Primary retinitis pigmentosa is a genetic retinal disease with progressive visual impairment as the main manifestation.
Typical manifestations: night blindness and progressive reduction of visual field; fundus manifestations: waxy disc, thinning of retinal vessels, and osteoblast-like pigment degeneration in the mid-periphery of the retina. The onset is mostly bilateral.
Night blindness: the earliest manifestation, often starting in childhood or adolescence, and mostly occurring before visible changes in the fundus. The symptoms are mild at the beginning and gradually worsen with age. This is because the lesion first starts in the mid-peripheral part of the retina, where there are many optic rods, which are responsible for dark vision.
Visual acuity and visual field changes: central vision is normal or near normal in the early stages, gradually decreases as the disease progresses, and finally becomes completely blind. In the early stage, there is a ring-shaped dark spot in the visual field, the location of which corresponds to the equatorial lesion, and then the ring-shaped dark spot slowly expands to the center and periphery to form a tubular visual field.
Color vision: Most patients have normal color vision in the early stages, which worsens with the progression of the disease. Typical changes: blue blindness, less red-green color vision impairment.
What tests are required.
Fundus examination.
1, the optic disc changes: early normal, late pale and slightly yellow, called “wax-like optic disc”.
2, retinal vascular lesions: retinal arteries and veins are narrowed, the arteries are significant, the arteries become thin lines in the late stage, usually not a white line.
3.Retinal pigmentation: Most of the pigmentation starts at the equatorial part, the pigmentation is in the form of small dots with protrusions, and then increases and becomes larger, osteoblast-like, sometimes in the form of irregular dots and lines, arranged in a ring of varying width around the equatorial part.
Fundus angiography (FFA): large non-fluorescent areas of background fluorescence suggesting atrophy of the choroidal capillary layer.
Dark adaptation test: early cone cell function is still normal, rod cell function decreases, resulting in an increase in the terminal threshold of the rod cell curve, resulting in a narrowing of the interphotoreceptor difference; late rod cell function is lost, and the cone cell threshold increases, resulting in a high monophasic curve.
Visual electrophysiology: Early in the lesion, a significant decrease in the light peak/dark valley of the electrooculogram can be detected or an extinguished change. As the disease progresses, the electroretinogram shows a low wave retardation pattern, especially the disappearance of b-wave is the typical manifestation of the disease.
What are the complications of primary retinitis pigmentosa: cataract, glaucoma, myopia, and hearing impairment?
How should primary retinitis pigmentosa be treated?
1, vasodilators and neurotrophic drugs: vitamin A, E, B12, lutein, anthocyanin, ginkgo biloba, etc;
2.Acupuncture therapy;
3, compound camptothecin injection therapy: accelerate the restoration of the normal level of vasoactive substances in the ischemic area of the eye, relieve vasospasm, improve the diastolic function of the choroidal vessels, increase blood flow, improve microcirculation, stop or slow down the progression of retinitis pigmentosa;
4.Direct current ion introduction treatment;
5.Other: periocular biofilm implantation, subscleral vascular grafting, superficial temporal artery bypass and other combined surgical methods. To speed up the blood flow, increase the blood flow and increase the blood circulation in the ophthalmic artery, central retinal artery and short posterior ciliary artery.
6.Retinal transplantation;
7.Artificial retinal transplantation.