By definition, primary congenital glaucoma is present at birth. It 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). An increase in IOP can damage the optic nerve and result in vision loss and eventually blindness.
Another way to think of high pressure inside the eye is to imagine a water balloon. The more water that is put into the balloon, the higher the pressure inside the balloon. The same situation exists with too much fluid inside the eye. The more fluid, the higher the pressure. Also, just like a water balloon can burst if too much water is put into it, the optic nerve in the eye can be damaged by too high of a pressure.
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.
Despite the rarity of primary congenital glaucoma, the impact on a child’s visual development is oftentimes extreme. Early recognition and appropriate therapy for the glaucoma by an ophthalmologist can significantly improve the child's visual future and possibly prevent blindness.
