Endoscopic findings at porta hepatis and clinical outcome after portoenterostomy in biliary atresia

Abstract

Postoperative endoscopic examination of the porta hepatis through an exteriorized enterostomy was undertaken in 19 children with biliary atresia (BA). Endoscopic findings were classified into three types: (1) ductal (D) type with distinct bile-duct orifice and good bile drainage; (2) oozing (O) type without distinct bile-duct orifice but good bile flow; and (3) covered (C) type with no bile-duct orifice and no bile discharge. The postoperative course of the D-type patients was excellent with immediate disappearance of jaundice and good liver function. In the O type, all but 1 patient became jaundice-free, but the duration of disappearance of jaundice after the operation was significantly longer than for the D type (P <0.05). All of the patients with type C died within 3 years after portoenterostomy. Histologic studies of the extrahepatic biliary remnant showed no significant difference in the area of the largest bile-duct lumen or the total area of all bile duct openings among the three types. The age at operation was youngest in type D, and there was a significant difference between types D and C (P <0.05). Endoscopic examination of the porta hepatis reaffirmed the importance of early diagnosis and early operation in the treatment of BA. Type D may have an excellent prognosis while type C may require liver transplantation.