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Alternative names Return to top
RPDefinition Return to top
Retinitis pigmentosa is a progressive degeneration of the retina (part of the eye) which affects night vision and peripheral vision.Causes, incidence, and risk factors Return to top
Retinitis pigmentosa commonly runs in families. The disorder can be caused by defects in a number of different genes which have recently been identified.
The cells controlling night vision, called rods, are most likely to be affected. However, in some cases, retinal cone cells are most damaged. The hallmark of the disease is the presence of dark pigmented spots in the retina.
As the disease progresses, peripheral vision is greadually lost. The condition may eventually lead to blindness, but usually not complete blindness. Signs and symptoms often first appear in childhood, but severe visual problems do not usually develop until early adulthood.
The main risk factor is a family history of retinitis pigmentosa. It is an uncommon condition affecting about 1 in 4,000 people in the U.S.
Symptoms Return to top
Signs and tests Return to top
Tests determine the integrity of the retina:
Treatment Return to top
There is no effective treatment for this condition. The use of sunglasses to protect the retina from ultraviolet light may have a vision-preserving effect.
Though controversial, recent studies have indicated that treatment with antioxidant agents (such as Vitamin A palmitate) may delay the progression of this disease.
Referral to a low vision specialist is very helpful in maintaining patient independence. Regular visits to your eye care specialist are important to monitor for the development of cataracts or retinal swelling -- both of which can be treated.
Expectations (prognosis) Return to top
The disorder will continue to progress, though at a very slow pace. Complete blindness is uncommon.
Complications Return to top
Peripheral and central loss of vision will eventually occur.
Many other syndromes with features similar to retinitis pigmentosa have been described, including:
Calling your health care provider Return to top
Call your health care provider if night vision becomes difficult or if other symptoms of this disorder develop.
Prevention Return to top
Genetic counseling may determine the risk of this disease occurring in a person's offspring.
Update Date: 4/22/2004 Updated by: Dominique Brooks, M.D., M.B.A, Ophthalmologist. Review provided by VeriMed Healthcare Network.
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Page last updated: 28 October 2004 |