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

Service du Professeur H. Hamard, Centre Hospitalier National d'Ophthalmologie des Quinze-Vingts, Paris.

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.

PMID: 7759754 [PubMed - indexed for MEDLINE]