Biliary atresia with extrahepatic biliary cysts--cholangiographic patterns influencing the prognosis.

PURPOSE: Biliary atresia (BA) with extrahepatic biliary cysts (EHBC) has been recognized generally as "correctable" BA, which indicates a good prognosis. The variants of BA with EHBC according to cholangiographic findings and their outcomes were reviewed.

METHODS

An EHBC was observed in 8 (20%) of 40 patients with BA who underwent operation at our institute. Intraoperative cholangiographic patterns included visualization of the intrahepatic bile ducts (type I BA with EHBC) in 6 patients and no visualization (type III BA with EHBC) in 2. Intrahepatic biliary cysts (IHBC) and EHBC were observed simultaneously in 2 patients diagnosed at older age. The follow-up periods ranged between 4 months and 20 years.

RESULTS

Good bile drainage after a hepaticoenterostomy or portoenterostomy was obtained in all 6 patients with type I BA with EHBC. Two who showed IHBC on intraoperative cholangiography had complications caused by postoperative recurrent cholangitis, which led to a liver transplantation in 1. Revision after the portoenterostomy was required in 2 patients with type III BA with EHBC. One became jaundice free after revision, whereas the other died of hepatic failure without bile drainage.

CONCLUSION

Intraoperative cholangiographic findings showing IHBC and type III BA are poor prognostic factors in patients with BA with EHBC.