Treatment of Severely Visually Impairing Ectopia Lentis Related to Marfan Syndrome in Pre-school Children: Report of 2 Cases Now Approaching 3 and 5 Years Follow-up Respectively


SCIENTIFIC POSTER

Treatment of Severely Visually Impairing Ectopia Lentis Related to Marfan Syndrome in Pre-school Children by Enrico Bertelli


ABSTRACT

The Authors report results in terms of safety and efficacy of phacoaspiration, capsulectomy, anterior vitrectomy and posterior iris-claw intraocular lens implantation in severely visually impairing ectopia lentis in Marfan’s syndrome in 2 preschool age children. The technique offers the potential advantage of minimizing early/late opacification of the visual axis due to secondary reproliferation.

Methods:

This retrospective small case series comprises 4 eyes of two siblings, boy and girl, presenting with bilateral, severely visually impairing ectopia lentis. Phacoaspiration, lens capsule removal, anterior vitrectomy, posterior iris-fixated implantation, and peripheral superior iridectomy were performed in each and every eye. The indication for surgery was reduced visual acuity, with best corrected visual acuity (BCVA) ranging from 1 to 0,70 Log MAR At the time of the first surgery the age of the patients was 5 years and 2 months and 5 years and 3 months respectively. The second eye was operated on after 4 and 8 weeks, respectively. BCVA, postoperative refraction and endothelial cell count were main outcome measures.

Results:

Postoperative follow-up ranges now from 32 to 56 months. After surgery, BCVA kept gradually and steadily improving in all 4 eyes for the first 2 years after surgery. 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. It resolved after intensive topical anti-inflammatory therapy. Endothelial cell count has remained stable along the follow- up time in all 4 eyes.

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 underwent surgery at the age of 5. These encouraging results must be confirmed by larger studies and longer follow-up times.


CONTACT DETAILS

 

Enrico Bertelli
Bolzano, Italy
Email : enrbertelli@gmail.com
Cell Phone: +393409191017
Work Phone: +390471908605