Further Outpatient Care
Annual patient examinations usually are sufficient to measure Goldmann visual field and visual acuity.
If medical treatment is initiated, more frequent visits and laboratory blood work may be indicated.
Patients with systemic conditions that are associated with retinitis pigmentosa (RP) may require closer follow-up care.
Patient Education
To help them make wise decisions about driving or vocational rehabilitation, educate patients about their field defects.
Family pedigrees and, when available, genetic subtyping can be helpful in genetic counseling. Patients should understand that the visual degeneration, which usually occurs over 30-40 years, slowly progresses and varies with the type of RP.
Patients with Usher syndrome should understand the course of their hearing loss, as each of the 3 types of Usher syndrome has a different prognosis regarding hearing.
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Usher syndrome with typical retinitis pigmentosa appearance.
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Choroideremia.
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Bull's eye maculopathy seen in cone dystrophy.
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Polydactyly seen in Bardet-Biedl syndrome (associated with retinitis pigmentosa).
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Cone dystrophy.
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Gross pathology of an eye in a man with retinitis pigmentosa.
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Leber congenital amaurosis.
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Female carrier of choroideremia.
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Representative electroretinograms of patients with healthy eyes, rod-cone dystrophy, and congenital stationary night blindness. Courtesy of Dr. Nusinowitz, Jules Stein Eye Institute.
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Representative electroretinograms of patients with healthy eyes and X-linked retinoschisis. Courtesy of Dr. Nusinowitz, Jules Stein Eye Institute.
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Retinitis pigmentosa pigmentation pattern demonstrated with ultrawide fundus imaging using the scanning laser ophthalmoscope (Optomap; Optos PLC, Dunfermline, Scotland, United Kingdom).
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Fellow eye of same patient as in the image above, again demonstrating a typical retinitis pigmentosa pigmentation pattern demonstrated with ultrawide fundus imaging using the scanning laser ophthalmoscope (Optomap; Optos PLC, Dunfermline, Scotland, United Kingdom).
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Higher resolution image of typical bone spicule formation.
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Cone dystrophy demonstrating typical central macular atrophy found in this condition.
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Retinitis pigmentosa, rubella, a history of retinal detachment, and syphilis all may result in a hyperpigmented retinal pigment epithelium (RPE) with bone spicule appearance, restricted visual field and/or poor vision, and atrophic vessels.
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Retinitis pigmentosa progresses over decades. Associated cataract also is relevant, as seen in this image.
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Pigmentary changes are not always seen in retinitis pigmentosa but frequently are observed, as in this patient with Alström disease.
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Genetic screening may be helpful in identifying patients who are at risk, in counseling, and in directing treatment as new knowledge is acquired. Some varieties of retinitis pigmentosa may have increased vulnerability to environmental hazards; for example, one might avoid light exposure in some rhodopsin mutations or sildenafil in phosphodiesterase mutations. Patients with retinitis pigmentosa may have other findings. This patient with Alström disease shows acanthosis.
