Treatment of retinal dystrophy. Treatment of different types of retinal degeneration Retinal pigmentary dystrophy

Pigmentary dystrophy mesh shell is enough rare disease. It occurs in 1 person out of 5000, and in total there are approximately 100 million carriers of the defective gene that causes retinitis pigmentosa in the world. With the development of this pathology, the eye, initially a light-absorbing structure, turns into a light-reflecting structure, which inevitably becomes the cause of blindness. Men are more susceptible to the development of retinitis pigmentosa, and the disease can occur both in childhood and in mature age. According to statistics, the majority of patients with retinal pigmentary dystrophy under the age of 20 retain visual acuity above 0.1 and the ability to read, while in 45–50-year-old patients, visual acuity decreases to a value below 0.1, and they can no longer read.

Retinitis pigmentosa or, in other words, retinal pigmentary dystrophy is characterized by degeneration of photoreceptors and pigment epithelium, as a result of which the ability to transmit signals from the retina to the cerebral cortex is lost. With this pathology, a characteristic accumulation of pigment substance is also clearly observed in the fundus picture. The degenerative process with pigment deposition causes the disappearance of photoperceiving cells and receptors, causing the retina to lose sensitivity to light signals. As a result, the person loses his vision.

Pigmentary dystrophy of the retina was first described in 1857 by D. Donders, and after 4 years the hereditary nature of this pathology was established. The type of heredity affects important indicators diseases: age of onset, rate of progression and prognosis of visual functions. The following types of inheritance are distinguished:

• Sex-linked: transmission of a defective gene from mother to son with the X chromosome is the least common, but the prognosis is the most unfavorable;
• Autosomal recessive: defective genes are passed on from both parents;
• Autosomal dominant: transmission of disease genes from one of the parents.

Signs

Retinitis pigmentosa often occurs in childhood, it is characterized by three symptoms:

• Small accumulations of pigment in the fundus;
• Disk optic nerve has a waxy pallor;
• Constriction of arterioles.

In the future, the patient's visual acuity may deteriorate, while pigment changes are observed in the macular area, and macular edema may occur. In patients with retinitis pigmentosa, there is increased risk development of myopia, posterior subcapsular cataract and open-angle glaucoma.

In childhood, retinitis pigmentosa is quite difficult to diagnose. The first noticeable symptom that appears in a child is early stage pathological process is poor orientation in the dark - often the disease is detected precisely by this manifestation. This disorder is also characterized by the inability to distinguish colors in the dark - this is called night blindness. Besides, initial stages Pigmentary dystrophy, especially in children, manifests itself with headaches and flashes of light in the eyes.

As the disease progresses, a continuous process of degeneration of the retina occurs, eventually leading to impairment of peripheral vision. According to ophthalmologists, vision becomes tunnel vision - this occurs in the second stage of the disease.

On last stage Due to the disease, a person loses not only peripheral, but also central vision. Blindness sets in.

Treatment of retinitis pigmentosa

There are currently no effective methods for treating retinal pigmentary dystrophy. Although it should be noted that constant and unsuccessful research is being conducted in this direction. More recently, thanks to new advances in electronics and cybernetics, huge strides have been made in this area. The essence of the new technique is to install a small photodiode sensor with a transmitter on the retina of the eye. The received light information from the outside world comes to the transmitter through an ultra-thin wire. The perception of information occurs through special glasses with receiving glasses. Of course, this is not full-fledged vision, but, nevertheless, a person becomes able to see. Experiments have shown that a blind patient gains the ability to distinguish between light and dark, as well as to see the outlines of large objects. There is no doubt that this development can make the lives of blind people much easier. This experimental technique is currently undergoing testing.

To treat retinitis pigmentosa, a method of slowing down the degenerative process of the retina is also used. It includes the use of stimulating procedures, for example, magnetic stimulation, as well as taking medications with blueberry extract and vitamin A, and parabulbar injections of the drug "Retinalamine".

Video from our specialist about the disease

Eye dystrophy includes many degenerative pathologies affecting the cornea - the transparent part outer shell, the retina - the inner membrane with photoreceptor cells, as well as the vascular system of the eyes.

The retina is considered the most important part of the eyes, since it is an element of the visual analyzer that perceives light impulses. Although is it really possible to imagine normal vision without a healthy cornea - the light-refracting lens of the eye, which provides at least two-thirds of its optical power. As for the blood vessels of the eyes, their important role is evidenced by the fact that vascular ischemia can provoke a significant deterioration in vision.

ICD-10 code

H31.2 Hereditary dystrophy choroid eyes

Causes of eye dystrophy

Now, in the same order, we will consider the causes of eye dystrophy.

As is known, there are no blood vessels in the cornea itself, and the metabolism in its cells is ensured by the vascular system of the limbus (the growth zone between the cornea and the sclera) and fluids - intraocular and lacrimal. Therefore, for a long time it was believed that the causes of corneal dystrophy are structural changes and decreased transparency - are associated exclusively with disorders of local metabolism and, partly, innervation.

The genetically determined nature of most cases of corneal degenerations, which are transmitted according to an autosomal dominant principle and appear at different ages, is now recognized.

For example, the result of mutations in the KRT12 gene or the KRT3 gene, which ensure the synthesis of keratins in the corneal epithelium, is Messmann's corneal dystrophy. The reason for the development of spotted corneal dystrophy lies in mutations of the CHST6 gene, which disrupts the synthesis of polymeric sulfated glycosaminoglycans that make up the corneal tissue. And the etiology of basement membrane dystrophy and Bowman’s membrane type 1 (Reis-Bücklers corneal dystrophy), granular and lattice dystrophies is associated with problems with the TGFBI gene, which is responsible for the growth factor of corneal tissue.

Ophthalmologists attribute the main causes of the disease, firstly, to biochemical processes in the membranes of its cells associated with an age-related increase in lipid peroxidation. Secondly, a deficiency of lysosome hydrolytic enzymes, which promotes the accumulation of granular pigment lipofuscin in the pigment epithelium, which disables light-sensitive cells.

It is especially noted that diseases such as atherosclerosis and arterial hypertension– because of their ability to destabilize the entire vascular system– increase the risk of central retinal dystrophy by three and seven times, respectively. According to ophthalmologists, an important role in the development of degenerative retinopathy is played by myopia (myopia), in which stretching occurs eyeball, inflammation of the choroid, increased level cholesterol. The British Journal of Ophthalmology reported in 2006 that smoking triples the risk of developing age-related retinal degeneration.

After basic research Over the past two decades, the genetic etiology of many dystrophic changes in the retina has become clear. Autosomal dominant gene mutations provoke overexpression of the transmembrane G protein rhodopsin, a key visual pigment rod photoreceptors (rods). It is mutations in the gene of this chromoprotein that explain defects in the phototransduction cascade in retinal pigmentary dystrophy.

The causes of the disease may be associated with impaired local blood circulation and intracellular metabolism during arterial hypertension, atherosclerosis, chlamydial or toxoplasmic uveitis, autoimmune diseases (rheumatoid arthritis, systemic lupus erythematosus), diabetes mellitus both types or eye injuries. There is also an assumption that problems with blood vessels in the eyes are a consequence of vascular damage to the brain.

Symptoms of eye dystrophy

Symptoms of corneal dystrophy noted by ophthalmologists include:

• painful sensations of varying intensity in the eyes;
• feeling of a clogged eye (presence of a foreign object);
• painful eye sensitivity to light (photophobia or photophobia);
• excessive tearing;
• hyperemia of the sclera;
• swelling of the cornea;
• decreased transparency of the stratum corneum and decreased visual acuity.

Keratoconus also causes a sensation of itching in the eyes and visualization of multiple images of single objects (monocular polyopia).

It should be borne in mind that retinal dystrophy develops gradually and does not show any signs at the initial stages. And symptoms characteristic of degenerative pathologies of the retina can be expressed as:

• rapid eye fatigue;
• temporary refractive errors (hypermetropia, astigmatism);
• reduction or complete loss of contrast sensitivity of vision;
• metamorphopsia (distortion of straight lines and curvature of images);
• diplopia (double vision of visible objects);
• nyctalopia (deterioration of vision with decreasing illumination and at night);
• the appearance of colored spots, “floaters” or flashes of light before the eyes (photopsia);
• distortions in color perception;
• absence peripheral vision;
• scotoma (appearance in the field of vision of areas not perceived by the eye in the form of darkened spots).

Central retinal dystrophy(age-related, vitelliform, progressive cone, macular, etc.) begins to develop in people with changes in the PRPH2 gene, which encodes the membrane protein peripherin 2, which ensures the photosensitivity of photoreceptor cells (rods and cones).

Most often, the disease makes itself felt after 60-65 years. According to the American National Eye Institute, about 10% of people 66-74 years old have the prerequisites for macular degeneration of the eye, and in people aged 75-85 years this probability increases to 30%.

The peculiarity of central (macular) dystrophy is the presence of two clinical forms– non-exudative or dry (80-90% of all clinical cases) and wet or exudative.

Dry retinal dystrophy is characterized by the deposition of small yellowish accumulations (drusen) under the macula, in the subretinal area. Layer of photoreceptor cells macular spot due to accumulating deposits (metabolic products that are not broken down due to a genetically determined deficiency of hydrolytic enzymes), it begins to atrophy and die. These changes, in turn, lead to distortions in vision, which are most obvious when reading. Most often, both eyes are affected, although it can start in one eye, and the process lasts quite a long time. However, dry retinal degeneration does not usually lead to complete loss of vision.

Wet retinal dystrophy is considered a more severe form, as it causes blurred vision in short time. And this is due to the fact that, under the influence of the same factors, the process of subretinal neovascularization begins - the growth of new abnormal blood vessels under the macula. Damage to the vascular walls is accompanied by the release of bloody-serous transudate, which accumulates in the macular area and disrupts the trophism of retinal pigment epithelial cells. Vision deteriorates significantly, in nine cases out of every ten there is loss of central vision.

Ophthalmologists note that in 10-20% of patients, age-related retinal dystrophy begins as dry, and then progresses to an exudative form. Age-related macular degeneration is always bilateral, with dry degeneration in one eye and wet degeneration in the other. The course of the disease can be complicated by retinal detachment.

Retinal dystrophy in children

A sufficient range of varieties of degenerative pathologies of the eye is represented by retinal dystrophy in children.

Central retinal dystrophy in children is congenital pathology associated with gene mutation. First of all, this is Stargardt disease (juvenile macular form of the disease, juvenile macular degeneration) - a genetically determined disease associated with a defect in the ABCA4 gene, inherited in an autosomal recessive manner. Royal Statistics national institute blind (RNIB) suggests that this disease accounts for 7% of all cases of retinal dystrophy in British children.

This pathology affects both eyes and begins to appear in children after five years of age. Manifests itself in photophobia, decreased central vision and progressive color blindness– green and red dyschromatopsia.

To date, this disease is incurable, since the optic nerve atrophies over time, and the prognosis is usually unfavorable. However, active rehabilitation steps can preserve and maintain a certain level of visualization (no more than 0.2-0.1).

In Best's disease (macular degeneration), which is also congenital, a fluid-containing cyst-like formation appears in the central fovea of ​​the macula. This leads to a decrease in the acuity of central vision (blurred images with darkened areas) while maintaining peripheral vision. Patients with Best disease often have nearly normal vision for many decades. This disease is inherited, and often family members may not know that they have this pathology.

Juvenile (X-chromosomal) retinoschisis - splitting of the layers of the retina with subsequent damage and degradation of the vitreous - leads to loss of central vision, and in half of the cases lateral vision. Common signs of this disease– squint and involuntary eye movements (nystagmus); the vast majority of patients are boys. Some of them retain a sufficient percentage of vision into adulthood, while for others, vision deteriorates significantly in childhood.

Retinitis pigmentosa includes several inherited forms of the disease that cause gradual loss of vision. It all begins at the age of about ten years with the child’s complaints about vision problems in the dark or limited lateral vision. As ophthalmologists emphasize, this disease develops very slowly and extremely rarely leads to loss of vision.

Leber's amaurosis is a congenital incurable blindness transmitted in an autosomal recessive manner, that is, in order for children to be born with this pathology, both parents must have the mutated RPE65 gene. [More information by going to our publication Amaurosis Leber].

Retinal dystrophy during pregnancy

Possible retinal dystrophy during pregnancy threatens women with severe myopia (above 5-6 diopters), since the shape of their eyeball is deformed. And this creates the preconditions for the development of complications in the form of peripheral vitreochorioretinal dystrophies, which can cause ruptures and retinal detachment, especially during childbirth. That is why, in such cases, obstetricians perform cesarean section delivery.

In pregnant women with myopia - in the absence of complications (preeclampsia) - the retinal vessels narrow slightly to support blood circulation in the uterus-placenta-fetus system. But when pregnancy is complicated by high blood pressure, soft tissue swelling, anemia and nephrotic syndrome, the narrowing of the retinal vessels is more pronounced, and this causes problems with its normal blood supply.

According to ophthalmologists, peripheral dystrophies are most often a consequence of a decrease in the volume of circulating blood in all structures of the eyes (by more than 60%) and a deterioration in the trophism of their tissues.

Among the most common pathologies of the retina during pregnancy are: lattice dystrophy with thinning of the part of the retina in the outer upper part of the vitreal cavity, pigmented and dotted retinal dystrophy with areas of atrophy of the retinal epithelium, as well as dystrophy of the vessels of the eye with spasms of capillaries and venules. Retinoschisis occurs quite often: the retina moves away from the choroid (without a rupture or with a rupture of the retina).

What types of eye dystrophy are there?

If you follow the anatomical principle, then you need to start with the cornea. In total, according to the latest international classification, corneal dystrophy has more than two dozen types, depending on the location of the corneal pathological process.

Surface or endothelial dystrophies (in which amyloid deposits arise in the corneal epithelium) include basement membrane dystrophy, juvenile Messmann dystrophy (Messmann-Wilke syndrome), etc. Dystrophies of the second layer of the cornea (the so-called Bowman's membrane) include subepithelial Thiel-Behnke dystrophy, dystrophy Reis-Bouclera et al.; over time, they often extend into the superficial layers of the cornea, and some can affect the intermediate layer between the stroma and the endothelium (Descemet's membrane) and the endothelium itself.

Dystrophy of the cornea of ​​the eye with localization in the thickest layer, consisting of collagen fibers, fibro- and keratocytes, is defined as stromal dystrophy, which can be different in the morphology of the damage: lattice, granular, crystalline, spotty.

In case of damage to the inner layer of the cornea, endothelial forms of the disease are diagnosed (Fuchs, spotted and posterior polymorphic dystrophy, etc.). However, with the conical type of dystrophy - keratoconus - degenerative changes and deformation occur in all layers of the cornea.

In domestic ophthalmology, retinal dystrophy is divided into central and peripheral according to the place of occurrence, and according to etiology - into acquired and genetically determined. It should be noted that today there are many problems with the classification of retinal dystrophy, which lead to a variety of terminology. Here is just one, but very illustrative example: central retinal dystrophy can be called age-related, senile, central chorioretinal, central chorioretinitis, central involutional, age-related macular degeneration. While Western experts, as a rule, make do with one single definition - macular degeneration. And this is logical, since the macula (macula in Latin - spot) is a yellow spot (macula lutea) in the central zone of the retina, which has a depression with photoreceptor cells that convert the effects of light and color into a nerve impulse and send it along the visual cranial medullary nerve to the brain. Age-related dystrophy retina (in people over 55-65 years old) is perhaps the most common cause of vision loss.

Peripheral retinal dystrophy is represented by such a list of “modifications” that, given the lack of terminology, it is quite problematic to list it in full. These are pigmentary (tapetoretinal or retinitis pigmentosa), cone-rod, vitreoretinal dystrophy Goldmann-Favre, Leber amaurosis, Lefler-Wadsworth dystrophy, retinitis alba dotted (dot-white), etc. Peripheral dystrophy can cause retinal rupture and detachment.

Finally, vascular dystrophy of the eye, which can affect the ophthalmic artery and the central retinal artery that branches off from it, as well as the veins and venules of the eyes. The pathology first manifests itself in microscopic aneurysms (dilations with protruding walls) of the extremely thin blood vessels of the retina, and then can progress to proliferative forms when neovascularization, that is, the growth of new, abnormally fragile blood vessels, begins in response to tissue hypoxia. By themselves, they do not cause any symptoms, however, if the integrity of their walls is violated, then serious problems with vision.

Diagnosis of eye dystrophy

IN ophthalmological clinics Diagnosis is carried out using such methods and techniques as:

• visocontrastometry (determination of visual acuity);
• perimetry (visual field examination);
• campimetry (determining the size of the blind spot and location of scotomas);
• functional testing of the central area of ​​the visual field using the Amsler grid;
• color vision testing (allows you to determine the functional state of the cones);
• dark adaptation testing (gives an objective picture of the functioning of the rods);
• ophthalmoscopy (examination and assessment of the condition of the fundus);
• electrooculography (gives an idea of ​​eye movements, the potential of the retina and eye muscles);
• electroretinography (determination functional state various parts of the retina and visual analyzer);
• fluorescein angiography (allows you to visualize the blood vessels of the eye and detect the growth of new blood vessels and leaks from existing vessels);
• tonometry (level measurement intraocular pressure);
• Ultrasound internal structures eyes in two projections;
• optical layer-by-layer tomography (examination of the structures of the eyeball).

To diagnose corneal dystrophy, it is necessary to examine the cornea using a slit lamp, use pachymetry (to determine the thickness of the cornea), skiascopy (to determine the refraction of the eye), corneotopography (to determine the degree of curvature of the corneal surface), as well as confocal biomicroscopy.

Treatment of eye dystrophy

Symptomatic treatment of corneal dystrophy includes medications to improve the trophism of its tissues:

Taufon - 4% eye drops based on taurine, which promotes the restoration of tissue damaged by degenerative changes in the cornea. They should be instilled under the conjunctiva - 0.3 ml once a day, the course of treatment is 10 days, which is repeated after six months. Taufon in the form of an injection solution is used in more severe cases.

Drops Sulfated glycosaminoglycans (Balarpan), due to the content of a natural component of the corneal stroma, activate its regeneration. Two drops are prescribed in each eye, morning and evening, for 30 days. Oftan Katahrom drops, consisting of adenosine (ATP component), nicotinic acid and the enzyme cytochrome oxidase, stimulate interstitial energy exchange and restoration of damaged cornea; The dosage recommended by doctors is 1-2 drops three times a day by instillation, that is, into the conjunctival sac (throwing the head back and slightly retracting the lower eyelid).

In addition, magnetophoresis is used with Vita-Iodurol drops, which, in addition to nicotinic acid and adenosine, contain magnesium and calcium chlorides.

Treatment of retinal dystrophy

At first, treatment with drugs is aimed at stimulating local blood circulation - to improve tissue nutrition (the drops given above are prescribed).

For example, such treatment for retinal pigmentary dystrophy is carried out twice a year, but, according to experts, most often it does not give the desired effect. The last word remains with ophthalmological microsurgery: operations are performed to transplant a longitudinal flap of one of the six extraocular muscles into the area of ​​the choroid.

Treatment of retinal dystrophy localized in the macular area should take into account not only the etiology of the disease and concomitant and aggravating diseases of a particular patient, but also the form of the pathology - dry or exudative.

Accepted in domestic clinical practice Treatment of dry retinal dystrophy can be carried out with antioxidant drugs. One of them is Emoxipin (in the form of 1% and eye drops And injection solution). The solution can be injected through the conjunctiva or into the periocular area: once a day or every other day, the maximum course of treatment lasts a month.

In the treatment of retinal dystrophy with drugs that neutralize free radicals and prevent damage cell membranes, the antioxidant enzyme superoxide dismutase, which is part of the drug Erisod (in the form of powder for the preparation of drops), is used. These drops should be prepared in distilled water and instilled for at least 10 days - two drops three times a day.

Wet retinal dystrophy is treated with photodynamic therapy. This is a combined non-invasive method aimed at stopping the process of neovascularization. To do this, the patient is injected intravenously with the photosensitizing agent Visudin (Verteporfin), which, after activation by a cold red laser, causes the production of singlet oxygen, which destroys the rapidly proliferating cells of the walls of abnormal blood vessels. As a result, the cells die, and a hermetically sealed blockage of the newly formed vessels occurs.

Ophthalmologists use drugs that block the VEGF-A (Vascular Endothelial) protein to treat macular degenerative pathologies of the exudative type. Growth Factor), produced by the body for the growth of blood vessels. Drugs such as Ranibizumab (Lucentis) and Pegaptanib sodium (Macugen) suppress the activity of this protein.

which are injected into the vitreous body of the eye 5-7 times during the year.

A intramuscular injection synthetic hormone of the adrenal cortex Triamcinolone acetonide helps normalize catabolism in connective tissue, reduce the level of cell division of membrane proteins and stop infiltration in wet retinal dystrophy.

Treatment of retinal dystrophy with laser

It should be borne in mind that laser treatment cannot restore normal vision, since it has a different purpose - to slow down the progression of the disease and minimize the risk of retinal detachment. Yes, it is still impossible to revive dead photoreceptor cells.

That's why this method called peripheral prophylactic laser coagulation, and its principle of action is based on the coagulation of proteins in retinal epithelial cells. In this way, it is possible to strengthen the pathologically altered areas of the retina of the eye and prevent the separation of the inner layer of light-sensitive rods and cones from the pigment epithelium.

This is how treatment is done peripheral dystrophy retina using laser coagulation. And laser therapy for dry dystrophy is aimed at removing deposits formed there from the subretinal zone of the eye.

In addition, laser coagulation seals choroidal neovascularization in macular degeneration and reduces the growth of “leaky” blood vessels, preventing further visual impairment. This treatment leaves a scar that creates a permanent blind spot in the field of vision, but this is much better than a complete blind spot instead of vision.

By the way, none folk remedies You shouldn’t use it for retinal dystrophy: it still won’t help. So don’t try to be treated with an infusion of onion peels with honey or lotions with a decoction of chamomile and nettle...

Vitamins and nutrition for retinal dystrophy

The “correct” vitamins are very important for retinal dystrophy. Experts include all B vitamins (especially B6 - pyridoxine), as well as ascorbic acid(antioxidant), vitamins A, E and P.

Many eye doctors recommend taking vitamin complexes for vision, containing lutein - a carotenoid of natural yellow enzymes. Not only does it have strong antioxidant properties, but it also reduces the formation of lipofuscin, which is involved in central retinal dystrophy. Our body cannot produce lutein on its own, so it must be obtained from food.

Nutrition for retinal dystrophy can and should help fight, and best of all, prevent eye dystrophy. For example, spinach, parsley, green peas, broccoli, pumpkin, pistachios, and egg yolk contain the most lutein.

It is necessary to include in your diet fresh fruits and vegetables in sufficient quantities, healthy unsaturated fats vegetable oils and whole grain products. And don't forget about the fish! Mackerel, salmon, sardines, and herring contain omega-3 fatty acids, which may help reduce the risk of vision loss associated with macular degeneration. These acids can also be found in... walnuts.

Forecast and prevention of eye dystrophy

The prognosis of eye dystrophy - due to the progressive nature of this pathology - cannot be considered positive. However, according to foreign ophthalmologists, retinal dystrophy itself will not lead to complete blindness. In almost all cases, some percentage of vision, mostly peripheral, remains. It is also necessary to take into account that it is possible to lose vision, for example, due to a severe stroke, diabetes mellitus or injury.

According to the American Academy of Ophthalmology, in the United States, only 2.1% of patients with central retinal dystrophy completely lost their vision, while the rest retained a sufficient level of peripheral vision. And yet, despite its successful treatment, macular degeneration may reappear over time.

Prevention of eye dystrophy - a healthy lifestyle. The consumption of large amounts of animal fats contributes to degeneration of the retina and macula of the eyes. high level cholesterol and metabolic disorders such as obesity.

The role of oxidative stress of retinal cells in the development of retinal dystrophy is convincingly demonstrated increased speed the development of pathology in smokers and those exposed to UV radiation. Therefore, doctors advise their patients to quit smoking and avoid exposing the cornea to intense sunlight, that is, wear sunglasses and hats.

It is useful for older people to visit an ophthalmologist once a year, especially if there is a family history of eye dystrophy - retinal, corneal or vascular.

Despite the high level of technology development, in modern world Not all ophthalmic diseases are treatable. Retinal pigmentary degeneration is one such pathology. This disease usually manifests itself in early age, periods of deterioration and improvement of vision alternate with each other throughout life, and closer to 50 years of age, complete blindness most often occurs. Retinal dystrophy most often occurs in men, and can first manifest itself both in childhood and in adulthood.

To date, the causes of retinal pigmentary dystrophy have not yet been identified.

Causes and course of retinal pigmentary degeneration

Why retinal dystrophy occurs has not yet been fully studied. Ophthalmologists are considering several versions. Retinal pigmentary degeneration was named so because of changes that occur in the fundus of the eye, causing pigment spots to appear on the eyes. They are formed along the vessels located on the retina of the eye, there are different sizes and forms. Gradually, the pigment epithelium of the retina becomes discolored, as a result of which the fundus of the eye becomes visible like a web of orange-red vessels.

Over time, the disease only progresses, and age spots in the eye spread more and more. Densely dotting the retina, they turn into central part eyes, arise in the iris. The vessels become very narrow and practically invisible, and the nerve disc turns pale, later atrophying. Retinal pigmentary degeneration usually affects both eyes at the same time.

Many people are discussing that the pigment epithelium is formed against the background of diseases in endocrine pathologies and vitamin deficiency, in particular, with a significant lack of vitamin A. Some experts are inclined to believe that infections and toxins have a negative impact. It is believed that retinal dystrophy can be inherited from relatives.

Retinal pigmentary dystrophy is dangerous for the patient's vision loss.

If the disease manifests itself at an early age, then by the age of 25 the patient may lose his ability to work. But there are exceptions here too. Sometimes dystrophy is observed only in one eye, or only a separate fragment of the retina is damaged. People who have pigment spots in their eyes are at risk for other ophthalmological diseases: cataracts, glaucoma, lens opacification.

Symptoms of the problem

Due to the process of retinal pigmentary degeneration, patients see a somewhat distorted image. Retinal stains make it difficult to clearly see the outlines of surrounding objects. Often people suffer from perception disorders color range. Pigmentation causes blurred vision, especially in low light. Due to damage to the rods (components of the retina), so-called night blindness or hemeralopia is formed. Orientation skills in twilight disappear. The retina consists of rods, which are located at the edges, and cones in the center. First, the rods are affected, as a result of which the patient’s peripheral vision narrows, but a “clean” area remains in the center. Degradation of cones aggravates the situation and gradually provokes blindness.

Diagnostic procedures

When examining a patient, the doctor first examines the quality of peripheral vision. Retinal pigmentary abiotrophy may be noticed during examination of the fundus of the eye. Spots on the iris that resemble “spiders” will become the main feature of dystrophy. To put accurate diagnosis Electrophysiological study is used; it is considered the most objective in assessing the condition and functionality of the retina.

Treatment of the disease

Pigmentary dystrophy should be treated comprehensively. To get rid of age spots, the doctor prescribes the patient a complex of various vitamins and medications to nourish the retinal pigment epithelium. In addition to tablets and injections, drops are also used. Treatment is aimed at restoring the natural functionality of the retina. Additionally used therapeutic methods, which are aimed at improving the blood supply to the eye. This can really slow down the pathological process, and even start remission.

For home use, “Sidorenko glasses” were developed - a simulator for the eye muscles, which combines several methods of influence and is especially effective in the initial stages.

Features in children

In the early stages, retinal pigmentary abiotrophy in children is especially difficult to diagnose, and before the age of 6 it is almost impossible. Later, the presence of visual dystrophy can be detected by noticing the child’s difficulties with orientation in space at night. Children do not do their usual activities at such times and stop playing. Without seeing the periphery of the visual field, the child can bump into surrounding objects, since only the center of the eye works efficiently. If a child is diagnosed age spot on the eye, experts recommend that direct relatives undergo examination by a doctor in order to identify eye dystrophy at an early stage and prescribe treatment.

Pediatric retinal pigmentary dystrophy requires the selection of implants.

It is necessary to recall radically new experimental techniques for influencing the pigment layer of the retina, in particular, gene therapy. With its help, you can restore damaged genes, which means you can gain hope for improved vision. Specific eye implants have also been developed. Their task is to work similarly to the natural retina. And indeed, people with such implants are gradually beginning to navigate more freely not only within the walls of a room, but also on the street.

Retinitis pigmentosa (retinitis pigmentosa, abiotrophy) is a genetically inherited disease. Usually the process begins unnoticed, can last for years and lead to complete blindness.

Causes and mechanism of development of the disease

Treatment of retinitis pigmentosa

In advanced stages of the disease, cataracts or glaucoma of secondary origin are formed. In this case, central vision decreases sharply and quickly. Atrophy of the optic disc gradually develops, which leads to reflex immobilization of the pupils. As for peripheral vision, it may be completely absent and then this condition is called tunnel vision (as if a person is looking through a long and thin tube).

Occasionally found atypical forms retinal degeneration. In these cases, only changes in the optic nerve head, narrowing and tortuosity of blood vessels, impaired twilight vision. Unilateral degeneration occurs extremely rarely, and in almost all cases there is a cataract on the affected eye.

Treatment of retinitis pigmentosa

Initial treatment for retinal degeneration most often consists of medications. Their main effect is to improve metabolism in the retinal layer, restore the retina and dilate blood vessels.

For this purpose the following is prescribed:

• Emoxipin;
• Mildronate;
• Emoxipin;
• Taufon;
• Nicotinic acid;
• No-shpu with papaverine;
• Aloe extract;
• Retinalamin;
• Alloplant.

These drugs can be introduced into the body, either in the form of eye drops or by injection. It is also advisable to use a complex of nucleic acids in treatment - Encad, which in more than half of the cases significantly improves visual functions. It is prescribed intramuscularly, subconjunctivally, using iontophoresis, or local applications are made with it.

Often in parallel with drug treatment, physiotherapeutic measures are also used, the purpose of which is to stimulate regenerative processes in the retina and activate the remaining rods and cones. Electrical stimulation of the eye, magnetic resonance therapy, and ozone treatment are widely used. Recovery vascular bed Vaso-reconstructive surgeries can be used.

Surgery pigmentary degeneration of the retina is used to normalize the blood supply to the retina of the eyes; for this purpose, some eye muscles are transplanted into the suprachoroidal space.

Recently, encouraging data have been coming from genetic engineers who have found the ability to restore damaged genes responsible for the development of this disease. In addition, special implants have been developed - retinal substitutes.

And very recent experiments on mice conducted in Britain provide convincing evidence that blindness can be treated with the help of special light-sensitive cells injected. And although this technique has not yet been tested in humans, scientists hope that this drug can be used to treat people suffering from retinitis pigmentosa.

As for the prognosis of the disease, it is generally unfavorable, but with early detection of pathology and timely initiation of treatment, the process can be delayed and even an improvement in the condition can be achieved. All patients are advised to avoid prolonged stays in dark rooms and not engage in heavy physical labor.

Pigmentary retinal degeneration (RPD) is a relatively rare, inherited disease that is associated with disruption of the function and survival of the rod photoreceptors in the retina responsible for peripheral black-and-white twilight vision. Cones, another type of photoreceptor, are located mostly in the macula. They are responsible for central daytime color vision with high acuity. Cones are involved in the degenerative process for the second time.

Inheritance can be sex-linked (passed from mother to son with the X chromosome), autosomal recessive (the disease genes are needed from both parents) or autosomal dominant (the pathological gene from one of the parents is enough). Since the X chromosome is most often involved, men are affected more often than women.

People with PDS usually discover that they are ill by noticing a loss of peripheral vision and the ability to navigate in dimly lit spaces. Disease progression is highly variable. For some, vision is very weak; for others, the disease gradually leads to complete blindness.

Often the disease is detected in childhood when symptoms appear, but sometimes they can appear in adulthood.

Signs (symptoms)

Poor vision at dusk in both eyes

Frequent trips and collisions with surrounding objects in low light conditions

Gradual narrowing of the peripheral visual field

• Fast eye fatigue

Diagnostics

Retinal pigmentary degeneration is usually diagnosed before adulthood. It is often discovered when the patient begins to complain of difficulty seeing at dusk and at night. To make a diagnosis, it is usually enough for a doctor to look at the fundus of the eye with an ophthalmoscope to see the characteristic accumulations of pigment there. An electrophysiological test, called electroretinography, may be ordered to provide more detail about the severity of the disease. Perimetry allows you to assess the state of the visual fields.

Treatment

Standard and really effective treatment Pigmentary retinal degeneration does not exist. Stimulating procedures may be prescribed - electrical and magnetic stimulation - in order to “stir up” the diseased retina, force the surviving photoreceptors to take on the functions of the dead and try to somehow stop the natural course of the disease. In addition, doctors can resort to so-called vasoreconstructive surgery, with which they try to improve the blood supply to the retina. All of these procedures have limited effectiveness.

Despite the paucity of the ophthalmologist's current weapons in the battle with the PDS and others hereditary diseases retina, this area is being actively developed by scientists. It constantly acquires new information, which inspires cautious optimism that in the relatively near future a radically new approach to preserving and restoring vision in this category of patients will be proposed.