Internal Ileal Diversion as Treatment for Progressive Familial Intrahepatic Cholestasis Type 1-Associated Graft Inflammation and Steatosis after Liver Transplantation
Anna M. Kavallar,
Franka Messner,
Stefan Scheidl,
Rupert Oberhuber,
Stefan Schneeberger,
Denise Aldrian,
Valeria Berchtold,
Murat Sanal,
Andreas Entenmann,
Simon Straub,
Anna Gasser,
Andreas R. Janecke,
Thomas Müller,
Georg F. Vogel
Affiliations
Anna M. Kavallar
Department of Paediatrics I, Medical University of Innsbruck, 6020 Innsbruck, Austria
Franka Messner
Department of Visceral, Transplant and Thoracic Surgery, Medical University of Innsbruck, 6020 Innsbruck, Austria
Stefan Scheidl
Department of Visceral, Transplant and Thoracic Surgery, Medical University of Innsbruck, 6020 Innsbruck, Austria
Rupert Oberhuber
Department of Visceral, Transplant and Thoracic Surgery, Medical University of Innsbruck, 6020 Innsbruck, Austria
Stefan Schneeberger
Department of Visceral, Transplant and Thoracic Surgery, Medical University of Innsbruck, 6020 Innsbruck, Austria
Denise Aldrian
Department of Paediatrics I, Medical University of Innsbruck, 6020 Innsbruck, Austria
Valeria Berchtold
Department of Visceral, Transplant and Thoracic Surgery, Medical University of Innsbruck, 6020 Innsbruck, Austria
Murat Sanal
Department of Visceral, Transplant and Thoracic Surgery, Medical University of Innsbruck, 6020 Innsbruck, Austria
Andreas Entenmann
Department of Paediatrics I, Medical University of Innsbruck, 6020 Innsbruck, Austria
Simon Straub
Department of Paediatrics I, Medical University of Innsbruck, 6020 Innsbruck, Austria
Anna Gasser
Department of Paediatrics I, Medical University of Innsbruck, 6020 Innsbruck, Austria
Andreas R. Janecke
Department of Paediatrics I, Medical University of Innsbruck, 6020 Innsbruck, Austria
Thomas Müller
Department of Paediatrics I, Medical University of Innsbruck, 6020 Innsbruck, Austria
Georg F. Vogel
Department of Paediatrics I, Medical University of Innsbruck, 6020 Innsbruck, Austria
Background: Progressive Familial Intrahepatic cholestasis type I (PFIC1) is a rare congenital hepatopathy causing cholestasis with progressive liver disease. Surgical interruption of the enterohepatic circulation, e.g., surgical biliary diversion (SBD) can slow down development of liver cirrhosis. Eventually, end stage liver disease necessitates liver transplantation (LT). PFIC1 patients might develop diarrhea, graft steatosis and inflammation after LT. SBD after LT was shown to be effective in the alleviation of liver steatosis and graft injury. Case report: Three PFIC1 patients received LT at the ages of two, two and a half and five years. Shortly after LT diarrhea and graft steatosis was recognized, SBD to the terminal ileum was opted to prevent risk for ascending cholangitis. After SBD, inflammation and steatosis was found to be reduced to resolved, as seen by liver biochemistry and ultrasounds. Diarrhea was reported unchanged. Conclusion: We present three PFIC1 cases for whom SBD to the terminal ileum successfully helped to resolve graft inflammation and steatosis.
