Abstract: ObjectiveTo clarify the value of ultrasound, hepatobiliary scintigraphy and magnetic resonance cholangiopancreatography(MRCP) in the diagnosis of biliary atresia. MethodsAccording to search terms of biliary atresia and bile duct atresia, English literature was searched in Pubmed，Embase，Medline from the establishment of databases to April 26, 2020 and Chinese literature was searched in CBM from January 1, 2000 to April 26, 2020. Retrieved articles were screened and selected according to the inclusion and exclusion criteria. The QUADAS-2 was used to evaluate the risk of bias and applicability concerns. Data were extracted and diagnostic parameters were calculated for the combined data. ResultsThirty studies on ultrasound were included. The sensitivity and specificity of triangular cord sign (24 articles involved) were 79% (95%CI: 68%-86%) and 97% (95%CI: 94%-98%) respectively with I2>97%, and the diagnostic odds ratio (DOR) was 103 (95%CI: 52-203). The SROC curve suggested that the AUC was 0.97 (95%CI: 0.95-0.98), and the funnel plot indicated little publication bias (P=0.28). The sensitivity of abnormal gallbladder morphology (9 articles involved), gallbladder size (12 articles involved), absence of common bile duct (5 articles involved) and hepatic artery diameter (6 articles involved) was 73%, 78%, 92% and 83%, respectively and the specificity was 94%, 76%, 76% and 78% respectively with I2>70%, and DOR was 42, 11,12, and 17, respectively. The SROC curve suggested that AUC was 0.94, 0.81, 0.92 and 0.87, respectively. Thirty-two studies on hepatobiliary scintigraphy were included. The sensitivity and specificity were 98%(95%CI: 95%-99%) and 75%（95%CI: 69%-81%）respectively with I2＞75% and DOR=140（95%CI: 50-392）. The SROC curve suggested that the AUC was 0.93 (95%CI: 0.91-0.95), and the funnel plot indicated little publication bias (P=0.05). In subgroup analysis of liver enzyme inducer before examination (18 articles involved), technetium contrast agent (28 articles involved), detection of contract in intestine only (18 articles involved), and detection of contract in intestine or bile duct (11 articles involved), the sensitivity ranged from 97% to 99%, and the specificity ranged from 75% to 78% with I2>75%, and DOR was 239, 251, 246 and 109, respectively. Seven studies on MRCP were included. The sensitivity and specificity were 92% (95%CI: 79%-97%) and 82% (95%CI: 63%-92%) respectively with I2 of 75% and 83%, and the DOR was 52 (95%CI: 7-382). The SROC curve suggested that the AUC was 0.94 (95%CI: 0.91-0.96). ConclusionCompared with other ultrasonic features (abnormal gallbladder morphology, gallbladder size, absence of common bile duct and hepatic artery diameter), the triangular cord sign detected by ultrasound is more accurate in the diagnosis of biliary atresia. The misdiagnosis rate of hepatobiliary scintigraphy is still high by liver enzyme inducer before examination, technetium contrast agent, detection of contract in intestine or bile duct. MRCP is not stable in terms of diagnosis accuracy of biliary atresia.