Changes in the eye with congenital glaucoma include: Types and symptoms of congenital glaucoma

Glaucoma is a very serious disease characterized by an acute attack and a greenish tint to the pupil of the eye. It is also called green cataract. Full list There are no exact symptoms of glaucoma in our time, since there are always disagreements and they coincide with many other diseases.

Most often, the disease is hereditary in nature and is caused by visual impairment. In children, glaucoma usually causes enlarged and painful eyes, which leads to lethargy, photophobia and myopia. Diagnosis is best done during pregnancy, especially in people genetically susceptible to the disease.

Treatment, unfortunately, is only surgical and should be performed as quickly as possible so as not to lead to worse consequences. In this article we will talk about congenital glaucoma, its types, symptoms, developmental pathology and treatment methods.

Congenital glaucoma

Congenital glaucoma
Source: institutodavisaodelages.com.br Congenital (infantile) glaucoma is formed at the stage of intrauterine development and occurs already at birth - as a pathological condition of the visual organs or as a tendency to increase inside eye pressure.

The causes of this pathology are either a genetic factor or harmful effects on the developing fetus.

According to epidemiological statistics, more than half of children initial manifestations syndrome of increased intraocular pressure are observed and diagnosed before the age of 5 years.

Primary congenital glaucoma is a very rare pathology (1: 10,000), but occurs more often than other congenital glaucomas. Affects mainly boys (65%). In 90% of cases, the disease occurs spontaneously, in 10% there is a hereditary predisposition.

Congenital glaucoma (also called infantile) can be hereditary or result from exposure of the fetus to various adverse factors.

Congenital glaucoma is a disease in which intraocular pressure increases due to hereditary or congenital disorders in the structures of the eye through which intraocular fluid normally flows.

Normally, intraocular fluid provides metabolic processes and maintains the required pressure inside the eye.

It is formed during the filtration of blood from the capillaries of the ciliary body, part of the choroid of the eye, which is also involved in changing the shape of the lens, and flows mostly through a complex system of channels located in the corner of the anterior chamber. In this way, the constancy and regulation of intraocular pressure is maintained.

The anterior chamber angle is limited by the iris, ciliary body and cornea. The canal system is represented by the so-called trabecular meshwork, a system of very thin membranes different sizes, filtering intraocular fluid.

Congenital glaucoma most often occurs in both eyes. The first signs of the disease appear already in the first 6-12 months of life. At the same time, unfortunately, if the disease is not detected in time and timely treatment is not carried out, up to 50% of children become blind by the age of 5-7 years.

Congenital glaucoma is a severe eye disease in newborns and is a leading cause of blindness in children. It appears due to an abnormal increase in intraocular pressure during pregnancy in the fetus, and in some cases, glaucoma is accompanied by various developmental abnormalities of the child.

In some cases, glaucoma is noticed immediately after the baby is born, but most often this pathology is detected during the first year of life, since only a few weeks after birth the child begins to master the visual apparatus, and before that it is very difficult to notice visual impairments.

Primary

It is often hereditary. Signs of the disease can be found in relatives of a sick child.

The occurrence of congenital primary glaucoma is due to the fact that due to various problems during pregnancy (various infectious diseases, vitamin deficiencies, mechanical injuries, alcoholism, drug addiction and others), part of the tissue that should be absorbed in the prenatal period remains in the corner of the child’s anterior chamber.

Less common are congenital anomalies such as fusion or complete absence Schlemov's canal, proliferation of blood vessels in the corner of the anterior chamber.

Secondary

This form is already associated with some diseases or injuries directly to the eye suffered in the prenatal period:

• inflammation of the cornea, that is, keratitis,
• with a corneal ulcer,
• inflammation of the iris and ciliary body or iridocyclitis,
• eye injury, as part of a birth injury - in these conditions, outflow intraocular fluid worsens due to damage normal structure anterior chamber angle.

Due to these changes, the outflow of intraocular fluid from the eye is disrupted, while the production of fluid remains the same, as a result of which intraocular pressure increases, eye functions begin to decrease (visual acuity, peripheral visual field), and pain occurs.

Classification of congenital glaucoma

Source: MyShared.ru Dating various types congenital glaucoma:

1. hydrophthalmos, or simple congenital form;
2. glaucoma combined with certain developmental anomalies visual system;
3. congenital glaucoma as part or element of a more general, systemic congenital pathology.

• True congenital glaucoma occurs in 40% of cases, and increased intraocular pressure (IOP) begins in utero.
• Infantile glaucoma is detected in 55% of cases and diagnosed in the first 3 days of life.
• Juvenile glaucoma is the rarest type of primary congenital glaucoma. Increased IOP can be detected between the ages of 3 days and 16 years.

These changes can be mistaken for primary open-angle glaucoma. During gonioscopy, there may be no pathology, but in some cases signs of goniodysgenesis are noted.

Etiology of the disease

In the occurrence of congenital glaucoma in 80% of cases, the main role is played by a hereditary factor with transmission predominantly by an autosomal recessive type. In this case, the pathology is often combined (anomalies of both the eyeball and individual organs and systems are observed).

In some cases, the development of the disease is caused by exposure to various unfavorable factors on the fetus during its intrauterine development. Among them, measles rubella, toxoplasmosis, viral diseases, endocrine disorders, ionizing radiation, hypo- and avitaminosis play a negative role.

Pathogenesis

The pathogenesis of congenital glaucoma is varied, but the basis for increased IOP is underdevelopment or improper development of the drainage system of the eye.

Most common reasons blocking the trabecular zone and Schlemm's canal are unresolved embryonic mesodermal tissue, poor differentiation of angular structures, anterior attachment of the iris root, as well as a combination of various anomalies.

The severity of the process and the pace of its development depend on the degree of defect in the outflow tract of intraocular fluid: the more pronounced it is, the earlier the disease clinically manifests itself.

Impaired outflow of aqueous humor in congenital glaucoma is associated with a congenital structural feature of the anterior chamber angle and is not associated with other eye abnormalities. This disrupts the flow of fluid from the eye.

The reasons are the attachment of the iris directly to the surface of the trabecula, or the growth of embryonic trabecular tissue through which the intraocular fluid should flow.

Reasons

The direct cause of congenital glaucoma is, as a rule, abnormal development of the angle of the anterior chamber of the eye or defects in the formation of the drainage system.

In turn, such deviations from normal development can be caused (usually in the early stages of pregnancy) by the following factors:

1. viral infection (rubella, influenza, etc.);
2. intoxication;
3. alcoholism;
4. radiation damage.

The disease is based on congenital anomalies in the development of the angle of the anterior chamber and the drainage system of the eye, which create an obstacle to the outflow of intraocular fluid or significantly complicate it, which leads to an increase in intraocular pressure.

The reasons for this anomaly are various pathological conditions women, especially in the first months of pregnancy. They are caused by a wide variety of reasons: infections (measles, rubella, influenza, etc.), poisoning, alcoholism, ionizing radiation, etc.

In some cases, congenital glaucoma in children may be accompanied by other pathologies of internal organs, therefore, when diagnosing this disease, it is important to comprehensive examination body.

In addition to hereditary factors, the cause of pathology may be various states and maternal illnesses during pregnancy, such as rubella or chickenpox.

Abuse of alcohol, nicotine, and narcotic substances can also have an impact. Hereditary glaucoma can be passed down through generations, so if there are risk factors, it is advisable to diagnose it immediately after birth.

Signs of eye disease

Despite the natural diagnostic difficulties associated with early childhood (the inability of a young patient to follow instructions, accurately verbalize his own sensations, etc.), congenital glaucoma has quite specific and easily recognizable signs.

The leading one, of course, is increased intraocular pressure, which can be detected by tonometry (a simple ophthalmological measurement procedure). Other diagnostically significant manifestations of congenital glaucoma are:

• aggravated, noticeably painful reaction to bright light;
• constant lacrimation;
• progressive enlargement of the eyeball, with larger eyes, for human perception certainly beautiful and expressive, in this case they soon begin to be perceived as unnatural, anomalous;
• pathologically wide cornea (with severe glaucoma, its diameter reaches 2 cm or more);
• swelling, turbidity of the cornea;
• inhibition, to one degree or another, of pupillary reflexes;
• pathological changes in the optic nerve.

In many cases, congenital glaucoma develops simultaneously with other defects - both in the visual system (aniridia, cataracts, etc.) and in other systems and structures of the body ( birth defects heart, deafness, microcephaly, etc.).

Unlike many acquired forms of glaucoma, which primarily affect one eye, congenital glaucoma in most cases (up to 80%) develops in both eyes.

High blood pressure may not cause any noticeable subjective concern if there is no severe damage to the cornea, and then the diagnosis can only be established by objective methods. ophthalmological examination– based on the criteria listed above.

Over time, however, the progression of glaucoma can lead to the formation of staphylomas (protrusions) and ruptures of the sclera, stretching of the transparent membranes, etc.

Impaired blood supply and obstructed circulation of intraocular fluids can lead to optic nerve dystrophy. Cataracts are also a common complication of congenital glaucoma in the later stages.

The functional state of the visual system usually decreases quite quickly. Deterioration of vision acquires a particularly pronounced, malignant character at the stages of involvement in pathological process optic nerve and/or retinal cells.

Complicated cataracts often develop. In the initial stage of development of congenital glaucoma, the fundus is normal. As the disease progresses due to circulatory disorders, the optic nerve head undergoes dystrophic changes.

In the first 2-3 months, parents may be alarmed by the child’s restless behavior, bad dream and appetite, such children avoid bright light, slight lacrimation may occur.

The difficulty lies in the fact that the development of the child’s eye and brain occurs gradually from birth, so that only from 2 months the child has vision of the most insignificant quality, seeing only the silhouettes of objects and people.

So the decrease in visual functions, which suffer from glaucoma: visual acuity and peripheral visual field, cannot be determined during this period.

The severity of symptoms depends on the amount of intraocular pressure. With a significant increase in eye pressure, the eyeball increases in size, stretches, the cornea becomes cloudy, swollen, and the sclera, on the contrary, becomes thin due to stretching and acquires a blue tint.

Early signs of congenital glaucoma:

1. photophobia, blepharospasm;
2. restless behavior of the child;
3. slight clouding (dullness, swelling of the cornea);
4. deep anterior chamber (over 2 mm);
5. dilated pupil. Over 2 mm with a slow reaction to light.

The main symptom of congenital glaucoma is a combination of one of the above symptoms with increased intraocular pressure.

Late signs of congenital glaucoma:

• expansion and tortuosity of the anterior ciliary vessels in the sclera (“jellyfish head”, “cobra head”, emissary symptom);
• stretching everything anterior segment eyes;
• severe swelling and clouding of the cornea;
• trembling of the iris (iridodonesis);
• predominance of optic disc atrophy over glaucomatous excavation
• the appearance of axial myopia.

The presence of glaucoma can be suspected by large eyes, which look very beautiful in infants and usually do not cause concern. Most often this is a bilateral process, and the majority of patients are boys.

If the cause of the pathology is heredity, then glaucoma can occur in a child of any gender. Most often, this disease is detected in maternity hospital, but if timely diagnosis was not carried out and treatment was not started, then even before reaching school age The child will be completely blind.

Diagnostics

Source: Glaza.guru The diagnosis is made based on the origin, form, dynamics of the process, stage of the process, degree of compensation.

Differential diagnosis of congenital glaucoma is carried out from megalocornea - a large cornea (there are no other symptoms of the disease) and parenchymal keratitis. With the latter, there are characteristic changes in the cornea in the absence of other signs of the disease.

In case of congenital glaucoma in a child, when collecting an anamnesis from the mother, it is necessary to find out how restless the child is, whether he sleeps well, takes the breast, and often regurgitates food.

Necessary measures!

It is necessary to find out the impact of teratogenic factors (viral diseases, injuries, ionizing radiation, hyper- and hypovitaminosis, congenital hereditary factors) on the mother’s body during pregnancy.

The child's visual acuity is determined in accordance with his age. An examination is carried out using the method of lateral illumination, transmitted light, and intraocular pressure is determined by palpation.

You need to know that with a careful examination of the eye condition in newborns, even without special ophthalmological devices, it is possible to accurately diagnose in 90% of cases.

Using a millimeter ruler applied in the desired direction to the edges of the orbit, the size of the cornea is measured (9 mm in newborns, 10 mm in children one year old and 11 mm in children over 3 years old). Next, we identify early signs congenital glaucoma.

If you have the slightest suspicion of congenital glaucoma, you should immediately consult an ophthalmologist.

1. will check the visual functions of the eye, at least approximately.
2. will assess the level of intraocular pressure – in children early age it is checked with light finger pressure, that is, by palpation. With the help of devices it is measured in a state of sleep.
3. will examine the eye under a microscope in order to establish the correct diagnosis and, if necessary, prescribe treatment.

Differential diagnosis:

• Congenital corneal opacity
• Enlargement of the cornea like megalocornea or high myopia.
• Lacrimation as a result of delayed restoration of the nasolacrimal duct.
• Secondary infantile glaucoma

The earlier the disease is detected, the greater the chance of a full life for the child. That is why it is very important to visit doctors in a timely manner during the first year of life, as is currently the case, as this will enable early diagnosis.

In some cases, the manifestations of glaucoma are similar to conjunctivitis; photophobia and lacrimation make diagnosis difficult. They can be distinguished only by the size of the cornea and the absence of intraocular high blood pressure, therefore, it is necessary to carry out a diagnosis even if there is the slightest suspicion of glaucoma.

Clinic of congenital glaucoma

When symptoms appear early, the disease is most severe and has a poor prognosis. In children with congenital glaucoma, the first thing that attracts attention is the large and expressive initial stages) eyes.

The clinical symptoms of hydrophthalmos are influenced by the fact that the tissues of the child’s eye are easily extensible, and therefore changes occur in all its structures.

First signs

The initial symptoms of hydrophthalmos are a slight enlargement of the cornea, the appearance of cracks in Descemet's membrane, and initially transient and then persistent corneal edema.

As the disease progresses, the cornea continues to stretch, the sclera becomes thinner, acquires a bluish tint (translucent choroid), the limbus noticeably widens and the anterior chamber deepens.

Corresponding transformations also occur with the iris. Atrophic processes begin to develop in it, affecting the sphincter of the pupil. As a result, it expands and reacts sluggishly to light.

The lens usually has normal sizes, but is flattened and moves back as the anterior chamber deepens. With a significant increase in the size of the eyeball, rupture of the stretched and thinned ciliary ligaments may occur, accompanied by subluxation or dislocation of the lens.

In an advanced stage of the disease, it often becomes cloudy (cataracts develop). The fundus is initially unchanged, but then glaucomatous excavation of the optic nerve begins to develop quite quickly. At the same time, the retina stretches and thins, which in the future can lead to its detachment.

In the early stages of the disease, IOP increases slightly and periodically, and subsequently it becomes persistent.

The progression of the disease leads to a steady deterioration in visual functions, primarily central and peripheral vision. At the onset of the disease, a decrease in visual acuity is caused by corneal edema.

Subsequently, vision deteriorates due to atrophy of the optic nerve, which manifests itself as glaucomatous optic neuropathy.

For the same reason, a threshold decrease in photosensitivity occurs in the paracentral and peripheral parts of the retina, which leads to specific changes in the visual field of the affected eye.

At the same time, symptoms such as photophobia, lacrimation and photophobia are also observed. The child becomes restless, sleeps poorly, and is capricious for no apparent reason.

In terms of shape, all congenital glaucoma, both hereditary and intrauterine, are classified as angle-closure. However, the reasons for obstructing the outflow of intraocular fluid are different, which allows us to distinguish two main clinical types of the disease - A and B.

Treatment methods

The questions asked during the next “direct line” of the Zvyazda newspaper in January 2011 were answered by an associate professor of the Department of Ophthalmology of the Belarusian medical academy postgraduate education, candidate of medical sciences Galina SEMAK.

RETINAL DYSTROPHY

- Kletsk, Irina Ivanovna. I have retinal pigmentary dystrophy. What could be the treatment?
- Treatment can only be symptomatic, aimed at helping the eyes a little, maintaining vision, and improving metabolic processes in the eye. There are no radical methods, since retinal pigmentary dystrophy is a congenital pathology.
- But back in my school years, when my eyesight was checked, such a pathology was not found...
- This means that the manifestations of pathology were very weak. But I would like to draw your attention to the fact that since retinal pigmentary dystrophy is inherited, your children must be examined and observed.
- Can I pick up glasses?
- Dystrophy means that they died nerve cells retina. Of course, they can no longer work. Therefore, no glasses can restore their function. At least some pieces of glass might help you. You need to work with your ophthalmologist and be constantly monitored. Perhaps you'll pick something up.

- Stolbtsovsky district, Stepan Ivanovich. Diagnosis: retinal dystrophy of the right eye. I was given treatment, but I did not feel any improvement. Is it possible to improve my vision, where can this be done?
- In your case, it is very difficult to improve your vision, since vision is an indicator of a person’s overall health. The cause of retinal dystrophy in the right eye is most likely high blood pressure, possibly diabetes mellitus or illness thyroid gland. In other words, general pathology very often causes decreased visual acuity. Therefore, you need to be examined by a therapist, cardiologist, endocrinologist and bring all the examination data to an ophthalmologist, who will draw the right conclusions. If there are any doubts, you can seek advice from the Republican ophthalmological center 10th clinical hospital Minsk.

- Baranovichi, Tatyana Petrovna. I have hypertension, early cataracts, and am being referred for a consultation for laser photocoagulation of the retina. And the question is: does a drug like Okuwait Lutein help with the initial stages of cataracts? Or is this just prevention?
- You must understand that Okuwait Lutein is not needed to treat cataracts, but for your retina. This drug was specially developed by a number of leading countries in the world for older people. It is after 40 years of age that the retina often suffers due to arterial hypertension, and in order to preserve its pigment, Okuwait Lutein is prescribed. The drug is good and necessary.
A consultation has been scheduled for you so that laser surgeons can see whether a specific intervention is necessary, and to what extent it is needed... After 40 years, half of the adult population has initial cataracts. Therefore, there is no need to be upset here; you should take multivitamin drops and see an ophthalmologist to monitor whether the cataract is progressing. Cataracts cannot be treated conservatively - only surgically, but surgery is resorted to when the disease interferes with vision and worsens a person’s quality of life. So come to laser center and treat the retina.

- Kletsk district, Zinaida Ilyinichna. 72 years old. My vision has fallen and continues to fall, my eyes hurt.
- What is your blood pressure?
- Increased.
- Aren’t you surprised that you turned to me as an ophthalmologist, and I’m asking you about blood pressure? You must clearly understand that visual acuity is an indicator of the health of the body as a whole. If you have high blood pressure, it means that it is high in all organs and tissues, including the eyes. Therefore, the first thing you need to do is correct blood pressure. We need to go to a cardiologist and a therapist. Have you been examined by an ophthalmologist?
- He said that cataracts were developing. Drops prescribed...
- Cataracts are the result of high blood pressure, since metabolic processes in the eye occur incorrectly... Drops can and should be used, but you shouldn’t expect them to improve vision. Please take care of your blood pressure.
In Minsk regional hospital Now there is an excellent ophthalmology department. Ask the doctor at your place of residence to refer you there or to our 10th hospital in Minsk. You need to be examined.

GLAUCOMA

- Berezinsky district, Natalya. Eye pressure figures - 22 mm Hg. Could glaucoma begin to develop in such a situation? How can you determine for yourself what is coming? They showed on television how to do it yourself, how to measure the angle of view...
- Glaucoma can be detected using different ways, but what's the point of diagnosing yourself? We need to go to the doctor. Glaucoma does not affect central vision, but peripheral vision is affected. There are several methods for measuring intraocular pressure. If a pressure of 22 was shown by a non-contact tonometer, then this is not at all the same as on a contact one, according to Maklakov. However, we must keep in mind that there is also such a thing as low-pressure glaucoma, when the pathological process occurs against the background of low pressure numbers. To deal with such situations, there are glaucoma rooms today, where an ophthalmologist sends them after performing visometry, perimetry, and tonometry.

- Pruzhany, Zoya Ivanovna. My husband is 58 years old and has glaucoma in his right eye. We drip cimalon and tramadol. Are we doing the right thing?
- This is very strong drugs, which are used in the maximum treatment regimen. I believe you need laser or surgical treatment so as not to ingest such a large amount of drugs, especially since they also affect the body as a whole. Solve this issue with your ophthalmologist.
- Is there a laser in Brest?
- There must be. If it is not there, you will be referred to us.

BLEPHAROSPASM

- Dzerzhinsk, Sophia. What should I do with such an ailment as blepharospasm?
- It is very important to find out the etiology of blepharospasm. Very often it occurs if a person has chronic conjunctivitis, keratitis, if the eyes hurt and the eye seems to be protected from sunlight by squinting. And then, even with calm eyes, this blepharospasm remains. It is also possible that this increased sensitivity corneas to the light. In this case, they often help sunglasses. In this case, it is necessary to find out what the origin of blepharospasm is. Perhaps neurologists can help in the sense of improving conductivity nerve tissue and eliminate this increased sensitivity to light.

DISEASE DISEASE

- Grodno, Elena Mikhailovna. My brother has abruption disease. I lost my sight because of this. He recently had surgery on his left eye - the retina was repaired. How to save the retina and left eye?
- Where does your brother live?
- In the Vitebsk region.
- First of all, heavy physical work with the torso bent is contraindicated for your brother.
- He works as a teacher.
- Okay, but you need to remember about the weights. It is imperative to check the fundus and fundus lens once a year. If he notices that vision is decreasing, some changes or distortions appear, he needs to urgently go to a specialist.
- After cauterization of the retina, my brother’s vision began to deteriorate and a hematoma formed. Why did the blood get under the retina or on the retina?
- This is how the disease progresses. The retina ruptures, the blood vessels suffer. That’s why I say that we need to monitor all this and continue to treat it with a laser and surgical intervention.
- Maybe he needs to take some vitamins?
- There is a large arsenal vitamin complexes. If there are no specialists at your place of residence with whom you could discuss such issues, you need to drive to Vitebsk, and it is best to see one doctor.
- Can he be given a second group of vision? Now he's on his third...
- Recommendations regarding this are clearly stated, and such issues should be considered by specialists of a different kind.

ITCHY EYELID

- Minsk, Irina Nikolaevna. The eyelids around the eyes become inflamed - they itch, peel, and even cracks appear. I feel irritation around my eyelashes and itches a little. And this has been observed for several years now.
- What did you do?
- I applied hydrocortisone, sinaflan... I constantly use cosmetics. If I don’t use it, on weekends, for example, it seems to be a little better, but during the week the situation worsens.
- Have you been able to avoid using cosmetics for a longer period of time?
- Unfortunately no.
- Firstly, it is impossible to exclude an allergic component. You need to see an allergist and get tested for allergens. Hospital No. 10 has an allergy center where a therapist will refer you. The second direction of your examination is an examination by an ophthalmologist, who will look for such phenomena as blepharitis and dry eye. Finally, you yourself understand perfectly well that you need to remove the irritating factor. Choose a chic frame for yourself. We need to adapt somehow.
- What to do if eyelashes fall out?
- You need to submit your eyelashes for examination under a microscope for demodicosis. Often a mite lives on the eyelashes, and then they fall out because the follicles from which they grow suffer. The examination can be done at the dermatovenerological dispensary - on Prilukskaya or Smolyachkova.
- Is it possible to strengthen eyelashes with oils - castor oil, for example?
- You can do all this when it’s over inflammatory process. And while there is a reaction there, there is no need to strengthen it.
- Once upon a time they told me about the possibility allergic reaction, but prescribed drugs to improve digestion.
- This is right. Need to know golden rule allergy treatment: “Cold, hunger and rest.” And, of course, allergens must be removed from use. If you add paint to your face, you immediately support the diseased state.

Svetlana BORISENKO, Olga SHEVKO, newspaper “Zvyazda”, January-February 2011.
Original in Belarusian:
http://zvyazda.minsk.by/ru/archive/article.php?id=73437
http://zvyazda.minsk.by/ru/archive/article.php?id=73504
http://zvyazda.minsk.by/ru/archive/article.php?id=73605&idate=2011-02-01
http://zvyazda.minsk.by/ru/archive/article.php?id=73668&idate=2011-02-02

Congenital glaucoma is a group of diseases characterized by impaired development of aqueous humor outflow pathways, resulting in an increase in IOP. Increased IOP can occur in utero or at any time after the birth of the child. Early diagnosis and successful antihypertensive treatment may preserve visual function.

Congenital glaucoma is associated with mutations in the CYP1B1 gene, encoding cytochrome P450 1B1, and the LTBP2 gene, encoding latent-transforming growth factor beta-binding protein 2. The genetic components underlying primary open-angle or closed-angle glaucoma are largely unknown.

Epidemiology

Primary congenital glaucoma is a very rare pathology (1 in 10,000), but is more common than other congenital glaucomas. Affects mainly boys (65%). In 90% of cases the disease is sporadic, in 10% it is autosomal recessive with incomplete penetrance.

Congenital glaucoma (CH) includes diseases caused by abnormalities embryonic development eyes (primarily dysgenesis of the anterior chamber angle), caused by exposure to teratogenic factors ( x-ray studies, hypoxia, vitamin deficiency, toxicosis, infectious diseases).

• fusion or underdevelopment of Schlemm's canal,
• closure of the trabecula by the iris root or ciliary body,
• underdevelopment of trabeculae
• various other anomalies of the elements of the angle of the anterior chamber of the eye.

Classification

VG is divided into

• simple ( primary), - is not associated with any other ocular or general anomalies and diseases
  • Primary congenital glaucoma (or hydrophthalmos) manifests itself before the age of 3 years of life, inheritance is recessive (sporadic cases are possible),
  • Primary infantile glaucoma occurs in children from 3 to 10 years of age, the inheritance and pathomechanisms are the same as for primary congenital glaucoma. IOP is increased, the size of the cornea and eyeball are not changed, the excavation of the optic disc increases as glaucoma progresses.
  • Primary juvenile glaucoma occurs between the ages of 11 and 35 years (gene mutations on chromosomes 1-3, 7, 8, 10, including the TIGR myocilin gene).
• associated with other congenital ocular and general syndromes ( combined)
• secondary.

Primary infantile glaucoma

The reason for the increase in IOP is the remains of unresolved mesodermal tissue in the corner of the anterior chamber (goniodysgenesis) of varying degrees of severity. Due to the presence of an obstacle, the outflow of aqueous humor worsens (pathogenetic mechanism).

The disease develops in children aged 3 to 10 years, inheritance is usually sporadic, possibly as a recessive trait (up to 10%).

Clinical symptoms at the onset of the disease are scant: pain and corneal syndrome are not typical, the size of the cornea and eyeball are not changed.

The IOP is increased by more than 23 mm Hg, and there is an IOP asymmetry between the eyes of 4 mm Hg. and more without treatment. In the background increased IOP glaucomatous optic neuropathy develops, which is accompanied by a steady increase in the excavation of the optic disc. The ratio of excavation and disc diameters is more than 0.3.

The diameter of the cornea does not change, there is no corneal edema or buphthalmos. Gonioscopically, the open angle of the anterior chamber is determined, the structures of the APC are poorly differentiated, goniodysgenesis of varying severity and anterior attachment of the iris are observed.

Primary juvenile glaucoma

The term “juvenile glaucoma” indicates the age of the patient at the onset of the disease (from 11 to 35 years) and does not contain information about the nature of this disease (abnormalities in chromosome 1 and TIGR).

The second or third decade of life is the time of manifestation of congenital simple glaucoma and congenital glaucoma associated with defects in eye development (Axenfeld-Rieger, Frank-Kamenetsky, Peters, Marfan, Sturge-Weber syndromes, etc.). In these cases there is a characteristic clinical picture anterior segment of the eye and diagnosis does not cause difficulties. In cases early development In primary open-angle glaucoma (POAG), dystrophic changes in the anterior segment of the eye are observed (pigment imbibition or sclerosis of the trabecular meshwork and Schlemm’s canal).

In recent years, there has been an increase in the number of patients with clinical manifestations of glaucoma (glaucomatous triad) without visible anatomical reasons for increased intraocular pressure. In these cases, primary juvenile glaucoma (PJG) has a long latent period, masked by progressive acquired myopia. The unstable hypotensive effect of drug-induced reduction of intraocular pressure (IOP) in young patients suggests that surgery remains the main type of treatment.

To date, the etiology of this disease unknown. Presumably, the reasons for the increase in IOP may be a deterioration in the outflow of aqueous humor due to trabeculopathy, which in some cases is combined with elements of goniodysgenesis.

The clinical symptoms of primary juvenile glaucoma differ significantly from the manifestations of congenital glaucoma and are very similar to primary open-angle glaucoma. The size of the cornea and eyeball, as a rule, is not changed, there is no photophobia, lacrimation and symptoms associated with stretching and swelling of the cornea.

Early manifestations of PJH include: a decrease in the thickness of the peripapillary nerve fiber layer, an increase in the ratio of the excavation diameter to the disc diameter, an increase in the ratio of the excavation area to the area of ​​the optic nerve disc, and a decrease in the area of ​​the neuroretinal rim. In patients with PJH, variability was revealed; in most cases, in the temporal sector, the volume of the neuroretinal girdle and the thickness of the peripapillary nerve fiber layer (RNFL) decreased to 100 ± 2 μn (normally 116 ± 8 μn).

The maximum IOP during the Maklakov study exceeds 25 mm Hg. without antihypertensive treatment, IOP asymmetry between fellow eyes is more than 4 mm Hg.

Changes in the optic disc are characteristic of glaucoma. Typically diffuse damage to the neural rim of the optic disc and RNFL. Gonioscopically, a wide angle of the anterior chamber is determined, in some cases with elements of goniodysgenesis. With PJG, compared with POAG, degenerative-dystrophic changes in the endothelium of the drainage system of the eye develop faster (relative to the stages of the process), there is no pigment imbibition of the outer wall of the scleral sinus, there is no cellular infiltration of the JCT and sclera, metachromasia is pronounced structural components and intense metabolic processes connective tissue. Thus, there is reason to consider connective tissue pathology to be the leading factor in the pathogenesis of PJH.

There are three degrees of severity of goniodysgenesis:

• I degree - the angle is open, the ciliary body is visible, above it is a delicate grayish veil of unresolved mesenchymal tissue.
• II degree - the iris is attached at the level of the posterior Y3 trabecular zone, the space between the root of the iris and the anterior limiting ring of Schwalbe is filled with semi-translucent grayish tissue (on light irises) or a continuous layer in the form of Barkan’s membrane (on dark irises).
• III degree- the iris is attached at the level of the middle of the trabecula or closer to the anterior (at the level of the anterior limiting ring of Schwalbe).

According to the course of the disease, they are distinguished:

• typical- a clear clinical manifestation develops at the age of 3-4 months, which indicates a pronounced stretching of the anterior part of the eye with the occurrence of corneal syndrome;
• malignant- an advanced stage of glaucoma is detected already at the birth of a child, or hydrophthalmos rapidly progresses in the first 1-2 months of his life. The process is usually bilateral with a pronounced enlargement of the eyeball and clouding of the cornea;
• benign - clinical manifestations develop slowly, more often between the first and second years of life, the enlargement of the eye is slight (an intermediate option between primary congenital and primary infantile form glaucoma);
• abortifacient- IOP spontaneously normalizes and the progression of the process stops.

There are stages of glaucoma:

• initial, in which the size of the cornea of ​​the eye is increased slightly, the optic nerve is not changed, and vision is preserved. The initial and advanced stages of PJH are associated with polymorphic changes in the outer wall of Schlemm's canal and the adjacent JCT.
• advanced stage. At the same time, the dimensions of the eye and cornea are increased by 3 mm. Initial changes occur in the optic nerve head, and visual acuity is reduced by half.
• advanced stage. At this stage, vision is reduced to light perception, the size of the eye is increased by 4 mm, and changes in the optic nerve are pronounced. Dystrophic-degenerative processes dominate in the absence of compensatory proliferative reactions.
• terminal stage. It is characterized by a sharp increase in the size of the eye and complete blindness. Intraocular pressure may be normal, moderately elevated, or high above 33 mmHg. Art.

Secondary congenital glaucoma

Secondary congenital glaucoma differs significantly from adult glaucoma in the variety of forms and is a consequence of other diseases.

Secondary congenital ophthalmic glaucoma (SCOG) is caused by abnormalities in the development of the anterior segment of the eye:

• Aniridia in 50% of cases can be complicated by an increase in IOP. Most often, glaucoma manifests itself in adolescence. Children with aniridia should be under dispensary observation, with systematic IOP monitoring
• ectopic lens often results in the root of the iris being pressed against the trabecula or cornea by a displaced lens, causing glaucoma;
• Rieger's syndrome - mesodermal dysgenesis of the iris and cornea, hereditary disease with a dominant type of transmission. The syndrome includes hypoplasia of the anterior layer of the iris, embryotoxon, mesodermal bridges extending from the basal part of the iris to the embryotoxon. Glaucoma usually develops after the first decade of life, and therefore the eyeball usually does not enlarge;
• Frank-Kamenetsky syndrome is observed in men, but is transmitted to a recessive, X-linked type. A distinctive feature of this iris hypoplasia is its two-colored appearance: the pupillary zone of 1.5-2 mm has a grayish or blue or brown color, the wider ciliary zone looks chocolate brown due to the exposure of the pigment layer. Glaucoma develops in the second decade of life.

Secondary congenital syndromic glaucoma (SCG):

• Sterge-Weber syndrome (encephalooculofacial hemiangiomatosis, encephalotrigeminal angiomatosis). The etiology is not fully understood, but heredity is of great importance. A systemic disease manifested by capillary hemangioma of the face, hemangioma and focal changes in the brain. A characteristic feature is capillary hemangioma faces along the vegvey trigeminal nerve in the form of extensive purple spots.
  Depends on the degree of intracranial angiomatosis neurological symptoms, which can manifest itself as epilepsy, hydrocephalus, paresis, mental disorders, mental retardation. Glaucoma develops on the side of the nevus when the eyelids, especially the upper, and conjunctiva are affected. Such patients require systematic monitoring of IOP;
• neurofibromatosis. Congenital glaucoma can be observed in its generalized form with damage to the skin, bones, brain, endocrine system and especially when localized in the area upper eyelid and temple;
• Marfan syndrome, Marchesani - family-hereditary diseases affecting connective tissue, cardiovascular, musculoskeletal, endocrine and other body systems, accompanied by ectopia of the lens and, as a consequence, secondary glaucoma.

Secondary acquired glaucoma (SAG) - this type of glaucoma develops after diseases or injuries of the eye. The immediate cause of increased IOP in all forms of secondary glaucoma is a violation of the outflow of aqueous humor from the eye. Large quantity various forms Secondary glaucoma can be combined into several groups:

• uveal post-inflammatory glaucoma - increased IOP is caused by the formation of goniosynechia, accumulation of exudate in the trabecular zone, fusion and fusion of the pupil.
• phacogenic glaucoma; Phacogenic glaucoma occurs due to diseases of the lens:
  • phacotopic glaucoma is caused by a change in the position of the lens when it is subluxated or dislocated;
  • swelling of the cataract can also cause impaired hydrodynamics and increased ophthalmotonus - phacomorphic glaucoma;
  • overripe cataract causes phacolytic glaucoma due to the resorption of its masses and the macrophages involved in this close the trabecular membranes
• Vascular glaucoma - often develops after thrombosis of the central retinal vein, diabetes. Neovascularization in the APC impedes the drainage of fluid from the eye;
  Phlebohypertensive glaucoma occurs when pressure in the venous vessels of the orbit increases due to vascular tumors or compression of the superior vena cava
• traumatic glaucoma - divided into contusion and wound. In case of contusion from a hydrodynamic shock, the trabecular apparatus is often damaged and the UPC is split, which complicates the outflow of fluid from the eye. Traumatic glaucoma is caused by adhesive process in the Code of Criminal Procedure.
• glaucoma caused by degenerative processes - occurs with uveopathy, phakomatoses, old detachment retina, with retinopathy of various origins
• neoplastic glaucoma - develops with intraocular tumors, when their decay products impede the outflow of fluid from the eye.

Pathogenesis

Impaired outflow of aqueous humor in primary congenital glaucoma is associated with a congenital structural feature of the aqueous humor and is not combined with other ocular anomalies (isolated trabeculodysgenesis). Clinical signs of trabeculodysgenesis include attachment of the iris directly to the surface of the trabecula in the absence of signs of angle recession.

There are two varieties:

• flat attachment of the iris - it sharply passes from the horizontal surface to a thickened trabecula or is attached anterior to the scleral spur.
• concave attachment of the iris (less common) - the superficial tissue of the iris covers the area of ​​​​the junction of the iris and trabecula, while the structures of the UPC are covered with a dense mesh tissue of the iris like a sheet.

According to the localization of retention in congenital glaucoma, one should distinguish:

• pretrabecular open-angle glaucoma, which comes in first place in terms of frequency of occurrence (62%). The angle is open, but mesodermal tissue is defined pretrabecularly;
• pretrabecular angle-closure glaucoma (14.7%), which may be caused by closure of the trabecula by the root of the iris, the ciliary body;
• trabecular glaucoma, which may be caused by underdevelopment of trabeculae, sclerosis, its absence, pathological inclusion of ciliary muscle fibers in the trabecula;
• intrascleral retention, which occurs in the absence, deformation, dislocation of Schlemm's canal, with underdevelopment of the scleral spur, intramural outflow tracts, up to their complete absence.

Hydrophthalmos occurs before the age of three years, but more often appears in the first year of life (up to 50-60%) and in most cases (3/4) is bilateral.

Clinical picture

The clinical manifestations of the disease, the severity of its course and partly the prognosis depend on the severity of goniodysgenesis.

Features depend on age and IOP level. Corneal opacification is associated with edema of the epithelium and stroma as a result of increased IOP.

Clinical manifestations

• lacrimation and blepharospasm.
• by 2-3 months, cranial innervation improves and photophobia appears due to irritation of the corneal nerves
• young children become restless and capricious.
• the horizontal diameter of the cornea is slightly larger than the age norm. You can focus on the following dimensions: the diameter of the cornea in newborns is 9-9.3 mm; at 1 year - 10-10.5 mm; at 2-3 years - 10.5-11 mm. The depth of the anterior chamber in newborns is 1.5-2 mm; at 1 year - 2.5 mm; at 2-3 years - 3-3.5 mm. After 6 years, these data approach the size of an adult human eye - the diameter of the cornea is about 11.5 mm, the depth of the anterior chamber is about 3.5 mm;
• the anterior chamber deepens, the ligament of Zinn is stretched, followed by subluxation of the lens.
• with an increase in IOP, the limbus begins to stretch (expand), since in this place outer shell thinned and fragile. The limbus becomes wider than 1 mm;
• the anterior ciliary vessels dilate;
• The sclera stretches and the choroid shines through it - the sclera receives a delicate bluish tint
• a gentle (like morning fog) swelling of the cornea appears - opalescence. Stretching of the cornea leads to cracks in the endothelium and leakage of fluid into its thickness. Normally, in 15% of cases, newborns experience physiological opalescence of the cornea, which disappears within 1 week. For differential diagnosis a 5% glucose solution or glycerin is instilled into the child’s eye - the pathological edema disappears, physiological opalescence remains;
• ruptures of Descemet's membrane occur due to stretching of the cornea and are associated with saturation of the stroma with aqueous humor. Chronic stromal edema can lead to scar formation. Haab's striae are horizontal broken lines of the edges of Descemet's membrane.
• the pupil dilates and its sluggish reaction appears due to muscle atrophy
• a shift of the vascular bundle is noted in the fundus
• An increase in the anteroposterior axis of the eye can cause axial myopia and the formation of anisometropic amblyopia.

IN late stages diseases, scarring of the stroma occurs and persistent haze cornea. Also characteristic is an increase in the depth of the anterior chamber, atrophy of the iris stroma with exposure of its radial vessels.

Excavation of the optic nerve head in newborns may regress with normalization of IOP. Normally, in most of them, the excavation is not determined; in some, the excavation/disc ratio reaches 0.3 (in contrast to the high percentage of this ratio in congenital glaucoma).

The scleral canal in newborns increases along with the enlargement of the eyeball, and the thin cribriform plate bends posteriorly as IOP increases. Increased excavation size may be the result of progressive neuronal loss, widening of the scleral ring, or a combination of these reasons. In congenital glaucoma, the disc excavation quickly increases in size and becomes deep, the E/D ratio is >0.3. However, at the onset of the disease, excavation of the optic disc can be reversible and decrease with a decrease in IOP.

In the advanced stage of the disease, the eyeball and especially the cornea are significantly enlarged, the limbus is stretched, poorly contoured, the cornea is cloudy, often overgrown with blood vessels. This condition is called bull's eye (buphthalmos). Overstretching and rupture of the ligaments of Zinn lead to iridodonesis and subluxation of the lens. In the blind eye, due to trophic disturbances, corneal ulcers and hyphemas often occur. Perforation of a corneal ulcer or rupture of the thinned membranes of the eyeball may occur (with buphthalmos, the eyes are highly susceptible to injury). Optic nerve atrophy leads to irreversible blindness.

Diagnostics

Diagnosis is carried out under intravenous anesthesia, but it must be remembered that some drugs can lower IOP.

In children under three years of age, IOP when examined according to Maklakov does not exceed 22-23 mm Hg. With glaucoma, IOP can reach 40 mmHg. and higher.

The horizontal diameter of the cornea in a healthy newborn is 10 mm, increasing to 11.5 mm by the 1st year and to 12 mm by the 2nd year. In patients with congenital glaucoma, the diameter of the cornea already in the first year of life is increased to 12 mm or more, the thickness of the cornea is reduced and the radius of its curvature is increased. Measuring the diameter of the cornea in the horizontal and vertical meridians is performed using calipers. Diameter >11 mm before age 1 year or >13 mm at any age is considered suspicious. A diameter of 14 mm is characteristic of severe buphthalmos.

An approximate assessment of the optic disc without ophthalmoscopy is possible: an increase in the E/D diameter ratio by 0.2 corresponds to an increase in corneal diameter by 0.5 mm.

The length of the anterior-posterior axis of the eye of a newborn varies from 17 to 20 mm, reaching 22 mm by the end of the 1st year of life. With glaucoma, the size of the eyeball increases, sometimes quite significantly, but can be within normal values. It should be noted that changes in corneal diameter have higher value in the diagnosis of primary congenital glaucoma and assessment of the stage of the disease than increasing the length of the eye axis.

Diagnosis of congenital glaucoma

Carrying out a diagnostic study includes specific list examinations: checking intraocular pressure (tonometry);

frontal diagnostics eye camera(goniosopia);

analysis of the condition of the cornea (keratometry);

eye assessment using a slit lamp (biomicroscopy);

checking the fundus using a special instrument (ophthalmoscopy);

examination of the structure of the eye and collection of data about them through an ultrasound machine (ultrasound biometry);

examination for changes at the genetic level.

Can be noted in the diagnosis of congenital glaucoma clinical guidelines, developed by the Ministry of Health.

How to treat the disease

• First of all, to relieve the symptom of increased intraocular pressure, they can be prescribed drops for congenital glaucoma.

This treatment method is quite conservative and not particularly effective. But, nevertheless, drug treatment allows you to reduce the production and increase the outflow of fluid from the eye.

It is necessary to understand that this treatment regimen is determined by the attending physician depending on individual characteristics each patient, taking into account his other diseases. This is because hypersensitivity to any prescribed drug may occur. In this case, as a result of application, a burning sensation, discomfort occurs, redness appears in the eyes, it is also possible headache, increased frequency heart rate(arrhythmia). If these signs occur, you should immediately consult a doctor to prescribe another medicinal product or simply for advice on how to reduce the complications that appear after taking it.

The ophthalmologist also needs to know what chronic diseases the patient has. This is due to the fact that certain eye drops have serious contraindications for diabetics, asthmatics, people with chronic diseases respiratory organs, heart, etc. Those with similar diseases are mainly prescribed betaxolol (betoptik).

• Comprehensive drug treatment of glaucoma also includes drugs, aimed at normalizing blood circulation in the brain, accelerating metabolic processes, trental, vinpocetine, cavinton, as well as vitamin complexes.

There are situations when the patient has already been prescribed these medications by a neurologist or therapist, then the attending ophthalmologist can adjust the treatment.

• At the advanced stage of congenital glaucoma it can be used physiotherapy. For example, stimulating procedures for the death of the optic nerve and retina using electric current, laser, light exposure or even magnetic field. Such methods can be significantly effective, and, in addition, they are completely harmless to patients.

• But treatment with eye drops every day at a certain time has one big drawback - a deterioration in the quality of life of patients with glaucoma. However, often the drops may not help at all in treating the disease.

Therefore, if medicines turned out to be useless, it is recommended performing laser and surgical operations.

Completely safe, operations are carried out in medical institutions and are very important for the full and successful treatment congenital glaucoma.

The most optimal treatment in the fight against congenital glaucoma, which is most effective, is surgery. A child suffering from congenital glaucoma should be operated on as early as possible.

Treatment surgically is aimed at restoring the functionality of the visual organs. The surgeon performing the operation creates new or restores old methods of outflow of intraocular moisture, thereby rehabilitating the features of the normal anatomy of the eye.