Degeneration or loss of function of an organ or organ system without any apparent reason; e.g., retinal abiotrophy (tapetoretinalnoy degeneration, retinal abiotrophy) - progressive retinal degeneration leading to vision loss that occurs due to various genetic disorders, e.g., due to retinitis pigmentosa (inflammation retina).
Causes of abiotrophy
Two kinds of light-sensitive structures are retina - rods and cones, which are so called because of their appearance. The first type of light waves receptors located throughout the retina, but the longer sticks to the peripheral areas of the eyes, and their amount decreases towards the center. The function of these components - to provide black-and-white peripheral vision at relatively low light.
- The cones are located in the central regions of the retina, and the task - providing high-contrast color vision. If some of the damaged gene structures that are responsible for normal operation and the blood supply and nutrients retina, the destruction of its outer layers begins, where and are the rods and cones. Destruction of the shells begins with the periphery and goes to the center of the eye for decades.
- Usually defeat covers both eyes at once, and the first symptoms of the disease can be detected as early as childhood. Such patients 21 years lose their ability to see normally. With the development of the disease may be different options, for example, struck by one eye or the retina one segmental area. If the disease has appeared in his mature years, then the person can develop glaucoma, cataract, swelling of the central retina or cataract.
- Under certain conditions, the affected cells can regenerate a tumor that is cancerous in nature. This tumor is called the retina eye melanoma. The tumor grows rapidly, which can lead to the need for emergency surgery, or people at risk of losing an eye. Before surgery for retinal melanoma was the only treatment. Therefore, most people remained without an eye: it was removed completely to prevent neoplastic go to the other eye.
At the present stage of development of medicine to fight the spread of a tumor Radiosurgical (Irradiation using radioactive cobalt and other elements) that allows you to save patient bodies view. But such methods, the elimination of melanoma has to be very careful not to damage healthy cells and tissues in the retina. Therefore, special importance is the timely diagnosis. In the early stages of this disease rid easier than neglected diseases.
Classification
Depending on the localization of the pathological process in the retina, there are the following forms abiotrophy:
| peripheral dystrophy | These include a X-chromosome Juvenile Retinoschisis, Goldmann-Favre disease and polymyositis. |
| generalized dystrophy | By this form include retinitis pigmentosa, atypical forms of retinitis pigmentosa, dystrophy, which is associated with the system and metabolic diseases, congenital amaurosis Leber congenital stationary night blindness, as well as dysfunction of the glomerular system. |
| Central dystrophy | This includes the Stargardt's disease, macular degeneration and Best zheltopyatnistoe fundus. |
Depending on the type of inheritance of the retina abiotrophy following types:
- Early autosomal recessive form. This form of the disease progresses rapidly. Often, early autosomal recessive form of complications arise in the form of macular degeneration or cataracts. By early autosomal recessive forms are congenital amaurosis Leber, which develops during the first five years of life. With this disease has been a sharp decline in visual functions.
- Late autosomal recessive form. This type of retinal pigment abiotrophy eye begins at the age of 30 years. In this case, the function of the retina is reduced considerably, but the disease progresses slowly.
- Sex-linked form of retinal abiotrophy. This form of disease transmitted by X-chromosome. This disease is quite hard and is progressing rapidly.
- Autosomal dominant form. This kind of retinal disease progresses slowly. Sometimes the disease is complicated by cataracts and macular degeneration, but the frequency of such complications occur less frequently than in the autosomal recessive form of retinal pigment abiotrophy.
Symptoms of retinal abiotrophy
In such diseases as abiotrophy retinal symptoms rather are variable due to the large number of different mutations that lead to the development of this pathology. But at the same time, among the various embodiments dystrophy within the same group (peripheral, central or generalized abiotrophy) has a number of similar manifestations.
- Abiotrophy central retinal cone cells are characterized by a primary lesion, whose concentration is highest in the macula - that's why they are also called macular degeneration. The foreground sharp decline in visual acuity, impaired perception of colors, with the total destruction of the photoreceptors in the center of the fundus develops central scotoma. If the pathological process does not extend to the peripheral region of the retina, the peripheral and night vision is affected slightly. When abiotrophy forms, characterized by focal lesions photoreceptors develop blind spots in the visual field. In especially severe forms may occur fiber optic atrophy and blindness.
- The peripheral retinal abiotrophy (pigmentary degeneration, belotochechnaya abiotrophy) begin with a primary lesion rods, so one of the first symptoms of the disease will be day-blindness. With the progression of the disease, with the further destruction of sticks, decreased night vision may develop into its complete loss - night-blindness. Disrupted peripheral vision, there is a concentric scotoma, after which the field of vision is narrowed so that it becomes "tubular". more severe disorders often do not develop in the retina belotochechnoy abiotrophy, daylight vision and color perception is unchanged. In some cases, a pigment degeneration in the pathological process involved and cones, which leads to a drop in day view, reduce its severity and sometimes complete blindness. Course of the disease can take decades, though there are fleeting and juvenile forms.
The full form of congenital stationary night blindness is characterized by a pronounced night-blindness with saving daylight vision and color perception. Thus there is no progression of the disease. Symptoms of incomplete forms VSNS a moderate day-blindness, decreased visual acuity, color perception disorders, difficulty adapting to low light.
Diagnostics
When the survey draws attention to peripheral vision and sharpness. Diagnosis is to examine the state of the fundus, to identify specific changes in the retina.
Most often, when viewed from the expert notes areas of dystrophic damage of receptor cells (also called bone cells), compression of the arteries of the retina, the color change of the optic nerve, it becomes more pale. If there is doubt in the diagnosis, you can spend additional electrophysiological examination, which will enable a more accurate assessment of the retinal eye features.
To check the "night blindness" syndrome assesses the condition of the patient, with the aid of special techniques. If the diagnosis "pigmentary retinal abiotrophy" treatment, given the hereditary nature of the disease, is carried out with a parallel survey lines relative to allow initial stage disease.
Treatment of retinal abiotrophy
Etiotrop specific treatment of any form of retinal abiotrophy at the moment does not exist. As maintenance treatment, retarding progression of the disease, drugs used vitamins A, E, riboflavin.
Vasodilators can improve the blood supply to the retina, which also slow down the degenerative processes. In recent years, there is evidence of the successful use of bionic retina implant (Argus, Argus 2), allowed to partially restore vision to patients completely lost it due abiotrophy. Some experience in the use of stem cells, gene therapy also aim to find a method of treatment abiotrophy retina.
Due to the large number of mutations that cause abiotrophy, and different clinical course of degenerative processes in the retina prediction almost always uncertain. Some types of pigment dystrophy may limit night blindness and impaired peripheral vision, while other forms of this disease leads to total blindness. By supplementation of vitamin A in some cases it is possible to slow the progression abiotrophy retina, according to some sources the use of sunglasses also can achieve the same result.
So abiotrophy retina - a dangerous pathology, which not only interferes with their lifestyle, but it can cause blindness. You need to be careful about your vision, keep an eye on him. In the case of uncomfortable sensations in the eyes, immediately contact an ophthalmologist, he will check you, order tests, and select effective treatment.
prevention
Adhering to preventive measures, you can not only slow down the development of many diseases of the retina of the eyeball, but also to prevent them.
Normally, the following steps experts recommend:
- annual examination by an ophthalmologist;
- holding the first laser correction becomes necessary;
- Avoid daily, long-term visual loads;
- create in the workplace and at home the correct lighting conditions;
- wearing sunglasses;
- gymnastic exercises for your eyes.
On the question of the retinal pigment abiotrophy what it is, only one answer can be given. This is one of those rare diseases, whose diagnosis in the early stages to avoid the complete absence of vision.
