Primary infantile glaucoma is a rare birth defect that prevents fluid (aqueous humor) 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.
(See also Glaucoma in adults.)
Normally, the fluid that nourishes the eye (aqueous humor) 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 Aqueous Humor Drainage
Aqueous humor is the fluid that nourishes the eye. This 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. |
Primary infantile glaucoma occurs in infants and young children and may affect only one eye or more often both eyes. In primary infantile glaucoma, intraocular pressure increases above the normal range.
Secondary glaucoma occurs when another disorder that affects the eyes, such as Sturge-Weber syndrome, increases intraocular pressure. 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 eye or 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).
Diagnosis of Primary Infantile Glaucoma
Measurements of the eyeball and cornea
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.
Treatment of Primary Infantile Glaucoma
Surgery
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.
More Information
The following English-language resource may be useful. Please note that THE MANUAL is not responsible for the content of this resource.
Children's Eye Foundation of AAPOS: Practical information about prevention, detection, research, and education to protect the vision of children
