Primary Congenital Glaucoma
Robert C Urban, Jr, MD
Richard W Allinson, MD
Francisco Talavera, PharmD, PhD
Robert H Graham, MD
Primary Congenital Glaucoma Overview
By definition, primary congenital glaucoma is present at birth. It is usually diagnosed at birth or shortly thereafter, and most cases are diagnosed during the first year of life. However, sometimes, its symptoms are not recognized until later in infancy or into early childhood.
Primary congenital glaucoma is characterized by the improper development of the eye's drainage channels (called trabecular meshwork). Because of this, the channels that normally drain the fluid (called aqueous humor) from inside the eye do not function properly. More fluid is continually being produced but cannot be drained because of the improperly functioning drainage channels. This leads to high pressure inside the eye, called intraocular pressure (IOP).
Eye pressure is measured in millimeters of mercury (mm Hg). Normal eye pressure ranges from 10-21 mm Hg. Elevated IOP is an eye pressure of greater than 21 mm Hg. An increase in IOP can damage the optic nerve and result in vision loss and even blindness.
In approximately 75% of cases, primary congenital glaucoma is bilateral, that is, it occurs in both eyes.
Primary congenital glaucoma occurs more often in boys than in girls, with boys accounting for approximately 65% of cases.
Primary congenital glaucoma is relatively rare. In the United States, it reportedly affects fewer than 0.05% of children. However, various studies suggest that from 2-15% of children in institutions for the blind have been diagnosed with primary congenital glaucoma.
Despite the rarity of primary congenital glaucoma, the impact on a child’s visual development can be extreme. Early recognition and appropriate therapy for the glaucoma by an ophthalmologist (a medical doctor who specializes in eye care and surgery) can significantly improve the child's visual future and prevent blindness.
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