Retinitis Pigmentosa

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Medical Author:

Frank J. Weinstock, MD, FACS

Frank J. Weinstock, MD, FACS

Dr. Weinstock is a board-certified ophthalmologist. He practices general ophthalmology in Canton, Ohio, with a special interest in contact lenses. He holds faculty positions of Professor of Ophthalmology at the Northeastern Ohio Colleges of Medicine and Affiliate Clinical Professor in the Charles E. Schmidt College of Biomedical Science at Florida Atlantic University.

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Medical Editor:

Andrew A. Dahl, MD, FACS

Andrew A. Dahl, MD, FACS

Andrew A. Dahl, MD, is a board-certified ophthalmologist. Dr. Dahl's educational background includes a BA with Honors and Distinction from Wesleyan University, Middletown, CT, and an MD from Cornell University, where he was selected for Alpha Omega Alpha, the national medical honor society. He had an internal medical internship at the New York Hospital/Cornell Medical Center.

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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.

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. A thorough genetic pedigree, often with the aid of a genetic counselor, is essential in determining risk of future generations acquiring the disease.

Retinitis pigmentosa is usually diagnosed during the teenage years but may be present at birth. The latter congenital type is usually fairly stable and nonprogressive. Cases that are diagnosed later in life are often milder and may progress more slowly.

There are approximately 75,000 people in the United States with retinitis pigmentosa (RP). RP is sometimes associated with other systemic illnesses. Usher syndrome, characterized by retinitis pigmentosa and neural hearing loss, is the most common cause of deaf-blindness in the United States. The hearing loss usually is diagnosed earlier than the eye changes.