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

Abstract

Background: Post liver transplant (LT) patients with Progressive Familial Intrahepatic Cholestasis Type 1 (PFIC-1) often develop progressive graft steatohepatitis, intractable diarrhea & growth failure. A total internal biliary diversion (TIBD) during a LT may prevent or reverse these adverse events. Methods: Children with PFIC- 1 who underwent a LT at our institute were divided into two groups, A & B based on the timeline where we started offering a TIBD in association with LT. Pre LT parameters, intraoperative details, post transplant complications like graft steatosis and diarrhea also analysed between two groups and their growth velocity were measured in follow up. Results: Of 550 pediatric LT performed between 2011 and 2022, 13 children underwent LT for PFIC-1. Group A had 7 patients (A1-A7) and Group B had 6 (B1-B6). Patients A1,A4,B4,B5 had a failed partial internal biliary diversion prior offering them a LT. Patients A1, A2,& A6 in Group A died in the post LT period (2 early allograft dysfunction & 1 PTLD) whereas A3,A4 & A5 had graft steatosis in follow up. A4 was offered a TIBD 4 years post LT following which the graft steatosis fully resolved. In group B, B1, B2, B5, B6 underwent TIBD during the LT and B3 and B4 had it 24 & 5-months subsequently for intractable diarrhea and graft steatosis. None of the patients in group B demonstrated a graft steatosis or diarrhea and had good growth catch up during follow up. Conclusion: We demonstrate that simultaneous TIBD in patients undergoing LT should be standard practice as it helps dramatically improve outcomes in PFIC-1 as it prevents graft steatosis and/or fibrosis, diarrhea and improves growth catch-up.