This is a rare affliction faced by one in 5,000 people. Pigmented dystrophy, or retinitis is most often seen in males. This can happen at any age, including children.
The vast majority of those who are faced with this form of dystrophy before the age of 20, the ability to read remains unchanged, and the degree of visual acuity is more than 0.1. In the age group from 45 to 50 years, the degree of sharpness of visual functions is less than 0.1, the ability to read is completely lost. About the symptoms, causes and treatment of the disease further.
Symptoms of retinal pigment dystrophy
The main manifestations of the disease include:
Hemeralopia, or "night blindness" - is formed due to damage to one of the components of the retina, namely the sticks. At the same time, the orientation skill with an insufficient degree of illumination is almost lost. Hemeralopia should be considered the primary manifestation of the disease. It may occur five or even 10 years before the first obvious manifestations in the retina,
Steady degradation of peripheral vision - begins with a minimum narrowing of the boundaries of peripheral vision. As part of further development may lead to the full loss of it. This phenomenon is called a tubular or tunnel type of vision, in which there is only a small "visual" area in the center,
The aggravation of visual acuity and color perception is noted in the late stages of the disease and is caused by the degradation of cones in the central region of the retina. Systematic development of the disease provokes blindness.
Causes of retinal pigment dystrophy
The reason for the appearance of pigmentary dystrophy is only one, in order to understand it, it is necessary to understand the anatomy of the human eye. Thus, the light-sensitive membrane of the eye or retina includes two categories of receptor cells. We are talking about chopsticks and cones, which received such names solely because of their shape. The vast majority of cones are located in the central area of the retina, which ensures optimum sharpness and color perception. The rods are distributed over the retina itself, they control peripheral vision and provide an opportunity to see in poor light conditions.
When specific genes are damaged that provide nutrition and retinal function, systematic destruction of the outer area of the retina is noted. That is exactly the one where the rods and cones are placed. Such damage primarily affects the periphery and goes away within 15-20 years. It is this long process that is the only reason for the development of pigmental dystrophy.
Diagnosis of retinal pigment dystrophy
The diagnosis of pigment dystrophy in the primary stages of the disease has its own characteristics. First of all, because in childhood it is difficult. Its implementation is possible only in children aged six years or more. The disease is often detected already after the appearance of difficulties of orientation at dusk or at night. In this case, the disease progresses for quite some time.
As part of the examination revealed the degree of visual acuity and peripheral perception. The fundus of the eye is examined most carefully, because it is there that changes can occur. The most obvious sign of a change that is detected in the process of examining the fundus is bone bodies. They represent areas where progressive destruction of receptor-type cells occurs.
To confirm the diagnosis, one can resort to electrophysiological research, because it is they who fully reveal the “working” capabilities of the retina. Also, with the help of specialized equipment, the assessment of dark adaptation and the ability to navigate in a darkened room are carried out.
If there are suspicions and, moreover, a diagnosis of retinal pigment dystrophy has been established, taking into account the genetic component of the disease, it is recommended that an individual’s next of kin be examined. That is what will help to identify the disease as soon as possible.
Treatment of retinal pigment dystrophy
Today, the treatment of pigment dystrophy is complex. So for the suspension of the progression of the disease should be used vitamin complexes, drugs that improve the nutrition of the eye and blood supply to the retina. It can be not only injections, but also drops. In addition to these funds, the class of peptide bioregulators has been widely used. They are designed to optimize the nutrition and regenerative capacity of the retina.
Physiotherapeutic methods are also used, which improve everything related to blood supply in the area of the eyeball. Thus, the pathological process is slowed down. With constant monitoring it is possible to achieve remission. Effective apparatus, which is designed for use in the home, should be considered "glasses Sidorenko." They combine four methods of exposure at the same time and will be most effective in the early stages of pigmentary dystrophy.
It should be noted a list of fundamentally new experimental directions in the field of treatment of the disease presented. We are talking about gene therapy, which allows you to restore those genes that were damaged. Also, surgeons introduce into the eye area special mechanical implants that act similarly to the retina. They enable people who have lost their sight to freely navigate indoors and even on the street. Also, with the help of these implants, the self-care process becomes much easier.
Thus, pigmentary dystrophy is a serious problem with vision, which has a progressive nature. Her symptoms at the primary stage are difficult to recognize, but we should not forget about the genetic predisposition to the disease. In case of early diagnosis, pigment dystrophy will be much easier to treat.
Causes of development
Pigment degeneration of the retina of the eyeball is a disease that results in a gradual narrowing of the visual fields. One of the obvious symptoms of the disease is vision loss in twilight time. The disease can be caused by the failure of a particular gene. In rare cases, a violation of the interaction of several genomes. The disease is hereditary and is transmitted through the male line. The disease may be accompanied by impaired hearing aid.
The causes of failures in the gene system of the human body have not yet been identified. Overseas researchers have found that abnormalities in DNA are not one hundred percent cause of the development of pigmental dystrophy. According to experts, the disease provokes the occurrence of violations in the vascular system of the eyeball.
Despite the fact that the causes of the disease remain a mystery of medicine, experts have fairly reliably studied the issue of its development.
At the initial stage of the disease, a process of metabolism failure in the retina of the eyeball occurs. Violations also affect the vascular system. As a result of the development of the disease, the layer of the retina in which the pigment is located begins to break down. In the same layer are sensitive photoreceptors, rods and cones. In the early stages, the degeneration processes affect only the peripheral areas of the retina. That is why the patient does not feel discomfort and pain. The gradually changed area begins to increase in size until it covers the entire zone of the retina. When the retina is completely affected, the first serious symptoms of the disease begin to manifest, and the perception of colors and their shades deteriorates.
The disease can spread only to one eye, however, there are cases when the disease affects two visual organs at once. The first symptoms of the disease appear in early childhood, and by the age of twenty a person may lose his ability to work. Severe stages of retinal pigment dystrophy can be accompanied by complications such as cataracts and glaucoma.
The sluggish development of the disease leads to the fact that most patients seek the help of specialists when pathological changes began their rapid development. The first serious symptom of the disease is the difficulty of orientation in low light conditions. Pathologies occurring on the peripheral part of the retina, lead to the fact that the visual fields are narrowed.
Given the nature of the disease, the main group of patients are children who have not reached school age. At this age, minor eye problems are not noticed, which means that parents may not be aware of the development of the disease.
The first stages of development can take a long time - up to five years. Subsequently, the degeneration of the peripheral region of the retina begins to progress. The visual fields at this point are severely narrowed, in some patients there is a complete lack of lateral vision. Examination of the ophthalmologist may reveal areas with pathological changes, but if you do nothing, then soon they will spread throughout the retina. At this stage, gaps may appear in some parts of the retina. The vitreous body begins to lose its transparency, becoming dull yellow. At this stage, central vision is not affected.
The exact cause of the disease has not been established, but ophthalmologists call only versions of the development of retinal pigment degeneration
The disease in the advanced stage can be complicated by the occurrence of diseases such as glaucoma and cataracts. With complications, central vision loses its sharpness very sharply, and over time it can be irretrievably lost. Complications lead to the development of atrophy of the vitreous body.
There is another form of retinal degeneration - atypical. As a result of the disease, the appearance and structure of the vascular system changes. The patient has difficulty orienteering, in low light conditions.
One of the rarest types of retinal degeneration is a one-sided form, while a patient always develops a cataract.
Treatment of pigment dystrophy
Treatment of pigment degeneration of the retina, which is under development, is most often carried out with the help of medicines. The actions of drugs should be aimed at normalizing blood circulation and nutrient metabolism in the retina and vascular system. In most cases, the following drugs are prescribed by specialists:
- "Emoxipin". This drug corrects microcirculation in the body.
- "Taufon". Eye drops stimulating regeneration processes in the eye tissues.
- "Retinalamin". The drug, prescribed for dystrophy of the retina, has a regenerative effect.
- A nicotinic acid. Vitamin that stimulates the metabolism of nutrients in the body and blood circulation.
- No-spa with papaverine. An antispasmodic that relieves pressure in the vascular system.
These drugs can be prescribed by the doctor in the form of tablets, as well as injections or eye drops.
Very often, in addition to medication, a course of physiotherapy is prescribed to stimulate the processes of recovery and regeneration of the retina. Passing this course can activate photoreceptors. One of the popular methods today is the stimulation of electrical pulses, magnetic resonance and ozone therapy. If as a result of the disease, the choroid is affected, it is reasonable to perform a surgical procedure.
Using the operation, specialists normalize blood circulation in the reticular layer of the eyeball. To achieve this goal, it may be necessary to transplant certain tissues of the eyeball, under the perihoroidal space.
The use of devices that correct vision
Some experts recommend treatment of retinal pigment dystrophy with the help of photostimulation devices. The basis of their work is a technique that causes arousal in certain areas of the eyeball and slowing the development of the disease.
The radiation emitted by the apparatus stimulates blood circulation in the vascular system of the eyeball, and also normalizes the metabolism of nutrients. Using this technique, you can also remove puffiness from the retina of the eyeball. Photostimulation of the retina of the optic organs can have a positive effect on strengthening the retina and improving the circulation of beneficial substances in the inner layers of the eyeball.
Damage starts at the periphery and spreads over several decades to the central zone of the retina.
Unfortunately, today medicine is still far enough from the question of when the disease is in disrepair. Very often there is news that foreign researchers have found a way to restore certain genes responsible for the occurrence of the disease. Already today, special implants, capable of replacing the reticular membrane, are undergoing the final stage of testing.
Another approach of specialists has revealed that it is possible to completely restore lost vision by injecting a special substance containing cells that are sensitive to light. However, this technique is still at the experimental stage, and whether it will be possible for scientists to achieve the desired result is still unknown.
Many of those who have experienced this disease know that the prognosis of treatment success is unfavorable in most cases. But if the disease is detected in the initial stage, using certain methods of treatment, you can stop its progression. In some cases, experts achieved really tangible results. People who have been diagnosed with the disease, should avoid prolonged physical exertion, as well as loads on the visual organs.
Pathogenesis of the disease
The retina of the eyeball consists of two types of receptor cells. On it are located cells in the form of cones and rods. Cones on the retina are in the center. They are responsible for the perception of color and clarity of vision. The rods are evenly distributed throughout the retina. Their task is to identify the light and ensure the quality of vision in twilight and darkness.
Under the influence of a number of external and internal factors at the gene level, there is a degradation of the outer layer of the retina, a decrease in the functionality of the rods. Pathological changes begin at the edges of the eyes, gradually moving towards their center. This process can last from several years to decades. Visual acuity and light perception are gradually deteriorating. In most cases, the disease ends in complete blindness.
As a rule, pathology captures both eyes, striking them with the same speed. If the patient is provided with timely and qualified assistance, then he has a chance to retain the ability to distinguish between surrounding objects and move independently without assistance.
Causes of vision loss
Since a pathology such as retinal pigment abiotrophy has not been sufficiently studied and studied, ophthalmologists cannot clearly systematize the causes of its occurrence. The only thing that has been reliably established is that the disease is inherited. It is laid on the gene level and goes to children from one or both parents.
Scientists were able to establish why there is progressive retinal pigment dystrophy, if close relatives do not suffer from this pathology.
Among the factors that provoke PDS in genetically healthy people include:
- Disorders of fetal growth during pregnancy. Deviations in the development of the child can be caused by alcohol intake, smoking, potent therapeutic drugs. In some cases, the disease is a consequence of severe stress in the mother.
- Brain injury in an adult. The cause of the disease can be a wound, an operation, a severe injury or a stroke. All this leads to deterioration of the blood supply and metabolism.
- Age. Over time, vascular wear, cell death and other pathological changes in the body occur.
- Exposure to radiation or toxic substances. Because of this, mutations occur in the body, causing changes in the structure of DNA and individual genes.
Regardless of the cause of the disease, patients experience approximately the same symptoms. Attention should be paid to them in order to receive medical care in the early stages of the disease.
The disease is sluggish, not showing bright signs. This is precisely its danger. The first signal that the degradation of peripheral cells has begun in the retina is a decrease in a person’s ability to orient in poorly lit spaces. With the hereditary nature of the PDS, this is manifested already in childhood. However, parents rarely pay attention to this deviation, considering it a fear of the dark.
The fact that a person develops pigment-free retinal tissue degeneration, indicate these symptoms:
- fatigue of vision in twilight conditions, insufficient or artificial light,
- blurry vision of objects and surroundings in the dark,
- impaired coordination of movements when walking along dark streets, stairs and corridors,
- collisions with objects that are clearly visible to other people
- the weakening and disappearance of peripheral vision (pipe effect).
When such symptoms are detected, it is necessary to avoid being in dark places as much as possible. At the earliest opportunity you need to contact an oculist.
Diagnosis of retinal pigment-free abiotrophy
Diagnosis of the disease is carried out in a clinical setting. It begins with the fact that the ophthalmologist conducts a discussion with the patient of the problems that pursue the latter. Find out the symptoms of the disease and the time of their occurrence. Special attention is paid to heredity. The doctor may request the patient's medical history to examine how the retinal pathological changes took place in them. Sometimes relatives of the patient are invited for consultation.
After the survey, the following events are held:
- Examination of the fundus. The ophthalmologist focuses his attention on the spots that form the destroyed receptor cells with narrowed vessels. The degree of damage to the eye is determined by the presence of bone bodies and discoloration of the optic nerve disc. When abiotrophy of the retina is observed blanching.
- Checking visual acuity. For this purpose, special tables and interchangeable lenses are used. The doctor examines the width of the sector, which can cover the eyes (perimetry).
- Testing a patient for color blindness. He is invited to study the color pictures and say what is depicted on them. If the patient does not distinguish colors, then this is another sign of retinal pigment dystrophy.
- Electrophysiological examination. This procedure makes it possible to objectively evaluate the functional capabilities of the retina. Based on the survey results, preliminary conclusions are made about the possibility and prospects for further treatment.
- Evaluation of the mechanisms of dark adaptation and orientation of the patient in low light conditions. Loss of orientation in space indicates the presence of abiotrophy of the retina.
- Urine and blood tests. Their results determine the presence of infection, inflammation and internal parasites in the body. At the same time, tumor markers are being studied for signs of oncology.
If the patient has been diagnosed with retinal pigment dystrophy, treatment should begin as soon as possible. Delay is fraught with the development of inflammation, necrosis and total blindness.
Treatment of retinal pigment degeneration
Today, there is no single approach to the specific treatment of retinal pigment abiotrophy. Medicine could only find ways to halt the rate of development of the disease.
For this patient is recommended to take such means:
- vitamin complexes to strengthen tissues and improve metabolism
- drugs that improve blood supply and nutrition of the retina,
- peptide bioregulators that improve the nutrition and recovery capabilities of the retina.
The development of science and technological capabilities of modern medicine has allowed us to develop a number of new experimental directions in the treatment of retinal pigment abiotrophy.
In countries with a developed health care system, this disease is treated by the following methods:
- Gene therapy With the help of certain modifications with DNA, doctors manage to repair damaged genes. Due to this, the pathological processes are stopped, normal or limited vision is returned to the patients.
- Implantation of special electronic implants. These microscopic devices allow completely blind people to navigate relatively freely in space, to maintain themselves and move without assistance.
- Incentive procedures. Magnetostimulation and electrostimulation activate surviving photoreceptors. In living cells, the functionality increases, they replace the dead cells of the retina.
- Acceptance of nerve tissue regenerating drugs. They help to stimulate the proliferation of active receptors with the gradual replacement of damaged tissue. Today, doctors have not yet managed to achieve the desired therapeutic effect. But, the continuous development of ophthalmology and nanotechnology gives us hope that very soon this method will allow to fully restore the retina.
For patients in whom advanced retinal abiotrophy has been detected, treatment may be surgical. Surgeons perform procedures to repair blood vessels that supply blood to the retina. Along the way, damaged eye fragments are removed.
Such an approach allows to improve the metabolism in diseased organs, to launch the mechanism of regeneration of nerve cells. The surgical method of treatment allows to partially restore the vision lost due to gene mutations, injuries and infectious diseases.
Conclusion on the topic
Today, many countries are developing issues of treatment of retinal pigment degeneration. Scientists are actively studying the functional anatomy and biology of the organs of vision, share achievements in the field of neuro-ophthalmology, improve the methods of microsurgery and cell transplantation. All this makes the prospect of successful treatment of retinal pigment degeneration quite real.
What are the causes of the disease?
According to statistics, pigment dystrophy is diagnosed in 1 out of 5,000 people. In most cases, retinitis affects men.
Pathology occurs as a result of changes in the work of one or more genes. The cause of dystrophy is considered a genetic predisposition. The disease is inherited through the male line and can be combined with impaired ear function. Factor affecting gene damage was not identified. The name of the disease originated from changes in the eye. There are pigment spots of the fundus, located along the vessels of the retina. Over time, the pigmentation of the epithelium decreases, and the inner shell of the eye becomes similar to the translucent vascular web.
Pigment forming spots on the retina, accumulating, forms deposits, like bone bodies. When they spread, hyperpigmentation occurs. The visual cells are damaged, and the epithelium near the spots becomes thinner. Due to the progression of the disease, the pigmented zones spread over the eye. After retinal damage, they appear in the central part of the organ and on the iris. Vessels are narrowed, they are difficult to detect during the inspection. At the same time, blanching of the nerve disk and its depletion are observed. Retinal pigment degeneration is diagnosed immediately on both.
How is pathology manifested?
Dispigmentation is accompanied by the following symptoms:
- Hemeralopia. The patient loses the ability to distinguish objects in the dark, deteriorates the ability to navigate in space in low light. The cause of the violation is damage to the sticks. This symptom is considered the first manifestation of the dystrophic process that appears several years before the appearance of obvious changes in the retina.
- Deterioration of peripheral vision. Immediately damaged lateral sticks, and gradually the process moves to the center. This leads to a narrowing of the zone of visibility, the patient has tunnel vision.
- Decreased sharpness and color vision. It occurs in the last stages of the development of the disease, when cones are involved in the pathological process. In the future, the patient becomes blind.
In the main group of patients, children of preschool age. In children it is difficult to detect changes in vision, because the disease remains undetected for a long time. The initial stage of development of the PDS lasts up to 5 years. If untreated, complications such as glaucoma or cataracts develop. Gradually, atrophy of the vitreous body occurs. Loss of vision on the background of pigmental dystrophy is irreversible.
A pigment spot on the retina requires immediate treatment. Otherwise, the pigments will spread through the eyeball, reducing vision. Pathological hyperpigmentation and retinal dystrophy in children younger than 6 years of age is detected when the baby is no longer able to orient properly in space, therefore, in the presence of such a disease, relatives should regularly take the child to an ophthalmologist to examine the fundus.
To make a diagnosis, the sharpness and features of the peripheral vision are checked. The doctor examines the retina for changes characteristic of pigmental dystrophy. With the development of the disease, pigments are found that are collected in dense formations and zones of dystrophic epithelium damage. The optic nerve head with hyperpigmentation of the retina is pale, and the blood vessels are narrowed. Dystrophy of eye tissue occurs in a diabetic due to constantly elevated glucose levels. To confirm the diagnosis, a series of electrophysiological studies are assigned.
If the disease manifested itself in childhood, by the age of 25 the patient’s vision is reduced to the point of loss of efficiency.
In most cases, treatment of retinal pigment dystrophy is carried out with the help of a complex use of drugs, which allow normalizing blood circulation in the eye and providing the retina with the necessary nutrition. Prescribe such means as:
- "Emoxipin". Restores microcirculation.
- "Taufon". Eye drops. Stimulate the regeneration of the tissues of the visual apparatus.
- A nicotinic acid. Normalizes metabolic processes and blood circulation in the body.
- "No-shpa" + "Papaverin". The combination of drugs provides an antispasmodic effect and a decrease in pressure in the vessels.
Retinal pigmentation degeneration is treated with photostimulation. The technique consists in provoking excitation in some areas of the eye apparatus. As a result, the development of the disease slows down. Under the influence of radiation, blood circulates better through the eyeball, but restores metabolic processes. The technique removes swelling from the shell of the optic organ, strengthens the retina.
What other treatment methods are used?
Often, drug therapy is complemented by physiotherapy, which speeds up the process of restoring the damaged inner membrane of the eye and activates the photoreceptors. In most cases, patients are prescribed ozone therapy, magnetic resonance therapy and stimulation with electrical impulses. With the defeat of the choroid of the eyeball operation is performed.
What could be the consequences of the disease?
In case of pigmentary dystrophy, symptoms occur when the retina is already damaged, therefore it is difficult to detect a disorder at the initial stage of development. The depletion of tissues leads to such complications as:
- peripheral vision loss
- deterioration in the ability to see in the dark,
- reduced visual acuity
- development of cataracts or glaucoma,
- tunnel vision
Prevention and prognosis
Since the development of pigment dystrophy is caused by genetic disorders, it is impossible to prevent the disease. In order to detect pathology, it is recommended to undergo regular physical examinations at the first stages of development, especially if there are people with such a disease in the family. To strengthen the retina and provide the eyeballs with the necessary substances, the patient must eat a balanced diet, give up bad habits. When diagnosing the advanced form of dystrophy, the prognosis is poor, the probability of blindness is high.
YOU STILL EVEN THAT RETURN A CLEAR VISION IS IMPORTANT?
Judging by the fact that you are now reading these lines - the victory in the struggle with not clear vision is not on your side.
And have you already thought about surgery? It is understandable, because the eyes are very important organs, and their proper functioning is a guarantee of health and a comfortable life. Sharp pain in the eye, misting, dark spots, foreign body sensation, dryness or, on the contrary, tearing. All these symptoms are familiar to you firsthand.
But perhaps it is more correct to treat not the effect, but the cause? We recommend reading the story of Yuri Astakhov, which he recommends. Read the article >>
The disease proceeds with manifestation first generation son from father (autosomal dominant mode of transmission) and in the second and third generation from two parents (autosomal recessive mode of transmission). The malady usually progresses in men (through the mother’s line of blood) due to the clutch on the X chromosome.
The disease can first manifest itself in the very early, youthful or adulthood. In adults, pathology subsequently provokes development of cataracts or glaucoma. Greater effect from therapy is achieved when diagnosing the disease in children.
Early on pathology patients complain of reduced vision at night, a violation of orientation in space. External manifestations of pathology is not observed.
Later around the fundus becomes noticeable pigmentationresembling bone bodies. At first them a bitbut over time the number of pigment foci is growing. The size of the rings is getting bigger, they move from the periphery to the central part of the retina. The blood vessels of the eyes narrow. This leads to discoloration of collapsing areas of the retina.
Photo 1. Fundus picture for retinal pigment dystrophy: a lot of whitish patches are noticeable.
Optic Disc atrophies and becomes lighter. The quality of vision remains the same.
Attention! Narrowing of the field of view occurs with further progression of pathology. It leads to clouding of the vitreous body and the development of glaucoma or cataracts.