Stargardt disease. A, Fundus photograph shows retinal flecks (arrows) and focal or geographic RPE atrophy (between arrowheads) in the macular region. B, FAF imaging reveals increased FAF corresponding to retinal flecks and decreased FAF corresponding to areas of RPE atrophy (asterisks). C, Near-infrared imaging (left panel) reveals increased reflectance corresponding to areas of RPE atrophy (asterisks). SD-OCT (right panel) reveals hyperreflectivity at the level of the RPE (corresponding histologically with enlarged RPE cells with increased lipofuscin content) (arrows), markedly thinned retina in the foveal region, and focal attenuation or loss of the photoreceptor cell layer in areas corresponding to RPE atrophy, as seen on the near-infrared image and back-shadowing on the SD-OCT image (brackets). D, Histology with PAS stain reveals hypertrophic RPE cells with numerous PAS-positive cytoplasmic granules containing lipofuscin. This histologic finding corresponds to the retinal flecks seen clinically. E, In advanced stages of Stargardt disease, geographic RPE atrophy with loss of the photoreceptor cell layer (asterisks) may be observed (H&E stain).