Primary Congenital Glaucoma

Updated: Mar 13, 2017
  • Author: Gerhard W Cibis, MD; Chief Editor: Inci Irak Dersu, MD, MPH  more...
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Overview

Background

By definition, primary congenital glaucoma is present at birth; however, its manifestations may not be recognized until infancy or early childhood. It is characterized by improper development of the eye's aqueous outflow system, leading to increased intraocular pressure (IOP), with consequent damage to ocular structures, resulting in loss of vision. Although the disease is relatively rare, the impact on visual development can be extreme. Early recognition and appropriate therapy of the glaucoma can significantly improve the child's visual future.

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Pathophysiology

Primary congenital glaucoma is restricted to a developmental abnormality that affects the trabecular meshwork. This serves to distinguish it from other childhood glaucomas associated with other ocular and systemic congenital abnormalities, as well as childhood glaucomas that may be secondary to other ocular disorders, such as inflammation, trauma, and tumors.

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Epidemiology

Frequency

United States

Primary congenital glaucoma is estimated to affect less than 0.05% of ophthalmic patients and o.o5% of children. Although patients with the disease account for a significantly higher incidence in institutions for the blind, with various studies suggesting from 2-15%.

International

For genetic reasons, the incidence can be much higher, for example, in Saudi Arabia and among Romanian gypsies.

Mortality/Morbidity

The disease is bilateral in approximately 75% of cases.

Race

Congenital glaucoma affects all races

Sex

Male patients are found to have a higher incidence of the disease, comprising approximately 65% of cases.

Age

Primary congenital glaucoma usually is diagnosed at birth or shortly thereafter, and most cases are diagnosed in the first year of life.

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