Stargardt’s disease 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 eight and 14. Boys and
girls are equally susceptible and more than one child in a family can
have it.
Stargardt’s disease begins with slightly blurry vision that
gradually gets worse. By the late 20s, corrected 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 will
not drive, or read without using magnification devices.
A buildup 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, eyeglasses and magnification help affected
people adapt to the disease.
(c) 2007 The American Academy of Ophthalmology