Pigment retinitis: description and methods of treatment

Among the less common, but the most serious diseases of the eye, today is pigment retinitis, which can cause great harm to the quality of sight.The disease in this case is hereditary and develops due to mutation of the genes of the human body. However, it can manifest itself at an early age, so it is especially important to keep track of its signs from childhood.

Definition of disease

Pigment retinitis (the second name pigmentary dystrophy of the retina) is a whole complex of pathologies that are hereditary in nature.It proceeds against the background of anomalies of photosensitive receptors and retinal pigment epithelium. Often, patients suffering from injury suffer from night blindness and lack of peripheral vision.

The disease has the following types of inheritance:

• Isolated (often);
• Autosomal dominant (often);
• With the X-chromosome (rarely);
• Autosomal recessive (often).

The best prognosis of treatment, as a rule, is observed in autosomal dominant type, the worst - X-linked.

Kinds

At present, the following types of retinitis are known:

• Pigmented inverted.In this case, the lesions of the cones are more pronounced than the changes in the rods. Daylight vision can be significantly weakened.
• Without pigment.There are no pigment deposits in this form.

• White-spot.Throughout the eye, pigment changes and white spots are observed.
• Pseudo pigmentary.The causes of the appearance may be the use of medications, injuries, injuries.
• In violation of metabolism.Retinitis is associated with violations of lipid, carbohydrate and other types of metabolism, which lead to mutations and changes in the gene.

Causes

The main cause of the onset and development of the disease is the change and mutation of those genes that are responsible for the synthesis of proteins of a specific type during pregnancy (in the womb of the mother).Usually retinitis pigmentosa is inherited, but there are cases when it has an innate form without any prerequisites from relatives.

Most often the disease affects several generations of the same family, so when planning a child you need to be ready for it.

Symptoms

The development of pigmentary dystrophy is usually accompanied by a variety of different symptoms.Among them:

• Night blindness.Progresses gradually, beginning with a deterioration of vision during twilight. Eventually, the central zone remains small.
• Decreased sharpness.Usually observed in autosomal recessive and X-linked forms. In the rest - most often good sharpness is preserved.
• Anomalies of the fundus: minute pigmentation, narrowing of arterioles (first degree); distribution of pigment concentrations (medium degree); thinning of arterioles, deterioration of capillaries, pallor (late stage development).

Anomalies of the fundus

• Macular pathologies: edema (accompanied by the appearance of cysts), atrophy, wrinkling.

In addition, there may be myopia, cataracts, keratoconus, optic nerve damage, glaucoma.

One of the main signs of pigment retinitis is the changes in the pigment itself. The remaining signs can also occur among the symptoms of other serious diseases. The most accurate diagnosis can be made only by a highly qualified ophthalmologist.

Possible complications

Currently, the lack of treatment and the use of means to slow the progression of the disease can lead to a severe drop in visual acuity.Along with this, myopia, keratitis, cataract and other diseases can develop, which can not always be completely eliminated.

Patients with pigment dystrophy should regularly undergo preventive examinations from ophthalmologists and follow all the prescriptions and recommendations of the treating physician.

Treatment

Diagnosis of pathology, as a rule, requires the study of the fundus and ophthalmoscopy, conducted by an ophthalmologist. Also, during examination, it is necessary to determine the level of photosensitivity and color perception by the patient.A special role in diagnosis is played by a survey, during which the oculist finds out the presence of anomalies in the next of kin and the symptoms that the patient is experiencing.

An example of a color perception test

Medication

At present, there are no medicines that can cure this disease.There are several types of drugs that can get rid of the most pronounced and unpleasant symptoms. Main directions:

• Removal of edema:for this purpose prescribe drugs-diuretics;
• Vascular strengthening:eye drops and medications for oral administration;
• Nutrition of the tissues of the visual apparatus:vasodilators;
• Delay in progression of visual acuity reduction:retinol, vitamin complexes.

Vitamin complexes for slowing the progression of visual acuity reduction

In addition, it is useful to keep a diet with a low content of animal fats. Its basis includes products of plant origin.

Surgically

If a cataract occurs as a by-product of the development of this type of dystrophy, it can be eliminated by laser coagulation.In some cases, it is possible to correct myopia.

In advanced countries in the form of an experiment, pigment cells are transplanted into the affected eye. There are also developments in the field of stem cell treatment and the search for a method for replacing a defective gene.

One of the experimental methods of eliminating the consequences of the disease is also the installation of a retinal prosthesis.However, the samples available today are difficult to freely use and care without the help of a doctor. At the moment the direction is at the stage of development and improvement.

Prosthesis of the retina

During the period of treatment, it is strongly recommended that the medical diet be followed and sunglasses are worn.

Prevention

Pigmentary dystrophy is a hereditary disease and occurs against the background of a gene mutation. That is why preventive measures to eliminate the possible occurrence of the disease does not exist.However, during the planning of the child, a thorough medical examination is recommended to reduce the risk of having a sick child.

Children who have pathologies of this type should immediately, after the appearance of the first signs of an anomaly, register with an ophthalmologist and regularly undergo preventive examinations.Also, they are recommended to follow a diet with a low content of animal fats, use sunscreen products (glasses), lead a healthy lifestyle.

The earlier a patient turns to an ophthalmologist with this problem, the greater the probability of reducing the risk of rapid reduction in visual acuity and other complications.

Video

conclusions

Retinitis pigmentosa is an extremely rare disease of the eye, but serious enough, since it can lead to a significant deterioration in the quality of eyesight.In addition, it is inherited and capable of hitting several generations of the family.At the same time, it practically can not be prevented and treated. The only way to alleviate the problem is to slow the progression of the disease. However, developments in the field of treatment of pathology have already yielded positive results and will soon be introduced into traditional medicine.