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Eye Glossary - Stargardt's Disease

Stargardt's Disease

Stargardt's is an inherited disease that affects the retina, the layer of light-sensitive cells lining the back of the eye. It usually becomes apparent between the ages of 8 and 14. Boys and girls are equally susceptible and more than one child in a family may have it.

Stargardt's disease begins with slightly blurry vision that gradually gets worse. By the late 20s, vision is typically about 20/200, the level labeled legally blind. Remaining vision is good enough for most people to live fairly normal lives, though they won't drive, or read without using magnification devices.

A build-up of lipofuscin (fatty substance) in retinal cells is thought to cause Stargardt's disease. The buildup typically happens in the central retina, or macula, where it resembles beaten bronze. Or, it can occur in the side retina where it causes small white flecks. This form is called fundus flavimaculatus. Angiography, a special photograph of the retina, may aid in the diagnosis. Although no specific medical or surgical treatment is available, glasses and magnification help affected people adapt to the disease.