Stargardt Disease with Excellent Central Vision

These images are from a 31 year old, male patient who has Stargardt disease. Notice the yellowish flecks (yellow arrows) on the color fundi. The yellow flecks (pisciform lipofuscin flecks) are caused by faulty metabolism of photoreceptor outer segments. The subjacent retinal pigment epithelium (RPE) eventually atrophies. There is an area of atrophic RPE (white arrows) that appears nasal to the fovea in the left eye. The right eye has an ellipsoid zone atrophy that has progressed farther away from the fovea (red circle). However, this patient has maintained excellent central vision due to noninvolvement of the foveal RPE (green arrows).