Outcomes after lensectomy for children with Marfan syndrome

To describe the long-term incidence of retinal detachment, intra- and postoperative complications, and visual and refractive outcomes of children with Marfan syndrome following lensectomy for ectopia lentis, with or without subsequent correction of aphakia using the Artisan aphakic iris-fixated anterior chamber intraocular lens.

The medical records of children treated at the Department of Ophthalmology, Children's University Hospital, Dublin, Ireland, from January 1, 1991, to December 31, 2011, were retrospectively reviewed. The primary outcome measure was rate of retinal detachment at final follow-up. Secondary outcomes included postoperative complications, endothelial cell count, visual acuity, refractive error, and postoperative refraction prediction error.

A total of 30 eyes of 15 patients underwent lensectomy. Of these, 16 eyes of 8 patients subsequently underwent Artisan implantation. Mean follow-up was 13.8 ± 5.9 years after lensectomy and 4.1 ± 2.8 years after Artisan implantation. There were no cases of retinal detachment. Best-corrected visual acuity was 0.11 ± 0.14 (logMAR) post-lensectomy and 0.12 ± 0.19 post-Artisan implantation. Endothelial cell count of the pseudophakic group was 3109 ± 458 preoperatively and 2632 ± 592 postoperatively. Mean cell loss was 15.4%. One eye required repeat surgical peripheral iridectomy for pseudophakic pupillary block. One eye required re-enclavation of a dislocated Artisan IOL haptic; the same eye required anterior vitrectomy for removal of retained lens fragment from the original lensectomy.

In this study cohort, there was no increased incidence of retinal detachment in children with Marfan syndrome and ectopia lentis in the setting of one or more prior intraocular procedures. A moderate rate of endothelial cell loss was observed.