Biliary Atresia-associated Cholangitis: The Central Role and Effective Management of Bile Lakes. 
Cholangitis and bile lakes are incompletely understood complications after portoenterostomy (PE).  We investigated relationships between recurrent cholangitis, bile lakes, and clinical outcomes as well as surgical management of bile lakes.  In this retrospective observational single institution study medical records and imaging studies of all patients who had undergone PE for biliary atresia during 1987 to 2016 (N = 61) were reviewed.  We related occurrence of cholangitis episodes with the presence of intrahepatic bile lakes, patient characteristics, and PE outcomes.  Risk factors for recurrent cholangitis and bile lakes, and management of bile lakes were analyzed.  Despite routine antibiotic prophylaxis median of 3.0 cholangitis episodes (0.75 episodes/year) occurred in 48 (79%) patients.  Intrahepatic bile lakes were discovered in 8 (13%) patients by 16 months after PE.  Overall, 54% had survived with their native liver at median age of 7.3 years and 28 (46%) patients had ≥1 cholangitis episodes/year.  Number and frequency of cholangitis episodes were >5 times higher among patients with bile lakes (P < 0.001).  Six patients underwent Roux-en-Y bile lake-jejunostomy, resulting in regression/disappearance of bile lakes and normalization of serum bilirubin in 5 with reduction of median yearly cholangitis rate from 8.8 to 1.1 (P = 0.028) and native liver survival of 6.3 (range, 1.3-17) years after the operation.  Bile lakes are a significant risk factor for recurrent cholangitis after PE and efficiently treated by operative intestinal drainage providing prolonged jaundice-free native liver survival.  Bile lakes should be actively screened among patients presenting with recurrent cholangitis after PE.