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.
http://emedicine.medscape.com/article/1206081-overview

Primary Congenital Glaucoma

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.
"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.

What Causes Primary Congenital Glaucoma?

The cause of primary congenital glaucoma is not clearly understood. Some cases are inherited.
But others are not. The problem of improper drainage of fluid is the result of the lack of full or
proper development before birth of the network of cells and tissue that make up the drain.

Who Is Likely to Have Primary Congenital Glaucoma?

It's hard to predict which babies will be born with primary congenital glaucoma. One risk factor
is a family history of congenital glaucoma. If the first and second child have the disease, the risk
increases significantly for later children.
About twice as many boys as girls are born with primary congenital glaucoma. It sometimes can
affect just one eye. But in three out of every four cases both eyes are affected.

What Are the Symptoms of Primary Congenital Glaucoma?

There are three main symptoms that a parent is likely to notice:

Involuntary protective closing of the eyelids

Painful oversensitivity to light
Excessive tearing
Depending how far the disease has progressed, other symptoms can include:

Cloudiness of the cornea, the normally clear front layer of the eye
Enlargement of one or both eyes
Red eye

How Is Primary Congenital Glaucoma Diagnosed?

A thorough eye exam is needed to accurately diagnose PCG and determine the proper
treatment.
It can be difficult to examine the eyes of a baby or a small child in the same way a doctor
examines the eyes of older children or adults. So the exam is almost always done in an
operating room under general anesthesia.
During the exam, the doctor will:

Measure the pressure inside the eye

Thoroughly examine all parts of the eye
A formal diagnosis of primary congenital glaucoma will be made only after ruling out other
possible conditions that could be responsible for the glaucoma or that could cause symptoms
similar to glaucoma.

How Is Primary Congenital Glaucoma Treated?

The first choice for treating primary congenital glaucoma is almost always surgery. And
because of the risk for young children associated with anesthesia, the surgery is often

performed at the same time the diagnosis is confirmed. If the primary congenital glaucoma
affects both eyes, the doctor will normally operate on both at the same time.

How Is Primary Congenital Glaucoma Treated? continued...

In some cases when surgery can't be performed immediately, the doctor will prescribe eye
drops, oral medicine, or a combination of both to help control pressure before surgery.
One common approach is microsurgery. This uses small surgical instruments to create a
drainage canal for the excess fluid.
Sometimes the doctor will implant a valve or small tube to allow the fluid to drain from the eye.
If the usual surgery or implanting a tube isn't effective, the doctor may perform laser surgery to
destroy the area where the fluid is produced.
In some cases following surgery, the doctor will prescribe medicine to help control pressure in
the eye.

What Are the Possible Complications From Surgery?

The most common complication is a reaction to the anesthesia. Other complications can
include:

Insufficient reduction of eye pressure or excessive lowering of eye pressure

Developing a lazy eye, called amblyopia
Retinal detachment
Astigmatism
Dislocation of the eye's lens
Because increased pressure can reoccur at any time, regular eye checkups are needed
throughout life.