What is Stargardt disease?

19 January 2023

Stargardt's disease is a rare genetic eye disease that occurs when fatty material builds up in the macula, the small part of the retina needed for sharp, detailed vision.

Vision loss usually begins in childhood, but some people with this condition do not begin to lose vision until adulthood. The disease has no treatment, but vision rehabilitation can help people make the most of their remaining vision.

The most common symptom of Stargardt's disease is a slow loss of central vision in both eyes. Some people suffer this loss more quickly than others.

Other symptoms may include:

• Gray, black or blurred spots in the center of vision.
• Sensitivity to light.
• Needing more time for your eyes to adjust between light and dark places.

Stargardt's disease is usually caused by alterations in a gene called ABCA4, which affects the way the body uses vitamin A. Vitamin A serves, among other functions, to help your eyes work properly. It serves, among other functions, to produce cells in the retina (the light-sensitive layer of tissue at the back of the eye); the ABCA4 gene then makes a protein that cleans out the excess fatty material. In people with Stargardt's disease this gene does not work, so the fatty material accumulates in yellowish clumps in the macula. Over time, this fatty material kills the light-sensitive cells and destroys central vision.

Stargardt disease is an inherited genetic disease, which means it is passed down through families.