Congenital glaucoma

Congenital glaucoma is genetically determined (primary congenital glaucoma), it can be caused by diseases or injuries of the fetus during embryonic development or during childbirth.

Elevated intraocular pressure in a child can be detected at birth, develop in the first weeks, months, but sometimes several years after birth.

[1], [2], [3], [4], [5], [6], [7]

Causes of congenital glaucoma

Congenital glaucoma is classified into primary, combined and secondary. Depending on the age of the child, the early congenital glaucoma that occurs in the first three years of life, the infantile and juvenile glaucoma, which manifests itself later in childhood or adolescence, are distinguished.

Primary early congenital glaucoma is diagnosed in 80% of cases of congenital glaucoma. Most of the disease manifests itself in the first year of a child's life.

As a rule, both eyes are affected, but to different degrees. Boys are more common than girls. The disease is associated with heredity. For the development of the eye, certain genes, mutations, including glaucoma and other genetic defects, are responsible. But children may also have sporadic cases that do not have a hereditary predisposition to the development of congenital glaucoma.

The increase in intraocular pressure is caused by the disruption of the formation of the anterior chamber angle and the trabecular network during intrauterine development, so these children are disturbed by the outflow of aqueous humor, which contributes to intraocular pressure.

Depending on the level of intraocular pressure, sooner or later, that is, within weeks, months and even years, glaucoma lesions develop. The mechanism of their development is the same as in adults, but in children there is an increase in the size of the eyeballs, due to greater extensibility of the sclera.

Stretching is also subject to the cornea, which can lead to small ruptures that cause opacity of the cornea. This can occur with a decrease in intraocular pressure. As a result of damage to the optic nerve or corneal opacity in children with congenital glaucoma, visual disturbances are observed.

[8], [9]

Children's glaucoma, or infantile congenital glaucoma

Infantile congenital glaucoma occurs at the age of 3-10 years. The cause of increased intraocular pressure is basically the same as with congenital glaucoma. However, this happens later, since the angle of the anterior chamber is more formed than with congenital glaucoma, the outflow of aqueous humor is normal, so intraocular pressure may be normal during the first years of life and only later will gradually increase.

There are some clinical differences between infantile congenital glaucoma and primary congenital glaucoma. The cornea and the eyeball are of normal size, there are no symptoms such as lacrimation, photophobia and corneal opacity. This type of glaucoma is diagnosed in a routine examination or when the child is examined sightingly due to the presence of glaucoma in the genus. In some children, glaucoma is accompanied by impaired vision and strabismus (strabismus). This type of glaucoma is often a hereditary disease. With increasing intraocular pressure in children's glaucoma, the same changes occur that adults with glaucoma: excavation of the optic nerve disk and narrowing of the visual fields. The size and depth of the excavation of the disc may decrease with the normalization of intraocular pressure. As a rule, children have normal blood circulation, so the prognosis of their disease is favorable provided that the intraocular pressure returns to normal.

[10], [11], [12], [13]

Juvenile glaucoma

With juvenile glaucoma, increased intraocular pressure occurs in older children or adolescents, is often hereditary and is combined with myopia. Increase in intraocular pressure is due to underdevelopment of the angle of the anterior chamber and trabecular tissue. Symptoms of the disease and treatment methods are the same as in open-angle primary glaucoma in adult patients.

It should be noted that children may suffer from other forms of glaucoma, such as secondary glaucoma as a result of trauma or inflammation.

Combined congenital glaucoma

Concomitant congenital glaucoma has much in common with primary congenital glaucoma. It develops due to underdevelopment of the angle of the anterior chamber and the drainage system of the eye. Congenital glaucoma is often combined with microcornea, anhy- dria, Morphan syndrome and Marcuseia, as well as with syndromes caused by intrauterine infection with the rubella virus.

[14], [15], [16], [17], [18], [19]

Secondary congenital glaucoma

The causes of secondary congenital glaucoma are trauma and uveitis, retinoblastoma, juvenile xanthogranulem, intraocular hemorrhage. With retinoblastomy and fibroplasia, there is a closed-angle glaucoma and a shift in front of the iridochrom and the diaphragm. With juvenile xanthogranuloma, a yellowish pigment is broken off in the iris.

Diagnosis of congenital glaucoma

The diagnosis of congenital glaucoma can be suspected if the child has specific signs and symptoms.

First of all, these are enlarged eyes. Often there are intense lacrimation, photophobia, sclera hyperemia.

Examination in newborns and young children is more difficult to perform than in adults. If there is a suspicion of glaucoma, complete diagnosis is necessary under general anesthesia. It is necessary to measure intraocular pressure, to conduct a survey of all parts of the eye, in particular, the disc of the optic nerve. For the primary congenital glaucoma characterized by a deepening of the anterior chamber and atrophy of the iris. Excavation of the optic disc develops rapidly, but at first it is reversible and decreases with a decrease in the eye pressure. In the late stage of the disease, the eyes and especially the cornea are enlarged in size, the corneal limb is stretched, the cornea is turbid, sprouted with vessels, and then a perforating ulcer of the cornea may form.

[20], [21], [22], [23], [24], [25], [26]

Treatment of congenital glaucoma

Treatment of congenital glaucoma is determined by the severity of the disease. With a moderate disease, therapy can begin with a decrease in intraocular pressure with eye drops. But the treatment of congenital glaucoma with drugs is ineffective. In order to reduce intraocular pressure, a surgical procedure is required.

Forecasts are satisfactory only with a timely surgical operation. If the operation is carried out at the initial stage of the disease, vision remains lifelong in 75% of patients and only 15-20% of late operated patients.