PMC10032466_01
Clinical fundus image showing pigmentary changes with crystalline
yellow-white deposits prominent at the posterior pole. The choroidal
vasculature was prominent seen in these areas. (a, b) FFA on Spectralis
shows area of central hypofluorescence with surrounding hyperfluorescence.
(c, d) A peripapillary ring and small central area was seen to be preserved.
The enhanced depth imaging - optical coherence tomography showed
disorganisation of the RPE, photoreceptors and choriocapillaris at macula
except a small central island. (e, f) Retinal and choroidal thinning was
prominent with intra-retinal hyper-reflective dots corresponding to
deposits. The ORT was also seen suggestive of degenerating RPE cells.. FFA, fundus fluorescein angiography; ORT, outer retinal tubulation; RPE,
retinal pigment epithelium.
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The choroidal vasculature was prominent in these areas (Figure 1a,b). A peripapillary ring and small central area were seen to be preserved (Figure 1c,d). OCT (EDI-OCT) (Spectralis, Heidelberg Engineering, Heidelberg, Germany) showed disorganisation of the RPE, photoreceptors and choriocapillaris at macula with exception of a small central island (Figure 1e, f). Retinal and choroidal thinning was prominent, with intra-retinal hyper-reflective dots corresponding to deposits. Outer retinal tubulation (ORT) suggestive of degenerating RPE cells was also seen in both eyes.
Optos ultra-widefield FAF showed central hypoautofluorescence with
surrounding mottled autofluorescence indicating RPE involvement in these
areas along with peripheral involvement. (a, b) Topcon FAF showed a few
hyperautofluorescence areas around the margin. (c, d) A peripapillary ring
and small central area was seen to be preserved.. FAF, fundus autofluorescence; RPE, retinal pigment epithelium.
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Ultra-widefield Optos (Optos California, P200 Dx, Marlborough, MA, USA) FAF showed central hypoautofluorescence with surrounding mottled autofluorescence indicating, retinal pigment epithelium (RPE) involvement in these areas along with peripheral involvement. (Figure 2a,b). But Topcon (Triton; Topcon Medical Systems, Tokyo, Japan) FAF also showed few hyper-autofluorescence (AF) areas around the margin (Figure 2c,d).
The Optos imaging using green reflectance (a, b) showed central macular
changes with few dispersed hyper-reflective spots while the red reflectance
(c, d) showed prominent choroidal vasculature.
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The Optos ultra-widefield green reflectance (Figure 3a, b) showed central macular changes with few dispersed hyper-reflective spots, whereas the red reflectance (Figure 3c, d) showed prominent choroidal vasculature with no hyper-reflective spots.
RF image on Topcon (a, b) and near infra-red imaging on spectralis (c, d)
showing central macular changes with dispersed hyper-reflective spots and
choroidal vasculature. Deposits can be seen distinctly on RF imaging.. RF, red-free.
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