Developmental issues with the eye during the fetal stage of the baby can hinder the proper drainage of the fluid that fills the front part of the eye. 

The fluid know as Aqueous humor plays a vital role in maintaining the eye’s shape, providing essential nutrients, and ensuring the eye’s health.

However, when developmental problems in a fetus disrupt the eye’s drainage system, the old fluid cannot exit, leading to its accumulation within the eye.

This fluid buildup exerts pressure on the optic nerve, resulting in reduced vision or complete blindness. 

This condition of the eye is known as Cogenital Glaucoma.

If the disease is found early, 80% to 90% of children respond well to treatment.

Read on to learn more about the causes, symptoms, and treatment of congenital Glaucoma.

Fact:
As per the CDC, Glaucoma ranks as the second most common cause of blindness globally and holds the top position for causing blindness in individuals aged 60 and above. In the United States, Glaucoma contributes to approximately 9–12% of all cases of blindness.

What is Congenital Glaucoma?

Congenital Glaucoma, also known as childhood Glaucoma, is a rare condition that impacts infants and young children. 

In this condition, the eye’s drainage system fails to develop correctly, resulting in elevated intraocular pressure. 

This heightened pressure harms the optic nerve, which conveys information from the retina to the brain. 

Typically, doctors diagnose it during a child’s first year of life. 

Without proper treatment, Glaucoma can put your vision at risk.

According to WebMD, it affects about one in every 10,000 infants, and untreated cases significantly cause childhood blindness.

Caution:
It’s essential to have regular eye exams that include measurements of your eye pressure. If Glaucoma is recognized early, vision loss can be slowed or prevented. You’ll need treatment or monitoring for the rest of your life if you have Glaucoma.

How many types of Congenital Glaucoma are there?

Congenital Glaucoma encompasses two distinct eye conditions.

Primary Congenital Glaucoma, which is present at birth due to abnormal eye development, and Secondary Congenital Glaucoma, typically arising from other eye or health issues.

Both conditions fall under the umbrella of childhood glaucoma.

Primary Glaucoma

Primary congenital Glaucoma, often called PCG, is an independent condition unrelated to other health issues at birth.

The occurence of PCG is approximately one in 10,000 newborns, with potential variations based on parental ethnicity.

Typically, PCG affects both eyes, but it can also manifest in just one eye, and it is more common in boys than in girls.

True Congenital Glaucoma

True Congenital Glaucoma, also known as Newborn Glaucoma, is a type of Primary Glaucoma. 

It is characterized by increased fluid pressure in the eyes.

This condition is noticeable within the first month of a child’s life.

Research suggests that elevated pressure at the optic nerve may occur during intrauterine development, contributing to the condition.

Newborn Glaucoma cases account for approximately 25 percent of all congenital Glaucoma cases, making it a relatively rare but significant form of the disease.

Infantile Glaucoma

Infantile Glaucoma, a subtype of primary congenital Glaucoma, typically presents in children aged 1 to 36 months.

This category comprises approximately 65 percent of all patients diagnosed with primary congenital Glaucoma.

Unlike True congenital or newborn Glaucoma, this condition appears within the first few years of life, making it a relatively common one.

Juvenile Glaucoma

Juvenile Glaucoma is a category that comprises children who display high eye pressure symptoms after the age of three but before reaching adulthood.

This specific form of Glaucoma accounts for approximately 10 percent of all diagnosed cases.

Unlike congenital or Infantile Glaucoma, Juvenile Glaucoma develops later in childhood, highlighting the importance of ongoing eye health monitoring.

Eye drops and oral medications are commonly utilized in the treatment of various forms of Glaucoma, including secondary and juvenile cases. Furthermore, they are frequently employed as part of the therapeutic practice after surgical treatments.

Secondary Glaucoma

Secondary congenital Glaucoma also begins at birth, resulting from a distinct eye condition or underlying health issue.

They occur as the result of the symptoms of another genetic disorder in the body such as Peters Anamoly and  juvenile idiopathic arthritis. 

Congenital Glaucoma Symptoms

Sensitivity to light
Sensitivity to light

Parents often seek the expertise of an eye doctor or pediatrician when they observe unusual characteristics related to their child’s eyes or vision.

The three most prevalent indicators of primary congenital Glaucoma encompass:

  • Sensitivity to light
  • Excessive tearing of the eyes
  • Abnormal eyelid twitching or blinking

Additional observations may include:

  • The cornea’s front layer displays cloudiness
  • The white part of the eye exhibits a bluish hue
  • An apparent enlargement of one or both eyes

An eye specialist will thoroughly examine specific signs indicative of PCG, which may be challenging or impossible for parents to discern independently. 

These signs include:

  • Enlargement of the cornea (the transparent front eye layer)
  • The presence of Haab’s striae, which are stretch marks inside the cornea
  • Irregularly shaped pupil (the small black aperture at the eye’s front)
  • Irregularities on the iris (the colored portion of the eye)
  • Elevated intraocular pressure 

Causes of Congenital Glaucoma

The eye continuously generates fluid to supply nutrients and moisture to other parts of the eye. 

It then expels the surplus fluid through a microscopic web called the trabecular meshwork, allowing it to re-enter the bloodstream.

Primary congenital Glaucoma typically occurs when:

  • The child’s drainage angle in the eye doesn’t develop correctly
  • Inadequate drainage leads to pressure buildup inside the eye
  • This elevated pressure leads to eye damage

Eye doctors aim to prevent or reverse these detrimental changes, as this damage can result in permanent vision loss.

Diagnosis of Congenital Glaucoma

Diagnosis of Congenital Glaucoma 
Diagnosis of Congenital Glaucoma 

Early diagnosis of congenital Glaucoma in children is crucial for preserving their vision, as this condition can progress silently without any apparent symptoms.

It is advised to have regular eye examinations by an eye doctor for timely detection.

These check-ups also rule out other eye conditions that may mimic primary or secondary Congenital Glaucoma. 

The American Optometric Association (AOA) provides a recommended examination schedule:

  • The first eye examination should be scheduled between 6 and 12 months of age
  • Then another one eye exam between ages 3 and 5
  • Another eye exam before entering 1st grade
  • Annual eye exams from 2nd grade onward

During an eye examination, the eye doctor employs specialized tools like ophthalmoscope and a slit lamp to assess your child’s eyes’ surface and internal structures.

While these Glaucoma tests are painless, they can be challenging for babies or young children. 

If congenital Glaucoma is suspected, the doctor may recommend examining under general anesthesia to ensure a thorough evaluation.

Treatment of Congenital Glaucoma

The primary objective of Glaucoma treatment is to diminish the additional pressure obstructing the function of the eye. 

Typically, this involves surgical interventions to facilitate fluid drainage to lower eye pressure, although medications can also be effective.

Surgical treatments encompass:

  • Angle surgery: This procedure repairs the eye to prevent the recurrence of congenital Glaucoma and enhances fluid drainage
  • Filtering surgery: It creates an outlet in the eye to allow fluid to escape, alleviating pressure on the optic nerve
  • Cycloablation: Employing a laser, this method targets and eliminates fluid-producing tissue within the eye

However, it is usually considered when other treatments prove ineffective.

Before surgery, a physician may recommend a regimen of topical or oral antiGlaucoma medications, such as eye drops or tablets.

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    • These medications aid in reducing intraocular pressure and corneal swelling.

    While treatment cannot recover lost vision, it can effectively stop the progression of symptoms and safeguard against further deterioration of vision. 

    Many children who undergo treatment can lead typical lives with their remaining vision intact.

    Takeaway

    Congenital Glaucoma, a rare condition affecting infants and children, occurs due to improper drainage of the eye’s fluid. 

    This elevated intraocular pressure damages the optic nerve, potentially leading to blindness. Early detection through regular eye exams is crucial. 

    There are two main types of Congenital Glaucoma: Primary (present at birth) and Secondary (resulting from other health issues).

    Treatment involves surgery and medication to lower eye pressure and preserve vision. 

    While treatment can’t restore lost vision, it can halt further deterioration, enabling affected children to lead normal lives with their remaining vision intact.

    Frequently Asked Questions

    What is the cause of congenital Glaucoma?

    Congenital Glaucoma results from birth-related structural eye defects hindering fluid drainage, causing heightened intraocular pressure. Untreated, this can harm the optic nerve, potentially leading to vision loss or blindness.

    What are signs of congenital Glaucoma?

    Signs of congenital Glaucoma in include photophobia, excessive tearing, abnormal eyelid twitching, cloudy corneas, eye enlargement, bluish sclerae, and poor visual responsiveness. Timely medical evaluation is essential to prevent vision loss or blindness associated with this condition.

    What is the most common symptom of congenital Glaucoma?

    The most common symptom of congenital Glaucoma is excessive tearing, also known as epiphora. Other common symptoms include sensitivity to light, abnormal blinking or twitching of the eyelids, cloudiness in the cornea, and enlargement of one or both eyes.

    When does congenital Glaucoma start?

    Congenital Glaucoma typically starts at birth or during an infant’s early months. It’s present from birth, with symptoms emerging early in a child’s development. Timely detection and intervention are vital to effectively manage the condition and prevent vision loss.

    What is the best treatment for congenital Glaucoma?

    The optimal treatment for congenital Glaucoma typically involves surgical procedures aimed at improving the drainage of fluid from the eye and reducing intraocular pressure. Medications may complement surgery in some cases. Early diagnosis and prompt surgical intervention are crucial for preserving vision in children with congenital Glaucoma.

    Can you cure congenital Glaucoma?

    Congenital Glaucoma is typically not curable but can be managed and controlled. Treatment aims to reduce intraocular pressure, preserving vision and preventing optic nerve damage. Early diagnosis and proper treatment enable many individuals with congenital Glaucoma to lead normal lives with manageable eye health.

    Citations:
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