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Purpose: It is difficult to distinguish cystic biliary atresia (CBA) from choledochal cyst (CC) before intraoperative cholangiography in neonates or young infants because of the similar ultrasonographic patterns and clinical manifestations. This study is to investigate the difference of clinical parameters between CBA and CC., Methods: 96 patients with cyst at hepatic hilum whose ages were less than 120 days during the period from Jan'2013 to Nov'2015 were retrospectively studied, they were divided into CBA group and CC group by intraoperative cholangiography, there were 29 cases of CBA and 67 cases of CC. It was compared and analyzed on laboratory data, preoperative ultrasonographic features, intraoperative cholangiography and histopathological results between the two groups. Data were quoted as means ± standard deviation (SD) or median (range), Student's t test, non-parametric test and χ2 test were used as appropriate. P < 0.05 was considered as significant statistical difference., Results: CBA group and CC group were comparable for the operative age (51.76 ± 23.99 days vs. 50.03 ± 26.38 days, P = 0.76), weight (4.60 ± 0.75 kg vs. 4.44 ± 1.03 kg, P = 0.46) and sex proportion (M/F: 9/20 vs. 20/47, P = 0.91). there were significant differences (CBA vs. CC) in ALT [59(3-375) vs. 30 (6-247) IU/L, P < 0.001], AST [140(30-694) vs. 44(18-410) IU/L, P < 0.001], T-Bil [190 (107-326) vs.87(5-310) μmol/L, P < 0.001], D-Bil [85 (31-174) vs.14(1-122) μmol/L, P < 0.001] and TBA [112 (23-269) vs.9(1-337) μmol/L, P < 0.001]. There was insignificant statistical difference in GGT [332(112-2154) vs. 226(21-1810) IU/L, P = 0.099] between CBA group and CC group. The cyst size in CBA group was obviously smaller than CC group (the maximum longitudinal diameter: 2.52 ± 1.16 cm vs. 4.71 ± 2.23 cm, P < 0.001; the maximum transverse diameter: 1.64 ± 0.75 cm vs. 3.41 ± 1.79 cm, P < 0.001). Significant differences presented in preoperative ultrasonographic features and histopathological results between the two groups., Conclusion: CBA should be suspected if ultrasonography indicates small cyst with characteristic manifestations of BA and elevated laboratory data. GGT was an insensitive indicator in distinguishing CBA from CC. Cholangiography should be done for the suspected CBA patients as soon as possible to confirm the diagnosis and have surgical intervention timely., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: With the advent of ultrasonography (US), perinatal diagnosis of biliary cystic malformation (BCM) has become more prevalent. BCM includes cystic biliary atresia (CBA) and choledochal cyst (CC). Both share similar ultrasonographic features and clinical manifestations, and yet the postnatal management is very different. This study was to assess whether CBA can be distinguished from CC by US., Methods: We retrospectively studied the clinical data of 98 BCM patients aged less than 130 days between January 2013 and November 2015. The patients were divided into the CBA group and the CC group based on intraoperative cholangiography. The ultrasonographic features in each group were then analyzed., Results: Thirty-one children had CBA (type I: 24, type III: 7) and 67 children had CC (all: type I). The cyst volume did not show a significant change in the CBA group while the cyst volume increased postnatally in the CC group. Preoperative cyst volume can be used as a sensitive indicator to distinguish choledochal cyst from cystic biliary atresia. Gallbladder size in the two groups differed significantly (P = 0.034); 23 of 31 patients in the CBA group had atrophic gallbladder, whereas none of the patients in the CC group had atrophic gallbladder (P < 0.001). The triangular cord sign was detected in ten patients in the CBA group and in none of the patients in the CC group (P < 0.001)., Conclusion: Serial prenatal and postnatal ultrasonographic studies may help differentiate CBA from CC in the majority of BCM, facilitating prenatal counseling and postnatal management.