The Retina Reference

Stargardt Disease - Dark Choroid with Target Lesions

Stargardt disease is a bilateral macular condition with an autosomal recessive inheritance. It causes macular atrophy and results in decreased vision over time. Images above belong to a 32-year-old woman with a visual acuity of 20/40 in the right eye and 20/32 in the left. She did not have any visual complaints about her vision. However, ancillary testing confirmed she had Stargardt disease. In her fluorescein angiography images, both eyes had dark choroid signs (the choroidal vasculature is not seen) and target lesions (yellow arrows). Dark choroids are typical of this condition and are caused by the accumulation of lipofuscin in the retina. Her optical coherence tomography (OCT) scans also show retinal pigment epithelial (RPE) atrophy in the parafovea which gives rise to the target lesion on fluorescein angiography (green arrows). Excessive consumption of vitamin A can theoretically exacerbate the disease and is not advised. There is currently no treatment for this condition.