Department of Pediatric Surgery, Severance Children's Hospital, Department of Surgery, Yonsei University College of Medicine, Severance Pediatric Liver Diseases Research Group, Seoul, Korea.
Biliary atresia (BA) is an infantile cholestatic disease of progressive obliterative cholangiopathy with varying degrees of damage to both extra and intrahepatic bile ducts due to unknown causes. The diagnostic studies should be done to diagnose or exclude BA without unnecessary delay. Kasai portoenterostomy is the first choice of treatment for bile drainage from microscopic bile ductules present in the portal fibrous mass. The medical management after Kasai portoenterostomy should be done carefully to maintain bile excretion and prevent and treat complications including cholangitis, hepatic fibrosis, portal hypertension and nutritional problem. The reported five years-survival rates after Kasai portoenterostomy range from 30 to 60%. About 20% of all patients undergoing Kasai portoenterostomy during infancy survive into adulthood with their native liver. Even if Kasai portoenterostomy remains as the first line of treatment in BA, liver transplantation serves as a good salvage treatment when portoenterostomy fails or liver function gradually deteriorates after initially successful establishment of bile flow. Overall 5-year survival rate in BA is about 90% in recent series.
