Surgical management of non-traumatic pediatric ectopia lentis: A case series and review of the literature

Purpose: To report a small series of pediatric patients with ectopia lentis that underwent limbal-approach lensectomy and vitrectomy and scleral-fixated intraocular lens implantation and to review the literature on the topic of surgical management of ectopia lentis. Method: A retrospective review of 13 eyes of seven patients that underwent lensectomy, vitrectomy, and scleral-fixated intraocular lens implantation and a review of the ophthalmic literature. Results: In our series, the average age at surgery was 70.3 ± 13.8 months and the average length of follow-up was 23.8 ± 5.9 months. The mean pre-operative visual acuity was 0.86 ± 0.17 which improved to 0.23 ± 0.09 post-operatively (p 0.001). No complications were encountered in our series. A review of the literature found that amblyopia was the biggest vision-limiting factor. In general, the literature suggested that a higher percentage of eyes that were left aphakic achieved better vision than those implanted with a scleral-fixated intraocular lens. However, there may be selection bias in that more eyes receiving an intraocular lens may have pre-existing amblyopia. The complication rates for lensectomy or scleral-fixated intraocular lens implantation were low in the literature. In the latter group, suture breakage and resultant intraocular lens dislocation is a worrisome late complication. Conclusion: Surgical intervention for ectopia lentis via vitrectomy techniques yields good result. In cases of unilateral aphakia or in settings where compliance with aphakic refractive correction is questionable and amblyopia is a constant threat, scleral-fixated intraocular lens implantation is highly encouraged. However, long-term follow-up is required due to the risk of suture breakage and resultant intraocular lens dislocation over time.