title: Biliary atresia and orthotopic liver transplantation. 11 years of experience in Geneva.
abstract: Biliary atresia (BA) is a congenital malformation or an evolutive inflammatory process which, without treatment, leads to cirrhosis, hepatic failure and death within two years of birth. The literature gives a survival rate of 60% at five years and 25% to adulthood after an initial operation performed for BA. 30% of children do not survive beyond two years of age. BA has become the most frequent indication for liver transplantation (LT) in children. With LT, survival expectancy is 90%. Results of the operation designed for BA remain unsatisfactory, and seem to depend on the age of the infants, as well as on other factors such as liver histology, and centre experience. Since 1989, onset of the paediatric hepatic transplantation program in Geneva, to July 2000, 20 children have been referred for initial treatment of BA, and 26 for possible hepatic transplantation after initial treatment done in another centre. The aim of the current study is to analyse our own results of the initial operation and to present the results of liver transplantation in this particular group of patients. All the patients with a BA are included in this study. The initial operation for BA yielded 43% favourable outcome at five years and the survival in this group following LT reached 91.3% survival. The importance of the age of the patient at time of initial operation is underlined.
authors.affiliation: Clinique de chirurgie pédiatrique, Hôpitaux Universitaires de Genève.
authors.forename: C
authors.initials: C
authors.lastname: Le Coultre
authors.forename: C
authors.initials: C
authors.lastname: Battaglin
authors.forename: P
authors.initials: P
authors.lastname: Bugmann
authors.forename: B
authors.initials: B
authors.lastname: Genin
authors.forename: R
authors.initials: R
authors.lastname: Bachmann
authors.forename: V
authors.initials: V
authors.lastname: McLin
authors.forename: G
authors.initials: G
authors.lastname: Mentha
authors.forename: D
authors.initials: D
authors.lastname: Belli
mesh_terms: D001656:Biliary Atresia / Q000401:mortality / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008111:Liver Function Tests; D016031:Liver Transplantation; D008297:Male; D011183:Postoperative Complications / Q000401:mortality / Q000601:surgery; D012086:Reoperation; D015996:Survival Rate