Retinitis pigmentosa is an eye disease in which there is damage to the
Causes, incidence, and risk factors
Retinitis pigmentosa can run in families. The disorder can be caused by a number of genetic defects.
The cells controlling night vision (rods) are most likely to be affected. However, in some cases, retinal cone cells are damaged the most. The main sign of the disease is the presence of dark deposits in the retina.
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 United States.
Symptoms often first appear in childhood, but severe vision problems do not usually develop until early adulthood.
- Decreased vision at night or in low light
- Loss of side (peripheral) vision, causing “tunnel vision”
- Loss of
centralvision (in advanced cases)
Signs and tests
Tests to evaluate the retina:
Examination of the retina by
ophthalmoscopyafter the pupils have been dilated
Measurement of the electrical activity in the retina (
Pupil reflex response
Side vision test (visual field test)
Slit lamp examination Visual acuity
There is no effective treatment for this condition. Wearing sunglasses to protect the retina from ultraviolet light may help preserve vision.
Some studies have suggested that treatment with antioxidants (such as high doses of vitamin A palmitate) may slow the disease. However, taking high doses of vitamin A can cause serious liver problems. The benefit of treatment has to be weighed against risks to the liver.
Several clinical trials are in progress to investigate new treatments for retinitis pigmentosa, including the omega-3 fatty acid, DHA.
Microchip implants that go inside the retina and act like a microscopic video camera are in the early stages of development for treating blindness associated with this and other serious eye conditions.
It can help to see a low-vision specialist, who can help you adapt to vision loss. Make regular visits to an eye care specialist, who can detect
The disorder will continue to progress, although slowly. Complete blindness is uncommon.
Peripheral and central
Patients with retinitis pigmentosa often develop cataracts at an early age, or swelling of the retina (macular edema). Cataracts can be removed if they contribute to vision loss.
Many other conditions have similarities to retinitis pigmentosa, including:
- Laurence-Moon syndrome (also called Laurence-Moon-Bardet-Biedl syndrome)
Mucopolysaccharidosis M yotonic dystrophy
- Usher syndrome (a combination of retinitis pigmentosa and hearing loss)
Calling your health care provider
Call your health care provider if night vision becomes difficult or if you develop other symptoms of this disorder.
Genetic counseling and testing may help determine whether your children are at risk for this disease.