Background: Congenital ectopia lentis (CEL) is a hereditary eye disease which severely impacts preschool children's visual function and development. This study aimed to evaluate the longitudinal changes in spherical equivalent (SE) refractive error in preschool children with CEL.Methods: A retrospective cohort study was conducted at Zhongshan Ophthalmic Center, Guangzhou, China. Medical records of CEL patients under 6-year-old who were diagnosed with Marfan syndrome at the initial visit from January, 2014 to March, 2022 were collected and were divided into surgery and nonsurgery groups. Mean change rate of SE in the two groups were evaluated and the potential associated factors of SE change rate were investigated by mixed-effect regression model.Results: A total of 94 preschool patients from 14 provinces of China were included. Among the 42 children of the surgery group, the mean age with standard deviation (SD) was 5.02±0.81 years and patients experienced a myopic shift of -0.05±0.09 D/month in average. The mean age with SD of the 52 children of the non-surgery group was 4.34±1.02 years and the mean myopic shift was -0.09±0.14 D/month. The mixed-effect regression model identi ed that higher degree of myopia at baseline was associated with slower myopic shift both in surgery (β= 0.901, 95% CI: 0.822~0.980, P< 0.001) and in non-surgery group (β= 1.006, 95% CI: 0.977~1.034, P< 0.001) in CEL patients. Surgical treatment (β= 2.635, 95% CI: 1.376~3.894, P< 0.001) was associated with slower myopic shift in all participants CEL patients.Conclusions: Myopic progression was slower in the surgery group than in the non-surgery group of CEL. Preschool CEL patients who met the surgical indication are suggested being performed with timely surgery to slow down the myopic progression.This study was approved by the Ethical Committee of the Zhongshan Ophthalmic Center, Sun Yat-sen University (ID: 2020KYPJ146) and was conducted in accordance with the tenets of the Declaration of Helsinki (as revised in 2013). Due to the retrospective nature of the study, informed consent was waived.
SubjectsThis retrospective study was conducted at Zhongshan Ophthalmic Center, Guangzhou, China. All paediatric CEL patients with age < 6 years diagnosed with Marfan syndrome at the initial visit from January, 2014 to March, 2022 were included, since the age from 0 to 6 was regarded as a crucial period for ocular development. [10,19]. Binocular symmetrical subluxated lenses without history of ocular trauma, neoplasm, glaucoma or uveitis, were regarded as CEL. Marfan syndrome was diagnosed according to the revised Ghent nosology. [20] Exclusion criteria were as follows: (a) with history of corneal or fundus disorders, (b) with severe postoperational complications such as uveitis, glaucoma and retinal detachment, (c) with invalid refraction or less than 6 months of follow-up visits. Eligible subjects were divided into surgery group or non-surgery group according to the treatment procedure. Surgical indications in our clinical practice are as follows: (a)S...