Congenital Cataract Surgery: A Case Series from a Tertiary Center
DOI:
https://doi.org/10.48560/rspo.38215Keywords:
Cataract Extraction, Cataract/congenital, ChildAbstract
INTRODUCTION: Congenital cataract is considered a rare disease by the World Health Organization and has diverse causes, including genetic mutations, infections and metabolic disorders. Despite advancements in surgical management, patients remain at risk for complications such as amblyopia, strabismus and glaucoma. Visual outcomes heavily depend on early diagnosis, age at surgery, and adherence to postoperative care.
METHODS: Retrospective observational study including pediatric patients who underwent congenital cataract surgery at the Ophthalmology Department of Unidade Local de Saúde de Santo António in Porto, Portugal, between 2010 and 2023.
RESULTS: A total of 49 eyes (from 32 patients) with congenital cataract operated on a pediatric age were included. Most cases included bilateral cataracts (n=34; 69.4%) and the most frequent location was posterior (n=22; 52.4%). The median age at surgery was 3.55 years old (0.06 – 16.90) and a family history of congenital cataract was positive in 4 (12.5%) patients. Laterality did not impact age at surgery (p=0.686). Primary intraocular lens (IOL) implantation was performed in 71.4% (n=35) of eyes, predominantly at older ages (5.81 (2.17-16.90) vs 0.30 (0.06-0.87); p<0.001). Patients submitted to surgery over 1 year of age had an improved final best-corrected visual acuity (p<0.001) and lower myopization rates across follow-up (p<0.001). A posterior Nd:YAG capsulotomy was required in 91.7% (n=11) of eyes not submitted to primary anterior vitrectomy/posterior capsulothexis (n=12) at a median time of 4.04 years postoperatively and surgical reintervention was needed in 4 eyes. A diagnosis of glaucoma was made in 5 eyes (2 with persistent primary hyperplastic vitreous) at a median of 0.25 years postoperatively, with 3 eyes requiring glaucoma surgery. Additional variables significantly associated with an improved final BCVA were the absence of ocular structural anomalies and having bilateral cataracts.
CONCLUSION: Studying rare conditions like congenital cataracts is challenging due to the heterogeneous nature of real-life scenarios. However, documenting real-life management decisions and outcomes is crucial. Bilateral cataracts, being operated over the year of age and not having ocular structural anomalies, related to an improved final BCVA.
Downloads
References
Sheeladevi S, Lawrenson JC, Fielder AR, Suttle CM. Global prevalence of childhood cataract: a systematic review. Eye. 2016;30:1160-9. doi: 10.1038/eye.2016.156.
Widdus R. Public-private partnerships for health: their main targets, their diversity, and their future directions. Bull World Health Organ. 2001;79:713-20.
Bell SJ, Oluonye N, Harding P, Moossiee M. Congenital cataract: a guide to genetic and clinical management. Ther Adv Rare Dis. 2020;1:2633004020938061. doi: 10.1177/2633004020938061.
Tätaru CJ, Tätaru CP, Costache A, Boruga O, Zemba M, Ciuluvick RC, et al. Congenital cataract - clinical and morphological aspects. Rom J Morphol Embryol. 2020;61:105-12. doi: 10.47162/RJME.61.1.11.
Nihalani BR, Vanderveen DK. Technological advances in pediatric cataract surgery. Semin Ophthalmol. 2010;25:271-4. doi: 10.3109/08820538.2010.518836.
Lambert SR, Lyons CJ. Taylor and Hoyt's Pediatric Ophthalmology and Strahismus. 5th ed. Amsterdam: Elsevier;2016.
Feng Y, Collignon O, Maurer D, Yao K, Gao X. Brief Postnatal Visual Deprivation Triggers Long-Lasting Interactive Structural and Functional Reorganization of the Human Cortex. Front Med. 2021;8:752021. doi: 10.3389/fmed.2021.752021.
Oshika T, Endo T, Kurosaka D, Matsuki N, Miyagi M, Mori T, et al. Long-term surgical outcomes of pediatric cataract-multivariate analysis of prognostic factors. Sci Rep. 2023;13:21645. doi: 10.1038/s41598-023-49166-2.
Drews-Botsch C, Celano M, Cotsonis G, Hartmann EE, Lambert SR; Infant Aphasia Treatment Study Group. Association Between Occlusion Therapy and Optotype Visual Acuity in Children Using Data From the Infant Aphasia Treatment Study: A Secondary Analysis of a Randomized Clinical Trial. JAMA Ophthalmol. 2016;134:863-9. doi: 10.1001/jamaophthalmol.2016.1365.
Bothun ED, Lynn MJ, Christiansen SP, Neely DE, Vanderveen DK, Kruger SJ, et al. Sensorimotor outcomes by age 5 years after monocular cataract surgery in the Infant Aphasia Treatment Study (IATS). J AAPOS. 2016;204:9-53. doi: 10.1016/j.jaapos.2015.11.002.
Lee SJ, Kim WS. Factors associated with strahismus after cataract extraction and primary intraocular lens implantation in congenital cataracts. Int J Ophthalmol. 2014;7:522-7. doi: 10.3980/j.issn.2222-
Freedman SF, Lynn MJ, Beck AD, Bothun ED, Örge FH, Lambert SR, et al. Glaucoma-Related Adverse Events in the First 5 Years After Unilateral Cataract Removal in the Infant Aphasia Treatment Study. JAMA Ophthalmol. 2015;133:907-14. doi: 10.1001/jamaophthalmol.2015.1329.
Matatisi A, Haidich AB, Kokkali S, Rabiah PK, Birch E, Stager DR Jr, et al. Postoperative glaucoma following infantile cataract surgery: an individual patient data meta-analysis. JAMA Ophthalmol. 2014;132:1059-67. doi: 10.1001/jamaophthalmol.2014.1042.
Sharma AK, Shah DN, Upadhyay MP, Thapa M. Demography and Etiology of Congenital Cataract in a Tertiary Eye Centre of Kathmandu, Nepal. Health Renaissance. 2015; 12. doi: 10.3126/hren.v12i1.11975.
Katibeh M, Eskandari A, Yaseri M, Hosseini S, Ziaei H. The gender issue in congenital and developmental cataract surgery. J Ophthalmic Vis Res. 2013;8:308-13.
Amaya L, Taylor D, Russell-Eggitt I, Nischal KK, Lengyel D. The morphology and natural history of childhood cataracts. Surv Ophthalmol. 2003;48:125-44. doi: 10.1016/s0039-6257(02)00462-9.
Patel A, Hayward JD, Tailor V, Nyamlete R, Ahlfors H, Gabriel C, et al. The Oculome Panel Test: Next-Generation Sequencing to Diagnose a Diverse Range of Genetic Developmental Eye Disorders. Ophthalmology. 2019;126:888-907. doi: 10.1016/j.ophtha.2018.12.050.
Snead MP, McNinch AM, Poulson AV, Bearcroft P, Silverman B, Gomersall P, et al. Stickler syndrome, ocular-only variants and a key diagnostic role for the ophthalmologist. Eye. 2011;25:1389-400. doi: 10.1038/eye.2011.201.
Moramarco A, Himmelblau E, Miraglia E, Mallone F, Robert V, Franzone F, et al. Ocular manifestations in Gorlin-Goltz syndrome. Orphanet J Rare Dis. 2019;14:218. doi: 10.1186/s13023-019-1190-6.
Jeon HS, Choi M, Byun SJ, Hyon JY, Park KH, Park SJ. Association of Pediatric Atopic Dermatitis and Cataract Development and Surgery. JAMA Ophthalmol. 2018;136:912-8. doi: 10.1001/jamaophthalmol.2018.2166.
Infant Aphakia Treatment Study Group; Lambert SR, Buckley EG, Drews-Botsch C, DuBois L, Hartmann E, et al. The infant aphakia treatment study: design and clinical measures at enrollment. Arch Ophthalmol. 2010;128:21-7. doi: 10.1001/archophthalmol.2009.350.
David R, Davelman J, Mechoulam H, Cohen E, Karshai I, Anteby I. Strabsimus developing after unilateral and bilateral cataract surgery in children. Eye. 2016;30:1210-4. doi: 10.1038/eye.2016.162.
Kim IG, Park JM, Lee SJ. Factors associated with the direction of ocular deviation in sensory horizontal strabismus and unilateral organic ocular problems. Korean J Ophthalmol. 2012;26:199-202. doi: 10.3341/kjo.2012.26.3.199.
Havertape SA, Cruz OA, Chu FC. Sensory strabismus - Eso or exo? J Pediatr Ophthalmol Strabismus. 2001;38:327-30; quiz 354-5. doi: 10.3928/0191-3913-20011101-05.
France TD, Frank JW. The association of strabismus and aphakia in children. J Pediatr Ophthalmol Strabismus. 1984;21:223-6. doi: 10.3928/0191-3913-19841101-06.
Lambert SR, Cotsonis G, DuBois L, Nizam Ms A, Kruger SJ, Hartmann EE, et al. 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;138:365-72. doi: 10.1001/jamaophthalmol.2020.0006.
Infant Aphakia Treatment Study Group; Lambert SR, Lynn MJ, Hartmann EE, DuBois L, Drews-Botsch C, et al. Comparison of contact lens and intraocular lens correction of monocular aphakia during infancy: a randomized clinical trial of HOTV optotype acuity at age 4.5 years and clinical findings at age 5 years. JAMA Ophthalmol. 2014;132:676-82. doi: 10.1001/jamaophthalmol.2014.531.
Infant Aphakia Treatment Study Group; Lambert SR, Buckley EG, Drews-Botsch C, DuBois L, Hartmann EE, et al. A randomized clinical trial comparing contact lens with intraocular lens correction of monocular aphakia during infancy: grating acuity and adverse events at age 1 year. Arch Ophthalmol. 2010;128:810-8. doi: 10.1001/archophthalmol.2010.101.
Touze R, Dureau P, Edelson C, Borella Y, Barjol A, de Laage de Meux P, et al. Congenital cataract surgery: long-term refractive outcomes of a new intraocular lens power correction formula. Acta Ophthalmol. 2022;100:e1641-5. doi: 10.1111/casos.15134.
Lambert SR, Nizam A, DuBois L, Cotsonis G, Weakley DR Jr, Wilson ME, et al. The Myopic Shift in Aphakic Eyes in the Infant Aphakia Treatment Study After 10 Years of Follow-up. Eye Contact Lens. 2021;47:108-12. doi: 10.1097/JCL.0000000000000718.
Egbert JE, Christiansen SP, Wright MM, Young TL, Summers CG. The natural history of glaucoma and ocular hypertension after pediatric cataract surgery. J AAPOS. 2006;10:54-7. doi: 10.1016/j.jaapos.2006.07.002.
Lambert SR, Purohit A, Superak HM, Lynn MJ, Beck AD. Long-term risk of glaucoma after congenital cataract surgery. Am J Ophthalmol. 2013;156:355-361.e2. doi: 10.1016/j.aio.2013.03.013.
Beck AD, Freedman SF, Lynn MJ, Bothun E, Neely DE, Lambert SR, et al. Glaucoma-related adverse events in the Infant Aphakia Treatment Study: 1-year results. Arch Ophthalmol. 2012;130:300-5. doi: 10.1001/archophthalmol.2011.347.
Chak M, Wade A, Rahi JS. Long-term visual acuity and its predictors after surgery for congenital cataract: Findings of the British Congenital Cataract Study. Invest Ophthalmol Vis Sci. 2006;47:4262-9. doi: 10.1167/iovs.05-1160.
Bonaparte LA, Trivedi RH, Ramakrishnan V, Wilson ME. Visual acuity and its predictors after surgery for bilateral cataracts in children. Eye. 2016;30:1229-33. doi: 10.1038/eye.2016.166.
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2025 Revista Sociedade Portuguesa de Oftalmologia
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
Do not forget to download the Authorship responsibility statement/Authorization for Publication and Conflict of Interest.
The article can only be submitted with these two documents.
To obtain the Authorship responsibility statement/Authorization for Publication file, click here.
To obtain the Conflict of Interest file (ICMJE template), click here
