title: Biliary atresia in England and Wales: results of centralization and new benchmark.
abstract: BACKGROUND
  Biliary atresia (BA) is a rare, potentially life-threatening condition of the newborn presenting with conjugated jaundice. Typically, it is treated by an initial attempt to restore bile flow (the Kasai portoenterostomy [KP]) as soon as possible after diagnosis and, if this fails, liver transplantation. Since 1999, the treatment of BA has been centralized to 3 centers in England and Wales able to offer both treatment options. The aim of this study was to review the outcome of this policy change and provide a national benchmark.
  
  
  METHODS
  The management of all infants born within England and Wales during the period January 1999 to December 2009 was assessed using 3 key performance indicators such as median time to KP, percentage clearance of jaundice (≤20 mol/L) post-KP, and 5- and 10-year native liver and true survival estimates. Data are quoted as median (range), and P < .05 was considered significant.
  
  
  RESULTS
  A total of 443 infants had confirmed BA; and of these, most were isolated BA (n = 359), with 84 having other significant anomalies (but predominantly BA splenic malformation syndrome). Four infants died before any biliary intervention. Kasai portoenterostomy was performed in 424 infants (median age, 54 [range 7-209] days), and a primary liver transplant was performed in 15. Clearance of jaundice post-KP was achieved in 232 (55%). There were 41 deaths, including 4 (10%) without any intervention, 24 (58%) post-KP usually because of end-stage liver disease and mostly on a transplant waiting list, and 13 (32%) post-LT usually because of multiorgan failure. Overall, the 5- and 10-year native liver survival estimates were 46% (95% confidence interval [CI], 41-51) and 40% (95% CI, 34-46), respectively. The 5- and 10-year true patient survival estimates were 90% (95% CI, 88-93) and 89% (95% CI, 86-93), respectively. Outcome was worse for those with other anomalies (lower clearance of jaundice post-KP [43% vs 57%; odds ratio, 1.7; 95% CI, 1.04-2.8]; P = .02) and an increased mortality overall (eg, at 5 years, 72 [95% CI, 64-83] vs 94 [95% CI, 91-96]; χ(2) = 33; P < .0001).
  
  
  CONCLUSIONS
  National outcome measures in BA appear better than those from previously published series from comparable countries and may be attributed to centralization of surgical and medical resources.
authors.affiliation: Paediatric Liver Centre, Kings College Hospital, SE5 9RS London, UK. markdav2@ntlworld.com
authors.forename: Mark
authors.initials: M
authors.lastname: Davenport
authors.forename: Evelyn
authors.initials: E
authors.lastname: Ong
authors.forename: Khalid
authors.initials: K
authors.lastname: Sharif
authors.forename: Naved
authors.initials: N
authors.lastname: Alizai
authors.forename: Patricia
authors.initials: P
authors.lastname: McClean
authors.forename: Nedim
authors.initials: N
authors.lastname: Hadzic
authors.forename: Deirdre A
authors.initials: DA
authors.lastname: Kelly
mesh_terms: D019985:Benchmarking; D001656:Biliary Atresia / Q000601:surgery*; D004739:England; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D016031:Liver Transplantation / Q000592:standards; D011171:Portoenterostomy, Hepatic / Q000592:standards; D011446:Prospective Studies; D014852:Wales