title: Total internal biliary diversion during liver transplantation for type 1 progressive familial intrahepatic cholestasis: a novel approach.
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 (t1/234, 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.
authors.affiliation: Organ Transplantation Center, National Center for Child Health and Development, Tokyo, Japan. survpm@nus.edu.sg.
authors.forename: V P
authors.initials: VP
authors.lastname: Mali
authors.affiliation: Organ Transplantation Center, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: A
authors.initials: A
authors.lastname: Fukuda
authors.affiliation: Organ Transplantation Center, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Shigeta
authors.affiliation: Organ Transplantation Center, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: H
authors.initials: H
authors.lastname: Uchida
authors.affiliation: Organ Transplantation Center, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Hirata
authors.affiliation: Organ Transplantation Center, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: T H
authors.initials: TH
authors.lastname: Rahayatri
authors.affiliation: Organ Transplantation Center, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: H
authors.initials: H
authors.lastname: Kanazawa
authors.affiliation: Organ Transplantation Center, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Sasaki
authors.affiliation: Department of Surgery and Transplantation Centre, Bambino Gesù Children's Hospital, Rome, Italy.|Paediatric Surgery Chair, Università di Roma Tor Vergata, Rome, Italy.
authors.forename: J
authors.initials: J
authors.lastname: de Ville de Goyet
authors.affiliation: Organ Transplantation Center, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Kasahara
mesh_terms: D000251:Adenosine Triphosphatases / Q000235:genetics; D001646:Bile; D001652:Bile Ducts / Q000503:physiopathology / Q000601:surgery; D002675:Child, Preschool; D002780:Cholestasis, Intrahepatic / Q000601:surgery*; D003967:Diarrhea / Q000209:etiology; D005234:Fatty Liver / Q000209:etiology; D005260:Female; D006579:Heterozygote; D006801:Humans; D007565:Jaundice / Q000209:etiology; D007583:Jejunum / Q000601:surgery; D016031:Liver Transplantation; D011183:Postoperative Complications; D011537:Pruritus / Q000209:etiology; D016896:Treatment Outcome
chemical_list: D000251:Adenosine Triphosphatases; C111067:ATP8B1 protein, human
keywords: biliary diversion; liver transplantation; living donor liver transplantation; progressive familial intrahepatic cholestasis type 1