What is the management of congenital cataract in infants and young children?

Management of Congenital Cataract in Infants and Young Children

For visually significant unilateral congenital cataracts, surgery should be performed by 6 weeks of age, and for bilateral cases between 6-8 weeks of age, followed by immediate optical rehabilitation with contact lenses or glasses and aggressive amblyopia treatment to prevent irreversible vision loss. 1

Surgical Timing and Indications

When to Operate

• Unilateral cataracts: Surgery at 6 weeks of age provides the best prognosis when optical correction is in place by 2 months of age 1
• Bilateral cataracts: Surgery between 6-8 weeks of age is recommended 2
• Absolute indications: Any lens opacity obscuring the red reflex in preverbal children or visual acuity less than 20/40 warrants lens aspiration 2
• Dense central cataracts are highly likely to cause amblyopia and require prompt surgical intervention 1
• Partial cataracts (polar or lamellar) may cause mild to moderate amblyopia or have no effect on visual development, requiring individualized assessment 1

Surgical Technique

• Recommended approach: Lens aspiration via vitrector with posterior capsulotomy and anterior vitrectomy in children younger than 6 years 2
• The procedure prevents visual axis opacification (VAO), which is a major postoperative complication 3
• Small-incision techniques are preferred to minimize inflammation and complications 2

Intraocular Lens (IOL) Implantation Decisions

Age-Based Guidelines

• Under 1 year: IOL implantation is not recommended; leave the child aphakic with contact lens or glasses correction 2
• 1-2 years: IOL implantation can be considered but remains controversial 2, 3
• Over 2 years: IOL insertion is advised when sufficient capsular support exists 2

Primary vs Secondary IOL Implantation

• Evidence suggests little difference in visual outcomes between primary IOL implantation and primary aphakia with contact lenses at 5-year follow-up (mean logMAR 0.50 vs 0.59) 3
• Secondary IOL implantation after a period of contact lens wear (mean 32 months) shows similar long-term visual outcomes and complication rates compared to primary implantation 4
• IOL power selection: Under-correction based on the child's age is recommended due to continuous ocular growth and biometric changes 2
• Hydrophobic foldable acrylic posterior chamber IOLs are preferred due to lower postoperative inflammation 2

Optical Rehabilitation (Critical Component)

Immediate Post-Surgical Correction

• Contact lenses: Rigid gas-permeable (GP) contact lenses are the preferred method for aphakic rehabilitation in infants and young children 5
• GP lenses are well-tolerated with acceptable parental compliance and achieve desirable visual outcomes 5
• Mean initial base curve of 7.86±0.39 mm and power of 23.29±5.52 diopters are typical starting parameters 5
• Aphakic glasses: Alternative option when contact lenses cannot be used, though less optimal for unilateral cases 3

Refractive Correction Requirements

• Correction of any associated refractive error is mandatory, as many partial cataracts have coexisting refractive errors 1
• Bifocal correction becomes necessary for school-age children 2
• Regular updates in optical correction are required due to rapid ocular growth 1

Amblyopia Management (Essential for Visual Outcome)

Treatment Protocol

• Unilateral cataracts: These cause the most severe deprivation amblyopia due to interocular competition, often resulting in visual acuity of 20/200 or worse 1
• Patching therapy: Occlusion of the fellow eye is typically first-line treatment for deprivation amblyopia 1
• For moderate amblyopia (20/40-20/80), prescribe 2 hours of daily patching 1
• For severe amblyopia (worse than 20/80), prescribe 6 or more hours of daily patching 1
• Pharmacologic treatment: Atropine 1% solution to the fellow eye is an effective alternative, particularly when patching compliance is poor 1

Monitoring for Reverse Amblyopia

• Occlusion amblyopia (reverse amblyopia) can occur in the patched eye, affecting 1% with 6+ hours patching and 9% with daily atropine 1
• Most cases resolve spontaneously with discontinuation of therapy while maintaining optical correction 1
• Regular monitoring of both eyes is essential during amblyopia treatment 1

Critical Pitfall

• Parental compliance: Only 27.6% of parents report non-adherence to scheduled part-time patching programs, but this must be actively monitored as compliance directly impacts visual outcomes 5

Postoperative Complications and Long-Term Management

Major Complications Requiring Surveillance

• Glaucoma: Occurs in approximately 10% of cases and requires lifelong monitoring 5, 3
• Visual axis opacification (VAO): Prevented by posterior capsulotomy and anterior vitrectomy during initial surgery 2, 3
• Posterior capsule opacification: Common complication requiring YAG laser or surgical intervention 2
• Inflammatory sequelae: More common with certain surgical techniques; posterior synechiae develop in some cases 3
• Strabismus: May develop or worsen during treatment, though some children show improvement 1

Refractive Changes

• Myopic shift: More frequent in eyes with primary IOL implantation due to continued ocular growth 4
• Regular refraction and optical correction updates are mandatory throughout childhood 1, 2

Follow-Up Schedule

Frequency of Monitoring

• Immediate postoperative period: Intensive monitoring for inflammation, glaucoma, and optical rehabilitation 2
• Long-term: Lifelong follow-up is required for proper management of complications 2
• Mean follow-up time of 12.44±26.28 months is typical in specialized contact lens clinics 5
• Regular ophthalmologic examinations to monitor for glaucoma development, refractive changes, and amblyopia progression 1

Multidisciplinary Coordination

• Communication with primary care providers and other specialists is essential 1
• Parental education about the importance of optical correction compliance and amblyopia treatment significantly improves outcomes 1
• Assessment of barriers to care including transportation, medication administration, and ability to attend postoperative visits 1

Special Considerations

Bilateral vs Unilateral Disease

• Bilateral cataracts: Generally have better visual prognosis than unilateral cases because interocular competition is absent 1
• Unilateral cataracts: Require more aggressive amblyopia treatment due to severe deprivation and competitive inhibition from the fellow eye 1

Associated Conditions

• Screen for systemic syndromes and genetic conditions when congenital cataract is diagnosed 1
• Evaluate for other ocular abnormalities including glaucoma, retinal abnormalities, and optic nerve hypoplasia 1
• Consider genetic counseling for families when hereditary patterns are identified 1