Image 7: Macula OCT of Patient #2 at 5-years-old

Preserved inner and outer retinal layers with thickened and hyperreflective ELM present in both eyes.

Image 8: Macula OCT of Patient #2 at 17-years-old

Complete outer retinal atrophy across retina, with outer retinal hyperreflective flecks above the level of the RPE. Note that ELM is completely lost, hence appearing to be a transient biomarker.