Surgical management of non-syndromic ectopia lentis.

Abstract

To compare whether aphakic contact lenses or secondary iris-claw intraocular lenses are superior in the refractive management post-pars plana vitreolensectomy in a pedigree with an FBN1 mutation causing non-syndromic ectopia lentis (NSEL) with retinal detachment (RD). Eight affected individuals had pars plana vitreolensectomy for bilateral ectopia lentis (EL). Twelve eyes of 6 patients had secondary iris-claw intraocular lenses inserted and 4 eyes of 2 patients were managed with contact lenses. Rhegmatogenous retinal detachment (RRD) was treated when necessary. Pre- and post-operative assessment included visual acuity, endothelial cell count and dilated fundal examination. Macula-on RRD was present in all individuals >18y, 64% (7/11 eyes) presenting post-vitreolensectomy with 57% having bilateral non-synchronous RRD. Surgical aphakia was managed with iris-fixated intraocular lenses (IOL group, n=6), or contact lenses (CL group, n=2). Visual acuity ≥0.3 logMAR (driving standard) was achieved in 75% of IOL group eyes and 25% of the CL group eyes. Mean loss of corneal endothelial cell count in the IOL group was 4% at 2y post-operative. In this cohort, refractive management with iris-claw IOLs provided superior outcomes to contact lenses and the authors recommend this as the optimal refractive correction in EL patients.