Congenital glaucoma

Congenital glaucoma is one of the most severe types of eye pathology in newborns, leading early to blindness. The disease is relatively rare, its frequency is 1 case per 10,000 newborns. However, congenital glaucoma is the cause of vision loss in 2.5-7% of children, ie, almost every 10th child is blinded from congenital glaucoma. In 15% of patients, the disease is hereditary( familial) or due to various disorders during the period of fetal development. For the most part, the disease affects the boys. The ratio of boys and girls with primary congenital glaucoma is approximately 3: 2. In general, congenital glaucoma refers to the pathology of the first year of life of the child, since by this age it is manifested in more than 90% of cases.

With congenital glaucoma, there are gross signs of impaired vision, so it shows up early and progresses quickly, leading to a permanent loss of vision within 2-3 weeks. In this regard, a special role is played by early diagnosis and early surgical treatment, which is aimed at removing obstacles to the flow of the eye fluid and creating artificial outflow pathways.

Early diagnosis is critical for successful treatment. However, it is possible only with a good knowledge of the early signs of the disease by obstetricians, neonatologists and mikropediatrs of maternity hospitals, district pediatricians and nurses who carry out patronage of young children at home. This is due to the fact that a children's ophthalmologist examines all children in order to exclude congenital pathology only at 2-4 months of age.

Hereditary glaucoma is noted in 15% of cases of this disease. It is transmitted by an autosomal recessive type of inheritance and is often combined with other anomalies of the eye. Intrauterine glaucoma develops under the influence of such factors as frequent radiographic studies, chronic intrauterine hypoxia, beriberi, toxicosis, infectious diseases.

In the normal organ of vision, there are two cameras: the anterior and posterior chambers of the eye. These cells contain a liquid, which is a refractive medium for focusing all the rays that fall into the human eye, to create a certain picture of visibility. Intraocular fluid constantly circulates, that is, there is a drainage system in the organ of vision. The liquid is constantly formed in the vascular plexus of the eye, and the excess flows down through certain structures of the anterior and posterior chambers of the eye. With glaucoma, there are violations that lead to a violation of outflow of fluid, its excessive accumulation and increased intraocular pressure. Congenital glaucoma is the result of improper and incomplete cleavage of tissues in the corner of the anterior chamber during intrauterine development. At this point, the path of outflow of the eye fluid should normally be located. Anomalies of the anterior chamber angle with congenital glaucoma are clearly pronounced. Changes in the angle of the anterior chamber can be very diverse, up to its complete non-cleavage and the absence of many links in the outflow pathways. Such strong changes in the drainage system do not leave any hope for the success of medical treatment and require urgent surgical intervention.

The stages of glaucoma are indicated by Roman numerals, and they are classified according to certain criteria. The outer shell of the eye in children is thin, elastic, so the eye enlargement becomes the first and important symptom of the development of glaucoma. It is easier for children to control visual acuity than the field of vision, so it is the second criterion. The third criterion for the development of the glaucoma process is the changes in the optic nerve.

Intraocular pressure is the second characteristic of the pathological process in glaucoma. It can be normal, moderately elevated and high. Normal intraocular pressure is up to 27 mm Hg. Art. Moderately elevated ranges from 28-32 mm Hg. Art. High intraocular pressure is 33 mm Hg. Art.and more.

The next characteristic of the process that occurs with glaucoma is its stability. Stable glaucoma includes cases without eye enlargement, loss of vision, negative dynamics in the optic nerve, based on the results of systematic observations. In the absence of negative dynamics in 6 months or more, the process is considered to be stabilized. Unstable glaucoma includes cases with negative dynamics: marked increase in eye size, decreased vision, narrowing of the field of vision.

With congenital glaucoma, the outflow of intraocular fluid is greatly hampered, the fluid accumulates in excess, the intraocular pressure rises. The sharply increasing load on the outer shell of the eye causes it to stretch. The eyes of the child at the beginning of the disease are beautiful: large, expressive, the anterior chamber of the eye deepens, the sclera from stretching becomes bluish. With prolonged stretching, the eyeball sharply increases( "bull's eye"), becomes turbid, the sclera sharply becomes thinner and sticks out unevenly in the form of a staphylus. Blind eyes swing in nystagmus.

This disease requires urgent surgical treatment.

Secondary congenital glaucoma is different from glaucoma in adults in a variety of forms and is the result of other diseases.

Secondary congenital ophthalmological glaucoma with anterior developmental anomalies:

1) aniridia( absence of iris) in 50% of cases may be complicated by increased intraocular pressure. Glaucoma manifests itself often in adolescence. Children with aniridia should be under clinical supervision with regular monitoring of intraocular pressure;

2) ectopia( wrong position) of the lens often leads to the development of glaucoma;

3) Rieger's syndrome is an underdevelopment of the iris and cornea. This is a hereditary disease that is transmitted by a dominant type of inheritance. Glaucoma develops, as a rule, after the first decade of life, in connection with which the eyeball does not usually increase;

4) Franck-Kamenetsky syndrome is observed only in men. The disease is transmitted through a recessive, sex-linked type of inheritance. With this anomaly the iris is two-color: the area around the pupil is grayish, blue or brown, and the peripheral part( wider) looks chocolate brown. Glaucoma develops in the second decade of life.

Secondary congenital syndrome glaucoma:

1) Stirge-Weber syndrome. The cause of the development of this disease is not clear until the end, but a great place is given to heredity. The syndrome is manifested by capillary tumors( hemangiomas) of the face, vascular and focal changes in the brain. Capillary hemangioma of the face is localized along the branches of the trigeminal nerve in the form of extensive crimson spots.

Neurological symptoms depend on the degree of development of vascular tumors in the brain. Possible epilepsy, hydrocephalus( accumulation in the cavity of the skull of excess fluid), paresis, mental disorders, mental retardation. Glaucoma develops on the side of the birthmark when the lesions of the eyelids, especially the upper, and conjunctiva. Such people need systematic monitoring of intraocular pressure;

2) neurofibromatosis. Congenital glaucoma can be with its generalized( most severe) form, which occurs with the defeat of the skin, bones, brain, endocrine system and especially when located in the upper eyelid and temple;

3) Marfan syndrome and Markesei syndrome are genetically caused diseases that occur with the defeat of connective tissue, cardiovascular, musculoskeletal, endocrine and other body systems. Accompanying the wrong position of the lens and, as a result, secondary glaucoma.