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September 1996

Management of Ectopia Lentis in a Family With Marfan Syndrome

Author Affiliations

From the Department of Ophthalmology, Eye Institute, Medical College of Wisconsin, Milwaukee. The authors have no proprietary interest in any instrument or technique described in this article.

Arch Ophthalmol. 1996;114(9):1058-1061. doi:10.1001/archopht.1996.01100140260001

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.