Image 3: Macula OCT of Patient #1 at 8-years-old

Complete outer retinal atrophy at the foveae, preservation of ellipsoid zone (EZ) aka photoreceptor integrity line (PIL) outside of foveae. Small outer retinal hyperreflective flecks present above the RPE.
Image 4: Macula OCT of Patient #1 at 19-years-old

Progressive outer retinal atrophy, now extending diffusely across retina rather than isolated to foveae. Outer retinal hyperreflective flecks present above the RPE.


