[Surgery of ectopia lentis in Marfan disease in children and young adults].

INTRODUCTION

To determine the visual outcome of surgical therapy and conservative management, we retrospectively reviewed all our patients with ectopia lentis and Marfan's disease.

MATERIAL AND METHOD

Thirty-five eyes of 18 patients (age 8 to 47, average 25.3) were studied. Eight eyes were followed conservatively, the remaining 27 eyes underwent a lensectomy either with the Klöti vitreous stripper (18) or using other techniques (9).

RESULTS

Over follow-up periods ranging from 0.5 to 13 years (average 6.2 years) low visual acuity was found in non-operated eyes. Retinal detachment occurred in 2 out of 8 eyes. Eyes undergoing other surgical techniques had a mean final visual acuity of 0.2. Retinal detachment occurred with high frequency (6 out of 9 eyes). Lensectomy-vitrectomy was a safe and effective procedure with a mean final visual acuity of 0.6. No retinal detachment or secondary glaucoma were found.

COMMENT

Our study suggests that performing lensectomy-vitrectomy with the Klöti vitreous stripper is the best surgical approach in ectopia lentis with Marfan's disease. This technique allows the vitreous to be handled more effectively and has led to improve results.