Retropupillary iris-claw intraocular lens in ectopia lentis in Marfan syndrome

Objective: To report visual outcomes, complication rate, and safety of retropupillary iris-claw intraocular lens (ICIOL) in ectopia lentis in Marfan syndrome (MFS). Design: Retrospective study. Methods: Six eyes of three MFS patients with ectopia lentis underwent surgery for subluxation lens and retropupillary ICIOL implantation from October 2014 to October 2015 at the Department of Ophthalmology, Santa Maria Hospital in Lisbon, Portugal. Demographics, preoperative and postoperative best-corrected visual acuity (BCVA), and intraocular pressure were evaluated. Endothelium cell count was assessed using specular microscopy; anterior chamber depth was measured using Pentacam postoperatively; and intraocular lens position was viewed by ultrasound biomicroscopy. All patients were female; mean age was 20 +/- 14.264 years (range: 7-38 years). Results: The average follow-up period was 6.66 months (range: 4-16 months). Preoperative BCVA was 0.568 +/- 0.149 logMAR units, and postoperative BCVA was 0.066 +/- 0.121 logMAR units. The mean BCVA gain was -0.502 +/- 0.221 on the logMAR scale. Postoperative average astigmatism and intraocular pressure were 1.292 +/- 0.697 mmHg (range: 0.5-2.25 mmHg) and 16 mmHg (range: 12-18 mmHg), respectively. The average endothelial cell density decreased from 3,121 +/- 178 cells/mm(2) before surgery to 2,835 +/- 533 cells/mm(2) after surgery (measured at last follow-up visit) and in the last follow-up, representing an average endothelial cell loss of 9.16%. Mean anterior chamber depth was 4.01 mm (+/- 0.77 mm), as measured by Pentacam. No complications were found intra-or postoperatively in any of the six studied eyes. Conclusion: Retropupillary ICIOL implantation is a safe and effective procedure in the treatment of aphakia in MFS eyes, without capsular support after surgery for ectopia lens. The six eyes that underwent lensectomy and retropupillary ICIOL implantation have had excellent visual outcomes with no complications so far.