Surgical management of lens subluxation in Marfan syndrome.
OBJECTIVE
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.
Abrahamson Eye Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
Virginia
V
Miraldi Utz
Faculty of Medicine, McGill University, Montreal, Canada.
Razek Georges
RG
Coussa
The Cole Eye Institute, Ophthalmology, Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, Ohio. Electronic address: traboue@ccf.org.
Elias I
EI
Traboulsi
D000293:Adolescent; D000328:Adult; D000869:Anterior Eye Segment / Q000601:surgery; D002387:Cataract Extraction / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D005260:Female; D000071837:Fibrillins; D006801:Humans; D007906:Lens Subluxation / Q000209:etiology / Q000235:genetics / Q000601:surgery*; D008297:Male; D008382:Marfan Syndrome / Q000150:complications / Q000235:genetics / Q000601:surgery*; D008840:Microfilament Proteins / Q000235:genetics; D011183:Postoperative Complications; D012189:Retrospective Studies; D014792:Visual Acuity / Q000502:physiology; D055815:Young Adult
D000071837:Fibrillins; D008840:Microfilament Proteins