Introduction

Childhood glaucoma encompasses a group of potentially blinding eye conditions characterized by elevated intraocular pressure (IOP) and optic nerve damage, occurring in the pediatric age group.

Key Points:

• Incidence: 1 in 10,000-20,000 live births
• Leading cause of pediatric blindness
• Early diagnosis crucial for visual prognosis
• Requires lifelong monitoring
• Genetic counseling often necessary

Classification

1. Primary Childhood Glaucoma:

• Primary Congenital Glaucoma (PCG)
  • Present at birth or early infancy
  • Isolated angle abnormality
  • Most common form (50-70%)
  • CYP1B1 gene mutations
• Juvenile Open Angle Glaucoma (JOAG)
  • Onset between 3-20 years
  • MYOC gene mutations
  • Family history often present

2. Secondary Childhood Glaucoma:

• Associated with Ocular Anomalies
  • Axenfeld-Rieger syndrome
  • Peters anomaly
  • Aniridia
  • Sturge-Weber syndrome
• Following Ocular Conditions/Surgery
  • Aphakic/pseudophakic glaucoma
  • Post-traumatic
  • Post-uveitic
  • Steroid-induced

Clinical Features

1. Classical Triad:

• Epiphora (Tearing)
• Photophobia
• Blepharospasm

2. Age-Specific Signs:

• Infants (< 3 years)
  • Enlarged cornea (>11mm)
  • Buphthalmos
  • Haab's striae
  • Corneal haze
• Older Children
  • Optic disc cupping
  • Visual field defects
  • Myopic shift
  • Reduced vision

3. Associated Features:

• High IOP (>21 mmHg)
• Corneal edema
• Iris abnormalities
• Lens abnormalities
• Angle abnormalities

Diagnosis

1. Initial Evaluation:

• Examination Under Anesthesia (EUA)
  • IOP measurement
  • Corneal diameter measurement
  • Gonioscopy
  • Optic nerve evaluation
• Measurements
  • Axial length
  • Keratometry
  • Refraction

2. Imaging:

• Anterior Segment
  • Anterior segment OCT
  • UBM when indicated
  • Photography
• Posterior Segment
  • Fundus photography
  • OCT of optic nerve and macula
  • Visual fields (when age appropriate)

3. Genetic Testing:

• CYP1B1 gene analysis
• MYOC gene testing
• PITX2, FOXC1 testing when indicated
• Family screening

Management

1. Medical Management:

• First-line Medications
  • Beta-blockers (timolol)
  • Carbonic anhydrase inhibitors
  • Prostaglandin analogues
• Considerations
  • Age-appropriate dosing
  • Systemic side effects
  • Compliance issues

2. Surgical Management:

• Primary Surgery
  • Angle surgery (goniotomy/trabeculotomy)
  • Combined trabeculotomy-trabeculectomy
  • Success rate 80-90% in PCG
• Secondary Procedures
  • Trabeculectomy with antimetabolites
  • Glaucoma drainage devices
  • Cyclodestructive procedures

3. Rehabilitation:

• Amblyopia management
• Refractive correction
• Visual rehabilitation
• Early intervention services

Prognosis & Follow-up

1. Prognostic Factors:

• Favorable
  • Early diagnosis
  • Good initial response to treatment
  • Unilateral cases
  • Primary glaucoma
• Poor
  • Delayed presentation
  • Associated anomalies
  • Failed initial surgery
  • Secondary glaucoma

2. Monitoring Schedule:

• Early Post-op
  • Weekly for first month
  • Monthly for 3-6 months
• Long-term
  • 3-6 monthly follow-up
  • Regular EUA if needed
  • Visual field testing
  • OCT monitoring

3. Quality of Life Considerations:

• Educational support
• Psychological support
• Family counseling
• Genetic counseling
• Support groups