Bilateral Crystalline Lens Subluxation in a Child With Marfan Syndrome

Ectopia lentis refers to displacement of the crystalline lens in the setting of various systemic and metabolic disorders. A literature review was conducted to investigate the management of non-traumatic ectopia lentis in the pediatric population, particularly focusing on surgical intervention. Both limbal and pars plana approaches for lensectomy are well established in the literature. Surgical options for intraocular lens implantation in pediatric eyes with ectopia lentis include anterior chamber intraocular lenses and iris-fixated or scleral-fixated posterior chamber intraocular lenses. Recently, the use of capsular tension rings has also been described with promising results. Visual rehabilitation and treatment of amblyopia are essential for patients within the amblyogenic age group following surgical intervention.

To evaluate the visual outcomes, intraoperative and postoperative complications of Cionni ring implantation in eyes with a subluxated lens associated with Marfan Syndrome. This case series included 15 eyes of 12 patients aged 19-56 years with a subluxated lens secondary to Marfan's syndrome operataed at the Departament of Ophtalmology, Toronto Western Hospital between 2003 and 2007. A 2-eyelet Cionni ring and an AcrySof (Alcon) foldable intraocular lens (IOL) were implanted in 13 eyes. Two eyes had trans-scleral IOL fixation in the ciliary sulcus. Best-corrected visual acuity, wavefront evaluation of eye aberration and complication rates were analysed. In all eyes, capsular bag centration was excellent. Preoperative visual acuity ranged from 20/50 to counting fingers, and improved to better than 20/40 in all eyes. The mean follow-up duration was 14+/-9.49 months. The most frequent postoperative complication was posterior capsule opacification, which occurred in 3 eyes (18.7%), 2 of which required a neodymium:YAG posterior capsulotomy. No eye developed retinal detachment. Total eye aberration, tilt and high-order aberrations with a 6-mm pupil diameter decreased significantly after surgery (n = 5): Total eye aberration decreased from a mean of 14.8+/-5.5 preoperatively to 2.1+/-4.3 microns after the operation. Tilt was decreased from 4.1+/-2.5 to 0.12+/-2.1 microns, and high-order aberrations decreased from 4.37+/-3.8 microns, before the operation, to 1.47+/-3.5 after the operation. Cionni ring implantation is an effective procedure to correct partial lens subluxation and has few complications (during 14 months of follow-up) in patients with Marfan's Syndrome.