Total internal biliary diversion during liver transplantation for type 1 progressive familial intrahepatic cholestasis: A novel approach

V. P. Mali, A. Fukuda, T. Shigeta, H. Uchida, Y. Hirata, T. H. Rahayatri, H. Kanazawa, K. Sasaki, J. de Ville de Goyet, M. Kasahara

Research output: Contribution to journalArticlepeer-review

Abstract

LT for PFIC type 1 is often complicated by postoperative diarrhea and recurrent graft steatosis. A 26-month-old female child with cholestatic jaundice, pruritus, diarrhea, and growth retardation revealed total bilirubin 9.1 mg/dL, gamma-glutamyl transpeptidase 64 IU/L, and TBA 295.8 μmol/L. Genetic analysis confirmed ATP8B1 defects. A LT (segment 2, 3 graft) from the heterozygous father was performed. Biliary diversion was performed by a 35-cm jejunum conduit between the graft hepatic duct and the mid-transverse colon. Stools became pigmented immediately. Follow-up at 138 days revealed resolution of jaundice and pruritus and soft-to-hard stools (6-8 daily). Radioisotope hepato-biliary scintigraphy (days 26, 68, and 139) confirmed unobstructed bile drainage into the colon (t1/2 34, 27, and 19 minutes, respectively). Contrast meal follow-through at day 62 confirmed the absence of any colo-jejuno-hepatic reflux. At 140 days, contrast follow-through via the biliary stent revealed patent jejuno-colonic anastomosis and satisfactory transit. Graft biopsy at LT, 138 days, and 9 months follow-up revealed comparable grades of macrovesicular steatosis (<20%). TIBD during LT may be a clinically effective stoma-free biliary diversion and may prevent recurrent graft steatosis following LT for PFIC type 1.

Original languageEnglish
JournalPediatric Transplantation
DOIs
Publication statusAccepted/In press - 2016

Keywords

  • Biliary diversion
  • Liver transplantation
  • Living donor liver transplantation
  • Progressive familial intrahepatic cholestasis type 1

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Transplantation

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