BACKGROUND/PURPOSE: It is difficult to discriminate between choledochal cyst[corrected]with obstructive jaundice and biliary atresia with a cyst at the porta hepatis in neonates or young infants. This review evaluates whether it is possible to differentiate between these two diseases. We here also provide an overview of our experience with type I cyst biliary atresia patients.

METHODS

Among all the biliary atresia infants who we treated, the infants who were diagnosed with type I cyst biliary atresia were identified and reviewed for their management and outcome. The clinical course and management in different reports were reviewed and compared to the cases presented to our institution.

RESULTS

Among the 220 biliary atresia cases, 11 (5 %; male/female: 4/7) were diagnosed to be type I cyst biliary atresia. Two received hepaticoenterostomy and nine received hepatic portoenteros. Three patients had severe late complications; overall, nine (81.8 %) were alive with their native liver and without jaundice.

CONCLUSIONS

Patient with choledochal cyst [corrected] are likely to represent larger cysts and inversely, smaller, static, anechoic cysts are more likely to represent cystic biliary atresia. However, exceptional cases were yet presented, and a definitive diagnosis may not be reached. Thus a complete differentiation between choledochal cyst [corrected] from type I cyst biliary atresia is yet hard to reach.