What is the appropriate management and treatment for an infant or child with congenital corneal opacity?

Management of Congenital Corneal Opacity

For infants and children with congenital corneal opacity, surgical intervention with penetrating keratoplasty (PK) should be performed between 1 to 3 months of age for optimal visual development, with mandatory concurrent amblyopia management to maximize long-term visual outcomes. 1

Critical Timing Considerations

The timing of surgical intervention is paramount for visual development:

• The critical period for visual development is 2 to 6 months after birth, with a shorter window for unilateral versus bilateral opacities 1
• Pediatric corneal ophthalmologists prefer surgical intervention between 1 to 3 months of age to prevent irreversible amblyopia 1
• However, one study showed no difference in visual acuity or graft survival when keratoplasty was performed at 0-90 days versus 3-12 months of age, suggesting some flexibility in timing for bilateral cases 1

Diagnostic Evaluation

Before proceeding with surgery, comprehensive evaluation must include:

• Anterior segment OCT is invaluable for characterizing the type and extent of anterior segment disorder, even in neonates as young as 2 days old, as it is fast and noncontact 2
• Ultrasound biomicroscopy (UBM) is particularly helpful in congenital cases to image ruptured or dislocated Descemet membrane, retrocorneal membranes, and iridocorneal adhesions 1
• Assessment of laterality (unilateral vs bilateral), overall health status, and family support system all influence surgical timing and expected outcomes 1
• Evaluate for associated ocular abnormalities: iridocorneal adhesions (52.1% of cases) and cataracts (18.8%) are most common 3
• Screen for systemic abnormalities including growth retardation and congenital brain defects 3

Surgical Management Algorithm

Primary Surgical Approach by Etiology:

Penetrating Keratoplasty (PK) is the primary procedure for:

• Peters anomaly (95.9% of cases require PK) 3
• Sclerocornea (100% require PK) 3
• Congenital glaucoma-related opacities 4

Lamellar Keratoplasty (LK) is preferred for:

• Dermoids (100% treated with LK) 3
• Superficial opacities not involving endothelium 1

Key Surgical Considerations:

• Peters anomaly represents 40.3% of congenital corneal opacities and is the most common indication for pediatric keratoplasty 4
• Deep anterior lamellar keratoplasty (DALK) has lower rejection rates (1.9% vs 7.8% for PK) but carries a 60% graft failure rate in complex cases, mostly from poor endothelial function 1, 5
• Send all removed corneal tissue for pathologic and microbiologic analysis during therapeutic keratoplasty 5

Mandatory Amblyopia Management

Co-management with a pediatric optometrist or ophthalmologist for amblyopia treatment is essential to achieve optimal visual outcomes 1:

• Amblyopia therapy must begin immediately postoperatively
• Success rates are significantly reduced in African-American and Latinx children, requiring heightened vigilance and support 1
• Unilateral amblyopia carries a doubled lifetime risk of bilateral visual impairment, often from trauma to the fellow eye 1

Common Pitfalls to Avoid

• Do NOT delay surgery beyond 6 months in unilateral cases due to the critical period for visual development 1
• Do NOT use corticosteroids during active infection, especially with fungal or atypical organisms 5
• Do NOT assume isolated corneal pathology: 52.1% have iridocorneal adhesions and 18.8% have cataracts requiring concurrent management 3
• Do NOT neglect systemic evaluation: 5.6% have associated systemic abnormalities 3

Postoperative Monitoring

• Graft failure occurs in approximately 8 cases during follow-up periods averaging 33 months 4
• Monitor for glaucoma development: pre-existing in 72-86% of complex cases, with 14-39.3% developing new or progressive glaucoma 1
• Retroprosthetic membrane formation occurs in 25-55% of cases requiring long-term surveillance 1
• Endothelial cell loss after DALK is biphasic: initial loss from intraoperative manipulation, then 3.9% annual decline 1

Medical Management Role

Medical management alone is insufficient for visually significant congenital opacities but may temporize:

• Hyperosmotic sodium chloride 5% drops or ointment have limited benefit and should be discontinued after weeks if ineffective 1
• Control elevated IOP when present, avoiding prostaglandin analogues due to inflammatory potential 1
• Topical antibiotics may be necessary to reduce infection risk when epithelial bullae rupture 1