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Ocular findings in 22q11.2DS16951.3 Cochrane search termsSearch terms used in CochraneDomain ((refraction OR vision OR visual OR ophthalm* OR eye OR eyes OR conjunctiv* OR cornea* OR ocular OR optic* OR orbit* OR retina* OR sclera* OR uvea* OR optic-nerve OR eyelid* OR lacrima* OR lens* OR pupil* OR iris OR intra-ocular OR intraocular OR cataract OR blindness OR embryotoxon OR tortuous-retinalveins OR retinal-vascular-tortuosity OR tortuous-retinal-vessels OR refract* OR strabismus OR exotropia OR esotropia OR amblyopia OR ptosis OR distichiasis or astigmati*):ti,ab,kwOutcome (22q11* OR del22q* OR DiGeorge OR di-george OR Velocardiofacial OR velocardio-facial OR VCF-syndrome OR Conotruncal anomal* face OR CTAF OR Autosomal dominant Opitz OR opitz G OR GBBB OR G BBB OR sedlackova OR Cayler OR catch22 OR catch 22 OR shprintzen OR thymic aplasia OR 22q11.2 Deletion Syndrome):ti,ab,kw)2. Critical appraisal formRisk of bias item ScoringExternal validity1. Was the study’s target population a close representation of the standard 22q11.2DS population?1 = yes 0 = no or unclear description2. Was the diagnosis genetically confirmed in all patients?1 = yes 0 = no or unclear description3. Was some form of random selection used to select the sample, or was a census undertaken?1 = yes 0 = no or unclear description4. Was the response rate for the study ≥ 75% orwas an analysis performed that showed no significant difference in relevant demographic characteristics between responders and nonresponders?1 = yes 0 = no or unclear descriptionInternal validity5. Were data collected directly from the subjects (as opposed to proxy)?1 = yes 0 = no or unclear description6. Was an acceptable definition of ocular features measured in the study used?1 = yes 0 = no or unclear description7. Was methods of eye examination described? 1 = yes 0 = no or unclear description8. Was the same mode of data collection used for all subjects?1 = yes 0 = no or unclear description9. Was the length of the shortest prevalence period for the parameter of interest appropriate?1 = yes 0 = no or unclear description10. Were the numerator(s) and denominator(s) for the parameter of interest appropriate?1 = yes 0 = no or unclear descriptionSource: Risk of bias assessment tool for prevalence studies, Hoy D, Brooks P, Woolf A, Blyth F, March L, Bain C, et al. Assessing risk of bias in prevalence studies: modification of an existing tool andevidence of interrater agreement. J Clin Epidemiol. 2012;65(9):934-9.