Primary congenital glaucoma (PCG) is an eye disease that affects children between the ages of birth and 3 years. It is one type of glaucoma. Glaucoma refers to a group of diseases in which there is permanent and characteristic damage to the optic nerve which is usually associated with abnormally high intraocular pressure.
Primary congenital glaucoma is rare. It affects about one in every 10,000 infants. But it is serious and needs attention. Untreated primary congenital glaucoma is a major cause of childhood blindness.
I've been going blind my whole life. I was born with choroideremia, a rare, inherited disorder that causes gradual vision loss. My doctors diagnosed it when I was 14, after my pediatrician saw small spots in my eyes. I had known I was having trouble seeing, especially at night, but at that age I didn't care. But then the doctors said, “You'll have a hard time in your 20s, a very hard time in your 30s, and you'll be blind by 60."
They were right. I am 49 now and almost completely blind, except for...
"Primary" means the disease does not result from another illness or condition, such as a tumor. "Congenital" means it is present at birth.
Although babies are born with primary congenital glaucoma, there may be no sign of it at first. It's commonly found between the ages of 3-6 months. But it can be diagnosed as late as 3 years of age.
If the disease is found and treated early, most children -- 80% to 90% -- respond well. They will not have vision problems in the future.
How Does Primary Congenital Glaucoma Affect the Eye?
This type of glaucoma is generally caused by increased pressure inside the eye. The abnormally high pressure is due to resistance to the flow of the normal circulation of eye fluid (aqueous). This eye fluid is needed to provide proper pressure to the eye. It also delivers nutrients to the interior parts of the eye.
In a healthy eye, the fluid leaves through a network of cells and tissue that functions as a tiny drain. To replace the fluid that drains, the eye continuously makes just the right amount of more fluid.
With glaucoma, something happens to this balance. In most cases, the fluid doesn't drain properly. The buildup of fluid causes the eye pressure to rise.
Increased pressure from the excess fluid damages the fibers that make up the optic nerve. The optic nerve is at the back of the eye. It sends signals to the brain to let you know what you see.
With most kinds of glaucoma, this damage occurs slowly over time. Often, by the time a person notices symptoms, the damage is already extensive. Once vision loss has occurred it is irreversible and cannot be restored.