Hepatobiliary and Pancreatic: Extrahepatic biliary atresia in an adult

Abstract

A teenager, aged 18, was referred for evaluation because of elevated serum levels of alanine aminotransferase (ALT) and aspartate aminotransferase (AST) for 16 years. There were no significant abdominal or other symptoms. However, on physical examination, he had a large spleen, 8 cm below the left costal margin. Routine blood tests showed anemia, leucopenia and thrombocytopenia. Liver function tests revealed a normal serum bilirubin but elevated levels of ALT, AST, alkaline phosphatase (ALP) and gamma-glutamyl transpeptidase (GGT). Various investigations for viral hepatitis, autoimmune disease and Wilson's disease were unhelpful. An abdominal ultrasound study and computed tomography (CT) scan showed dilatation of intrahepatic ducts and splenomegaly. Magnetic resonance cholangiopancreatography (MRCP) showed cystic dilatation of the hilar bile duct and moderate dilatation of intrahepatic ducts. The gallbladder (GB) and common bile duct (BD) were clearly shown and the gallbladder appeared to be linked to intrahepatic ducts (Figure 1). Endoscopic retrograde cholangiopancreatography (ERCP) was also performed and showed contrast passing from the common bile duct into the cystic duct and gallbladder (Figure 2). The common hepatic duct was not outlined. Endoscopy revealed esophageal and gastric varices while CT angiography showed a normal hepatic artery, portal vein and inferior vena cava. Figure 1 Figure 2 At laparotomy, the patient had features of cirrhosis. An operative cholangiogram was performed by injection of contrast into the gallbladder and only showed contrast in the common bile duct, similar to findings at ERCP. In the process of mobilizing the gallbladder, bile ducts in the gallbladder bed were shown to communicate with the gallbladder. The common hepatic duct could not be identified but dissection revealed a hilar pouch containing bile. Two hepaticojejunostomies were performed to drain bile from the gallbladder bed and from the hilar pouch. Splenectomy was also performed. The anastomoses in the gallbladder bed were shown to be patent by passage of contrast through a stent. Liver function tests returned to normal after 8 months. This may be the first report of biliary atresia diagnosed in an adult. In this case, he had an unusual variant characterized by absence of the common hepatic duct (type II). The diagnosis was delayed because of the development of anastomoses between branches of the right hepatic duct and the gallbladder. Despite this, there was persistent cholestasis with the development of biliary cirrhosis and portal hypertension. The surgical procedure appears to have been helpful in the short-term but the longer-term outcome remains unclear. Contributed by