Contents

Congenital Cataract

Edited by:

Last updated: March 1st, 2022

Menu

Summary of Evidence

Visual axis opacification

Evidence exists for the care of children with congenital or developmental bilateral cataracts to reduce the occurrence of visual axis opacification. This was achieved with techniques that included an anterior vitrectomy or optic capture. Posterior capsulotomy alone was inadequate except in older children.{Long V, Chen S, Hatt S. Surgical interventions for bilateral congenital cataract. Cochrane Database Syst Rev. 2006 Jul 19;(3):CD003171.}

Visual outcomes in IOL vs contact lens correction

In patients with unilateral congenital cataract, implanting an IOL at the time of cataract extraction between 1 and 6 months of age was neither beneficial nor detrimental to the visual outcome at age 10.5 years.(IATS, 2020){Lambert SR, Cotsonis G, DuBois L, Nizam Ms A, Kruger SJ, Hartmann EE, Weakley DR Jr, Drews-Botsch C; Infant Aphakia Treatment Study Group. Long-term Effect of Intraocular Lens vs Contact Lens Correction on Visual Acuity After Cataract Surgery During Infancy: A Randomized Clinical Trial. JAMA Ophthalmol. 2020 Apr 1;138(4):365-372.}

Risk of Glaucoma

Glaucoma-related adverse events are common and increase between ages 1 and 5 years in infants after unilateral cataract removal at 1 to 6 months of age; primary IOL placement does not mitigate their risk but surgery at a younger age increases the risk.(IATS, 2015){Freedman SF, Lynn MJ, Beck AD, Bothun ED, Örge FH, Lambert SR; Infant Aphakia Treatment Study Group. Glaucoma-Related Adverse Events in the First 5 Years After Unilateral Cataract Removal in the Infant Aphakia Treatment Study. JAMA Ophthalmol. 2015 Aug;133(8):907-14.}

Evidence

1. Background

1.1 Epidemiology

Review

2016 Wu et.al.

Article link | Metrics cited by count

2016
Review

The overall prevalence of childhood cataract and congenital cataract was in the range from 0.32 to 22.9/10000 children (median=1.03) and 0.63 to 9.74/10000 (median=1.71), respectively. The prevalence of childhood cataract in low-income economies was 0.42 to 2.05 compared with 0.63 to 13.6/10000 in high-income economies.{Sheeladevi S, Lawrenson JG, Fielder AR, Suttle CM. Global prevalence of childhood cataract: a systematic review. Eye (Lond). 2016 Sep;30(9):1160-9.}

Wu et.al.
Journal full text Metrics

cited by count
Review

2016 Sheeladevi et.al.

Article link | Metrics cited by count

2016
Review

The pooled prevalence estimate of congenital cataract was 4.24 per 10,000 people. Subgroup analyses revealed the highest CC prevalence in Asia, and an increasing prevalence trend through 2000.{Wu X, Long E, Lin H, Liu Y. Prevalence and epidemiological characteristics of congenital cataract: a systematic review and meta-analysis. Sci Rep. 2016 Jun 23;6:28564.}

Sheeladevi et.al.
Journal full text Metrics

cited by count

2. Surgery

2.1 Management of visual axis opacification

Systematic review

2019 Cao et.al.

Article link | Metrics cited by count

2019
Systematic review

Vitrectomy helps lower the PCO risk and reoperation risk after congenital cataract surgery, and also, vitrectomy helps patients gain a better BCVA and achieve a better low-contrast sensitivity, with no trade-off on IOP control, IOL deposit, synechias, uveitis and secondary glaucoma.{Cao K, Wang J, Zhang J, Yusufu M, Jin S, Hou S, Zhu G, Wang B, Xiong Y, Li J, Li X, Chai L, He H, Wan XH. Efficacy and safety of vitrectomy for congenital cataract surgery: a systematic review and meta-analysis based on randomized and controlled trials. Acta Ophthalmol. 2019 May;97(3):233-239.}

Cao et.al.
Journal full text Metrics

cited by count
Systematic review

2011 Vasavada et.al.

Article link | Metrics cited by count

2011
Systematic review

Review of options available to prevent PCO, including pars plicata posterior capsulorhexis, sutureless vitrectomy, sealed-capsule irrigation, and bag-in-the-lens IOL.{Vasavada AR, Praveen MR, Tassignon MJ, Shah SK, Vasavada VA, Vasavada VA, Van Looveren J, De Veuster I, Trivedi RH. Posterior capsule management in congenital cataract surgery. J Cataract Refract Surg. 2011 Jan;37(1):173-93.}

Vasavada et.al.
Journal full text Metrics

cited by count
Systematic review

2006 Long et.al. (Cochrane review)

Article link | Metrics cited by count

2006
Systematic review

Evidence exists for the care of children with congenital or developmental bilateral cataracts to reduce the occurrence of visual axis opacification. This was achieved with techniques that included an anterior vitrectomy or optic capture. Posterior capsulotomy alone was inadequate except in older children.{Long V, Chen S, Hatt S. Surgical interventions for bilateral congenital cataract. Cochrane Database Syst Rev. 2006 Jul 19;(3):CD003171.}

Long et.al. (Cochrane review)
Journal full text Metrics

cited by count

2.2 Management of ocular inflammation

Clinical Trial

2014 Ventura et.al.

Article link | Metrics cited by count

2014
Clinical Trial

Intraoperative intracameral triamcinolone injection and postoperative oral prednisolone for modulating inflammation after congenital cataract surgery had similar outcomes at 1-year post-operatively.{Ventura MC, Ventura BV, Ventura CV, Ventura LO, Arantes TE, Nosé W. Outcomes of congenital cataract surgery: intraoperative intracameral triamcinolone injection versus postoperative oral prednisolone. J Cataract Refract Surg. 2014 Apr;40(4):601-8.}

  • Randomized controlled trial of intraoperative intracameral injection of triamcinolone acetonide vs  prednisolone syrup for 15 days postoperatively, tapered over the following 2 weeks, in children younger than 2 years unergoing congenital cataract surgery (60 eyes).
  • Findings
    •  In both groups, the mean IOP and CCT did not change significantly postoperatively. The groups had similar incidences of cell deposits  and posterior synechiae. No eye developed visual axis obscuration or had additional surgical procedures.

Ventura et.al.
Journal full text Metrics

cited by count

2.3 Intraocular lens power calculation

Case series

2013 Vanderveen et.al. (IATS)

Article link | Metrics cited by count

2013
Case series

The Holladay 1 and SRK/T formulae gave equally good results and had the best predictive value for infant eyes out of multiple formulae (Hoffer Q, Holladay 1, Holladay 2, Sanders-Retzlaff-Kraff (SRK) II, and Sanders-Retzlaff-Kraff theoretic (SRK/T) formulae) used to calculate predicted postoperative refraction for eyes that received primary IOL implantation in the Infant Aphakia Treatment Study.{Vanderveen DK, Trivedi RH, Nizam A, Lynn MJ, Lambert SR; Infant Aphakia Treatment Study Group. Predictability of intraocular lens power calculation formulae in infantile eyes with unilateral congenital cataract: results from the Infant Aphakia Treatment Study. Am J Ophthalmol. 2013 Dec;156(6):1252-1260.e2.}

Vanderveen et.al. (IATS)
Journal full text Metrics

cited by count

3. Outcomes of surgery

3.1 Long-term visual outcomes

Clinical Trial

2020 Lambert et.al. (IATS)

Article link | Metrics cited by count

2020
Clinical Trial

In patients with unilateral congenital cataract, implanting an IOL at the time of cataract extraction between 1 and 6 months of age was neither beneficial nor detrimental to the visual outcome at age 10.5 years.{Lambert SR, Cotsonis G, DuBois L, Nizam Ms A, Kruger SJ, Hartmann EE, Weakley DR Jr, Drews-Botsch C; Infant Aphakia Treatment Study Group. Long-term Effect of Intraocular Lens vs Contact Lens Correction on Visual Acuity After Cataract Surgery During Infancy: A Randomized Clinical Trial. JAMA Ophthalmol. 2020 Apr 1;138(4):365-372.}

  • Randomized controlled trial of cataract surgery with or without primary IOL implantation between 1 and 6 months of age in infants with unilateral congenital cataract (114 patients)
  • Findings:
    • Visual acuity outcomes were highly variable with only 27 children (25%) achieving excellent visual acuity (20/40 or better) in their treated eye and 50 children (44%) having poor vision (20/200 or worse) in the treated eye

Lambert et.al. (IATS)
Journal full text Metrics

cited by count

3.2 Costs

Clinical Trial

2015 Kruger et.al. (IATS)

Article link | Metrics cited by count

2015
Clinical Trial

At 5 years, unilateral cataract surgery in infancy coupled with primary IOL implantation is approximately 7% more expensive than aphakia and CL correction. Patient costs are more than double with CL versus IOL treatment.{Kruger SJ, DuBois L, Becker ER, Morrison D, Wilson L, Wilson ME Jr, Lambert SR; Infant Aphakia Treatment Study Group. Cost of intraocular lens versus contact lens treatment after unilateral congenital cataract surgery in the infant aphakia treatment study at age 5 years. Ophthalmology. 2015 Feb;122(2):288-92.}

  • Retrospective cost analysis of a prospective, randomized clinical trial based on Georgia Medicaid data and the actual costs of supplies used (114 infants).

Kruger et.al. (IATS)
Journal full text Metrics

cited by count
Clinical Trial

2013 Carrigan et.al. (IATS)

Article link | Metrics cited by count

2013
Clinical Trial

For IATS patients up to 12 months of age, cataract surgery coupled with IOL implantation and spectacle correction was 37.5% (∼$4000) more expensive than cataract surgery coupled with contact lens correction.

  • Retrospective cost analysis of a prospective, randomized clinical trial based on Georgia Medicaid data and the actual costs of supplies used (114 infants).

Carrigan et.al. (IATS)
Journal full text Metrics

cited by count

4. Complications

4.1 Secondary glaucoma

4.1.1 Primary IOL implantation vs aphakia/secondary IOL implantation

Meta-analysis

2019 Zhang et.al.

Article link | Metrics cited by count

2019
Meta-analysis

In patients under 2 years of age, primary IOL implantation for bilateral congenital cataract surgery is associated with a lower risk of secondary glaucoma (long-term incidence of secondary glaucoma in general population of eyes with congenital cataract was 6.7% vs 16.7%). For unilateral congenital cataract surgery, the incidence was very similar in eyes with and without primary IOL.{Zhang S, Wang J, Li Y, Liu Y, He L, Xia X. The role of primary intraocular lens implantation in the risk of secondary glaucoma following congenital cataract surgery: A systematic review and meta-analysis. PLoS One. 2019 Apr 1;14(4):e0214684.}

  • Meta-analysis of 8 publications involving 892 eyes

Zhang et.al.
Journal full text Metrics

cited by count
Clinical trial

2015 Freedman et.al. (IATS)

Article link | Metrics cited by count

2015
Clinical trial

Glaucoma-related adverse events are common and increase between ages 1 and 5 years in infants after unilateral cataract removal at 1 to 6 months of age; primary IOL placement does not mitigate their risk but surgery at a younger age increases the risk.{Freedman SF, Lynn MJ, Beck AD, Bothun ED, Örge FH, Lambert SR; Infant Aphakia Treatment Study Group. Glaucoma-Related Adverse Events in the First 5 Years After Unilateral Cataract Removal in the Infant Aphakia Treatment Study. JAMA Ophthalmol. 2015 Aug;133(8):907-14.}

  • Randomized controlled trial of primary IOL or no IOL implantation (contact lens) in infants with unilateral congenital cataract undergoing surgery between ages 1-6 (114 patients).
  • Findings:
    • Risk for glaucoma and glaucoma + glaucoma suspect at 4.8 years after surgery were 17%  and 31%, respectively
    • The contact lens and IOL groups were not significantly different for either outcome
    • Younger age at surgery conferred an increased risk for glaucoma (26% vs 9%, respectively)

Freedman et.al. (IATS)
Journal full text Metrics

cited by count

References

Comments

Subscribe
Notify of
guest
0 Comments
Inline Feedbacks
View all comments

© 2024 - sourceshed