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Eye diseases. Congenital glaucoma (most important)

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50. Congenital glaucoma

There is hereditary congenital glaucoma (about 15% of cases) and intrauterine (about 85% of cases), which occurs as a result of exposure to various pathological factors on the fetal eye, which is a consequence of malformations of the anterior part of the eye. An increase in intraocular pressure occurs due to a violation of the outflow of intraocular fluid due to the closure of the iridocorneal angle of the anterior chamber by unresolved embryonic mesodermal tissue. Less common causes of aqueous humor retention are the anterior attachment of the iris and intratrabecular and intrascleral changes.

Congenital glaucoma manifests itself in three forms: simple (actual hydrophthalmos) with changes in the angle of the anterior chamber of the eye (the most common); congenital glaucoma with anomalies in the anterior part of the eye or the entire eye (aniridia, ectopia of the lens, microphthalmos, etc.); congenital glaucoma with phakomatoses (angiomatosis, neurofibromatosis).

Often, congenital glaucoma appears in newborns or in the first six months of a child's life, as well as in the first year of life. Congenital glaucoma is characterized by a progressive course. There are the following stages of the disease: initial, advanced, advanced, almost absolute and absolute. According to the state of intraocular pressure, it is possible to distinguish compensated, uncompensated and decompensated congenital glaucoma.

The onset of the disease is manifested by photophobia, lacrimation, dullness of the cornea; the length of the sagittal axis of the eye and the diameter of the cornea are normal or slightly enlarged. An increase in the length of the sagittal axis of the eye, the diameter of the cornea and an increase in corneal edema occur in the advanced stage due to further stretching of the membranes of the eyeball. There are ruptures of the Descemet's membrane and clouding of the cornea.

The anterior chamber becomes deeper. Changes occur in the iris in the form of atrophy and stromal hypoplasia, depigmentation. The pupil is dilated. Excavation of the optic nerve head, decreased visual acuity, and narrowing of the field of view on the nasal side to 45-35° are observed (if the child’s age allows them to be examined). The progressive stage of the disease is determined by a sharp increase in the length of the sagittal axis of the eye and the diameter of the cornea. The limbus is stretched. The sclera becomes thinner, and the choroid appears through it in a bluish-bluish color. The anterior chamber is deep. There are degenerative changes in the cornea. The pupil is wide. The optic disc is grayish in color, its excavation increases. There is a sharp decrease in visual acuity, a concentric narrowing of the field of vision, mainly on the nasal side (up to 15°). Treatment of congenital glaucoma is surgical. In order to eliminate embryonic tissue and improve the outflow of intraocular fluid into Schlemm's canal, in most cases, operations are performed in the area of ​​the anterior chamber angle, as they are the most effective. Despite the age of the child, the operation must be performed urgently.

Author: Shilnikov L.V.

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