Liver transplantation for congenital biliary dilatation: a single-center experience.

Abstract

BACKGROUND:

Congenital biliary dilatation is a rare disease. Although the possibility of refractory cholangitis and/or the frequency of malignant tumors legitimize hepatobiliary surgery, repeated cholangitis and biliary obstruction result in secondary liver cirrhosis even after polysurgery. There are no definitive guidelines on liver transplantation for congenital biliary dilatation.

PATIENTS:

A total of 1,101 liver transplantation recipients were enrolled in this study. Eleven patients with congenital biliary dilatation including 5 patients with Caroli's disease were retrospectively analyzed in detail.

RESULTS:

Nine of 11 patients underwent initial operations before liver transplantation while 2 patients with Caroli's disease received liver transplantation as initial surgery, with good outcomes. All patients had intractable symptoms caused by liver cirrhosis, and growth delay was considerable in patients aged <20 years. Histopathological analysis of the native liver revealed hepatic fibrosis (≥F2). One patient with ABO incompatibility died. One patient with Caroli's disease accompanied with intrahepatic carcinoma survives 11.8 years after liver transplantation without any recurrences.

CONCLUSIONS:

Patients with congenital biliary dilatation with refractory symptoms and complications secondary to liver failure are appropriate candidates for liver transplantation. We suggest that liver transplantation is an effective therapeutic option for patients with congenital biliary dilatation with due consideration to many accompanying factors, such as clinical course, growth delay, image findings and histopathological analysis.

• Authors: Tomohide Hori, Fumitaka Oike, Yasuhiro Ogura, Kohei Ogawa, Koichiro Hata, Yukihide Yonekawa, Mikiko Ueda, Seisuke Sakamoto, Mureo Kasahara, Hiroto Egawa, Yasutsugu Takada, Toshimi Kaido, Etsuro Hatano, Justin H Nguyen, Feng Chen, Ann-Marie T Baine, Shinji Uemoto
• Journal: Digestive surgery (Dig Surg) Vol. 27 Issue 6 Pg. 492-501 ( 2010) ISSN: 1421-9883 [Electronic] Switzerland
• PMID: 21063126 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: Copyright © 2010 S. Karger AG, Basel.

Topics

• Adolescent
• Adult
• Biliary Tract (abnormalities)
• Biopsy, Needle
• Caroli Disease (surgery)
• Child
• Child, Preschool
• Cholangiopancreatography, Magnetic Resonance
• Dilatation, Pathologic
• Female
• Humans
• Immunosuppression Therapy
• Infant
• Liver (diagnostic imaging, pathology)
• Liver Cirrhosis (pathology)
• Liver Failure (complications)
• Liver Transplantation
• Male
• Radionuclide Imaging
• Tomography, X-Ray Computed
• Treatment Outcome