus  HD-OCT Model 4000; Carl Zeiss Meditec AG) using the HD 5-line raster scan protocol (horizontal scan of 6 mm) revealed thinning of the central retina with subretinal tissue in the right eye (Figure 1G), but no obvious abnormality was seen in the neighborhood of the fovea in the left eye (Figure 1H).
Appearance of the skin.. Note: Characteristic skin lesions on (A) the neck, and (B) the right axilla, suggestive of pseudoxanthoma elasticum.
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Examination of the skin revealed characteristic skin lesions on the neck (Figure 2A) and axillae on both the right (Figure 2B) and left side, suggestive of pseudoxanthoma elasticum.
Left fundus findings 3 months after injury.. Notes: (A) Fundus photograph: Well-defined choroidal neovascularization (CNV), one disc diameter in size, is evident, somewhat above the macular region; (B and C) Optical coherence tomography: CNV is clearly located in the (B) juxtafoveal and (C) subfoveal regions, extending across the retinal pigment epithelium line into the retina, resulting in Gass type II CNV; (D and E) Fluorescein angiography: (D) Well-defined CNV is evident in the early phase (20 seconds after injection). (E) A high level of fluorescent leakage consistent with CNV is apparent in the late phase (10 minutes after injection); (F and G) Indocyanine green angiography: CNV is visualized from (F) the early phase (1 minute after injection) to (G) the late phase (13 minutes after injection).
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One month later (3 months after the injury), BCVA in the patient's left eye had decreased to 0.7, and well-defined CNV, one disc diameter in size, was evident somewhat above the macular region (Figure 3A). OCT demonstrated that CNV was clearly located in the juxtafoveal (Figure 3B) and subfoveal (Figure 3C) regions, extending across the retinal pigment epithelial line into the retina, resulting in Gass type II CNV. CNV in the early phase (Figure 3D) and a high level of fluorescein leakage coincident with CNV in the late phase (Figure 3E). CNV was also visualized on indocyanine green angiography (VISUCAMNM/FA) from the early phase (Figure 3F) to the late phase (Figure 3G).
Left fundus findings 2 months after the intravitreal injection of bevacizumab.. Notes: (A) Fundus photograph reveals pronounced regression of choroidal neovascularization (CNV); (B) Optical coherence tomography by horizontal scan shows disappearance of subfoveal CNV.
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Visual acuity gradually improved and by approximately 2 months after starting intravitreal bevacizumab (IVB) had recovered to 1.2 in the left eye, with pronounced regression of the CNV (Figure 4A) and disappearance of the subfoveal CNV (Figure 4B).