Primary Congenital Glaucoma



What is primary congenital glaucoma?

Primary congenital glaucoma (PCG) is a type of glaucoma, a group of diseases in which high fluid pressure in your eye damages the optic nerve. This disease is usually diagnosed at birth or shortly thereafter, and most cases are diagnosed during the first year of life. PCG is characterized by an abnormality in the development of the eye’s drainage channel (a structure in the eye called the trabecular meshwork). A clear fluid called aqueous continuously flows within the eye from the area behind the iris where it’s secreted to the sieve-like trabecular meshwork where it drains back into the bloodstream. Because the trabecular meshwork does not function properly in PCG, the aqueous outflow is impaired, resulting in high pressure inside the eye. This elevated intraocular pressure can result in damage to the optic nerve (glaucoma) and may result in permanent vision loss if not treated promptly.

How common is primary congenital glaucoma?

It affects children between birth and 3 years. PCG is a serious condition that needs attention. It affects about one in every 10,000 infants. Untreated cases are a major cause of childhood blindness.

However, it can be managed by reducing your risk factors. Please discuss with your doctor for further information.


What are the symptoms of primary congenital glaucoma?

It is assumed that there are three typical symptoms characterize primary congenital glaucoma:

  • Excessive tearing (called epiphora)
  • Sensitivity to light (called photophobia)
  • Spasms or squeezing of the eyelid (called blepharospasm)

If an infant or a young child shows any of these symptoms, parents or caregivers should seek medical care as soon as possible from an ophthalmologist as they may be signs of glaucoma.

When should I see my doctor?

Early diagnosis and treatment can stop this congenital condition from worsening and prevent another medical emergency, so talk to your doctor as soon as possible to prevent this serious condition.

If you have any signs or symptoms listed above or have any questions, please consulting with your doctor. Everyone’s body acts differently. It is always best to discuss with your doctor what is best for your situation.


What causes primary congenital glaucoma?

We know that in case a baby’s eye cells and tissues don’t grow normally before birth, he can have trouble with drainage after he’s born. But actually we don’t exactly understand what causes this condition at this time. Some cases are inherited, while others aren’t.

Risk factors

What increases my risk for primary congenital glaucoma?

It is unforeseeable that which babies will be born with PCG. Parents with a family history of this condition are more likely to have it. If your first and second children have it, later children probably will, too. About twice as many boys as girls are born with it. It sometimes appears only in one eye, but most of the time, it affects them both.

Diagnosis & Treatment

The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.


How is primary congenital glaucoma diagnosed?

Based on the child’s age and level of cooperation, some examination can be performed in the clinic. In infants, the examination is often less challenging if performed when the infant is relaxed or drowsy, such as during feeding or shortly after. In most cases, additional examination and testing will need to be performed under sedation or anesthesia, and arrangement can be made to treat at the time of diagnosis. Your doctor begins by asking the caregivers about duration of the child’s symptoms and any known family history of glaucoma or some other eye disorders. Some following tests are performed in the office and in the operating room:

  • Vision is examined: In an infant, this may be limited to checking if he/she can fixate on an object and follow a moving object with the eyes.
  • Refraction is measured: This is a measurement of nearsightedness, farsightedness, or astigmatism. In PCG, the high eye pressure can produce nearsightedness (myopia) resulting from an increase in the eye’s length and astigmatism resulting from scarring or swelling of the cornea.
  • Tonometry to measure the eye pressure: This measurement of the eye’s size determines if it is enlarged. The diameter of the cornea is measured using calipers, and the length of the eye is measured using A-scan ultrasound. Examination of the cornea checks for cloudiness and small breaks on the back layer that is sometimes presented when the cornea is stretched under high eye pressure. A measure of the cornea’s thickness might also be done.
  • Direct visualization of the trabecular meshwork: This is called gonioscopy and is performed with special contact lenses and mirrors. This test is important to detect if the angles (the area where the trabecular meshwork is located) are open, narrowed, or closed, and if other conditions such as scar tissue in the angle are present.
  • The optic nerves are carefully examined (using ophthalmoscopy): To look for signs of glaucomatous optic nerve damage, this is a good option. This may require the dilation of the pupils to ensure an adequate view.

How is primary congenital glaucoma treated?          

Surgery is always considered as the first choice. Because it’s quite risky for babies to get anesthesia, doctors prefer doing it right after the diagnosis is confirmed. If both eyes are affected, the doctor will operate on both at the same time.

If surgery can’t take place right away, the doctor may prescribe eye drops, medicine to be taken by mouth, or a combination of the two to help monitor the fluid pressure.

Many doctors perform a procedure called microsurgery. They use small tools to make a drainage canal for the excess fluid. Sometimes the doctor will implant a valve or small tube to carry fluid out of the eye.

If the normal methods don’t work, the doctor may perform laser surgery to destroy the area where fluid is produced. He may prescribe medicine to help control eye pressure after surgery.

Lifestyle changes & Home remedies

What are some lifestyle changes or home remedies that can help me manage primary congenital glaucoma?

While taking care of your baby, you should notice any signs or symptoms above to make sure that your baby are still normal. In case your baby experiences some signs and symptoms of this condition, you must look for an immediate healthcare as soon as possible to avoid it.

If you have any questions, please consult with your doctor to better understand the best solution for you.

Hello Health Group does not provide medical advice, diagnosis or treatment.


Review Date: February 26, 2017 | Last Modified: March 13, 2017

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