Childhood glaucoma is a rare type of eye condition that can cause cloudy vision or light sensitivity, as well as vision loss. This primary congenital glaucoma can be treated with eye drops, medications, and even eye surgery.

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Glaucoma is a very common eye condition that affects over 3 million people in the United States and is the second leading cause of blindness worldwide. While the general risk increases as you get older, there’s a rare type of glaucoma that specifically affects infants and children.

Known as congenital or childhood glaucoma, this eye condition can cause symptoms such as cloudy vision and sensitivity to light. It’s progressive and can lead to vision loss.

This article will explain more about this type of childhood glaucoma and the symptoms to look out for, and we’ll explain more about the possible treatment options that might include eye drops, other medications, and eye surgery.

Primary congenital glaucoma is also known as PCG. It occurs in very young children, and diagnosis is normally made in the first year of life. PCG is rare.

It can be genetic or caused by developmental abnormalities before birth, and it causes pressure inside the eye. Children with primary congenital glaucoma typically have symptoms that include enlarged eyes, sensitivity to light, and cloudiness over the cornea (the front of the eye).

Primary congenital glaucoma is distinct from standard glaucoma. Glaucoma is typically a chronic and progressive eye condition that affects older adults. Often, a buildup of eye pressure causes stress on the optic nerve. Over time, this leads to vision loss.

Primary congenital glaucoma is rare. When cases do occur, there’s often no clear cause.

However, there are some factors that might increase the risk of congenital glaucoma. Data suggests that boys always have a slightly higher risk than girls.

Additional possible risk factors include:

  • having a sibling with primary congenital glaucoma
  • a family history of primary congenital glaucoma
  • being the child of a marriage between first cousins, especially if this practice is common in your region, culture, or ethnic group

Researchers believe that some cases of congenital glaucoma are genetic. About 10% of congenital glaucoma cases are thought to have a genetic link.

The condition is recessive and has been associated with abnormalities on the CYP1B1 and LTBP2 genes.

The signs and symptoms of congenital glaucoma can vary.

Some children will have almost no symptoms. In many cases, symptoms will progress as the child ages. Often, a cloudy or hazy cornea is the first symptom parents notice. Common symptoms include:

Children with primary congenital glaucoma are likely to display additional secondary symptoms. These are generally a result of the discomfort caused by glaucoma, and might include:

What is the Loch Ness Monster phenomenon?

Children with primary congenital glaucoma often have a visibly enlarged eyeball. This can cause the iris and blood vessels to take on unusual shapes and presentations, including the “Loch Ness Monster” phenomenon. Named after the mythical monster because of how the vessels look, this phenomenon describes the loops of eye blood vessels in the angle of the eye. The angle of the eye is the cushion layer between the iris and the pupil.

There are multiple available treatments for congenital glaucoma. The exact treatments will depend on the severity of glaucoma and on the severity of symptoms. All treatments will focus on relieving eye pressure to relieve symptoms. Treatment options include:

  • Filtering surgery: Filtering surgery uses small tools to create a drain in the eye. This relieves eye pressure.
  • Laser surgery: During laser surgery, a laser creates a small opening in the eye to let out eye pressure.
  • Angle surgery: Angle surgery cuts into the angle eye and repairs it. It creates new angle structures for the eye, relieving pressure and increasing the flow of eye moisture.
  • Topical eye drops: There are a few different types of eye drops that can help children with congenital glaucoma. They can be used to increase how much fluid leaves the eye or to decrease fluid overproduction inside the eye.
  • Oral medications: Like eye drops, there are a few options for oral medications. Some can relieve pain and pressure, while others can address eye moisture.

Vision that has already been lost as a result of congenital glaucoma can’t be restored.

However, it’s often possible to stop vision loss from progressing.

Once vision loss has stopped, doctors can help children make the most of their remaining vision with visual aids and assistive devices. Not all cases of congenital glaucoma will respond to surgery, and outcomes can vary depending on the specific procedure and specific child, but overall, success rates for congenital glaucoma are high.

Research from the British Infantile and Childhood Glaucoma Eye Study in 2020 found a more than 94% success rate when looking at a variety of common treatments.

Primary congenital glaucoma is a rare eye health condition that affects infants and children. Children with this condition have pressure inside their eyes that causes symptoms such as eye enlargement, light sensitivity, and a hazy cornea.

Over time, these symptoms can progress and can lead to vision loss. Treatment options include eye drops, medication, and surgery. Although treatment can’t restore lost vision, it can often stop further vision loss.

Many children with congenital glaucoma are able to maximize their remaining sight and lead full lives.