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Primary Infantile Glaucoma

(Infantile Glaucoma; Congenital Glaucoma; Buphthalmos)

By Christopher M. Fecarotta, MD, Attending Physician, Phoenix Children’s Hospital
Wendy W. Huang, MD, PhD, Attending Physician, Phoenix Children’s Hospital

(See also Glaucoma.)

Primary infantile glaucoma is a rare birth defect that prevents fluid from properly draining from the front part of the eye. This blockage increases pressure inside the eye, which, if left untreated, damages the optic nerve and can cause complete blindness.

Primary infantile glaucoma occurs in infants and young children and may affect only one eye (in 40% of children) or both eyes (in 60% of children). This disorder is considered primary because it is not caused by another disorder, such as Sturge-Weber syndrome. Pressure inside the eye (intraocular pressure) increases above the normal range. Intraocular pressure can also increase in infants after an injury or eye surgery (for example, removal of a cataract).

In primary infantile glaucoma or early childhood glaucoma, the affected eyes can enlarge because the sclera (the tough white fiber layer covering the eye) and cornea (the clear layer in front of the iris and pupil) stretch as a result of the increased intraocular pressure. This enlargement does not occur in adult glaucoma. The cornea is sometimes cloudy. The infant may have tearing and eye pain resulting from bright light (photophobia).

If untreated, clouding of the cornea progresses, the optic nerve is damaged, and blindness can occur. To prevent these complications, doctors do a surgical procedure to create a new drainage system (goniotomy, trabeculotomy, or trabeculectomy) as early as possible.