The surgical management of the unusual forms of biliary atresia

Of 50 infants undergoing laparotomy for biliary atresia during the last 9 yr at the Kobe Children's Hospital, 9 had variant forms, including (A) a gallbladder with a demonstrable connection with the intrahepatic biliary system, (B) a gallbladder with a connection with the extrahepatic biliary system and duodenum, and (C) a grossly visible subhepatic cyst. Three patients with form A underwent cholecystoduodenostomy. All these patients died of cholangitis and hepatic failure within 2 mo after operation. Two patients with form B were successfully treated by hepatic portocholecystostomy without any episodes of cholangitis. Two of the patients with form C, occurring in 1970, underwent an anastomosis between the subhepatic cyst and the duodenum. There was no bile excretion in these patients. The other two patients were treated subsequently by hepatic portoenterostomy with complete excision of the subhepatic cyst and excellent bile excretion was obtained. Infants with forms A and C should be treated by hepatic portoenterostomy instead of a direct anastomosis between the dilated portion of the extrahepatic biliary system and the alimentary tract. For patients with form B, hepatic portocholecystostomy seems to be the most suitable procedure relative to the prevention of postoperative cholangitis, which is the most serious complications in infants obtaining successful bile excretion.