Childhood Glaucoma

What is Childhood Glaucoma?

Childhood glaucoma, also called pediatric glaucoma, is rare. The condition is caused by increased intraocular pressure in one or both eyes. Elevated eye pressure damages the optic nerve, leading to vision loss.

There are three types of pediatric glaucoma, including:

1. Congenital glaucoma is present at birth
2. Infantile glaucoma develops between 1 and 24 months old
3. Juvenile glaucoma develops after age 2
   

Congenital glaucoma is caused by the abnormal development of the eye’s drainage system before birth. The eye's drainage system controls the flow of aqueous humor (clear liquid of the eye). It also creates eye shape, provides nutrients, and maintains intraocular pressure (IOP). 

If the drainage rate of aqueous humor is off even slightly, IOP increases and damages the optic nerve, leading to vision loss.

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. 

Childhood glaucoma is rare, affecting 1 in 10,000 American infants. Congenital glaucoma is discovered within the first year of life. If treated early, the child usually does not develop permanent vision loss.³

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 that develop glaucoma later in childhood may be asymptomatic until the disease progresses into permanent optic nerve damage and vision loss. Eye exams are recommended for children within their first year of life.

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 is causing an increase in intraocular pressure. 

Medical conditions linked to childhood glaucoma include:

• Axenfeld-Rieger Syndrome, a group of eye and other development disorders
• Aniridia, an underdeveloped iris (color part of the eye)
• Sturge-Weber Syndrome, a rare vascular disorder in the brain and eye 
• Neurofibromatosis, nervous system tumor growths
• Chronic steroid use, which is associated with elevated intraocular pressure
• Eye injury and blunt trauma
• Previous eye surgery, such as for congenital cataracts

What are the Risks and Complications of Childhood Glaucoma?

Childhood glaucoma is rare. 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. 

Treatment for Childhood Glaucoma

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 to measure vision ability
• Peripheral vision test for side vision
• Optical coherence tomography, a scan of the optic nerve
• Tonometry, an eye pressure test

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

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 to remove a section of tissue and drain fluid 
• Trabeculectomy to create a bleb (reservoir) to capture fluid and decrease IOP
• Tube shunt surgery, which implants a hollow tube called a shunt to divert fluid into a reservoir and away from the eye

Other surgeries used to treat childhood glaucoma include:

• Iridotomy, which 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. 

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

Can Childhood Glaucoma Be Prevented? 

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

Congenital glaucoma typically has visual warning signs shortly after birth. But 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. 

Congenital glaucoma has visual warning signs and is typically diagnosed shortly after birth. 

Secondary childhood glaucoma does not usually have symptoms until the disease progresses into vision loss and possible blindness if not treated.

Pediatric glaucoma treatment includes a combination of surgery and medication. If treated early, many children diagnosed with glaucoma lead healthy lives with minimal vision loss. 

Eye doctors recommend starting childhood eye exams within the first year of life.