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Glaucoma is normally found in older people but can be found in children from as early as birth. Glaucoma found in children 3 and under is called infantile or congenital glaucoma. Glaucoma is when the fluid in the eyes builds up instead of flowing out, causing immense pressure within the eyes. This causes damage to the optic nerve and furthermore the eyesight of the individual with glaucoma.

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Glaucoma is a condition in which the normal fluid pressure inside the eyes (intraocular pressure, or IOP) slowly rises as a result of the fluid aqueous humor - which normally flows in and out of the eye - not being able to drain properly. Instead, the fluid collects and causes pressure damage to the optic nerve (a bundle of more than 1 million nerve fibers that connects the retina with the brain) and loss of vision. Glaucoma is classified according to the age of onset. Glaucoma that begins before the child is 3 years old is called infantile or congenital (present at birth) glaucoma. Glaucoma that occurs in a child is called childhood glaucoma.

Glaucoma occurs when the fluid drainage from the eye is blocked by abnormal development or injury to the drainage tissues, thus, resulting in an increase in the intraocular pressure, damage to the optic nerve, and loss of vision.

There are many causes of childhood glaucoma. It can be hereditary or it can be associated with other eye disorders. If glaucoma cannot be attributed to any other cause, it is classified as primary. If glaucoma is a result of another eye disorder, eye injury, or other disease, it is classified as secondary.

In addition to a complete medical history and eye examination of your child, diagnostic procedures for childhood glaucoma may include:

visual acuity test - the common eye chart test (with letters and images), which measures vision ability at various distances.

pupil dilation - the pupil is widened with eyedrops to allow a close-up examination of the eye's retina and optic nerve.

visual field - a test to measure a child's side (peripheral) vision. Lost peripheral vision may be an indication of glaucoma.

tonometry - a standard test to determine the fluid pressure inside the eye.

Younger children may be examined with hand-held instruments, whereas older children are often examined with standard equipment that is used with adults. An eye examination can be difficult for a child. It is important that parents encourage cooperation. At times, the child may have to be examined under anesthesia, especially young children, in order to examine the eye and the fluid drainage system, and to determine the appropriate treatment.

Glaucoma is rare in children, as compared to the adult. However, when it does occur, the symptoms may not be as obvious in children. Many children are diagnosed before they are 6 months old. Glaucoma can affect one eye or both.

The following are the most common symptoms of childhood glaucoma. However, each child may experience symptoms differently. Symptoms may include:

excessive tearing

light sensitivity (photophobia)

closure of one or both eyes in the light

cloudy, enlarged cornea (large eye)

one eye may be larger than the other

vision loss

If the eye pressure increases rapidly, there may be pain and discomfort. Parents may notice that the child becomes irritable, fussy, and develops a poor appetite. Early detection and diagnosis is very important to prevent loss of vision. The symptoms of glaucoma may resemble other eye problems or medical conditions. Always consult your child's physician for a diagnosis.

Specific treatment for glaucoma will be determined by your child's physician based on:

your child's age, overall health, and medical history

extent of the disease

your child's tolerance for specific medications, procedures, or therapies

expectations for the course of the disease

your opinion or preference

It is important for treatment of childhood glaucoma to start as early as possible. Treatment may include:

medications
Some medications cause the eye to produce less fluid, while others lower pressure by helping fluid drain from the eye.

conventional surgery
The purpose of conventional surgery is to create a new opening for fluid to leave the eye.

surgery
Surgical procedures are performed by using microsurgery or lasers. The purpose of surgery is to create an opening for fluid to leave the eye. Surgical procedures used to treat glaucoma in children include the following:

trabeculotomy and goniotomy
A surgical opening is made into the drainage area of the eye (known as the trabecular meshwork drainage system), therefore establishing a more normal anterior chamber angle that allows the fluid to drain more freely, lowering the intraocular pressure (IOP). A goniotomy is an internal trabeculotomy procedure that is used in congenital glaucoma.

trabeculectomy
A surgical procedure that involves the removal of part of the trabecular meshwork drainage system, allowing the fluid to drain from the eye.

iridotomy
In this procedure, a small hole is made through the iris - the colored part of the eye - to allow fluid to flow more freely in the eye. The surgeon may use a laser to create this hole (laser iridotomy).

cyclophotocoagulation
A procedure that uses a laser beam to freeze selected areas of the ciliary body - the part of the eye that produces aqueous humor - to reduce the production of fluid. This type of surgery may be performed with severe cases of childhood glaucoma.

Both medications and surgery have been successfully used to treat childhood glaucoma.

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Detailed information on childhood glaucoma, including causes, symptoms, diagnosis, and treatment.

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Luckily, early childhood glaucoma is easy for doctors to recognize and diagnose. Watery/tearful eyes, frequent blinking, bulging eyes, sensitivity to light and a cloudy outer layer or cornea on the eye are some of the most common or noticeable symptoms. If the pressure in the eye increases rapidly the child may end up with a loss of appetite, moderate pain and irritability. Unfortunately, as children get older, they may develop glaucoma asymptomatically. That's one of many reasons children should have annual eye exams as early as age one. Doctors can catch it fairly easily through tests done during the exam.

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What is Childhood Glaucoma?
Childhood glaucoma, or "pediatric glaucoma," is a rare eye disease affecting 1 in 10,000 American infants. 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

Congenital glaucoma is usually noticeable right away. Common symptoms include:
What are the Symptoms of Childhood Glaucoma?

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.
There are three types of pediatric glaucoma, including:
Types of Pediatric Glaucoma

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

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

Age of the child

Overall health

Medical history

Child's tolerance to procedures and medications

Yes. Early detection and treatment can prevent pediatric glaucoma from progressing into blindness.
Can Childhood Glaucoma Be Prevented?

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.

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Childhood Glaucoma: Causes, Symptoms, and Treatments

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Find out everything you need to know about childhood glaucoma, including the causes and treatments.

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A fluid in the eye called "aqueous humor" builds up in the eye due to either too much being produced or never draining out. The aqueous humor is supposed to drain out of a tissue in the back of the cornea called the "trabecular meshwork", but it does not always function as need be. This causes pressure to build up in the eye, damaging the optic nerve and reducing vision. Children can be either born with glaucoma or may develop it due to a brain injury.

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Overview

Glaucoma is a group of eye conditions that damage the optic nerve, which is vital to good vision. This damage is often caused by an abnormally high pressure in your eye.

Glaucoma is one of the leading causes of blindness in the United States. It can occur at any age but is more common in older adults.

The most common form of glaucoma has no warning signs. The effect is so gradual that you may not notice a change in vision until the condition is at an advanced stage.

Vision loss due to glaucoma can't be recovered. So it's important to have regular eye exams that include measurements of your eye pressure. If glaucoma is recognized early, vision loss can be slowed or prevented. If you have the condition, you'll generally need treatment for the rest of your life.

Symptoms

The signs and symptoms of glaucoma vary depending on the type and stage of your condition. For example:

Open-angle glaucoma

Patchy blind spots in your side (peripheral) or central vision, frequently in both eyes

Tunnel vision in the advanced stages

Acute angle-closure glaucoma

Severe headache

Eye pain

Nausea and vomiting

Blurred vision

Halos around lights

Eye redness

If left untreated, glaucoma will eventually cause blindness. Even with treatment, about 15 percent of people with glaucoma become blind in at least one eye within 20 years.

When to see a doctor

Seek immediate medical care

Promptly go to an emergency room or an eye doctor's (ophthalmologist's) office if you experience some of the symptoms of acute angle-closure glaucoma, such as severe headache, eye pain and blurred vision.

Schedule eye exams

Open-angle glaucoma gives few warning signs until permanent damage has already occurred. Regular eye exams are the key to detecting glaucoma early enough to successfully slow or prevent vision loss.

The American Academy of Ophthalmology recommends glaucoma screening:

Every four years beginning at age 40 if you don't have any glaucoma risk factors

Every two years if you're at high risk or over 65

Causes

Glaucoma is the result of damage to the optic nerve. As this nerve gradually deteriorates, blind spots develop in your visual field. For reasons that doctors don't fully understand, this nerve damage is usually related to increased pressure in the eye.

Elevated eye pressure is due to a buildup of a fluid (aqueous humor) that flows throughout your eye. This fluid normally drains into the front of the eye (anterior chamber) through tissue (trabecular meshwork) at the angle where the iris and cornea meet. When fluid is overproduced or the drainage system doesn't work properly, the fluid can't flow out at its normal rate and pressure builds up.

Glaucoma tends to run in families. In some people, scientists have identified genes related to high eye pressure and optic nerve damage.

The types of glaucoma include the following:

Open-angle glaucoma

Open-angle glaucoma is the most common form of the disease. The drainage angle formed by the cornea and iris remains open, but the trabecular meshwork is partially blocked. This causes pressure in the eye to gradually increase. This pressure damages the optic nerve. It happens so slowly that you may lose vision before you're even aware of a problem.

Angle-closure glaucoma

Angle-closure glaucoma, also called closed-angle glaucoma, occurs when the iris bulges forward to narrow or block the drainage angle formed by the cornea and iris. As a result, fluid can't circulate through the eye and pressure increases. Some people have narrow drainage angles, putting them at increased risk of angle-closure glaucoma.

Angle-closure glaucoma may occur suddenly (acute angle-closure glaucoma) or gradually (chronic angle-closure glaucoma). Acute angle glaucoma is a medical emergency. It can be triggered by sudden dilation of your pupils.

Normal-tension glaucoma

In normal-tension glaucoma, your optic nerve becomes damaged even though your eye pressure is within the normal range. No one knows the exact reason for this. You may have a sensitive optic nerve, or you may have less blood being supplied to your optic nerve. This limited blood flow could be caused by atherosclerosis - the buildup of fatty deposits (plaques) in the arteries - or other conditions that impair circulation.

Glaucoma in children

It's possible for infants and children to have glaucoma. It may be present from birth or developed in the first few years of life. The optic nerve damage may be caused by drainage blockages or an underlying medical condition.

Pigmentary glaucoma

In pigmentary glaucoma, pigment granules from your iris build up in the drainage channels, slowing or blocking fluid exiting your eye. Activities such as jogging sometimes stir up the pigment granules, depositing them on the trabecular meshwork and causing intermittent pressure elevations.

Risk factors

Because chronic forms of glaucoma can destroy vision before any signs or symptoms are apparent, be aware of these risk factors:

Having high internal eye pressure (intraocular pressure)

Being over age 60

Being black or Hispanic

Having a family history of the condition

Having certain medical conditions, such as diabetes, heart disease, high blood pressure and sickle cell anemia

Having certain eye conditions, such as nearsightedness

Having had an eye injury or certain types of eye surgery

Early estrogen deficiency, such as can occur after removal of both ovaries (bilateral oophorectomy) before age 43

Taking corticosteroid medications, especially eyedrops, for a long time

Prevention

You may not be able to prevent glaucoma. But these self-care steps can help you detect it early, limit vision loss or slow its progress.

Get regular eye care. Regular comprehensive eye exams can help detect glaucoma in its early stages before irreversible damage occurs. As a general rule, have comprehensive eye exams every four years beginning at age 40 and every two years from age 65. You may need more frequent screening if you're at high risk of glaucoma. Ask your doctor to recommend the right screening schedule for you.

Know your family's eye health history. Glaucoma tends to run in families. If you're at increased risk, you may need more frequent screening.

Exercise safely. Regular, moderate exercise may help prevent glaucoma by reducing eye pressure. Talk with your doctor about an appropriate exercise program.

Take prescribed eyedrops regularly. Glaucoma eyedrops can significantly reduce the risk that high eye pressure will progress to glaucoma. To be effective, eyedrops prescribed by your doctor need to be used regularly even if you have no symptoms.

Wear eye protection. Serious eye injuries can lead to glaucoma. Wear eye protection when using power tools or playing high-speed racket sports on enclosed courts.

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Glaucoma is a group of eye conditions that damage the optic nerve, which is vital to good vision. This damage is often caused by an abnormally high pressure in your eye. Glaucoma is one of the leading causes of blindness in the United States.

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The treatment for children versus adults with glaucoma vary for the fact that some treatments are not as effective or safe for children. Medication may be used to treat this condition; however, adults can remain on the medication indefinitely while children should be on it for the least amount of time possible before surgery. A child may receive a trabeculotomy, removing a part of the trabecular meshwork and supporting structures to better drain the eye. More commonly for pediatric patients suffering from glaucoma is a goniotomy, where a small hole is made in the meshwork for drainage. Teaching a child with this condition would be a learning curve for the teacher for sure. Assistive devices such as text to speech on assignments, more test time would be needed since they would need more time to hear the question rather than see it. A student with this condition would teach classmates empathy, assisting the student by perhaps being quieter so they can hear better. This would also be great for team building, relying on everyone to work together to help each other out. This child's disability is just like any other, they may have strengths in subject and weaknesses just like any other student and would help other students with understanding that.

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Ta Chen Peter Chang, MD, assumed that he would never again see the 16-year-old who came to him for a second opinion. She had previously been diagnosed with juvenile open-angle glaucoma, was doing well on eyedrops, and did not need surgery. He confirmed that her pressure was controlled and that her condition should remain stable.

Flash forward to 1 year later when she returned with "incredibly high intraocular pressure [IOP]" and significant vision loss in both eyes. "Her right eye had a dense superior arcuate scotoma and a nasal step; her left had a dense inferior arcuate scotoma," said Dr. Chang, at the Bascom Palmer Eye Institute. "I was shocked. I thought I'd dropped the ball. What had I missed?"

He'd missed the fact that the patient's mother had always administered the drops, with 100% adherence. Then the young woman went off to college and stopped taking her meds. "That's how she fell off the cliff," Dr. Chang said.

Dr. Chang performed emergency surgery, and the patient no longer even needs drops, so the story ends well. Still, he frets that he had not emphasized the importance of treatment adherence. "I had not given her the tools to deal with the disease." And he can't stop wondering: What if? What if he'd performed surgery when he first saw her? But surgery isn't benign, and she was doing well with drops. "When do I pull the trigger?" he said. "That's a conundrum."

In Search of Answers

Childhood glaucoma is full of such conundrums, and pediatric glaucoma specialists have more questions than answers. For instance, which children with risk factors will go on to develop the disease? Why do some children respond to treatment and others do not? Can you predict, based on some severity score index, which child will benefit from treatment and which one will not? If so, how should you proceed? Finally, what if you suspect that nothing will work?

In some children, a single surgery may be curative "and change the next 75 years of the child's life," Dr. Chang said. In others, the prognosis may be so poor that it may be best to forgo further treatment. "As ophthalmologists, we want the children to see as well as they possibly can. We also want the patient to lead as good a life as possible. Sometimes, I think those 2 [desired outcomes] may be in conflict," he said.

In particular, he said, "Every time we do another surgery, put the child to sleep, we take away from the quality of life" for both the child and his or her parents. For instance, the multiple challenges of missing school and home life, traveling to the hospital, and preparing for and recovering from surgery means that, from the child's perspective, "childhood halts," he said. The child's family is under significant strain as well. "Every examination under anesthesia is a stressful event."

Dr. Chang is looking for a balance. "If you know further surgeries will not yield much benefit, wouldn't it be better to help prepare the family to raise their child as a blind person, so they don't spend their life tethered to a disease? These are the questions I struggle with at night."

A High-Stakes Game

Ophthalmologists who treat childhood glaucoma emphasize that there is no room for error. "This is a very vulnerable population. This is not an 80-year-old whose vision is slowly going," said Faruk H. Örge, MD, at Case Western Reserve University. "In childhood, glaucoma can be very aggressive. The patient can lose sight very quickly."

In children, it can be "an all-or-none" situation, said Alana L. Grajewski, MD, at Bascom Palmer. But with prompt diagnosis and surgery, some children with congenital glaucoma "stand a good chance of having normal or near-normal vision the rest of their lives," she said. "That's why it's important to know when to refer."

"That's one of the reasons some of us are attracted to this field," said Dr. Chang. "It's a high-stakes game. There are children who, with 1 procedure, never need another surgery and probably won't need drops. And there are others who, even after many surgeries, have poor prognoses."

The Impact of Puberty on IOP

When Dr. Toris ran out of rabbits in the middle of a study, she got new ones. And when the replacement rabbits yielded "all kinds of different results" from their predecessors, Dr. Toris was stumped. The replacements were the same gender, the same breed, and from the same vendor. "The only thing I could think of was age." As it happened, the second group of rabbits was a lot younger than the first group.

Something similar happened in a separate study involving primates. The results obtained with younger monkeys were inconsistent with those of the older ones they replaced, Dr. Toris said.

Around the same time, Dr. Toris started hearing from glaucoma specialists that young patients were responding differently to medical treatment than adults. This sparked a "Eureka!" moment. Perhaps there was a link between what the doctors were saying and what she was observing in her lab's replacement animals. "The first thing you think about is aqueous humor dynamics," said Dr. Toris.

As a result, she began treating young rabbits with pressure-lowering drops-timolol, latanoprost, or acetazolomide. She then measured the rabbits' IOP and aqueous humor at various time points, from when they were just weaned to when they reached sexual maturity. Her findings, which she is preparing for publication, indicate that IOP increases until puberty. At this point, it drops, and then it rises again after puberty.

None of these drugs worked in young rabbits. They only worked after sexual maturity. The question, Dr. Toris said, is: Why isn't IOP progression linear, as it is in adults?

"Certain things that work in adults don't work so well in children," she said. "We know these drugs were designed and tested in adults, but children are not miniature adults. They're quite different."

Dr. Toris said there's precedent for this finding in children with diabetes, whose blood sugar is well controlled before puberty, difficult to maintain during puberty, and controlled once again after puberty. 1 In either scenario-glaucoma or diabetes-children experience rapid growth and development in a short time as well as hormonal changes that "throw the body for a loop." And, as with diabetes, knowing that IOP changes are nonlinear in childhood may have clinical implications, she said.

___________________________

1 Cho YH et al. Pediatr Diabetes. 2014;15(1):18-26.

Not Just Small Adults

Childhood glaucoma is more than just glaucoma with a very early onset. "It's a completely different type of glaucoma, and it is managed differently," said Dr. Grajewski.

A panocular disorder. In adults, glaucoma is diagnosed based on the pathology of the optic nerve. But in children, glaucoma is characterized by IOP-related damage to the entire eye.

Primary congenital glaucoma, which accounts for 50% to 70% of all childhood glaucoma (and occurs in about 1:10,000 births in Western developed countries), is not an isolated disease of the optic nerve. Rather, it is a panocular disorder that is defined by structural, functional, and physiological parameters, Dr. Grajewski said. "It affects every part of the eye."

Other types of childhood glaucoma can occur at any time. It may be acquired (e.g., as a result of trauma or uveitis); alternatively, it may be associated with either a systemic disorder, such as Sturge-Weber syndrome, or an ocular disorder, such as aniridia or Peters anomaly.

Developmental complications. Because the immature eye is elastic, elevated IOP causes structural changes. The cornea may stretch, grow, and bulge outward, leading to the descriptive name buphthalmos, or ox-eye. Globe distension also results in iris stromal hypoplasia, ectopia lentis, and Descemet breaks, which degrade optical quality. Corneal haze is another hallmark of the disease.

Moreover, a child's eye is growing and the visual system is still immature, thus raising the risk of amblyopia, the most common cause of visual impairment in children with glaucoma. ¹

Diagnostic challenges. Eighty percent of cases are diagnosed during the first year of the child's life. ² But young children can be fidgety and uncooperative, so imaging is a challenge, which complicates the diagnosis, Dr. Örge said.

Moreover, IOP readings can be difficult to obtain. For instance, if the child is crying or holding his breath, IOP tends to be artificially elevated, making it more difficult to recognize if there's a problem. While examination under sedation or anesthesia can calm the patient, these agents can affect IOP. "Most sedation and anesthesia agents lower IOP, so if we obtain the IOP 5 minutes after [anesthesia is given], the IOP would be falsely lower than what it actually is," Dr. Örge said. (One exception to this is ketamine, which elevates pressure, as it tightens the extraocular muscles, he noted.)

Specialty clinics have portable slit lamps and tonometers that make the job easier. It's important to have this armamentarium at hand so that you can care for the patient, Dr. Örge said. "Our job is to make sense of what is real and not real."

Treatment challenges. Because of the myriad potential complications in childhood glaucoma, treatment involves much more than just lowering IOP. "If you look at the outcome as being [reduction of] pressure, you can win the battle of pressure but lose the war on amblyopia," Dr. Grajewski said.

Even if the glaucoma is controlled, the child can lose vision if the refractive error is not corrected, Dr. Örge noted. Other problems may develop, such as cataracts resulting from surgical procedures. "One disease can give birth to others," he said.

"For anybody treating childhood glaucoma, meticulous attention to visual development and the amblyopia treatment is part of the game," Dr. Chang said. This requires knowledge of prescribing glasses, checking vision, and patching. The stakes are highest in younger children. "You have to monitor [them] very carefully because the brain can turn the eye off very quickly when they're young."

Thus, as Dr. Grajewski recommended, "If you are uncomfortable treating childhood glaucoma, refer to a center that has a specialist. If you treat pediatric glaucoma yourself, involve your colleagues early to be sure you are not just treating the pressure but the final vision outcome as well."

A note on puberty. "Most of what we know about eye physiology is from adults, and that doesn't always transfer to children. Certain treatments that work well in adults may be unpredictable in children," said Carol B. Toris, PhD, at Case Western Reserve University.

And that unpredictability may be exacerbated during puberty. Dr. Toris' current research indicates that IOP progression isn't linear in children, as it usually is in adults (see " The Impact of Puberty on IOP "). The bottom line: You might treat a patient who is in the middle of puberty differently than you would a prepubescent child or an adult, she said.

A note on surgery. In adults, the treatment paradigm generally moves from medications to laser to incisional surgery. But in children, surgery is the mainstay, with the most common surgical interventions being trabeculotomy and goniotomy. 3,4 "In children, meds are more of a bridge until you do surgery," Dr. Grajewski said.

Dr. Örge elaborated on this point: "In adults, drainage starts failing after years of working. In pediatrics [in congenital glaucoma], this mechanism is defective from the get-go," he said. In a defective drainage system, medications are only temporary, he added.

A note on family dynamics. "When you're sitting in the exam room and looking at an adult, it's the adult. But with a child, everyone in the room is affected," said Dr. Grajewski. "You're treating the entire family, plus the glaucoma, the amblyopia, and the genetics," she said. This means treatment is time intensive and requires the integration of services, including social services, she noted.

"The referring physician should let families know that childhood glaucoma is a very complex problem," Dr. Örge said. "Hearing this from the referring physician makes it much easier to take in and reinforces the message that the disease can be controlled but that it needs a very high level of compliance."

In addition, the child's future needs to be addressed. "Most adults [diagnosed with glaucoma] have had fruitful lives, and we try to make sure they don't lose useful vision before the end of their lives," Dr. Chang said. "With pediatric glaucoma, the outlook can be very different. That's actually becoming a larger part of my conversation with parents. I want them to have a broad view"-that is, to focus not only on the disease but also on the child's quality of life.

Childhood Glaucoma Research Network Powers Up

Pediatric glaucoma specialists have a feel for what works and what doesn't in terms of treatments, said Dr. Chang. For example, he knows that if 2 prior surgeries failed, a third probably won't work either. "So we do have some inkling," he said. Still, he wonders, "Is this an eye where 1 surgery is a slam dunk? Or is this child going to be blind down the road? Can we support our hunches with good evidence?"

In the past, studies have lacked the statistical power to answer such questions because of small numbers of participants. That has changed, however, with the establishment of the Childhood Glaucoma Research Network (CGRN). Thanks to the network, clinicians and scientists who specialize in childhood glaucoma now have a place to share their expertise and pool information on outcomes.

CGRN has grown from an informal discussion with a few dozen researchers at the 2012 ARVO meeting into a full-fledged organization that has more than 150 members in 31 countries, said Dr. Grajewski, who raised funding for the group, which is now located at the Samuel and Ethel Balkan International Pediatric Center at Bascom Palmer. "Anybody with an interest in caring for children should become a member," Dr. Grajewski said. Already, CGRN has created a childhood glaucoma classification system to overcome inconsistent terminology (see " Classification of Childhood Glaucoma "). It has established registries at Wills Eye Hospital and Moorfields Eye Hospital to capture data worldwide. And it has developed a website for parents of children with glaucoma.

Other issues CGRN is addressing include the following: How does the disease start? What are the long-term outcomes? How can vision loss be prevented? "Looking at genetics is high on our list. Treatment options are, too," Dr. Grajewski said. She reminds clinicians that not all children with pediatric glaucoma are alike. "With uveitic glaucoma and Sturge-Weber, for example, what do you do first? Second? We don't know the best treatment pathway."

Perhaps the collective wisdom and experience of CGRN members will generate answers. As Dr. Örge put it: "If you really want to understand whether goniotomy works better than trabeculotomy, you need lots of patients. For this reason, the network becomes an integral part of what we do."

Volatile and Enduring

It's never static. The experts also emphasized the volatile nature of pediatric glaucoma. "With childhood glaucoma, it's always something," Dr. Grajewski said. "That's one of the biggest challenges we face. The condition changes as time goes on."

"Pediatric glaucoma is much more complex than many glaucomas we take care of in adults," Dr. Örge agreed. "What you do with a 1-month-old is very different from a 1- or 18-year-old. Being suspicious about this condition is the key."

It's never over. "The pathologic elevations of pressure in infancy result in considerable changes in adult eyes," Dr. Grajewski said. It's important to keep this in mind, she said, because when ophthalmologists first see these patients as adults, there's a tendency to think that "they had glaucoma as kids and then it's over. But these problems are ongoing, because they occurred at a sensitive time."

The residual effects of increased axial length caused by early stretching may include high myopia, a thinner sclera, an enlarged cornea, and an increased risk of retinal detachment. Corneal haze or altered corneal physiology can cause glare; in addition, they may complicate cataract surgery.

These are lifelong issues. "Any surgeries on an adult eye with a history of childhood glaucoma can be magnitudes more complex," Dr. Chang said.

On the Horizon

Dr. Örge is conducting research using ultrasound biomicroscopy to observe outflow mechanisms. It's not a new instrument, he noted, but he's using it in a different way to see pathology that is ordinarily obscured by a hazy cornea or a structural abnormality.

Dr. Örge is hopeful that a better understanding of outflow mechanisms will lead to better treatment decisions. In current practice, he said, "Sometimes you do 2 or 3 angle procedures in each eye before you realize that won't work," he said. With regard to outflow issues, he said, "If you know the trabecular meshwork is impaired but Schlemm's canal is okay, you know that the angle procedure is going to work." But if Schlemm's and the collector channels are not working, a drainage device may be a better choice.

But there is reason for optimism, said Dr. Chang. The outcomes for childhood glaucoma are becoming better defined through the use of information technology, he said. In addition, a new network is helping to link doctors with others who have an interest in the field (see " Childhood Glaucoma Research Network Powers Up ").

"With timely, accurate diagnosis, expert surgery, and meticulous follow-up [to monitor] visual development, many children previously destined to be blind will now lead lives as sighted persons," said Dr. Chang.

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1 Chang TC et al. Curr Ophthalmol Rep. 2015;3(2):85-90.

2 www.glaucomafoundation.org/childhood_glaucoma.htm.

3 Chen TC et al. Ophthalmology. 2014;121(11):2107-2115.

4 Papadopoulos M et al. Eye. 2014;28(8):931-943.

Meet the Experts

Ta Chen Peter Chang, MD Assistant professor of clinical ophthalmology and pediatrics at the Bascom Palmer Eye Institute in Miami. Relevant financial disclosures: None.

Alana L. Grajewski, MD Director of the Samuel and Ethel Balkan International Pediatric Glaucoma Center, director of pediatric glaucoma, and professor of clinical ophthalmology at the Bascom Palmer Eye Institute in Miami; founding director of the Childhood Glaucoma Research Network. Relevant financial disclosures: None.

Faruk H. Örge, MD William R. and Margaret E. Althans Chair and Professor of Pediatric Ophthalmology and director of the Center for Pediatric Ophthalmology and Adult Strabismus, University Hospitals Case Medical Center; chief of pediatric ophthalmology, Rainbow Babies and Children's Hospital and University Hospitals Eye Institute; and assistant professor of pediatrics and associate professor of ophthalmology, Case Western Reserve University School of Medicine in Cleveland. Relevant financial disclosures: None.

Carol B. Toris, PHD Professor of ophthalmology and visual sciences at Case Western Reserve University in Cleveland. Relevant financial disclosures: None.

For full disclosures and the disclosure key, see below.

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A look at the multiple challenges that come with treating this volatile and enduring disease. Web Extra: The CGRN's diagnos­tic flow chart.

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Glaucoma (Animation of Why It Happens and How It Can Cause Blindness)