Anterior Chamber Intraocular Lens (ACIOL) Placement After Pars Plana Lensectomy in Pediatric Marfan Syndrome

Introduction: The management of subluxed crystalline lenses in patients with Marfan syndrome is challenging and may require lensectomy. Options for postoperative optical correction include aphakic spectacles, aphakic contact lens, posterior chamber IOLs sutured to the ciliary sulcus, and open-loop flexible anterior chamber IOLs (ACIOL). We recently began placing ACIOLs in all patients with Marfan Syndrome who were younger than 18 years of age and required lensectomy.

Methods: We used a retrospective chart review design for this study.

Results: Eight eyes of 5 patients were treated with pars plana vitrectomy, pars plana lensectomy, and primary ACIOL placement. Follow-up ranged from 9 to 16 months, with a mean of 12.7 months. Ages ranged from 5 to 17 years with a mean of 9 years. Three eyes were functionally aphakic at the time of surgery. Of the 5 eyes with a portion of the lens remaining in the visual axis, none maintained accommodative function by near visual acuity testing. Best-corrected distance visual acuity preoperatively ranged from 20/50 to 20/400, with an average of 20/80 (0.65 logMAR). Postoperative acuity ranged from 20/20 to 20/50, with an average acuity of 20/32 (0.20 logMAR). One patient had an iatrogenic sector iridectomy. No patients experienced corneal decompensation, increased intraocular pressure, persistent inflammation, IOL displacement, or explantation during the follow-up period.

Conclusions: Although long-term follow-up data are unavailable, our cases of primary ACIOL placement in children with Marfan syndrome indicate that this therapy should be considered.