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Research Article: Evaluation of functional vision and eye-related quality of life in children with congenital ectopia lentis: a prospective cross-sectional study

Date Published: 2023-12-21

Abstract:
Objectives This study aims to evaluate the effect of congenital ectopia lentis (CEL) on functional vision and eye-related quality of life (ER-QOL) in children and their families using the Paediatric Eye Questionnaire (PedEyeQ). Design A questionnaire survey administered via in-person interviews of patients with CEL and their parents. Participants 51 children with CEL and 53 visually normal controls accompanied by 1 parent completed the survey questionnaires for the study from March 2022 to September 2022. Outcome measures PedEyeQ domain scores. Functional vision and ER-QOL of children and their families were evaluated by calculating and comparing the Rasch domain scores of the PedEyeQ. Results PedEyeQ domain scores were significantly worse with CEL compared with controls (p<0.01 for each), with the exception of the Proxy Social domain among children aged 0–4 years (p=0.283). Child PedEyeQ greatest differences were in the functional vision domain (5–11 years, ?20 points (95%?CI ?27 to ?12)) and frustration/worry domain (12–17 years, ?41 (95%?CI ?37 to ?6)). Proxy PedEyeQ greatest differences were in the functional vision domain (0–4 years, ?34 (95%?CI ?45 to ?22)) and frustration/worry domain (5–11 years, ?27 (95%?CI ?39 to ?14); 12–17 years, ?37(95%?CI (?48 to ?26))). Parent PedEyeQ greatest difference was in the ‘worry about child’s eye condition’ (?57 (95%?CI (?63 to ?51))). Conclusions In this study, children with CEL had reduced functional vision and ER-QOL compared with controls. Parents of children with CEL also experience reduced quality of life.

Introduction:
Congenital ectopia lentis (CEL) is defined as the displacement of the crystalline lens from its normal position caused by congenital dysplasia of the zonule. The prevalence rate of CEL is approximately 6.4/10?million. 1 CEL may present with symptoms such as blurred vision, fluctuating vision and monocular diplopia, 2–4 and can also occur in conjunction with abnormalities in the skeletal and cardiovascular system, as seen in Marfan’s syndrome (MFS), homocystinuria and Weill-Marchesani syndrome. 5–7 MFS accounts for…

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