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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 can cause increases in the intraocular pressure, which if untreated can damage the optic nerve.
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 aniridia or Lowe syndrome or Sturge-Weber syndrome is called secondary glaucoma.
The eye becomes enlarged because the collagen of the sclera and cornea can stretch from the increased intraocular pressure. 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 may occur. Early surgical intervention (eg, goniotomy, trabeculotomy, trabeculectomy) is the mainstay of treatment.
Last full review/revision June 2007 by Albert W. Biglan, MD
Content last modified June 2007
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