摘要： PURPOSE. We evaluated the incongruous observation whereby ?ecks in recessive Stargardt disease (STGD1) can exhibit increased short-wavelength auto?uorescence (SW-AF) that originates from retinal pigment epithelium (RPE) lipofuscin, while near-infrared AF (NIR-AF), emitted primarily from RPE melanin, is usually reduced or absent at ?eck positions. METHODS. Flecks in SW- and NIR-AF images and spectral-domain optical coherence tomography (SD-OCT) scans were studied in 19 STGD1 patients carrying disease-causing ABCA4 mutations. Fleck spatial distribution and progression were recorded in serial AF images. RESULTS. Flecks observed in SW-AF images typically colocalized with darkened foci in NIR-AF images; the NIR-AF pro?les were larger. The decreased NIR-AF signal from ?ecks preceded apparent changes in SW-AF. Spatiotemporal changes in ?eck distribution usually progressed centrifugally, but in one case centripetal expansion was observed. Flecks in SW-AF images corresponded to hyperre?ective deposits that progressively traversed photoreceptor-attributable bands in SD-OCT images. Outer nuclear layer (ONL) thickness negatively correlated with expansion of ?ecks from outer to inner retina. CONCLUSIONS. In the healthy retina, RPE lipofuscin ?uorophores form in photoreceptor cells but are transferred to RPE; thus the SW-AF signal from photoreceptor cells is negligible. In STGD1, NIR-AF imaging reveals that ?ecks are predominantly hypo?uorescent and larger and that NIR-AF darkening occurs prior to heightened SW-AF signal. These observations indicate that RPE cells associated with ?ecks in STGD1 are considerably changed or lost. Spectral-domain OCT ?ndings are indicative of ongoing photoreceptor cell degeneration. The bright SW-AF signal of ?ecks likely originates from augmented lipofuscin formation in degenerating photoreceptor cells impaired by the failure of RPE.