Definition

What is Stargardt disease?

Stargardt disease is an inherited disorder of the retina – the tissue at the back of the eye that senses light. The disease typically causes vision loss during childhood or adolescence, although in some forms, vision loss may not be noticed until later in adulthood. It is rare for people with the disease to become completely blind. For most people, vision loss progresses slowly over time to 20/200 or worse. (Normal vision is 20/20).

Stargardt disease is also called Stargardt macular dystrophy, juvenile macular degeneration, or fundus flavimaculatus. The disease causes progressive damage—or degeneration—of the macula, which is a small area in the center of the retina that is responsible for sharp, straight-ahead vision. Stargardt disease is one of several genetic disorders that cause macular degeneration.

How common is Stargardt disease?

Experts estimate that 1 in 8-10 thousand people have Stargardt disease. Please discuss with your doctor for further information.

Symptoms

What are the symptoms of Stargardt disease?

The most common symptom of Stargardt disease is variable, often slow loss of central vision in both eyes. People with the disease might notice gray, black, or hazy spots in the center of their vision, or that it takes longer than usual for their eyes to adjust when moving from light to dark environments. Their eyes may be more sensitive to bright light. Some people also develop color blindness later in the disease.

The progression of symptoms in Stargardt disease is different for each person. People with an earlier onset of disease tend to have more rapid vision loss. Vision loss may decrease slowly at first, then worsen rapidly until it levels off. Most people with Stargardt disease will end up with 20/200 vision or worse. People with Stargardt disease may also begin to lose some of their peripheral (side) vision as they get older.

There may be some symptoms not listed above. If you have any concerns about a symptom, please consult your doctor.

When should I see my doctor?

If you have any signs or symptoms listed above or have any questions, please consult with your doctor. Everyone’s body acts differently. It is always best to discuss with your doctor what is best for your situation.

Causes

What causes Stargardt disease?

Stargardt’s is an inherited disease passed along to children when both parents carry mutations of a gene associated with vitamin A processing in the eye. Parents can carry recessive genetic traits responsible for Stargardt’s, even though they themselves may not have the disease.

Researchers have found that about 5 percent of people carry gene mutations that cause inherited retinal diseases such as Stargardt’s disease and retinitis pigmentosa. However, the inheritance pattern of Stargardt’s is variable, and it’s possible for up to half of the children of a single affected parent to develop the condition. Also, you can’t predict how much vision a child may lose to Stargardt’s disease based on the affected parent’s vision loss.

Risk factors

What increases my risk for Stargardt disease?

Please discuss with your doctor for further information.

Diagnosis & treatment

The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.

How is Stargardt disease diagnosed?

An eye care professional can make a positive diagnosis of Stargardt disease by examining the retina. Lipofuscin deposits can be seen as yellowish flecks in the macula. The flecks are irregular in shape and usually extend outward from the macula in a ring-like pattern. The number, size, color, and appearance of these flecks are widely variable.

A standard eye chart and other tests may be used to assess symptoms of vision loss in Stargardt disease, including:

  • Visual field testing. Visual fields testing attempts to measure distribution and sensitivity of field of vision. Multiple methods are available for testing; none is painful and most share a requirement for the patient to indicate ability to see a stimulus / target. This process results in a map of the person’s visual field, and can point to a loss of central vision or peripheral vision.
  • Color Testing: There are several tests that can be used to detect loss of color vision, which can occur late in Stargardt disease. Three tests are often used to get additional information: fundus photography combined with autofluorescence, electroretinography, and optical coherence tomography.
  • A fundus photo is a picture of the retina. These photos may reveal the presence of lipofuscin deposits. In fundus autofluorescence (FAF), a special filter is used to detect lipofuscin. Lipofuscin is naturally fluorescent (it glows in the dark) when a specific wavelength of light is shined into the eye. This test can detect lipofuscin that might not be visible with standard fundus photography, making it possible to diagnose Stargardt disease earlier.
  • Electroretinography (ERG) measures the electrical response of rods and cones to light. During the test, an electrode is placed on the cornea and light is flashed into the eye. The electrical responses are viewed and recorded on a monitor. Abnormal patterns of light response suggest the presence of Stargardt disease or other diseases that involve retinal degeneration.
  • Optical coherence tomography (OCT) is a scanning device that works a little like ultrasound. While ultrasound captures images by bouncing sound waves off of living tissues, OCT does it with light waves. The patient places his or her head on a chin rest while invisible, near-infrared light is focused on the retina. Because the eye is designed to allow light in, it’s possible to get detailed pictures deep within the retina. These pictures are then analyzed for any abnormalities in the thickness of the retinal layers, which could indicate retinal degeneration. OCT is sometimes combined with infrared scanning laser ophthalmoscope (ISLO) to provide additional surface images of the retina.

How is Stargardt disease treated?

Currently, there is no treatment for Stargardt disease. Some ophthalmologists encourage people with Stargardt disease to wear dark glasses and hats when out in bright light to reduce the buildup of lipofuscin. Cigarette smoking and second hand smoke should be avoided. Animal studies suggest that high-dose vitamin A may increase lipofuscin accumulation and potentially accelerate vision loss. Therefore, supplements containing more than the recommended daily allowance of vitamin A should be avoided, or taken only under a doctor’s supervision. There is no need to worry about getting too much vitamin A through food.

A number of services and devices can help people with Stargardt disease carry out daily activities and maintain their independence. Low-vision aids can be helpful for many daily tasks and range from simple hand-held lenses to electronic devices such as electronic reading machines or closed circuit video magnification systems. Because many people with Stargardt disease will become visually disabled by their 20s, the disease can have a significant emotional impact. Work, socializing, driving and other activities that may have come easily in the past are likely to become challenging. So counseling and occupational therapies often need to be part of the treatment plan.

Lifestyle changes & home remedies

What are some lifestyle changes or home remedies that can help me manage Stargardt disease?

The following lifestyles and home remedies might help you cope with Stargardt disease:

  • Wear eyeglasses or sunglasses that block 100 percent of UV light to reduce the possibility of additional eye damage caused by the sun.
  • Wear eyeglasses with specially tinted lenses to block certain wavelengths of light.

The American Macular Degeneration Foundation recommends that people with Stargardt’s or a history of the eye disease in close family members obtain genetic counseling before starting their own families.

Because vision loss often appears in young children with Stargardt’s, low vision counseling from your eye doctor is essential to make sure that classroom learning is not hampered. For example, a child with Stargardt’s may need to use large print books and special devices that magnify print.

If you have any questions, please consult with your doctor to better understand the best solution for you.

Hello Health Group does not provide medical advice, diagnosis or treatment.

Sources

Review Date: January 3, 2018 | Last Modified: January 3, 2018

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