The role of liver transplantation in patients with Caroli's disease.
Caroli's disease, characterized by segmental or diffuse dilation of the intrahepatic biliary ducts, is a rare disease which is difficult to treat. The course of the disorder is characterized by recurrent episodes of cholangitis and hospital stays, with a consequent loss of quality-of-life and productive capacity, often ending in death due to uncontrolled infection. Endoscopic drainage of the bile duct, percutaneously or surgically, is palliative, and presents bad results in the follow-up of these patients. Orthotopic liver transplantation appears to be an effective curative option for the treatment of patients with Caroli's disease associated to complications. The authors present the course of two cases of this disease, associated with congenital fibrosis of the liver worsened by repeated episodes of cholangitis, submitted to orthotopic liver transplantation.
Waechter
F L
FL
Liver Transplantation Program, Surgery and Gastroenterology Department, Hospital de Clínicas de Porto Alegre, Universidade Federal do Rio Grande do Sul, Brasil. waechter@zaz.com.br
Sampaio
J A
JA
Pinto
R D
RD
Alvares-da-Silva
M R
MR
Cardoso
F G
FG
Francisconi
C
C
Pereira-Lima
L
L
D000328:Adult; D016767:Caroli Disease / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery*; D002769:Cholelithiasis / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery; D005500:Follow-Up Studies; D006801:Humans; D008099:Liver / Q000473:pathology; D008103:Liver Cirrhosis / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery; D016031:Liver Transplantation; D008297:Male; D011788:Quality of Life; D014057:Tomography, X-Ray Computed