Surgical management of non-syndromic ectopia lentis

AIM: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).METHODS: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.RESULTS:Macula-on RRD was present in all individuals>18 y,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 log MAR(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 2 y post-operative.CONCLUSION: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.