What is the recommended treatment for a patient with congenital cataract?

Treatment of Congenital Cataract

Surgical removal of the cataract is the definitive treatment for congenital cataract, and must be performed urgently—ideally within 2 months of birth for dense bilateral cataracts—to prevent irreversible amblyopia and nystagmus. 1

Timing of Surgery: Critical for Visual Outcomes

• Surgery must be performed promptly in cases with dense congenital cataract; if nystagmus has developed, the amblyopia is irreversible. 1
• For bilateral congenital cataracts, intervention within the first 2 months of life combined with immediate optical correction and aggressive occlusion therapy yields the best visual outcomes. 1
• The urgency stems from the critical period of visual development—delay results in permanent vision loss that cannot be recovered even with later intervention. 1

Surgical Technique Considerations

Primary Surgical Approach

• Both anterior and posterior capsulorhexes should be performed in most children to reduce the risk of visual axis opacification. 1
• Anterior dry vitrectomy is recommended in preschool children to prevent after-cataract formation, which is the most common complication of pediatric cataract surgery. 1
• Lensectomy and lens aspiration with primary capsulotomy produce similar visual acuity outcomes, but lensectomy results in significantly lower rates of secondary opacification (2% vs 66%). 2

Intraocular Lens (IOL) Implantation vs Primary Aphakia

• IOL implantation can be performed safely in children older than 1 year. 1
• For children under 2 years with bilateral congenital cataracts, the evidence comparing IOL implantation to primary aphakia (leaving the child without a lens) is very uncertain, with no clear difference in visual acuity at 5 years (mean logMAR 0.50 for pseudophakia vs 0.59 for aphakia). 3
• The choice between IOL implantation and aphakia remains controversial in very young children due to concerns about ocular growth, refractive changes, and long-term complications. 3
• If primary aphakia is chosen, contact lenses or aphakic glasses are required for optical correction. 3

Postoperative Management

Inflammation Control

• Either intraoperative intracameral triamcinolone injection (1.2 mg/0.03 mL) or postoperative oral prednisolone (1 mg/kg/day for 15 days, then tapered over 2 weeks) effectively controls postoperative inflammation with similar outcomes at 1 year. 4
• Both approaches result in comparable rates of cell deposits, posterior synechiae, and intraocular pressure changes. 4

Amblyopia Treatment

• Aggressive occlusion therapy with frequent follow-up is essential following surgery and optical correction. 1
• For unilateral congenital cataracts, patching protocols should be individualized, but evidence suggests that optotype acuity measures obtained at age 4-5 years are reliable predictors of final visual outcomes. 5
• The amount of patching between ages 4-5 years shows minimal correlation with changes in visual acuity, suggesting less aggressive protocols may be justified in some children once reliable acuity measurements can be obtained. 5

Critical Complications Requiring Lifelong Surveillance

Visual Axis Opacification

• This is the most common complication of pediatric cataract surgery and necessitates performing both anterior and posterior capsulorhexes with anterior vitrectomy. 1

Secondary Glaucoma

• Secondary glaucoma is the most sight-threatening complication and occurs more frequently when surgery is performed early in life. 1
• Life-long follow-up is essential to monitor for this devastating complication. 1

Other Complications

• Posterior synechiae, retinal detachment, and need for reoperation must be monitored throughout the patient's life. 3

Common Pitfalls to Avoid

• Delaying surgery in dense bilateral cataracts beyond 2 months of age risks irreversible amblyopia and nystagmus development. 1
• Failing to perform posterior capsulotomy and anterior vitrectomy in young children leads to high rates of visual axis opacification requiring additional procedures. 1, 2
• Inadequate long-term follow-up misses the development of secondary glaucoma, which can cause permanent vision loss. 1
• Insufficient amblyopia treatment after surgery undermines the visual potential gained from timely surgical intervention. 1