Congenital Glaucoma

👶 Congenital Glaucoma (Pediatric Glaucoma)

Also known as Primary Congenital Glaucoma (PCG) or Infantile Glaucoma

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🧠 Definition

Congenital glaucoma is a rare condition present at birth or within the first few years of life, caused by abnormal development of the eye’s aqueous outflow system, especially the trabecular meshwork and anterior chamber angle, leading to increased intraocular pressure (IOP).

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🔍 Types of Congenital Glaucoma

1. Primary Congenital Glaucoma

   • Isolated developmental abnormality

   • Bilateral in 70–80% of cases

   • Most common form of glaucoma in infancy

2. Secondary Congenital Glaucoma

   • Associated with ocular or systemic conditions like:

     • Axenfeld-Rieger syndrome

     • Peter’s anomaly

     • Sturge-Weber syndrome

     • Aniridia

3. Infantile and Juvenile Glaucoma

   • Infantile: onset after 1 month but before 3 years

   • Juvenile: onset after age 3 to early adulthood

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🧬 Etiology & Pathogenesis

• Developmental anomaly of the trabecular meshwork and anterior chamber angle obstructs aqueous humor outflow.

• Leads to elevated IOP, stretching the eye structures (as the infantile eye is still elastic), and eventually damaging the optic nerve.

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👁️ Clinical Features (Classic Triad)

Symptom	Explanation
Epiphora (Tearing)	Due to corneal irritation from high IOP
Photophobia	Light sensitivity caused by corneal edema
Blepharospasm	Involuntary tight closure of eyelids

Additional signs:

• Buphthalmos: Enlargement of the eyeball

• Corneal clouding due to edema

• Haab’s striae: Horizontal breaks in Descemet’s membrane

• Enlarged corneal diameter (>12 mm)

• Optic disc cupping

• Elevated IOP (>21 mmHg)

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🩺 Diagnosis

1. Examination under anesthesia (EUA)

   • Accurate IOP measurement

   • Corneal diameter

   • Gonioscopy (angle evaluation)

   • Fundus examination (optic disc)

2. Tonometry

   • Use pediatric-friendly instruments (e.g., Perkins, Tono-Pen)

3. Ultrasound Biomicroscopy (UBM) or AS-OCT

   • Evaluates anterior segment structures

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💊 Management

1. Surgical Treatment (Mainstay)

Procedure	Indication
Goniotomy	First-line if cornea is clear
Trabeculotomy	If cornea is cloudy
Combined Trabeculotomy-Trabeculectomy	For advanced or resistant cases
Glaucoma Drainage Devices	For failed previous surgeries
Cycloablation (Cyclophotocoagulation)	As a last resort

2. Medical Treatment (Supportive/Pre-op)

• Used temporarily to control IOP before surgery

• Beta-blockers (e.g., Timolol) – caution in infants

• Carbonic anhydrase inhibitors (e.g., Acetazolamide)

• Prostaglandin analogs – variable effectiveness

• Miotics (Pilocarpine) – limited role

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🔄 Follow-Up & Prognosis

• Regular monitoring of IOP, optic nerve, visual acuity, and eye growth

• Lifelong follow-up needed

• Prognosis depends on age at diagnosis, timing of surgery, and degree of optic nerve damage

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⚠️ Complications if Untreated

• Permanent vision loss / blindness

• Amblyopia

• Strabismus

• Corneal scarring

• Photophobia & cosmetic disfigurement

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📝 Summary Table

Feature	Details
Onset	Birth to 3 years
Triad	Epiphora, Photophobia, Blepharospasm
Eye enlargement	Buphthalmos
Diagnosis	EUA, tonometry, gonioscopy
Treatment	Surgical (goniotomy/trabeculotomy)
Medical Rx	Temporarily lowers IOP pre-surgery
Prognosis	Better with early detection & treatment