Congenital glaucoma

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

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

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Causes of congenital glaucoma

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

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

Typically, both eyes are affected, but to varying degrees. It is more common in boys than in girls. The disease is hereditary. Certain genes, mutations, including glaucoma and other genetic defects are responsible for the development of the eye. However, sporadic cases may also occur in children who do not have a hereditary predisposition to the development of congenital glaucoma.

Increased intraocular pressure is caused by a disruption in the formation of the anterior chamber angle and trabecular meshwork during intrauterine development, which is why such children have a disruption in the outflow of aqueous humor, which contributes to intraocular pressure.

Depending on the level of intraocular pressure, sooner or later, i.e. over 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 the greater elasticity of the sclera.

The cornea is also subject to stretching, which can lead to small tears that cause corneal clouding. This may resolve with a decrease in intraocular pressure. Children with congenital glaucoma experience visual impairment as a result of damage to the optic nerve or corneal clouding.

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Childhood 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 in congenital glaucoma. However, it occurs later, since the angle of the anterior chamber is more developed than in congenital glaucoma, the outflow of aqueous humor is normal, so intraocular pressure can be normal during the first years of life and only later will it gradually begin to increase.

There are some clinical differences between infantile congenital glaucoma and primary congenital glaucoma. The cornea and eyeball are of normal size, there are no symptoms such as lacrimation, photophobia and corneal opacity. This type of glaucoma is diagnosed during a routine examination or when a child is examined specifically due to the presence of glaucoma in the family. In some children, glaucoma is accompanied by visual impairment and strabismus (crossed eyes). This type of glaucoma is often a hereditary disease. With an increase in intraocular pressure in childhood glaucoma, the same changes occur as in adult patients with glaucoma: excavation of the optic disc and narrowing of the visual fields. The size and depth of the excavation of the disc may decrease with normalization of intraocular pressure. As a rule, children have normal blood circulation, so the prognosis for their disease is favorable, provided that the intraocular pressure returns to normal values.

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Juvenile glaucoma

In juvenile glaucoma, the increase in intraocular pressure occurs in late childhood or adolescence, is often hereditary and is combined with myopia. The increase in intraocular pressure is due to underdevelopment of the anterior chamber angle and trabecular tissue. The symptoms of the disease and treatment methods are the same as for open-angle primary glaucoma in adult patients.

It should be noted that children can also suffer from other forms of glaucoma, such as secondary glaucoma due to injury or inflammation.

Combined congenital glaucoma

Combined congenital glaucoma has much in common with primary congenital glaucoma. It develops due to underdevelopment of the anterior chamber angle and the drainage system of the eye. Congenital glaucoma is often combined with microcornea, anhydria, Morfan and Marchesia syndromes, as well as syndromes caused by intrauterine infection with the rubella virus.

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Secondary congenital glaucoma

The causes of secondary congenital glaucoma are trauma and uveitis, retinoblastoma, juvenile xanthogranuloma, intraocular hemorrhages. With retinoblastoma and fibroplasia, closed-angle glaucoma and anterior displacement of the iris-lens diaphragm occur. With juvenile xanthogranuloma, the yellowish pigment in the iris breaks off.

Diagnosis of congenital glaucoma

The diagnosis of congenital glaucoma can be suspected in the presence of specific signs and symptoms in children.

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

Examination of newborns and young children is more difficult than that of adults. If glaucoma is suspected, a full diagnosis under general anesthesia is necessary. Intraocular pressure should be measured, all parts of the eye should be examined, in particular the optic disc. Primary congenital glaucoma is characterized by a deepening of the anterior chamber and atrophy of the iris. Excavation of the optic disc develops quickly, but at first it is reversible and decreases with a decrease in intraocular pressure. In the late stage of the disease, the eye and especially the cornea are enlarged, the corneal limbus is stretched, the cornea is cloudy, overgrown with vessels, and a perforating corneal ulcer may subsequently form.

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Treatment of congenital glaucoma

Treatment of congenital glaucoma is determined by the severity of the disease. In case of moderate disease, therapy can be started by lowering intraocular pressure with eye drops. But treatment of congenital glaucoma with medications is ineffective. In order to reduce intraocular pressure, surgical operation is required.

The prognosis is satisfactory only if the surgery is performed in a timely manner. If the surgery is performed at the initial stage of the disease, vision is preserved throughout life in 75% of patients and only in 15-20% of late operated patients.