Stargardt’s Disease – Juvenile Macular Degeneration

Stargardt’s disease (fundus flavimaculatus) is a form of macular degeneration that can affect both children and young adults. While macular degeneration is considered a disease that primarily affects the aging population, stargardt’s disease is an inherited disease that causes the light-sensitive cells in the retina to deteriorate. AS with macular degeneration, stargardt’s disease affects the macula, the portion of the eye that controls central vision.

Stargardt’s disease is thought to be inherited when both parents carry genetic mutations that causes the eye disease. Both parents can have the gene but not have the eye disease themselves. Stargardt’s disease is considered the most common form of juvenile macular degeneration.

Stargardt’s disease was named when Dr. Stargardt described this eye disease in 1909. Approximately 1 in 20,000 children over the age may be diagnosed with this eye disease and it is usually diagnosed before the age of 20. This disease affects both boys and girls equally.

With this form of macular degeneration, the macula and the surrounding retina are affected. Early in Stargardt’s disease, the macula may appear normal and over time change to allow diagnosis using a fluorescein angiography. With this test, a dye is injected into the arm and as the flow of this dye enters the eye it is observed. During the test, patients with stargardt’s disease has the flow of light blocked causing a “dark choroid” which is used to aid the diagnosis.

As the eye disease progresses, the retina appears to be “beaten metal” with small yellowish-white flecks (fundus flavimaculatus) in the peripheral retina. These may resemble drusen deposits that are noticeable in other forms of macular degeneration.

Children diagnosed with Stargardt’s Disease may experience a mild loss of visual acuity and a loss of sharpness of vision. Vision may appear somewhat normal in the early stages. As the eye disease progresses, there may be blind spots developing as the retina becomes more and more damaged. Images may appear and disappear.

As with other forms of macular degeneration, Stargardt’s Disease causes the loss of central vision. This is referred to as a central scotoma or blindspot in central vision. Patients may also develop abnormal color vision, photophobia (an abnormal intolerance of light) and night blindness.

There is currently no treatment for Stargardt’s disease.