Stargardt Disease

Color fundus photography image from a Stargardt disease patient showing a central macular scar with some pigmentary changes and surrounding perimacular flecks.What is Stargardt disease?

Stargardt disease is a disorder of the macula of the retina, the part of the eye that senses light and sends signals to the brain to process the images you see. People with Stargardt disease do not become completely blind, but they may experience slow vision loss that progresses to 20/200 or worse—most often during childhood or adolescence. Stargardt disease is also called Stargardt macular dystrophy, juvenile macular degeneration, or fundus flavimaculatus.Fundus autofluorescence image of a patient with Stargardt disease.

What causes Stargardt disease?

Stargardt disease is an inherited disorder, most often due to a mutation in a gene called ABCA4. Rods and cones are the cells in the retina that sense light. Rods help us see in dim and dark lighting, while cones help us see detail and color. In Stargardt disease, both rods and cones die away, especially the cones.

What are the symptoms of Stargardt disease?

If you have Stargardt disease, you may notice:

  • Blurred or distorted vision
  • Seeing dark areas in your central vision
  • Trouble seeing colors
  • Difficulty adjusting from bright to dark areas

How is Stargardt disease diagnosed?

An eye care professional can determine if you have Stargardt disease by examining your retina, which may show yellowish flecks on the macula called lipofuscin deposits. You may have tests to assess your visual field and ability to perceive color, measure the response of your retina rods and cones to light, and see detailed pictures deep within the retina.

Can Stargardt disease be treated?

Research is under way to evaluate new approaches such as gene therapy and stem cell therapies for people with Stargardt disease, but there is currently no cure. There are things you can do, however, to learn how to live with Stargardt disease, such as:

  • Wearing dark glasses and hats when out in bright light, which reduces the buildup of lipofuscin deposits in your eyes
  • Avoiding cigarette smoke
  • Not taking vitamin A supplements, which increase lipofuscin buildup
  • Learning how to use low vision aids, such as handheld lenses, electronic reading machines, or closed circuit video magnification systems
  • Seeking counseling and occupational therapy to help you cope with the effects of Stargardt disease on your ability to work, socialize, drive, and perform other daily activities