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Retinitis Pigmentosa

By Gretchyn Bailey; reviewed by Vance Thompson, MD

Retinitis pigmentosa (RP) is a rare, inherited disease in which the light-sensitive retina of the eye slowly and progressively degenerates. Eventually, blindness results.

Retinitis Pigmentosa Symptoms and Signs

The first signs of retinitis pigmentosa usually occur in early childhood, when both eyes typically are affected. Night vision can be poor, and the field of vision may begin to narrow.

During later stages of retinitis pigmentosa, only a small area of central vision remains, along with slight peripheral vision.

What Causes RP?

Not much is known about what causes retinitis pigmentosa, except that the disease is inherited. Even if your mother and father don't have retinitis pigmentosa, you can still have the eye disease when at least one parent carries an altered gene associated with the trait.

A shrinking field of vision is one of the early warning signs of retinitis pigmentosa.

In fact, about 1 percent of the population can be considered carriers of recessive genetic tendencies for retinitis pigmentosa that, in certain circumstances, can be passed on to a child who then develops the disease (Ophthalmology 2004).

Rather than being considered a single disease, retinitis pigmentosa instead is viewed as a group of diseases affecting how light-sensitive cells in the back of the eye (retina) function.

Rods — the light-sensing retinal cells that are responsible for vision in dim light — gradually deteriorate until seeing at night becomes more difficult.

Retinitis Pigmentosa Tests and Treatment

Visual field testing likely will be done to determine the extent of peripheral vision loss. Other eye exams may be conducted to determine whether you have lost night or color vision.

 
Left: Retinitis pigmentosa causes deterioration of light-sensitive cells in the back of the eye. Right: No cure is known for people blinded by retinitis pigmentosa. But studies have focused on artificial retinas that may restore sight at least partially. The Argus II Retinal Prosthesis System shown here received FDA clearance in 2007 to begin clinical trials. (Image: Second Sight Medical Products)

No treatments are available currently for retinitis pigmentosa, although some practitioners believe that vitamin A may slightly delay vision loss.

Occupational therapy may be helpful, because it's easier to adjust to declining vision in earlier stages of vision loss.

People with retinitis pigmentosa also might consider using low vision devices that can help magnify and illuminate objects in home and work spaces.

Researchers are looking into ways to treat RP in the future, such as retinal implants and drug treatments.

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[Page updated May 2007]