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Catherine A. Cukras, Wai T. Wong, Rafael C. Caruso, Denise Cunningham, Wadih M. Zein, Paul A. Sieving; Radial Expansion of Fundus Autofluorescence Patterns in Stargardt Disease Over Time. Invest. Ophthalmol. Vis. Sci. 2011;52(14):5015.
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Changing lipofuscin and melanin content in RPE cells has been hypothesized to contribute to Stargardt disease pathogenesis. Longitudinal study of autofluorescence in Stargardt disease which reflect changing fluorophore compositions can reveal aspects of disease progression not previously evident.
We examined the temporal-spatial patterns of fundus autofluorescence with excitation at both 488 nm (standard fundus autofluorescence, FAF) and 795nm (near infrared autofluorescence, NIA) in a longitudinal case series involving 8 eyes of 4 patients (range of follow-up = 16 to 60 months; mean = 33 months). Image processing was performed to analyze spatial and temporal cross-modality associations.
Longitudinal FAF imaging of fleck lesions revealed hyperautofluorescent lesions that extended in a centrifugal direction from the fovea with time. Patterns of spread were non-random and followed a radial path that leaves behind a trail of diminishing autofluorescence. Longitudinal NIA imaging also demonstrated centripetal lesion spread, but with fewer hyperautofluorescent lesions, suggestive of more transient hyperautofluorescence and more rapid decay at longer wavelengths. FAF and NIA abnormalities were spatially correlated to each other, and together reflect systematic progressions in fleck distribution and fluorphore composition occurring during the natural history of the disease.
Stargardt disease fleck lesions do not evolve randomly in location but instead follow consistent patterns of radial expansion and a systematic decay of autofluorescence that reflect changing lipofuscin and melanin compositions in RPE cells. These progressive foveal-to-peripheral changes are helpful in elucidating molecular and cellular mechanisms underlying Stargardt disease and may constitute potential outcome measures in clinical trials.
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