The Retina Reference

Stargardt Disease - Severe Progression

Refer to normal photos for comparison. This is a 36-year-old female with severe Stargardt disease, a genetic condition that affects the central retina and retinal pigment epithelium. It has no racial predilection. At her initial visit in 2006, her vision was 20/400 in the right eye and count fingers (CF) in the left. In 2018, her visual acuity has dropped to CF in both eyes. Color fundus photos taken in 2013 show dark areas of retinal pigment epithelium (RPE) atrophy and scarring (yellow arrows). At her most current visit in 2018, fundus autofluorescence scans show large regions of hypoautofluorescence (green arrows) which are areas of the retina where cells have died. In her optical coherence tomography scans (OCTs), the RPE layer is noticeably absent in both eyes (white arrows) with a subretinal scar in the right (red arrow) and subretinal lipofuscin in the left (blue arrow). The pink arrow points to an epiretinal membrane (scar tissue) that is unrelated to Stargardt disease. Those who are diagnosed with Stargardt are advised not to take large doses of vitamin A as it could cause an acceleration in vision loss. There is currently no treatment for Stargardt disease, although there is research involving subretinal transplantation of stem cells.