Bilateral phacoaspiration, capsulectomy, anterior vitrectomy and posterior iris-claw intraocular lens implantation in the treatment of severely visually impairing ectopia lentis related to Marfan's syndrome in preschool age

Session Details

Session Title: Presented Poster Session: Cataract - Paediatric

Session Date/Time: Monday 07/09/2015 | 09:30-11:00

Paper Time: 10:20

Venue: Poster Village: Pod 1

First Author: : E.Bertelli ITALY

Co Author(s): :    P. Santorum              

Abstract Details

Purpose:

To report results in terms of safety and efficacy of phacoaspiration, capsulectomy, anterior vitrectomy and posterior iris-claw intraocular lens implantation for severely visually impairing ectopia lentis in Marfan's syndrome in children of preschool age. The technique offers the potential advantage of minimizing early/late opacification of the visual axis due to secondary reproliferation in this delicate phase of functional development.

Setting:

Dept. of Ophthalmology, San Maurizio Regional Hospital, Bolzano, Italy

Methods:

This retrospective small case series comprises 4 eyes of two siblings, boy and girl, presenting with bilateral, severely visually impairing ectopia lentis that had phacoaspiration, lens capsule removal, anterior vitrectomy, posterior iris-fixated implantation, and peripheral superior iridectomy. The indication for surgery was reduced visual acuity, with best corrected visual acuity (BCVA) ranging from 1 to 0,70 Log MAR . Age at time of first surgery was 5 years and 2 months and 5 years and 3 months respectively. Second eye was operated on after 4 and 8 weeks, respectively. Main outcome measures were change in BCVA and postoperative refraction.

Results:

Postoperative follow-up ranged from 8 to 30 months. After surgery, BCVA kept gradually and steadily improving in all 4 eyes. Postoperative BCVA ranged from 0,30 to 0,10. Postoperative spherical equivalent was within +/-1.00D from refraction target (+1D) in all eyes. Intraoperatively a small iatrogenic iris sphincter defect required a single iris suture in 1 eye. A moderate postoperative intraocular inflammation was observed on postoperative day 3 in 1 eye, that resolved after intensive topical anti-inflammatory therapy.

Conclusions:

Phacoaspiration, capsulectomy, anterior vitrectomy and posterior iris-claw intraocular lens implantation significantly improved visual acuity in 4 eyes of two siblings, affected by Marfan's syndrome and severely visually impairing ectopia lentis, who were operated on at the age of 5. Studies with a larger number of patients and longer follow-up are necessary to confirm these encouraging results.

Financial Interest:

NONE