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Chapter 5152resolved by discussion. Reference lists of the included studies were handsearched for additional relevant articles.Quality assessment To assess the relevance and validity of the included articles, we performed a critical appraisal using the Risk of bias assessment tool for prevalence studies,10 which was adapted and specified to our research question (See Supplementary 2). The quality of the studies was assessed independently by two reviewers (EV, MN and/or EVS) and discrepancies were resolved by discussion. In the absence of reference scores, we decided to exclude studies with a very high risk of bias (≥7/10 points) for data extraction. Risk of bias assessment included selection bias, standardization, measurement bias and non-response bias. In case of overlap of populations of the same research group, the study with the lowest risk of bias was included. Data extraction Data on visual acuity (VA), refractive errors, eye position and motility, eye lid abnormalities, biomicroscopic and fundoscopic results were extracted by one reviewer and verified by a second reviewer. VA measurements were transformed to logarithm of the minimum angle of resolution (LogMAR) for uniformity. We categorized VA as (near) normal (≤0.30 LogMAR), mild (>0.30– <0.50 LogMAR), moderate (≥0.50 – <1.0 LogMAR) or severely impaired(≥1.0 LogMAR) according to criteria of the World Health organization.11Dutch multicenter cross-sectional study Study design and setting The study was approved by the institutional ethical committees of the University Medical Center Utrecht (UMCU, #18-510/C), Máxima Medical Center Veldhoven (MMCV, #L20.044), and Maastricht University Medical Center+ (MUMC+, #2019-1321). Through a review of medical records, we systematically compiled data of 22q11.2DS patients that visited the ophthalmological outpatient clinic of UMCU, MMCV, and/or MUMC+ between January 1992 and January 2021. All centers are multidisciplinary outpatient clinics for 22q11.2DS. Ophthalmic screening was carried out as regular screening after diagnosis or referral in all clinics and only in a minority of cases upon clinical indication.