Long-term Results of Anterior Chamber Iris Claw Intraocular Lens Implantation in Children with Ectopia Lentis in Marfan Syndrome

PURPOSE:To report the long-term clinical and endothelial cell count (ECC) results of lensectomy with primary anterior chamber iris claw lens implantation in the eyes of patients ≤18-year-old with ectopia lentis due to Marfan syndrome.METHODS:The medical records of Marfan patients operated on at a single institution from September 2007 to August 2020, with minimum follow-up of 2 years, were reviewed retrospectively. The following data were analyzed: sex, age at surgery, indication for surgery, the position of the lens in relation to the undilated and dilated pupil, corneal endothelial cell counts (ECC), peri- and postoperative complications, pre- and postoperative best-corrected visual acuity.RESULTS:A total of forty-two eyes of 23 patients (12 girls and 11 boys) were included. At least two or more postoperative ECCs were collected from 33 eyes (17 patients). Median age at IOL implantation was 6.1 years (range, 1.8-18). Median overall follow-up time was 6.2 years (range, 2-13.5). Median ECC follow-up time was 6.2 years (range, 2-10). Mean best-corrected visual acuity was 0.71 ± 0.38 logMAR before surgery and 0.02 ± 0.25 logMAR at final follow-up. The mean annual ECC decline was 0.71% ± 2.24. Total cell loss from first to last postoperative measurement was 150 cells ± 394 cells/mm2 (4.81%). Pre- and first postoperative data were available for 17 eyes of 10 patients, with a mean cell loss before and directly after surgery of 269 ± 268 cells (7.94%). Surgery related complications were iris bombé due to blockage of peripheral iridectomy in 3 eyes and claw dislocation due to direct impact trauma in 3 eyes.CONCLUSIONS:In our large, pediatric study cohort, anterior chamber iris claw IOL implantation resulted in an excellent visual outcome and normal endothelial cell loss compared with normative data. Safety measures are recommended to avoid traumatic dislocation of IOLs.