What Is Congenital Glaucoma? Causes, Symptoms & Treatment Explained
Glaucoma is often regarded as a silent thief of sight, typically associated with aging. Very rarely, it can affect toddlers and infants, a condition which is referred to as congenital glaucoma. Unlike adult-onset glaucoma, this form of the disease is present at birth or develops within the first few years of life. To maintain vision and avoid lifelong complications, it is important to manage this disorder as soon as it is diagnosed. In this blog, we will equip you with the causes, symptoms, treatment options and prognosis of the condition so as to provide value for you as a parent or a medical enthusiast.
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What Is Congenital Glaucoma?
Congenital glaucoma is an uncommon form of glaucoma that impacts infants and toddlers, generally within the first year of life. It results from inadequate development of the eye’s drainage system during the intrauterine period, resulting in intraocular pressure (IOP) elevation, which is characteristically glaucoma.
In a normal glaucoma eye, aqueous humor (the fluid inside the eye) circulates through a drain located at the intersection between the iris and cornea, which euros. In congenital cases, this drainage system is either absent or disformed, leading to fluid retention and raised pressure. The increased pressure in turn, damages the optic nerve – responsible for relaying the visual signals to the brain.
In the absence of any other ocular or systemic abnormality, it’s also referred to as primary congenital glaucoma (PCG). In case of association with other conditions, it’s known as secondary congenital glaucoma.
How Common Is Congenital Glaucoma?
Congenital glaucoma, albeit rare, remains a prominent cause of vision loss and blindness among children. The condition is estimated to occur in 1 in 10,000 to 20,000 live births worldwide. It is more prevalent in populations that practice consanguineous (close-relative) marriages.
Boys tend to be slightly more affected than girls, and in approximately 70% of occurrences the condition is bilateral (affects both eyes).
Glaucoma Causes: What are the Common Causes of Congenital Glaucoma?
The congenital glaucoma causes are mainly associated to the congenital anomalies of the trabecular meshwork and anterior chamber angle which are responsible for the outflow system of the aqueous humor.
Key Causes Include:
1. Genetic Causes
- Genetic alterations, including CYP1B1 gene mutations, are associated with primary congenital glaucoma.
- Autosomal recessive is the most prevalent mode of inheritance.
2. Lack of Proper Development
- Incomplete development of the drainage angle and meshwork during embryonic life.
3. Secondary Causes
- Sturge-Weber and Axenfeld-Rieger syndromes are some of the secondary congenital glaucoma causing syndromes.
Important Note: Despite the role of family history, many congenital glaucoma cases are idiopathic.
Warning Signs and Symptoms of Congenital Glaucoma
Unlike the adult form of glaucoma, which tends to be asymptomatic in the early stages, congenital glaucoma symptoms typically present early and can be quite concerning for caregivers.
Key Early Signs and Symptoms Are:
1, Buphthalmos or abnormal ocular enlargement.
- Because of the relatively soft structure of an infant’s eye, a proportion of elevated intraocular pressure can result in stretching of the eyeball.
2. Epiphora (excessive tearing)
- This condition manifests as chronic tears.
3. Photophobia (light sensitivity)
- In infants, this can present as avoiding bright lights with their eyes either closing or turning away.
4. Cloudy or hazy cornea
This shows the presence of corneal edema associated with elevated intraocular pressure.
- Frequent eye rubbing or squinting
- Irritability and poor vision development
Vision problems in infants usually appear alongside developmental delays, which many parents notice. Seeking professional help from a pediatric ophthalmologist is imperative, as treatment avenues reduce with age and can lead to irreversible vision damage.
Congenital Glaucoma Diagnosis
An accurate diagnosis of congenital glaucoma is critical to effective management and outcomes, which in turn depends on early intervention. The comprehensive assessment may involve:
- Clinical evaluation using sedation or anesthesia
Infants often require general anesthesia for still examinations, so they do not move around during the procedure.
- Measurement of intraocular pressure (IOP)
- Checking corneal diameter and clarity
- Anterior chamber angle examination with gonioscopy
- Evaluation of optic nerve
- B-scan ultrasound if cloudy cornea obstructs view
Some cases may benefit from genetic assessment or consultation, especially if there is a known history in the family.
Treatment Approaches for Congenital Glaucoma
The main purpose of congenital glaucoma treatment is to manage intraocular pressure and prevent progressive damage to the optic nerve while attempting to preserve vision. In contrast to adult glaucoma, surgery is often the first treatment approach in children, as medications are unlikely to be effective on their own.
1. Surgical Treatments:
i). Goniotomy
- One of the more common first surgical approaches.
- Involves opening the trabecular meshwork and the overlying portion of the cornea.
ii). Trabeculotomy
- Access and open the drainage canal from outside the eye.
iii). Combined Trabeculotomy and Trabeculectomy
- Usually performed in more advanced or severe cases.
iv). Glaucoma Drainage Devices
- Used when initial surgeries are unsuccessful.
v). Cyclophotocoagulation
- Last resort laser treatment which reduces fluid production.
2. Medications:
The medications are not ideal for the long term, but may offer short-term solutions and should be prescribed by an experienced and learned doctor. These can be prescribed in the lead-up to surgery or when there are delays in surgical intervention.
Important Note: Consistent monitoring remains vital even with successful surgical intervention due to the risk of intraocular pressure rising after some time.
Prognosis and Long-Term Outlook
If congenital glaucoma is treated early, many children are able to preserve some level of vision. However, prognoses may differ based on:
- Diagnosis and Detection Age
- Condition’s Severity
- Operative Procedure(s)
- Associated eye or systemic conditions, if any
Post-operative treatment may require children to undergo:
- Vision therapy sessions
- Spectacles or contact lenses
- Ongoing pediatric ophthalmologist appointments
- Amblyopia management in unilateral cases “lazy eye”
Close follow up is crucial to assess for potential IOP re-elevation, scarring, or other refractive changes.
Caring for a Child Blinded by Glaucoma: Things Parents Should Know
Looking after a glaucoma eye child may be difficult both emotionally and in day-to-day logistics. Parents can follow these steps to make it easier:
- Adhere to regular checkups as advised by your doctor.
- Look for signs of eye discomfort, redness, or photophobia, which can indicate elevated IOP.
- Aid with visual therapies and aids if necessary.
- Informed educators to support the child appropriately.
- Be concerned of hereditary risks for future siblings and if needed, seek family planning advice from a genetic counsellor.
Also Read – How to Know When I Need to See a Neuro ophthalmologist?
Conclusion
Congenital glaucoma, although rare, is a condition of great concern due to its ability to cause permanent vision impairment. Untreated, it has the potential to severely diminish a child’s eyesight. Understanding the early signs and symptoms, identifying the glaucoma causes, and receiving timely congenital glaucoma treatment can significantly aid in a child’s visual refinement and overall development.
A glaucoma eye can be addressed through surgical, medical, or rehabilitative means, requiring collaboration from multiple health professionals and sustained effort. It is heartening to note that with increased advocacy and improvement in pediatric ophthalmology, there is a significantly better outcome for children who are diagnosed early with congenital glaucoma.
As a parent or caregiver, do monitor for changes in eye symptoms and act on them. Often, sustained early action leads to safeguarding vision along with boundless possibilities.
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