Stargardt Disease

Stargardt Disease (fundus flavimaculatus, Stargardt macular dystrophy) is a common type of macular degeneration affecting young people. It arises from an inherited recessive gene and manifests as a very severe type of macular degeneration beginning in late childhood and resulting in becoming legally blind.

Stargardt Disease

Stargardt Disease (fundus flavimaculatus, Stargardt macular dystrophy) is a common type of macular degeneration affecting young people. It arises from an inherited recessive gene and manifests as a very severe type of macular degeneration beginning in late childhood and resulting in becoming legally blind.

“With further progression, oily deposits begin to accumulate in the layer of tissue comprising the retinal pigment layer of cells (RPE) which is the layer behind the macula. ”

Symptoms & Progression

The symptoms of Stargardt are similar to those of age-related macular degeneration (ARMD). It is diagnosed in 1 in 20,000 children over 6, usually identified before age 20. The first symptom is decreased vision.

  1. Poor vision in dim light.
  2. Difficulty in close vision, such as reading, although general vision may seem fine.
  3. Loss of vision sharpness, poor color vision and small blind spots.
  4. Blurriness and distorted vision
  5. Difficulty adapting to the dark after sunlight exposure, and/or light sensitivity.
  6. In more severe stages of vision loss, patients may experience Phantom vision or visual hallucinations. These episodes are not usually related to underlying psychiatric problems, but rather are normal attempt by the brain to make sense of impaired sensory information. The brain may embellish the image making it very real just as it does in our dreams.

Early Stage

Stargardt disease results in progressive central vision loss, first reported as difficulty in reading and seeing in low light situations, although vision is generally good. These problems are generally noticed between ages 6 to 12, though the symptoms may also arise as an adult.

Disease Progression

With further progression, oily deposits begin to accumulate in the layer of tissue comprising the retinal pigment layer of cells (RPE) which is the layer behind the macula. The RPE is a layer lying between the retina and the choroid, providing nourishment to photoreceptor cells. These deposits, called "lipofuscin", look like yellowish-tinted specks.