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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

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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.

(See also Overview of Glaucoma.)

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, is called secondary glaucoma.

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.