Congenital Glaucoma: Types, Symptoms, Causes, Signs, Treatment and Prognosis

Doctors usually detect it between the ages of 3 to 6 months, but there may be no signs at the beginning. It can be diagnosed until 3 years old.

Primary congenital glaucoma is a disease in which the optic nerve is damaged by pressure caused by fluid, the most affected are children up to 3 years of age; This disease is serious and requires your prompt attention.

It affects one in ten thousand babies, and if it is not treated in time it can cause childhood blindness. The term “Primary” means that the disease is not the result of another or condition, such as a tumor. “Congenital” means that it is present from birth.

If the disease is detected early, between 80% and 90% of children respond well to treatment. They will not have vision problems in the future.

Congenital glaucoma is a global problem and poses a diagnostic and therapeutic challenge for the ophthalmologist . A detailed evaluation is recommended under general anesthesia to establish the diagnosis and plan for treatment.

Medical therapy has a limited role and surgery remains the main therapeutic modality.

Trabeculectomy with antifibrotic agent and glaucoma drainage devices has a role in the treatment of refractory cases, and cyclodestructive procedures should be reserved for patients where these procedures have failed.

Early diagnosis, immediate therapeutic intervention and adequate refractive correction are key to success.

The management of residual vision and visual rehabilitation should be an integral part of the treatment of children with low vision and lifelong follow-up is essential.

How Glaucoma Affects Vision

Glaucoma involves loss of vision due to damage to the optic nerve. The optic nerve carries visual images to the brain and any damage to the nerve damages the eyesight.

For the eye to work properly, a certain level of pressure is needed for the eye to maintain its shape, but if the eye’s pressure is too high, it squeezes the optic nerve and destroys some of the nerve fibers, leading to loss of vision.

The first areas that will be affected are the decentered parts of the vision. If glaucoma is not treated, the damage can progress to tunnel vision and eventual loss of central vision, although blindness is rare.

Generally, but not always, the damage occurs because the pressure inside the eye increases and presses on the nerve, which damages it.

Types of Glaucoma

There are four main types of glaucoma: primary open-angle glaucoma, primary angle-closure glaucoma, secondary glaucoma, developmental glaucoma (congenital glaucoma).

Primary open-angle glaucoma : This is the most common type of glaucoma and develops very slowly.

Closed angle glaucoma : This is rare and may occur slowly (chronic) or may develop rapidly (acute) with a sudden and painful buildup of pressure in the eye.

Secondary glaucoma : occurs as a result of an eye injury or other ocular condition, such as uveitis (inflammation of the middle layer of the eye).

Developmental glaucoma (congenital glaucoma): This is rare, but it can be serious. It is usually present at birth or develops soon after birth. It is caused by an abnormality of the eye.

You also have an increased risk of developing open-angle glaucoma if you are of African-American or black Caribbean origin.

The other types of glaucoma, such as acute angle-closure glaucoma, are much less common. However, people of Asian origin are more at risk of getting this type of glaucoma than those of other ethnic groups.

What are the symptoms?

Many people with glaucoma suffer from high pressure inside the eye and, if you have this, it can be a risk factor for glaucoma. However, unfortunately for many people, to the point where you start to lose vision, there are very few symptoms: in fact, it is estimated that up to 50% of people in developed countries do not know they have it during the early stages .

What is scary with glaucoma is that you can have it but not be aware of it, by the time you realize that something is wrong in your vision, the damage is already done. Then you can prevent it from getting worse but you can not recover your previous vision.

What are the causes of primary congenital glaucoma?

Most cases of primary congenital glaucoma occur sporadically. However, some cases are genetic and, therefore, inherited. In these cases, genetic testing of the parents and siblings of the affected child can be considered.

What are the primary signs of congenital glaucoma?

The high eye pressure can exert a very strong touch on the wall of the eye and cause expansion. The eye may appear enlarged. This is called Buftalmos. The cornea (the transparent front layer of the eye) can also become cloudy and appear whitish gray.

When should someone seek medical attention for primary congenital glaucoma?

Any child suspected of having glaucoma should be seen as soon as possible by an ophthalmologist. The sooner the diagnosis is made and the treatment is started, the greater the chances of preventing permanent loss of vision. The initial eye examination is done in the office, and additional tests can be performed in an operating room under sedation, if warranted.

What tests and tests do health professionals use to diagnose primary congenital glaucoma?

Depending on the age of the child and the level of cooperation, part of the examination can be done in the office. In babies, the test is often less challenging if it is done when the baby is relaxed or sleepy, such as during feeding or soon after.

In most cases, additional tests and tests should be performed under sedation or anesthesia, and arrangements can be made to treat them at the time of diagnosis.

The ophthalmologist begins by asking caregivers about the duration of the child’s symptoms and any known family history of glaucoma or other eye disorders. The following tests are performed in the office and / or in the operating room:

  • Vision check : in a baby, this can be limited to checking if you can look at an object and follow a moving object with your eyes.
  • Refraction is measured : this is a measure of myopia, hyperopia or astigmatism. In PCG, high eye pressure can cause myopia (myopia) as a result of an increase in eye length and astigmatism as a result of scarring or swelling of the cornea.
  • Tonometry to measure the pressure of the eye : this measure of the size of the eye determines if it is enlarged (Buftalmos). The diameter of the cornea is measured using gauges, and the length of the eye is measured using an ultrasound.

Examination of the cornea checks for cloudiness and small cuts in the posterior layer (called Haab striae) that sometimes occur when the cornea is stretched under high eye pressure.

  • You can also make a measure of the thickness of the cornea ( pachymetry ).
  • Direct visualization of the trabecular network : it is called gonioscopy and it is done with contact lenses and special mirrors. This test is important in determining whether the angles (the area where the trabecular meshwork is located) are open, narrowed or closed, and whether there are other conditions, such as scar tissue in the angle.
  • The optic nerves are carefully examined : (using ophthalmoscopy) to detect signs of glaucomatous optic nerve damage. This may require dilating the students to ensure adequate vision.
  • Photographs of the fundus of the eye : which are images of the optic nerve, can be taken for future references and comparisons.

What is the treatment for primary congenital glaucoma?

Primary congenital glaucoma is almost always treated surgically.

Drugs in the form of eye drops are used initially to reduce eye pressure before surgery. Other medications may also be used to help decrease the opacity of the cornea in preparation for surgery.

The surgery is done to allow the aqueous fluid to drain more easily into the bloodstream.

The most common surgical procedures for primary congenital glaucoma are goniotomy and trabeculotomy. In both procedures, the ophthalmologist makes an incision through the trabecular fibers of the mesh that are preventing the flow of water.

In goniotomy, the procedure is performed by inserting an instrument into the eye and visualizing the angle directly with a gonioscopy lens. This can only be done if the cornea is clear.

In a trabeculotomy, the trabecular meshwork is approached through an incision made in the outer wall of the eye; This is the preferred procedure if the cornea is too cloudy for a goniotomy.

Goniotomy and trabeculotomy have similar success rates of approximately 80%.

If medications and goniotomies and / or trabeculotomies fail to reduce eye pressure sufficiently, the remaining options are to perform surgery that allows the aqueous to avoid the trabecular meshwork (trabeculectomy or surgery of drainage implants) or reduce the production of bodies aqueous (ciliary body ablation procedures).

In a trabeculectomy, the surgeon creates a new drainage channel that allows the aqueous to drain directly from the inside of the eye into a small tissue pocket (called a blister) and then back into the bloodstream, which reduces eye pressure.

Medications called antimetabolites are sometimes used together with trabeculectomy to reduce the chances of scarring the blister.

In drainage implant surgery, the aqueous is drilled into the bloodstream through an implanted drainage device (eg, Molteno, Baerveldt or Ahmed implants).

Ciliary body ablation procedures are generally reserved as a treatment of last resort.

In this procedure, the ophthalmologist uses a diode laser or a freezing probe (cryotherapy) to intentionally destroy a part of the ciliary body, which is the organ in the eye that produces the aqueous.

Eye pressure is reduced because less fluid accumulates inside the eye. After any surgery, it is important to perform a thorough follow-up since complications can occur with any surgical procedure.

Serious complications can include the accumulation of blood in the eye (hyphema), infection, damage to the lens, uveitis or internal swelling (inflammation) and too low eye pressure (hypotonia).

Because children can also have complications with general anesthesia, both eyes can be treated in the same session in the operating room (bilateral procedure).

Is a follow-up necessary after treatment of primary congenital glaucoma?

Frequent follow-up visits are necessary after surgery and close monitoring if eye pressures and optic nerves will be needed throughout the patient’s life. The optic nerve remains vulnerable if the eye pressure starts to rise again for any reason.

In addition, children will need close supervision of their refractions. If the eye is found to have significant myopia (myopia), hyperopia (farsightedness) or astigmatism, glasses or contact lenses can be used to prevent amblyopia (blurred or loose eyes).

If amblyopia is already present, a regime of eyeglasses or contact lenses may be needed along with patches.

If the cornea, lens, or other parts of the eye have scars or damage as a result of eye pressure, swelling (inflammation), or surgical treatment, additional procedures may also be necessary.

Is it possible to prevent primary congenital glaucoma?

Primary congenital glaucoma can not be prevented. Many cases are genetic, inherited as a recessive trait, which means that it could bypass generations and it can be difficult to trace them in family history.

Once suspected, early treatment by an ophthalmologist can reduce the chances of permanent vision loss.

What is the prognosis of primary congenital glaucoma?

The prognosis depends on both the age of onset and the time of initial treatment. PCG that is present at birth carries the greatest risk of vision loss.

The most favorable result is seen in babies whose symptoms appear between 3 and 12 months of age and undergo rapid treatment.

The exhaustive treatment includes not only reducing eye pressure but also quickly addressing any additional factors such as refractive error, corneal / lens damage and amblyopia.

Close follow-up to detect any new complications as they arise will also improve the prognosis.

Support and counseling groups for primary congenital glaucoma

Parents and caregivers should understand that high eye pressure can recur at any age in a child with primary congenital glaucoma. For this reason, regular eye exams with an ophthalmologist are very important.

Support groups and counseling may also be available through several organizations specializing in vision care.

History of the disease in a baby

A baby was born without complications during her pregnancy, a month later the mother notes that her baby’s eyes were crying and she did not like the high lights, she noticed that sometimes it seemed that her baby would close her eyes. The baby’s pediatrician noticed that his cornea appeared cloudy and he was referred to an ophthalmologist.

The Exam in the Baby

The ophthalmologist detected a high pressure inside the eye, the diameter of the cornea increased fifteen millimeters and microcystic edema with Haab striae. On examination, the child’s optic nerve heads appeared normal.

Treatment

It started with a topical prostaglandin analog. The pressure of the liquid was reduced in both eyes, then it was taken to an ophthalmology center specialized in the treatment of congenital glaucoma, after a complete examination and under anesthesia, a goniotomy was performed in both eyes; the baby grew and has good visual acuity.