What is Congenital Glaucoma?

is an eye disease that affects children between the ages of birth and 3 years. It is one type
of glaucoma. Glaucoma refers to a group of diseases in which there is permanent and
characteristic damage to the optic nerve which is usually associated with abnormally high
intraocular pressure.
is characterized by the improper development of the eye's drainage channels (called
trabecular meshwork). Because of this, the channels that normally drain the fluid (called
aqueous humor) from inside the eye do not function properly. More fluid is continually
being produced but cannot be drained because of the improperly functioning drainage
channels. This leads to high pressure inside the eye, called intraocular pressure (IOP).
Eye pressure is measured in millimeters of mercury (mm Hg). Normal eye pressure
ranges from 10-21 mm Hg. Elevated IOP is an eye pressure of greater than 21 mm Hg.
An increase in IOP can damage the optic nerve and result in vision loss and even
blindness.

Congenital Glaucoma Symptoms

An infant or young child who has congenital glaucoma may exhibit cloudy corneas, sensitivity to
light, excessive tearing, and eyelid spasm. Congenital glaucoma can be difficult to recognize
because children often have trouble discerning the signs and symptoms. Consult a pediatric
optician if you notice a cloudy, white, hazy, enlarged, or protruding eye. Congenital glaucoma is
more common in boys than girls. A pediatric optician can help you diagnose congenital
glaucoma symptoms and arrange treatment.

How is it Diagnosed?
Primary congenital glaucoma is also abbreviated as PCG. This eye disease affects children up to
the age of 3. Primary congenital glaucoma is a glaucoma that causes damage to the optic nerve
and affects the eye. This type of glaucoma is rare and is believed to affect 1 in every 10,000
infants.
Primary congenital glaucoma is also considered to cause childhood blindness. Increased eye
pressure is one of the major causes of this type of eye disease. There is increased eye pressure
caused by resistance to the flow of circulation of fluid in the eye. Eye fluid is required for
maintaining accurate eye pressure. Causes of this type of glaucoma are not completely known.

Diagnosis of Primary Congenital Glaucoma

There may not be any sign of glaucoma in the earlier stages. Babies born with this type of
glaucoma are usually diagnosed with this disease between the age of 3 and 6 months. Diagnosis
and testing of this glaucoma can be as late as the age of 3 years as the baby grows. If diagnosis of
this disease is done early, vision problems in the future can be prevented.
Diagnosis of primary congenital glaucoma is done through complete eye testing. Eye testing is
difficult for small children. Hence, the diagnosis procedure is conducted in an operating room.
Diagnosis is performed under general anesthesia. This diagnosis is performed only after the
possibility of all other eye disease or conditions has been ruled out. Diagnosis of primary
congenital glaucoma includes measuring eye pressure. A thorough examination and testing of all
parts of the eye is part of the diagnosis. The diagnosis may include taking pictures of the eye for
testing and proper diagnosis.
Ophthalmology
The standard procedure followed for eye testing is called ophthalmology. Ophthalmology testing
is devised for infants as well as children. Pediatric ophthalmology has tests for determining
asymmetry between the eyes. Intraocular pressure may also be measured in pediatric
ophthalmology testing.
Ophthalmology has tests to determine abnormalities in the iris, cornea, and lens. Refraction can
be performed after dilation in pediatric ophthalmology testing. Careful examination of the optic
nerve for any kind of asymmetry and abnormality is also conducted as part of pediatric
ophthalmology. Anterior segment exam in the pediatric ophthalmology testing examines the
abnormality in the corneal diameter. Clarity of the cornea is also taken into account in pediatric
ophthalmology.

Exams and Tests:

Often in the childhood patient, the eye examination must be performed under general anesthesia
to obtain important information regarding the disease.
The ophthalmologist performs the following examinations in the operating room:
Tonometry is a method used to measure the pressure inside the eye. Pressures that are
recorded while the child is under general anesthesia are usually lower than those obtained in
the office because of the effects of the anesthesia.
The cornea is measured and examined.

The average diameter of the cornea at birth is less than 10.5 millimeters.
Because of elevated IOP, the cornea becomes enlarged. If the cornea is more than 12
millimeters in diameter in the first year of life, glaucoma is a strong possibility.
The cornea may be swollen as a result of the elevated IOP. It may also appear
hazy. This haze usually clears when the pressure returns to normal. In advanced cases,
severe clouding of the cornea may occur due to the corneal swelling. This cloudiness of the
cornea may persist even after the pressure is reduced inside the eye.
Haab striae may be seen as a result of elevated IOP. Haab striae are tears in the
Descemet membrane, which is the inner tissue layer of the cornea.

Gonioscopy is performed to check the drainage angle of the eye. This test is important to
determine if the angles are open, narrowed, or closed and to rule out any other conditions that
could cause elevated IOP. To view the angle, a special contact lens is placed on the eye.
The angle of the eye is formed where the iris and the cornea come together.
Because the iris is frequently abnormal in many types of childhood glaucomas, the angle is
often affected. Abnormalities in the angle can cause glaucoma. Therefore, the iris and the
angle are always carefully studied with a thorough gonioscopy.
In children, glaucoma may deepen the anterior chamber. A deep anterior
chamber hampers the flow of fluid from the eye and allows it to build up inside the eye,
thereby causing high pressure.

Ophthalmoscopy is a method used to examine the optic nerves for any damage or
abnormalities. This may require dilation of the pupils to ensure an adequate examination of
the optic nerves.
The most important indicator of the extent and severity of the disease is any
changes in the optic nerve. Unfortunately, by the time that glaucoma is diagnosed in a
child, the optic nerve is usually abnormal.
Fundus photographs, which are pictures of the optic disc (the front surface of the
optic nerve), may be taken for future reference and comparison.

Surgery
Surgery is performed to allow the fluid inside the eye to drain outside the eye and lower the
pressure.

If surgery is needed, the ophthalmologist will discuss the appropriate surgical procedure for the
child with the parents or caregivers. The risks and benefits of each procedure will also be
explained.
Common surgical procedures
The most common surgical procedures for primary congenital glaucoma are goniotomy and
trabeculotomy.
In both procedures, the ophthalmologist makes an incision into the trabecular meshwork to allow
the fluid (aqueous humor) to flow out better from the eye using the normal drainage channel.
In goniotomy, the fibers of the trabecular meshwork are cut to eliminate any resistance to fluid
flow imposed by an incompletely developed trabecular meshwork.
In trabeculotomy, a probe is used to tear through the trabecular meshwork to open it and allow
fluid flow.
One advantage of trabeculotomy is that it can be performed in eyes with cloudy or clear corneas,
whereas goniotomy can only be performed in eyes with clear corneas.
Goniotomy and trabeculotomy have similar success rates of approximately 80%.
Alternate surgical procedures
When multiple goniotomies and/or trabeculotomies have failed to reduce IOP, the eye surgeon
may perform trabeculectomy, drainage implant surgery, or ciliary body destructive procedures.
Trabeculectomy
During trabeculectomy, the ophthalmologist creates an alternate pathway (or
drainage channel) in the eye to increase the passage of fluid from the eye. By constructing a
new drainage channel, the fluid is able to flow better outside the eye. As a result, IOP is
lowered.
Medicines, called antimetabolites, are sometimes used in conjunction with
trabeculectomy. They help reduce scarring and increase the chance of IOP being lowered.
Drainage implant surgery
If goniotomy and trabeculotomy fail, drainage implant surgery may be
performed.
In drainage implant surgery, the ophthalmologist places a tube in the anterior
chamber to shunt the aqueous humor.
Different types of implants can be used, but most function by allowing drainage
of the aqueous humor from the anterior chamber, thereby lowering IOP.
Ciliary body destructive procedures

In those situations in which all else has failed, ciliary body destructive
procedures (also called ciliary body ablation or cyclodestructive surgery) may be useful.
In this procedure, the ophthalmologist uses a laser to destroy a portion of the
ciliary body, thereby limiting the production of aqueous humor. IOP is lowered because of
less fluid being able to build up inside the eye.
Possible surgical complications
As with any surgical procedure, complications may occur but are rare. Serious complications
associated with surgery may include the following:
Hyphema (bleeding in the anterior chamber)
Infection
Lens damage
Uveitis (inflammation of the uvea, that is, the iris, the ciliary body, and the choroid)
The most serious complications in children are often caused by general anesthesia. In some
children, because of the risks associated with anesthesia, both eyes may be operated on at the
same time (called a bilateral procedure).
Examination
It is important to do a complete ophthalmic exam in a child suspected of glaucoma. This exam
must include IOP measurement, gonioscopy, inspection of the optic nerve and refraction. Check
for the childs ability to fix and follow and for the presence of nystagmus. Examination of the
cornea is crucial, with respect to the size and clarity of the cornea and the presence of Haab
striae. The anterior chamber angle of a healthy infant differs from that of a healthy adult and
from that of a child with glaucoma, as described in Angle Comparisons (previous page). The
optic nerve exam usually reveals a cup-to-disc ratio greater than 0.3 in infants with glaucoma.
This ratio is usually less than 0.3 in healthy newborns. Asymmetry in the cup-to-disc ratios is
also suggestive of glaucoma. Although cupping may occur rapidly in infants, it is reversible with
surgical management and IOP control. This reversibility is most common in early stages of
glaucoma in children, especially in the first year of life when the connective tissue of the optic
nerve is relatively elastic.1,2
Although measuring IOP can be challenging, this is a necessary part of the exam and can be done
in the office with a conscious, swaddled infant. Bottle-feeding may help to calm the infant. A
Tono-Pen or a Perkins or Goldmann applanation tonometer may be used. In infants with healthy
eyes, the IOP is in the range of 10 to 12 mmHg, and it reaches approximately 14 mmHg by age 7
or 8 years. An IOP measurement of greater than 20 mmHg in a calm resting infant raises the
suspicion of glaucoma when other signs and symptoms suggest the disease. Likewise,
asymmetry of more than 5 mmHg is of concern. IOP measurements taken while a child is crying
are unreliable, since the Valsalva maneuver and eyelid squeezing can cause an IOP of 30 to 40
mmHg even in healthy eyes.

If it is not possible to get an IOP measurement in the office, an exam under anesthesia that
includes optic nerve examination should be performed. General anesthetics can lower the IOP.
However, in primary congenital glaucoma, measurements made soon after induction of general
anesthesia are still usually greater than 20 mmHg, though pressure typically drops after this early
stage.
For the sake of comparison, the reported IOP in a healthy infant under halothane anesthesia is 9
to 10 mmHg. It appears that conscious sedation with chloral hydrate has the least effect on IOP
measurement, with no statistical significance noted in studies.3 A study using chloral hydrate
found IOP in healthy children to range from 11 to 17 mmHg. Some agents are known to increase
IOP, including ketamine (minimal to mild) and succinylcholine (marked).

Conclusion
The main goal in managing primary congenital glaucoma is early diagnosis and therefore early
surgical treatment to normalize IOP, in order to allow possible reversal of cupping and thus to
minimize the impact on vision.
Treatment
Medical therapy is generally used as a temporizing measure to help control IOP and clear the
cornea prior to surgery. However, it may become more integral to the treatment strategy in cases
where surgery is not possible due to medical reasons or when surgery is inadequate. Medical
treatment can be challenging since most of the relevant medications are not approved for use in
children.
Surgery. The main treatment options are surgical and include goniotomy and trabeculotomy.
Surgical planning begins with a comprehensive examination under anesthesia. As long as the
cornea is clear, either procedure may be performed.
In goniotomy, an incision is made across the trabecular meshwork under direct gonioscopic
visualization. In trabeculotomy, the trabecular meshwork is incised by way of an external
approach through Schlemms canal. If the cornea is hazy or opaque, trabeculotomy is the better
choice.
These procedures have high success rates, up to 90 percent or greater in some series. Most
infants with primary congenital glaucoma who present between 3 months and 1 year of age will
have their IOP controlled by one or two angle surgeries. If IOP control is not achieved with the
first surgery, at least one other angle surgery is attempted before trying another strategy. When
angle surgery fails and medical therapy is inadequate, other options may be considered, such as
tube implant procedures, trabeculectomy and cryoablation. Successful combined trabeculotomytrabeculectomy has been reported.4 Shunt procedures were found to be more successful than
trabeculectomy in patients under 2 years of age