PMC4333364_01
Clinical profile and ancillary investigations at baseline. Color fundus photography demonstrates the presence of irregular, ill defined, circular area of white grey discoloration of the right macula with intraretinal haemorrhage, intraretinal thickening and retinal pigmentary changes temporal to the fovea (A). Infrared imaging showing the presence of a figure of eight or dumb-bell-shaped lesion with disruption of the inner segment-outer segment (IS-OS) junction and hyperreflective debris at the apical side of the retinal pigment epithelium on the corresponding spectral domain ocular coherence tomography (SD-OCT) (B). Fundus fluorescein angiography showing a large area of intense subretinal hyperfluorescence indicating significant macular leakage (C) along with a small central area of pooling (yellow arrow) with no extra-macular abnormalities and no vasculitis. Indocyanine green angiography demonstrating irregular heterogenous patches of blocked fluorescence in the corresponding area (D).
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Fig1
Fundus examination revealed right macular intraretinal haemorrhage, intraretinal thickening and retinal pigmentary changes temporal to the fovea (Figure 1A). Infrared imaging showed the presence of a figure of eight or dumb-bell-shaped lesion with abnormalities of the inner segment-outer segment (IS-OS) junction and hyperreflective debris along the retinal pigment epithelium (RPE) on the corresponding spectral domain ocular coherence tomography (SD-OCT), (Figure 1B). Fundus fluorescein angiography (FFA) showed significant macular leakage (Figure 1C) with no extra-macular abnormalities and no vasculitis. Indocyanine green angiography (ICGA) demonstrated blocked fluorescence in the corresponding area (Figure 1D).
Follow-up investigations. Funds fluorescein angiography (A) demonstrates late staining with reduced leakage, and indocyanine green angiography (B) illustrates irregular 'moth-eaten' choroidal vasculature with tortous choroidal vessels around the foveal region. A residual scar remained in the right macula with intraretinal pigmentary change and resolution of subretinal fluid with a subretinal fibrotic scar on SD-OCT (C). There is a presence of disrupted irregular photoreceptor layer with hyperreflective debris on the apical side of the retinal pigment epithelium.
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Fig2
ICGA did not reveal the presence of active disease (Figure 2A,B). ICGA showed irregular moth-eaten appearance of the choroidal vasculature with no areas of abnormal hypercyanescence. Residual scar remained in the right macula with intraretinal pigmentary change and resolution of subretinal fluid with a subretinal fibrotic scar on SD-OCT (Figure 2C).
Autofluorescence images. Autofluorescence at baseline revealed a irregular hypo-autofluorescent area with a strip of hyperautofluorescence at temporal edge (A), which over the next 4 days became morphologically similar to a dumb-bell-shaped maculopathy with increasing hyperautofluorescence at the rim of hypo-autofluorescent lesion (B). The lesion showed more stippled hyperautofluorescence at 1 month (C) with constricting lesion and loss of background fluorescence on subsequent follow-up at 2 months (D) and at 4 months (E).
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Fig3
Autofluorescence revealed a hypofluorescent lesion at base line (Figure 3A), which over the next 4 days became morphologically similar to a dumb-bell-shaped maculopathy (Figure 3B). The autofluorescence lesion started constricting on subsequent follow-up (Figure 3C,D,E).