Surgical management of lens subluxation in Marfan syndrome.

PURPOSE: To review the literature on the surgical management, describe a simplified surgical technique, and to report the postoperative clinical course of ectopia lentis removal in patients with Marfan syndrome.

METHODS

The medical records of patients with a clinical diagnosis of Marfan syndrome and clinically significant lens subluxation were retrospectively reviewed. Patients underwent lens extraction by a single surgeon via a simplified anterior segment approach. The pre- and postoperative best-corrected visual acuity, biometric measurements, intraocular pressure, and incidence of surgery-related complications were reviewed.

RESULTS

A total of 42 eyes of 22 patients were included. Mean postoperative follow-up was 4.9 ± 2.9 years (range, 1-10 years). Average age at surgery was 10.2 ± 9.2 years (range, 2-37 years), with 18 patients (36 eyes) ≤ 18 years of age. The average preoperative best-corrected visual acuity was 20/80, and the average postoperative best-corrected visual acuity at last follow-up was 20/25, with an average improvement of 6 lines on the Snellen chart. All eyes had a best-corrected visual acuity > 20/30 at last follow-up with aphakic correction. One eye of 1 patient developed a retinal detachment following blunt trauma. No other intra- or postoperative complications were reported.

CONCLUSIONS

Anterior lensectomy and limited vitrectomy with aphakic correction is safe and provides a consistent visual outcome in patients with lens subluxation secondary to Marfan syndrome. This is especially important in pediatric patients, in whom long-term follow-up for iris- and scleral-fixated intraocular lenses is limited.