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.