Retinitis Pigmentosa

Overview

Retinitis pigmentosa (RP) is a group of inherited eye diseases characterized by progressive loss of night vision and peripheral vision. 1 in 4000 Americans develop retinitis pigmentosa.

In retinitis pigmentosa, photoreceptor cells begin to deteriorate, starting with those photoreceptors that are responsible for night vision (known as rods).Individuals with retinitis pigmentosa possess a gene mutation (or absence of a gene) that leads to deterioration of photoreceptors. There are many different ways in which retinitis pigmentosa can occur, but in all cases, there is a progressive and relentless loss of photoreceptors, which leads to legal blindness and tunnel vision.

Retinitis Pigmentosa Symptoms & Signs

Figure: Retinitis Pigmentosa. Courtesy of EyeAtlas.com.

The first symptoms of retinitis pigmentosa tend to occur during childhood, but severe vision loss generally does not take hold until early adulthood.

Reduction in night vision or vision in dim light is a classic symptom of retinitis pigmentosa. Progressive loss of peripheral vision follows. In severe cases of retinitis pigmentosa, central vision eventually becomes reduced as well. Upon examination of the inside of the eye, retinitis pigmentosa has an extremely characteristic appearance: the Figure shows the hallmark pigmented spots in the retina of a person with retinitis pigmentosa.

Detection & Diagnosis of Retinitis Pigmentosa

An eye doctor has a number of different tests at his/her disposal to examine the eyes of a patient with retinitis pigmentosa. This helps to evaluate the progression of the eye disease.

Retinitis Pigmentosa Treatment

Currently there is no effective treatment for retinitis pigmentosa. Patients are instead provided with special sunglasses to block UV radiation and to help them cope with their vision loss. Vitamin A supplements may have a vision-preserving effect, but this is still controversial.

The unfortunate reality of retinitis pigmentosa is that it is a progressive eye disease that will eventually lead to legal blindness and tunnel vision. Complete blindness, however, is uncommon.

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