The Retina Reference

Pattern Dystrophy of the Retinal Pigment Epithelium

Please refer to the images from a normal eye for comparison. This 71 year old woman presented with wavy vision and a visual acuity of 20/30 in both eyes. She was diagnosed with pattern dystrophy of the retinal pigment epithelium (RPE). This disease is an autosomal dominant condition that primarily targets the macula. Subretinal yellow-orange or gray pigments are deposited in the macula resulting in decreased visual acuity. Her fundus images taken in 2009 shows the pigmentary changes in both eyes (white arrows). Optical coherence tomography (OCT) scans that were also taken in 2009 show subretinal deposits under the macula (red arrows). By 2016, her left eye had developed more pigment epithelial atrophy than her right eye. Her visual acuity was 20/50 in the right eye and 20/125 in the left. The OCT of her right eye shows similar features to the 2009 scan with an epiretinal membrane (yellow arrow) that is unrelated to the disease. Unlike the scan of her right eye, the scan of her left eye shows RPE atrophy (blue arrow) which is consistent with her decreased vision. This disease does not cause total blindness; however, it can impair central vision. Low vision aids were recommended.