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Congenital Glaucoma

What is glaucoma?

Glaucoma is a disease caused by elevated intraocular pressure (IOP). The elevated IOP damages and destroys the axons of the optic nerve, leading to progressive blindness.

Does glaucoma occur in young people?

The number of younger people (under 35 to 40 years) with glaucoma has been vastly underestimated in the past. A major reason for this is that for a long time, ophthalmologists as well as patients have thought of glaucoma as a disease with variations. However, realizing that glaucoma represents a final common pathway resulting from a number of different conditions which can affect the eye, it should be almost a priori that some of these conditions would affect infants, children, adolescents, and young adults.

What is congenital glaucoma?

Congenital glaucoma, or infantile glaucoma, affects about 1 in 10,000 live births. It may occur without other findings (primary congenital glaucoma), associated with other syndromes, or after injury, congenital cataract extraction, or inflammation. Primary congenital glaucoma is due to failure of development or abnormal development of the trabecular meshwork. Most cases of primary congenital glaucoma are sporadic in occurrence. In the approximately 10% in which a hereditary pattern is evident, it is generally believed to be autosomal recessive in most cases.

How is congenital glaucoma detected?

Congenital glaucoma is usually detected by the parents. In infancy, the sclera of the eye is easily distensible, so that the eye enlarges when intraocular pressure is increased. Breaks occur in the corneal endothelium, leading to edema, or swelling, of the cornea, which produces a hazy, frosted glass appearance. The baby is sensitive to light and tearing may be present. As the cornea stretches, ruptures allow more aqueous into the corneal stroma and epithelium, causing a sudden increase in edema and haze and an increase of tearing and photophobia. The infant may become irritable to the point of burying its head in a pillow to avoid the pain caused by bright lights.

What is the treatment for someone with congenital glaucoma?

Treatment is surgical and often successful, although more than one operation may be necessary. The prognosis is worse if the glaucoma is present at birth.

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