Centrifugal expansion of fundus autofluorescence patterns in Stargardt disease over time.

Abstract

Clinical hallmarks of Stargardt disease include the accumulation of yellow flecks in the retina in the earlier phase¹ and the onset of central retinal and retinal pigment epithelial (RPE) atrophy and central vision loss in the later phase.² Histopathological studies indicate that Stargardt disease–related fleck lesions are composed mainly of lipofuscin,^3-5 a by-product of the visual cycle that consists of multiple fluorophores, including A2E.⁶The accumulation of lipofuscin in the retina occurs normally with aging⁷ but has an accelerated course in certain retinal diseases such as Stargardt disease.⁸