Merck Manual

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Primary Infantile Glaucoma

(Infantile Glaucoma; Congenital Glaucoma; Buphthalmos)

By

Leila M. Khazaeni

, MD, Loma Linda University School of Medicine

Last full review/revision Jun 2020| Content last modified Jun 2020
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Primary infantile glaucoma is a rare developmental defect in the iridocorneal filtration angle of the anterior chamber that prevents aqueous fluid from properly draining from the eye. This obstruction increases the intraocular pressure, which, if untreated, damages the optic nerve. Infantile glaucoma can cause complete blindness if left untreated.

The disorder occurs in infants and young children and may be unilateral (40%) or bilateral (60%). Intraocular pressure increases above the normal range (10 to 22 mm Hg). Glaucoma can also occur in infants after trauma or intraocular surgery (eg, cataract extraction). Glaucoma associated with another ocular disorder, such as aniridia, Lowe syndrome, or Sturge-Weber syndrome Sturge-Weber Syndrome Sturge-Weber syndrome is a congenital vascular disorder characterized by a facial port-wine nevus, a leptomeningeal angioma, and neurologic complications (eg, seizures, focal neurologic deficits... read more Sturge-Weber Syndrome , is called secondary glaucoma.

Aqueous humor production and flow

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 or the uveoscleral pathway (black arrows).

Aqueous humor production and flow

In primary infantile glaucoma or early childhood glaucoma, the affected eyes become enlarged because the collagen of the sclera and cornea can stretch because of the increased intraocular pressure. This enlargement does not occur in adult glaucoma. The large-diameter (> 12 mm) cornea is thinned and sometimes cloudy. The infant may have tearing and photophobia. If untreated, corneal clouding progresses, the optic nerve is damaged (as evidenced clinically by optic nerve cupping), and blindness can occur.

Early surgical intervention (eg, goniotomy, trabeculotomy, trabeculectomy) is the mainstay of treatment.

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