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

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...

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Bibliographic Details
Published in:Pediatric transplantation 2016-11, Vol.20 (7), p.981-986
Main Authors: Mali, V. P., Fukuda, A., Shigeta, T., Uchida, H., Hirata, Y., Rahayatri, T. H., Kanazawa, H., Sasaki, K., Ville de Goyet, J., Kasahara, M.
Format: Article
Language:English
Subjects:
Adenosine Triphosphatases - genetics
Bile
Bile Ducts - physiopathology
Bile Ducts - surgery
biliary diversion
Child, Preschool
Cholestasis, Intrahepatic - surgery
Diarrhea - etiology
Fatty Liver - etiology
Female
Heterozygote
Humans
Jaundice - etiology
Jejunum - surgery
Liver Transplantation
living donor liver transplantation
Postoperative Complications
progressive familial intrahepatic cholestasis type 1
Pruritus - etiology
Treatment Outcome
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Summary: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 (
ISSN:1397-3142
1399-3046
DOI:10.1111/petr.12782