Retinitis pigmentosa (RP) is a clinically and genetically heterogeneous group of disorders characterized by progressive loss of vision and night-blindness. Etiologically, the disorder can be classified as a cone-rod dystrophy caused by apoptotic changes in the photoreceptor cells of the eye. Clinically, affected patients begin to show the symptoms of nyctalopia and vision loss in early adulthood. The latter usually begins early on as a peripheral vision loss, resulting in tunnel vision. Over time, however, the condition may progress to include central vision loss as well. Photopsia, or the visual perception of flashes of light, is also seen in many patients. Although RP is usually found in isolation, it can also form a part of several syndromic conditions, such as Usher syndrome, Kearn Sayre syndrome, and abetalipoproteinemia.
RP is a fairly common disorder, affecting approximately 1 in 4,000 individuals. Although the disease is not curable, techniques are available to ameliorate the condition. Night vision difficulties can be eased with the help of ocular devices. Clinical trials are underway to test new treatment strategies, including the use of vitamin A and omega-3 fatty acids in the treatment of RP. Doctors recommend annual eye examinations and evaluations for patients since there may be a tendency for affected patients to develop cataracts and/or retinal swelling.
