Internal Ileal Diversion as Treatment for Progressive Familial Intrahepatic Cholestasis Type 1-Associated Graft Inflammation and Steatosis after Liver Transplantation

Abstract

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.

Management and outcomes after liver transplantation for progressive familial intrahepatic cholestasis: A systematic review and meta-analysis

“…This emphasizes first the role of the FIC1-deficient enterohepatic circulation underlying this phenomenon and second, as described in earlier reports 23 – 27 that SBD post-LT seems an efficient treatment to protect the graft from injury. Total external biliary diversion or total internal diversion to the colon seems to be the preferable solution.…”

Simultaneous total internal biliary diversion during liver transplantation for progressive familial intrahepatic cholestasis type 1: Standard of care?

“…One patient had ascending cholangitis which the authors attributed to a short roux limb. [11] The role of biliary diversions especially PIBD for relieving pruritus in patients with PFIC-1 is welldocumented in literature. In our previously published series of 9 patients with PFIC-1 who underwent a PIBD, 6 had a complete resolution of their pruritus; others, however, required an LT. [12] Biliary diversion is effective because it works by reducing the circulating pool of the pruritogenic bile acids and altering the cholic acid:chenodeoxycholic acid ratio.…”

“…There was resolution of graft steatosis in all 3 patients and improvement of diarrhea in 1 patient. One patient had ascending cholangitis which the authors attributed to a short roux limb 11 …”