[Partial External Biliary Diversion in Progressive Familial Intrahepatic Cholestasis and Alagille Syndrome: A Case...

Partial external biliary diversion in patients with progressive familial intrahepatic cholestasis and Alagille syndrome reduces plasma bile salt levels by modifying their enterohepatic circulation. When this surgical technique is performed before the onset of cirrhosis, the symptoms may improve. It can also modify hepatic fibrosis evolution and, thus, disease progression by avoiding or delaying liver transplantation. We present eight patients undergoing partial external biliary diversion: five patients were diagnosed with progressive familial intrahepatic cholestasis, and three of them with Alagille syndrome and severe pruritus. Presence of cirrhosis was ruled out in all cases after liver biopsy was performed. Two out of five patients with partial external biliary diversion are alive with their native liver and are almost asymptomatic four and twenty years after the procedure respectively. The three patients with Alagille syndrome improved their symptoms; two of them had complete symptom resolution.