Internal biliary diversion using appendix during liver transplantation for progressive familial intrahepatic cholestasis type 1: A case report

Abstract

BACKGROUND

Progressive familial intrahepatic cholestasis type 1 (PFIC-1) is a genetic cholestatic disease causing end-stage liver disease, which needs liver transplantation (LT). Simultaneous biliary diversion (BD) was recommended to prevent allograft steatosis after transplantation, while increasing the risk of infection. Here, an attempt was made to perform BD using appendix to prevent bacterial translocation after LT.

CASE SUMMARY

An 11-month-old boy diagnosed with PFIC-1 received ABO compatible living donor LT due to refractory jaundice and pruritus. His mother donated her left lateral segment with a graft-to-recipient weight ratio of 2.9%. Internal BD was constructed during LT using the appendix by connecting its proximal end with the intrahepatic biliary duct and the distal end with colon. Biliary leakage was suspected on the 5^th day after transplantation and exploratory laparotomy indicated biliary leakage at the cutting surface of liver. The liver function returned to normal on the 9^th day post-operation and maintained normal during the 15-month follow-up. Cholangiography at 10 months after transplantation confirmed the direct secretion of bile into colon. Computerized tomography scan (4 months and 10 months) and liver biopsy (10 months) indicated no steatosis in the allograft. No complaint of recurrent diarrhea, infection or growth retardation was reported during follow-up.

CONCLUSION

Internal BD using appendix during LT is effective in preventing allograft steatosis and post-transplant infection in PFIC-1 recipients.

Keywords: Liver transplantation; Progressive familial intrahepatic cholestasis type 1; Biliary diversion; Appendix; Case report

Core Tip: Liver transplantation indicated in end-stage liver disease caused by progressive familial intrahepatic cholestasis type 1 is often performed with biliary diversion (BD). Although effectively preventing allograft steatosis, traditional BD increases post-operative infection risk. Here, we present a case of an 11-month-old progressive familial intrahepatic cholestasis type 1 patient receiving liver transplantation and BD using appendix to prevent post-operative infection. The proximal end and distal end of appendix were connected with intrahepatic biliary duct and colon respectively. Post-operative exams confirmed successful BD and absence of allograft steatosis. Thus, using appendix for BD might be a new surgical approach preventing allograft steatosis and post-transplant infection.