Long-term outcome after surgery for biliary atresia

Biliary atresia—a fifteen-year review of clinical and pathologic factors associated with liver transplantation

“…8 Our series showed that the age at which the Kasai procedure was initially performed was not significantly different between the groups (55 v 56 days). The initial duration of jaundice before Kasai hepatic portoenterostomy, however, was significantly longer in the group that subsequently required transplantation.…”

Acute intrahepatic portal vein thrombosis complicating cholangitis in biliary atresia

“…Despite successful biliary drainage by portoenterostomy, patients with biliary atresia typically progress towards cirrhosis and portal hypertension [3,8]. Herein, we report a case of acute intrahepatic portal vein thrombosis in a patient post-Kasai portoenterostomy for biliary atresia.…”

“…Recurrent episodes of cholangitis are associated with an accelerated progression to biliary cirrhosis disrupting the hepatic architecture, further increasing portal pressure [8]. It has previously been proposed that children with cirrhosis associated with bile duct damage are particularly susceptible to hepatic infarction and had more severe portal hypertension as a consequence of intra or extrahepatic portal vein thrombosis [5,6,13].…”

“…Of children with biliary atresia after a successful Kasai procedure 25% are well after 5 years of age, and 20% are well after 10 years of age. Biliary atresia remains the most frequent indication for liver transplantation and accounts for 50% of the total of all paediatric cases [8].…”

Biliary Atresia

“…If successful, any patent intrahepatic bile ducts will drain into the roux limb allowing relief of the biliary obstruction. If possible, a Kasai procedure should be performed before 60 days of age when its short-term success is 80% 16,83,84. Efficacy of the procedure drops with age of the patient, decreasing to 60% by 90 days of age.…”