Images from a patient with late-stage Sjögren-Larsson syndrome. A, Fundus images demonstrate RPE changes and atrophy with lipofuscin deposits in the macula. B, Fundus autofluorescence reveals hyperautofluorescence of lipofuscin deposits. C, D, Increased flow voids, vessel dilation, and decreased capillary density in the superficial and deep retinal capillary plexuses are displayed with optical coherence tomography (OCT) angiography. E, Spectral domain OCT (SD-OCT) shows hyperreflective dots in the inner retina and RPE and disruption of the ellipsoid zone.