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What is Congenital Glaucoma?

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Glaucoma is a serious disease of the eyes that can lead to a permanent loss of vision if left untreated. Anyone can develop glaucoma, from babies to older adults. While adult glaucoma is more common, some babies are born with congenital glaucoma or childhood glaucoma. 

According to the Glaucoma Research Foundation, childhood glaucoma is a rare disease. Approximately 1 in 10,000 babies born in the United States has primary childhood glaucoma. 

Please continue reading to learn more about congenital glaucoma, including what causes it, why early diagnosis is important, and how it is treated.

What is glaucoma? 

Glaucoma is an eye disease in which the optic nerve is damaged due to high intraocular pressure IOP (pressure inside the eye). This typically happens because of the buildup of a fluid called aqueous humor in the anterior chamber (front part of the eye). 

In a healthy eye, there is constant aqueous inflow and aqueous outflow. Meaning, a certain amount of aqueous humor flows into the eye, and the same amount drains out. This maintains a stable intraocular pressure at normal levels. However, if an area in the eye called the trabecular meshwork or drainage canal is not working properly, the fluid can build up in the eye increasing the IOP andleading to optic nerve damage over time. 

The optic nerve is made up of more than 1 million nerve fibers that carry visual signals to the brain. If these nerve fibers are damaged or die, blind spots develop in the vision, ultimately leading to blindness. However, there are no early symptoms from angle abnormalities or increased eye pressure, and glaucoma can sneak up on you. That’s why it is often called the “silent thief of sight.” 

Some 3 million Americans have glaucoma, but only half the people who have this condition are aware of the problem. Glaucoma accounts for approximately 10% of cases of blindness in the US and is the second leading cause of blindness worldwide. 

What is childhood glaucoma or primary congenital glaucoma?

Childhood glaucoma is also called congenital glaucoma, infantile glaucoma, juvenile glaucoma, pediatric glaucoma, or early-onset glaucoma, depending on the age at which it is diagnosed. Congenital glaucoma or infantile glaucoma occurs in babies under 12 months. Childhood glaucoma is diagnosed in young children under 3 years old. Juvenile glaucoma affects teenagers and young adults. Early-onset glaucoma occurs before age 40. All these types of glaucoma are associated with increased eye pressure.

There are two types of congenital glaucoma or childhood glaucoma:

  • Primary congenital glaucoma or primary infantile glaucoma is present from birth and is not caused by other conditions.
  • Secondary congenital glaucoma occurs due to another condition, such as an infection or tumor. 

What causes congenital glaucoma?

Childhood glaucoma or pediatric glaucoma is a rare condition. It can be inherited. It can also be caused by abnormal development of the drainage system in the eye before birth. 

What are the symptoms of childhood glaucoma?

Signs and symptoms of childhood glaucoma include:

  • Enlarged eyes
  • Hazy or cloudy cornea
  • Excessive tearing
  • Redness of the eye
  • Photosensitivity (sensitivity to light)
  • Blepharospasm (involuntary twitching of the eye)

Does congenital glaucoma cause blindness?

Glaucoma can cause severe vision loss and blindness if it is left untreated. Congenital glaucoma is not curable. The lost vision cannot be restored. However, oral medications, eye drops, or a surgical procedure can reduce intraocular pressure and stop further vision loss. 

Childhood glaucoma is a chronic condition, and affected children need to be monitored lifelong. However, children with infantile glaucoma or pediatric glaucoma can live full lives with the proper glaucoma management to optimize their remaining vision. 

Can childhood glaucoma be cured?

As mentioned above, infantile glaucoma or juvenile glaucoma can't be cured. However, early diagnosis and treatment can control it. 

How is congenital glaucoma treated?

In most children with glaucoma, surgery is done to correct the structural defects, for example, at the anterior chamber angle to allow the flow of fluid out of the eye and lower intraocular pressure. 

There are two types of surgery that can be done in the operating room to reduce increased intraocular pressure and reduce the risk of severe complications like vision loss. These are called trabeculectomy and goniotomy. Eye surgeons can perform filtering surgery or microsurgery with small surgical tools to create an opening in the drainage system. They can also perform laser surgery using a powerful beam of light to open up the eye’s drainage system and lower eye pressure.

Medications are available to reduce the increased pressure. They can be in the form of topical eye drops or oral medications. Some children need both medications and surgery to treat childhood glaucoma.

Children with glaucoma can develop recurrent pediatric glaucoma. This is associated with a recurrence of increased intraocular pressure after treatment. Repeat treatments can usually reduce elevated intraocular pressure in such children. 

Wrapping Up

Congenital glaucoma is an inherited condition or a developmental disorder in which the anterior chamber angle or trabecular meshwork does not allow proper drainage of the aqueous humor. As a result, elevated IOP occurs (increased eye pressure), leading to optic nerve damage and vision loss. 

Early diagnosis of primary congenital glaucoma is essential so that the appropriate treatment can be started before there is irreversible damage to the optic nerve. 

People with a positive family history of pediatric glaucoma should consider genetic counseling and prenatal testing. 

The treatment plan for each child with childhood glaucoma is individualized based on their symptoms and clinical presentation. Treatment for childhood glaucoma may include surgery, medications, or both.

References:

  1. https://www.aao.org/eye-health/diseases/what-is-glaucoma
  2. https://www.glaucoma.org/glaucoma/glaucoma-facts-and-stats.php
  3. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6950954/
  4. https://www.hindawi.com/journals/joph/2013/763735/