What Are the Signs and Symptoms of Childhood Glaucoma?

What is Childhood Glaucoma?

Childhood glaucoma, or “pediatric glaucoma,” is a rare eye disease affecting 1 in 10,000 American infants.³ The condition is caused by increased intraocular pressure (IOP) in one or both eyes. 

Childhood glaucoma can be classified as primary glaucoma if the cause is not associated with another medical condition. If an underlying medical condition causes elevated intraocular pressure, it is called secondary glaucoma. 

What Is Glaucoma?

Glaucoma is an eye disease that damages the optic nerve, causing vision loss and blindness. It occurs when normal fluid pressure inside the eye increases. 

This can happen when the aqueous humor (clear liquid in the eye) doesn’t drain properly. The eye's drainage system is responsible for functions such as: 

• Creating eye shape
• Providing nutrients
• Maintaining IOP
• Aqueous humor flow

What Causes Childhood Glaucoma?

There are many causes of pediatric glaucoma. Primary congenital glaucoma is caused by the drainage system not developing correctly before birth. 

Secondary childhood glaucoma occurs when an underlying medical condition causes an increase in IOP. Medical conditions linked to childhood glaucoma include:

• Axenfeld-Rieger Syndrome. A group of eye and other developmental disorders
• Aniridia. An underdeveloped iris (colored part of the eye)
• Sturge-Weber Syndrome. A rare vascular disorder in the brain and eye 
• Neurofibromatosis. Nervous system tumor growths
• Chronic steroid use. Often associated with elevated intraocular pressure
• Eye injury and blunt trauma. Which can affect IOP
• Previous eye surgery. Complications with procedures such as congenital cataracts

What are the Symptoms of Childhood Glaucoma?

Congenital glaucoma is usually noticeable right away. Common symptoms include:

• Light sensitivity
• Cloudy cornea (transparent outer layer)
• Extra tears
• Frequent blinking
• Large, bulging eyes

Children may experience pain, poor appetite, and irritability if eye pressure increases rapidly. Children who develop glaucoma later in childhood may be asymptomatic.

However, the disease will progress into permanent optic nerve damage and vision loss. Because of this, eye exams are recommended for children within their first year of life.

How is Childhood Glaucoma Diagnosed?

Childhood glaucoma is diagnosed during a comprehensive eye examination. This exam includes a series of tests and assessments, including: 

• Pupil dilation, allowing the eye doctor to look at the retina and optic nerve
• Visual acuity test, which measures vision ability
• Peripheral vision test for side vision
• Optical coherence tomography, a scan of the optic nerve
• Tonometry, an eye pressure test

Surgery and medication are usually effective at treating pediatric glaucoma if treated early. Many children with glaucoma live healthy lives with minimal vision loss. 

Types of Pediatric Glaucoma

There are three types of pediatric glaucoma, including:

1. Congenital glaucoma. Present at birth, caused by abnormal development in the eye’s drainage system before birth
2. Infantile glaucoma. Develops between 1 and 24 months old
3. Juvenile glaucoma. Develops after age 2

Congenital glaucoma is discovered within the first year of life. If treated early, the child usually does not develop permanent vision loss.³

Treatment for Childhood Glaucoma

Surgery is the best way to treat primary congenital glaucoma. Treatment involves opening up the drainage system to lower intraocular pressure.

Surgical procedures for congenital glaucoma include:

• Goniotomy. Removes a section of tissue and drains fluid 
• Trabeculectomy. Creates a bleb (reservoir) to capture fluid and decrease IOP
• Tube shunt surgery. Implants a hollow tube called a shunt to divert fluid into a reservoir and away from the eye

• Iridotomy. Involves creating a small hole in the iris (colored part of the eye) to drain all fluid
• Cyclophotocoagulation. A laser surgery that reduces fluid production

Medicated eye drops are routinely used to lower fluid pressure inside the eye. They are used both before and after glaucoma surgery. 

What are the Risks and Complications of Childhood Glaucoma?

Ten percent of primary congenital glaucoma cases are genetic.¹ Family history is also a risk factor for secondary childhood glaucoma. 

Neurofibromatosis and aniridia are linked to genetics, with 50% of cases inheriting the disease from parents.¹ Other risk factors for pediatric glaucoma include:

• Suspicious optic discs (high degree of optic disc cupping)
• Elevated eye pressure 
• Family history of glaucoma
• Sturge-weber syndrome,  a rare vascular disorder in the brain and eye 

If not treated early, elevated eye pressure causes permanent damage to the optic nerve. This results in irreversible vision loss. 

Factors that Affect Pediatric Glaucoma

Treatment of pediatric glaucoma will depend on several factors, including:

• Age of the child
• Overall health
• Medical history
• Child’s tolerance to procedures and medications

The goal of treating secondary glaucoma first focuses on reducing eye pressure and treating the underlying medical condition. 

Can Childhood Glaucoma Be Prevented? 

Yes. Early detection and treatment can prevent pediatric glaucoma from progressing into blindness.

Congenital glaucoma typically has visual warning signs shortly after birth. However, other types of childhood glaucoma are usually asymptomatic until the disease progresses into permanent optic nerve damage and vision loss.

If your child is at risk for infantile or juvenile glaucoma, it is vital to have an annual comprehensive eye exam by a pediatric ophthalmologist. They will check eye pressure and look for signs of optic nerve damage. 

Summary

Childhood glaucoma is a rare condition caused by elevated eye pressure. The condition can lead to permanent optic nerve damage, vision loss, and blindness. 

Depending on the age of onset, children with glaucoma might be diagnosed with congenital, infantile, or juvenile glaucoma. Many types of childhood glaucoma are hereditary. 

Pediatric glaucoma treatment includes a combination of medical eye drops and surgery. Treating it early can help prevent optic nerve damage and vision loss. Because of this, doctors recommend eye exams for children within the first year of their life.