Management of ectopia lentis in a family with Marfan syndrome

Objective: To determine the effectiveness of combined pars plana vitrectomy-lensectomy and open-loop anterior chamber lens implantation for the management of ectopia lentis associated with Marfan syndrome. Methods: Retrospective review of the medical records of 4 consecutive patients with Marfan syndrome who underwent combined pars plana lensectomy-vitrectomy and anterior chamber lens implantation at our institution between August 1994 and July 1995. Results: All patients demonstrated postoperative visual acuity of 20/25 or better during an average follow-up period of 6 months (range, 4-9 months). Two patients developed pseudophakic pupillary block and required YAG laser iridotomy postoperatively. Conclusions: Pars plana lensectomy-vitrectomy and anterior chamber intraocular lens implantation appears to be an excellent technique for the management of ectopia lentis associated with Marfan syndrome. A bimanual, closed-system endosurgical technique avoids many of the complications previously associated with surgery for ectopia lentis.