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Primary Infantile Glaucoma

(Infantile Glaucoma; Congenital Glaucoma; Buphthalmos)


Leila M. Khazaeni

, MD, Loma Linda University School of Medicine

Reviewed/Revised Feb 2022 | Modified Sep 2022
Topic Resources

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.

Normally the aqueous fluid, which nourishes the eye, is produced by the ciliary body behind the iris (in the posterior chamber) and flows through the pupil to the front of the eye (anterior chamber), where it exits into drainage canals between the iris and cornea. When fluid drainage is blocked, pressure inside the eye (intraocular pressure) increases.

Normal Fluid Drainage

Fluid is produced in the ciliary body behind the iris (in the posterior chamber), passes into the front of the eye (anterior chamber), and then exits through the drainage canals.

Normal Fluid Drainage

Primary infantile glaucoma occurs in infants and young children and may affect only one eye or more often both eyes. This disorder is considered primary because it is not caused by another disorder, such as Sturge-Weber syndrome Sturge-Weber Syndrome Sturge-Weber syndrome involves an abnormal growth of small blood vessels. It is characterized by a port-wine birthmark on the face, overgrowth of blood vessels (angioma) in the tissues that... read more Sturge-Weber Syndrome . 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).

Doctors suspect primary infantile glaucoma when a baby has tearing, frequent blinking, and light sensitivity. They confirm the diagnosis by doing a thorough eye examination in an office or clinic, followed by a more detailed examination under general anesthesia in an operating room so that special measurements of the eyeball and cornea can be taken.

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 Surgery ) as early as possible.

More Information

The following is an English-language resource that may be useful. Please note that THE MANUAL is not responsible for the content of this resource.

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