The Retina Reference

Pattern Dystrophy of the Retinal Pigment Epithelium - with peripheral vitelliform lesions

Pattern dystrophy of the retinal pigment epithelium (RPE) is a genetic disease that causes pigment and lipofuscin to deposit in the macula, generally resulting in the loss of sharp central vision. This 69-year-old African American female was seen in 2011 for this condition and at the time her vision was 20/25 in the right eye and 20/32 in the left. 2011 fundus images show a normal right macula that corresponds to her normal optical coherence tomography (OCT) scan. In her left eye, a vitelliform lesion is present (yellow arrow). On the OCT the vitelliform material is solid (blue arrow). Additional lesions were also documented in the periphery of both her eyes, which is uncommon for this disease (black arrows). At her 2016 visit, her vision was 20/32 in the right eye and count fingers at 3 feet in the left. Her fundus images showed a vitelliform lesion with a pseodohypopyon appearance in the right macula (red arrow). Transverse OCT scans of her right eye shows subretinal fluid which corresponds to the darker area of the lesion (white arrows). A longitudinal scan shows the hyperreflective lipofuscin that has collected inferior to her fovea (green arrow). In the left eye the retinal pigment epithelium (RPE) layer has atrophied under the fovea (blue arrow) and the volume of vitelliform material has decreased.