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Keywords:

  • biliary atresia;
  • cholangitis;
  • hepatic portoenterostomy;
  • Kasai operation;
  • liver transplantation

Abstract: Although the prognosis of biliary atresia has been improved in recent years, particularly in the era of liver transplantation, hepatic portoenterostomy, e.g., the Kasai operation, is still the first line of surgical treatment. Successful hepatic portoenterostomy depends on early diagnosis and operation, adequate operative technique, prevention of postoperative cholangitis, and precise postoperative management. The pathophysiology of the liver and of the intrahepatic bile ducts in this disease is still controversial.