Jaume Català-Mora1,2,3, Daniel Cuadras4, Jesús Díaz-Cascajosa1, Marta Castany-Aregall5, Joan Prat-Bartomeu1, José García-Arumí3,5. 1. Hospital Sant Joan de Déu, Esplugues de Llobregat, Barcelona, Spain. 2. Ophthalmological Institute del Pilar, Barcelona, Spain. 3. Surgery Department, Universitat Autònoma Barcelona, Barcelona, Spain. 4. Advisory Service and Statistical Methodology Research, Fundació Sant Joan de Déu, Esplugues de Llobregat, Barcelona, Spain. 5. Hospital Vall d'Hebron, Barcelona, Spain.
Abstract
PURPOSE: To report the feasibility and long-term safety of lensectomy and iris-claw intraocular lens (IOL) implantation to treat children with severe ectopia lentis in a paediatric tertiary hospital. METHODS: Prospective cohort study of 21 eyes from 12 patients with severe ectopia lentis and visual acuity <20/63. All eyes underwent 23-gauge pars plana vitrectomy, lensectomy, iridectomy and Artisan IOL implantation in the anterior chamber with iris-claw enclavation via pars plana. Mean age at surgery was 8.0 ± 5.3 yo (range 3-17 years). A full ophthalmologic examination including best-corrected visual acuity (BCVA), biomicroscopy, intraocular pressure (IOP) measurement, fundus evaluation and central endothelial cell count (cECC) was performed pretreatment, at 3 months' postsurgery, and every 6 months thereafter. Ultrasound biomicroscopy (UBM) was performed 12 months after surgery. RESULTS: Mean follow-up was 39.3 ± 13.0 months. Best-corrected visual acuity (BCVA) (mean ± SD) improved from 0.91 ± 0.29 logMar preoperatively to 0.18 ± 0.23 logMar at final follow-up (p < 0.0001). Mean distance from the endothelium to the anterior IOL surface after surgery was 3.11 ± 0.61 mm. Postsurgically, cECC loss was 5.04% ± 9.58% with an annual cECC loss rate of 3.16% ± 4.46%. One patient developed IOL dislocation and retinal detachment after severe ocular contusion requiring vitrectomy, IOL refixation and gas tamponade. Another patient developed cystoid macular oedema, managed with intravitreal dexamethasone. CONCLUSION: This technique is both feasible and effective to manage severe ectopia lentis in children. Lifetime ophthalmic follow-up including cECC measurement, IOL position monitoring and fundus examination is mandatory in these patients.
PURPOSE: To report the feasibility and long-term safety of lensectomy and iris-claw intraocular lens (IOL) implantation to treat children with severe ectopia lentis in a paediatric tertiary hospital. METHODS: Prospective cohort study of 21 eyes from 12 patients with severe ectopia lentis and visual acuity <20/63. All eyes underwent 23-gauge pars plana vitrectomy, lensectomy, iridectomy and Artisan IOL implantation in the anterior chamber with iris-claw enclavation via pars plana. Mean age at surgery was 8.0 ± 5.3 yo (range 3-17 years). A full ophthalmologic examination including best-corrected visual acuity (BCVA), biomicroscopy, intraocular pressure (IOP) measurement, fundus evaluation and central endothelial cell count (cECC) was performed pretreatment, at 3 months' postsurgery, and every 6 months thereafter. Ultrasound biomicroscopy (UBM) was performed 12 months after surgery. RESULTS: Mean follow-up was 39.3 ± 13.0 months. Best-corrected visual acuity (BCVA) (mean ± SD) improved from 0.91 ± 0.29 logMar preoperatively to 0.18 ± 0.23 logMar at final follow-up (p < 0.0001). Mean distance from the endothelium to the anterior IOL surface after surgery was 3.11 ± 0.61 mm. Postsurgically, cECC loss was 5.04% ± 9.58% with an annual cECC loss rate of 3.16% ± 4.46%. One patient developed IOL dislocation and retinal detachment after severe ocular contusion requiring vitrectomy, IOL refixation and gas tamponade. Another patient developed cystoid macular oedema, managed with intravitreal dexamethasone. CONCLUSION: This technique is both feasible and effective to manage severe ectopia lentis in children. Lifetime ophthalmic follow-up including cECC measurement, IOL position monitoring and fundus examination is mandatory in these patients.