Retinitis pigmentosa is the most common of a group of hereditary progressive retinal degenerations or dystrophies. There is considerable variation and overlap among the various forms of retinitis pigmentosa. Common to all of them is progressive degeneration of the retina, specifically of the light receptors, known as the rods and cones. The rods of the retina are involved earlier in the course of the disease, and cone deterioration occurs later. In this progressive degeneration of the retina, the peripheral vision slowly constricts and central vision is usually retained until late in the disease.

Etiology

• Retinitis pigmentosa is an inherited condition which involves both eyes. If it starts in one eye, the other eye usually develops the same condition in a number of years. Most cases are familial, inherited in a variety of ways, including dominant, recessive, and sex-linked recessive. Some cases are sporadic and lack a family history of the disease.

Ophthalmic features

• Since retinitis pigmentosa begins as rod degeneration, the patient first notices increasing difficulty in night vision, followed by difficulty seeing in the periphery. Slowly progressive constriction of the visual field leads to tunnel vision. A small area of central vision in both eyes usually persists for years. Generally night blindness precedes tunnel vision by years or even decades. Total blindness eventually ensues in most cases. The age of appearance of legal blindness ranges from as early as childhood to as late as the 40s.

Diagnosis

• The history (especially the possibility of retinitis pigmentosa appearing in other family members) and complaints of the patient may make one suspect RP, it is primarily diagnosed by examination. The patient may complain of difficulty seeing at night or in low light condition. At some point, the ophthalmologist observes relatively characteristic bone spicule pigment during the fundus examination
• Visual fields -will find defects in the peripheral (side vision) with the degree of loss related to defects in relation to the damage occurring in this disease. Over time, the visual field may reduce to a small central island of vision causing "tunnel vision." The final progression may be the complete loss of the remaining central vision.
• Electoretinogram -Responses to flashes of light are measured via electrodes placed on the surface of the eye. It is a painless test. The electroretinogram(ERG), in conjunction with the visual field exam, will usually make the diagnosis. This will also determine if there is any cone involvement.
• Recently, gene testing for defects is being done to clarify the basic cause for RP and assist in ultimately finding a treatment

Treatment

• As of now there is no specific cure for retinitis pigmentosa. For years, vitamin A therapy has been recommended for many RP patients, based on research dating back to the early 1990s. A randomized, controlled trial of vitamins A and E found that 15,000 IU a day of vitamin A palmitate could slow the course of the condition among adults with typical forms of RP. Vitamin E, however, at a 400 IU a day dose appeared to have an adverse effect on the course of RP in the same study.
• Another study among adult patients with RP has shown that an omega-3-rich diet containing docosahexaenoic acid can further slow disease progression. Such a diet includes one to two 3-ounce servings per week of oily fish such as salmon, tuna, herring, mackerel, or sardine