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


METHODS
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.
Divisions of Hepato-Pancreato-Biliary, Transplant and Pediatric Surgery, Department of Surgery, Kyoto University Hospital, Kyoto, Japan. horit@kuhp.kyoto-u.ac.jp
Tomohide
T
Hori
Fumitaka
F
Oike
Yasuhiro
Y
Ogura
Kohei
K
Ogawa
Koichiro
K
Hata
Yukihide
Y
Yonekawa
Mikiko
M
Ueda
Seisuke
S
Sakamoto
Mureo
M
Kasahara
Hiroto
H
Egawa
Yasutsugu
Y
Takada
Toshimi
T
Kaido
Etsuro
E
Hatano
Justin H
JH
Nguyen
Feng
F
Chen
Ann-Marie T
AM
Baine
Shinji
S
Uemoto
D000293:Adolescent; D000328:Adult; D001659:Biliary Tract / Q000002:abnormalities*; D001707:Biopsy, Needle; D016767:Caroli Disease / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D049448:Cholangiopancreatography, Magnetic Resonance; D004108:Dilatation, Pathologic; D005260:Female; D006801:Humans; D007165:Immunosuppression Therapy; D007223:Infant; D008099:Liver / Q000000981:diagnostic imaging / Q000473:pathology; D008103:Liver Cirrhosis / Q000473:pathology; D017093:Liver Failure / Q000150:complications; D016031:Liver Transplantation; D008297:Male; D011877:Radionuclide Imaging; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome