What is the treatment for congenital glaucoma?

Treatment for Congenital Glaucoma

Surgery is the primary and definitive treatment for congenital glaucoma, with trabeculectomy being the recommended first-line surgical intervention, particularly in settings where presentation is late or surgeons may not be familiar with goniotomy techniques. 1

Diagnostic Evaluation

• Classic presentation includes:

  • Tearing (epiphora)
  • Photophobia
  • Blepharospasm
  • Enlarged cornea/eye (buphthalmos)
  • Corneal edema
  • Haab's striae (breaks in Descemet's membrane)
  • Elevated intraocular pressure (IOP)
  • Increased axial length

• Examination under anesthesia (EUA) is typically required for accurate diagnosis and assessment in infants and young children 2, 3

Treatment Algorithm

First-line Treatment: Surgery

1. Angle Surgery Options:

   • Trabeculectomy: Most effective surgical option with normalization of IOP in 76-84% of eyes long-term 1
   • Circumferential trabeculotomy: Recent advance allowing treatment of the entire angle in one operation 3
   • Goniotomy: Option for cases with good corneal clarity
   • Trabeculotomy: Alternative angle procedure

2. Timing of Surgery:

   • Perform as soon as possible after diagnosis
   • Early intervention is critical to prevent irreversible vision loss

Second-line Treatment Options (After Failed Initial Surgery):

1. Additional angle surgery if first procedure was partially successful

2. Glaucoma drainage device surgery (e.g., Ahmed glaucoma valve) for refractory cases 4

3. Medical therapy:

   • Limited effectiveness as primary treatment (successful in only 9.7% of cases long-term) 1
   • Often used as adjunctive therapy after surgery
   • May include:
     • Prostaglandin analogs
     • Beta-blockers (with caution in infants)
     • Alpha-2 agonists (contraindicated in very young children)
     • Carbonic anhydrase inhibitors

Special Considerations

• Genetic factors: PCG is associated with CYP1B1 gene mutations and is more common in populations with higher rates of consanguinity 3, 5

• Coexisting conditions: When congenital glaucoma occurs with other conditions like retinopathy of prematurity, coordinated care between subspecialties is essential 4

• Long-term management:

  • Regular follow-up examinations under anesthesia until reliable office examinations are possible
  • Ongoing monitoring of IOP, corneal diameter, axial length, and optic nerve appearance
  • Many patients require topical medications for IOP control even after successful surgery 4

Prognosis

• Early diagnosis and prompt surgical intervention significantly improve visual outcomes
• Without treatment, congenital glaucoma leads to blindness
• Even with successful treatment, patients require lifelong monitoring
• Multiple surgeries may be necessary throughout the patient's lifetime

Pitfalls and Caveats

• Delayed diagnosis can lead to irreversible vision loss
• Congenital glaucoma may initially present without the classic triad of symptoms, leading to diagnostic delays 2
• Medical therapy alone is rarely sufficient for long-term IOP control 1
• Patients often need continued medical therapy even after successful surgical intervention 4
• Regular follow-up is essential to monitor for disease progression and complications