title: Orthotopic liver transplantation in biliary atresia: a single-center experience.
abstract: BACKGROUND
  Biliary atresia (BA) is the leading indication for orthotopic liver transplantation (OLT) among children. However, there are technical difficulties, including the limited dimensions of anatomical structures, hypoplasia and/or thrombosis of the portal vein and previous portoenterostomy procedures.
  
  
  OBJECTIVE
  The objective of this study was to present our experience of 239 children with BA who underwent OLT between September 1989 and June 2010 compared with OLT performed for other causes.
  
  
  METHODS
  We performed a retrospective analysis of patient charts and analysis of complications and survival.
  
  
  RESULTS
  BA was the most common indication for OLT (207/409; 50.6%). The median age of subjects was 26 months (range, 7-192). Their median weight was 11 kg (range, 5-63) with 110 children (53.1%) weighing ≤10 kg. We performed 126 transplantations from cadaveric donors (60.8%) and 81 from living-related donors (LRD) (39.2%). Retransplantation was required for 31 recipients (14.9%), primarily due to hepatic artery thrombosis (HAT; 64.5%). Other complications included the following: portal vein thrombosis (PVT; 13.0%), biliary stenosis and/or fistula (22.2%), bowel perforation (7.0%), and posttransplantation lymphoproliferative disorder (PTLD; 5.3%). Among the cases of OLT for other causes, the median age of recipients was 81 months (range, 11-17 years), which was higher than that for children with BA. Retransplantation was required in 3.5% of these patients (P < .05), mostly due to HAT. The incidences of PVT, bowel perforation, and PTLD were significantly lower (P < .05). There was no significant difference between biliary complications in the 2 groups. The overall survival rates at 1 versus 5 years were 79.7% versus 68.1% for BA, and 81.2% versus 75.7% for other causes, respectively.
  
  
  CONCLUSIONS
  Children who undergo OLT for BA are younger than those engrafted for other causes, displaying a higher risk of complications and retransplantations.
authors.affiliation: Department of Pediatrics, Pediatric Surgery and Liver Transplantation Division, Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil.
authors.forename: A C A
authors.initials: AC
authors.lastname: Tannuri
authors.forename: N E M
authors.initials: NE
authors.lastname: Gibelli
authors.forename: L R S
authors.initials: LR
authors.lastname: Ricardi
authors.forename: M M
authors.initials: MM
authors.lastname: Silva
authors.forename: M M
authors.initials: MM
authors.lastname: Santos
authors.forename: M L
authors.initials: ML
authors.lastname: Pinho-Apezzato
authors.forename: J G
authors.initials: JG
authors.lastname: Maksoud-Filho
authors.forename: M C P
authors.initials: MC
authors.lastname: Velhote
authors.forename: A A R
authors.initials: AA
authors.lastname: Ayoub
authors.forename: W C
authors.initials: WC
authors.lastname: Andrade
authors.forename: A N
authors.initials: AN
authors.lastname: Backes
authors.forename: H T
authors.initials: HT
authors.lastname: Miyatani
authors.forename: U
authors.initials: U
authors.lastname: Tannuri
mesh_terms: D000293:Adolescent; D001656:Biliary Atresia / Q000601:surgery*; D002648:Child; D006801:Humans; D016031:Liver Transplantation; D012189:Retrospective Studies