Refractive growth in Marfan syndrome patients with ectopia lentis after intraocular lens implantation

PURPOSE: To evaluate refractive growth after intraocular lens (IOL) implantation for Marfan syndrome (MFS) patients with ectopia lentis (EL) and identify influencing factors. SETTING: Eye and ENT Hospital of Fudan University, Shanghai, China. DESIGN: Retrospective cohort study. METHODS: Medical data were collected from patients diagnosed with MFS and EL underwent IOL implantation. Spherical equivalent (SE) and ocular biometric parameters were recorded preoperatively and serially postoperatively. The rate of refractive growth (RRG3) was calculated using aphakic refraction at the IOL plane against log 10 (age + 0.6) years. Patients were stratified by surgical age with multivariate generalized estimating equation analyses identifying predictive factors. RESULTS: A total of 374 eyes of 232 patients were enrolled in this study. Median age at surgery was 6.38 years (interquartile range [IQR] 5.00 to 11.60), with a median follow-up of 3.17 years (IQR 2.17 to 4.54). The mean △SE/△axial length was -2.04 ± 1.84 diopters (D)/mm. The RRG3 model effectively controlled for age demonstrating a mean value of -14.1 ± 10.2 D. Greater postoperative myopia at baseline (β = 0.849, P = .022) and poorer postoperative corrected distance visual acuity (β = -10.333, P = .001) were associated with more negative RRG3 value in patients aged 8 years or younger. In patients aged 8 to 15 years at surgery, RRG3 was only correlated with postoperative SE (β = 1.812, P < .001). Age at surgery, IOL fixation technique, and preoperative ocular biometrics were not significantly associated with RRG3. CONCLUSIONS: Refraction growth of MFS eyes with EL undergoing primary IOL implantation followed a logarithmic progression. Postoperative refractive status and visual acuity significantly influence refractive growth, underscoring the importance of careful postoperative monitoring and strategic undercorrection setting.