Stargardt Disease- Absence of Central Scotoma

These images come from a 48-year-old female patient with Stargardt disease. She was diagnosed with Stargardt disease 17 years ago. The red arrows highlight areas of retinal pigment epithelial (RPE) atrophy with pigmented scarring. The yellow arrows highlight the characteristic flecks (areas of lipofuscin build-up) associated with Stargardt disease. The visual field testing shows scotomas in both eyes caused by the atrophy. Note that the scotomas are not central, which stems from the fact that the patient is viewing eccentrically to perform the visual field test. The patient fixates using a border locus of normal retina on the edge of the scar. The green arrows on the OCTs highlight areas of RPE atrophy. This patient's visual acuity is around 20/40 OD and 20/50 -2 OS, which is similar to that of a year ago. This case illustrates that visual acuity cannot be reliably determined by the appearance of the fundus lesion. The median time until visual acuity falls to 20/200 vision in a patient diagnosed after age 20 is approximately 20 years.