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Intrahepatic biliary tract abnormalities in children with corrected extrahepatic biliary atresia
Insights
Recurrent cholangitis in children with extrahepatic biliary atresia can stem from undrained bile lakes. Drainage of these bile lakes, alongside antibiotics, resolved infections and improved bile flow, highlighting a new cause for this condition.
Area of Science:
- Pediatric Surgery
- Hepatology
- Gastroenterology
Background:
- Extrahepatic biliary atresia (EHBA) is a serious neonatal liver condition requiring surgical correction.
- Recurrent cholangitis is a known complication following surgical correction of EHBA.
- The underlying mechanisms for persistent or recurrent cholangitis in EHBA patients are not fully understood.
Abstract:
Three children with surgically corrected extrahepatic biliary atresia developed recurrent cholangitis associated with bile lakes that failed to drain via the hepatoportoenterostomy. Surgical or percutaneous drainage of these cysts was followed by both resolution of the infection and spontaneous internal drainage. We postulate that the ongoing inflammatory process resulted in intrahepatic biliary obstruction, which caused cholangitis and bile cysts. Successful treatment required not only antibiotics but drainage of the bile lakes. Development of bile cysts is a new cause of recurrent cholangitis seen in extrahepatic biliary atresia.