Management of paediatric ectopia lentis at an Indonesian referral hospital: A retrospective review

Abstract

Background: Ectopia lentis in children is rare and often associated with systemic conditions. Left untreated, it can lead to visually debilitating complications. Although there are various techniques available, no current consensus for treatment of paediatric ectopia lentis exists.

Aim: To describe the management and visual outcomes of paediatric ectopia lentis in an academic referral hospital.

Setting: This study was conducted at Cipto Mangunkusumo National Central General Hospital, Jakarta, Indonesia.

Methods: A retrospective review was conducted on the medical records of all paediatric ectopia lentis patients treated at our hospital, from 2011 to 2020. Primary outcomes include uncorrected visual acuity (UCVA), best-corrected visual acuity (BCVA), treatment, surgical technique, selection of intraocular lens (IOL), and postoperative complications.

Results: This study included 66 eyes of 39 patients. Average age at initial presentation was 7.8 (2–17) years. Aetiology was mostly non-traumatic, whilst four were secondary to trauma. All eyes except one underwent surgery, and IOLs were inserted in 57.4% of the non-traumatic cohort, with iris-claw IOL as the most popular choice (85.7%). Uncorrected visual acuity and BCVA significantly improved in pseudophakic and aphakic groups (p < 0.05). Nearly 60% achieved a BCVA of 6/12 or greater, and the proportion was higher amongst pseudophakic group. Ten eyes had post-operative complications, with a median onset of 393 (1–1095) days after surgery.

Conclusion: With varied techniques used to manage paediatric ectopia lentis, the choice of technique was dependent on individual patient condition and surgeon preference. Whilst the proportion of eyes with BCVA of ≥ 6/12 was higher amongst the pseudophakic eyes than aphakic eyes, IOL implantation entails the risk of complications, whereas no complications occurred in those left aphakic. Therefore, no particular technique can be considered marginally superior to the other, and larger studies are needed.