Partial Internal Biliary Diversion in Progressive Familial Intrahepatic Cholestasis: Introduction of a New Approach

Abstract

Introduction: Facilitation of biliary salts secretion represents the mainstay of treatment for progressive familial intrahepatic cholestasis (PFIC). The purpose of this study was to introduce a new approach for the treatment of progressive familial intrahepatic cholestasis (PFIC) to avoid ostoma. Case Presentation: An 11-year-old girl with the diagnosis of PFIC underwent cholecystoappendicostomy with myotomy operation. Because of anastomosis stricture, she was reoperated with cholecystojejunocolic anastomosis and intussuscepted valve surgery. She was followed for 9 months. Despite disappointing outcomes of internal drainage with cholecystoappendicostomy, results of cholecystojejunocolic anastomosis with intussuscepted valve surgery were promising. Discussion: The cholecystojejunocolic anastomosis with intussuscepted valve surgery could be considered as a forthcoming approach in the treatment of intrahepatic cholestasis.