Problem

Juvenile macular degeneration

Other Names:
Best disease
Vitelliform macular degeneration
Stargardt's disease
Fundus flavimaculatus
Behr's dystrophy
Doyne's dystrophy
Honeycomb dystrophy
Nature:

There are rare forms of macular degeneration which afflict young people. Collectively these conditions are called juvenile macular degeneration. Each is caused by a mutation in a gene. The disease is the result of the death of the photoreceptors in the retina of the eye causing reduced vision. Though age-related macular degeneration can affect one or both eyes, Stargardt’s Disease often affects both eyes. With this comes partial vision loss: those with Stargardt’s Disease don’t lose their eyesight completely because it does not affect the periphery (side vision). Progressive loss of central vision in both eyes makes it difficult for children and teens for focus. Reading becomes more difficult and so does being in school as almost all learning is done visually. Despite this, many children with juvenile macular degeneration function very well, performing routine school tasks with only a minimum of help. Sports and other outdoor activities may also prove challenging for a child or teen suffering from the disease. Poor coordination may be less due to being bad at sports and have more to do with not being able to use their central vision.  Another symptom is seeing flecks or spots in their vision.  Light sensitivity is also a related symptom.  Often eyes with damaged or dead photoreceptors will have trouble adjusting to bright lights. Moving from dark setting into light ones and vice versa may take the eyes a while to adjust to. In the later stages of the disease, some people have developed color blindness as the photoreceptors are in charge of capturing color.

Glasses and low vision aids have been helpful in improving vision for patients with juvenile macular degeneration. Laser surgery is usually not effective in treating this condition.

Incidence:

Stargardt’s Disease is named after Karl Stargardt, a German ophthalmologist who was the first known person to have the disease in 1901. It affects young children and teens who have inherited certain genes from their parents that allow the disease to develop.  It is estimated that one in 10 thousand people have the disease.  Although vision loss is generally slow in Stargardt’s Disease patients, sometimes when the disease sets in early in life, vision loss can be quick. Vision loss will begin slow, then take a steep dive until it eventually levels off at 20/200 vision or worse.


 

Problem Type:
G: Very specific problems
Date of last update
08.11.2017 – 17:23 CET