Long-term results of biliary atresia in the era of liver transplantation

Pediatr Surg Int. 2013 Dec;29(12):1297-301. doi: 10.1007/s00383-013-3366-9. Epub 2013 Aug 15.


Purpose: The purpose of this study was to analyze the survival of patients with biliary atresia (BA) after Kasai operation and liver transplantation (LT) and to analyze the factors affecting survival.

Methods: Seventy-two patients diagnosed with BA were operated on between April 1995 and December 2009 and retrospectively analyzed.

Results: Out of the 72 patients, 59 received Kasai operation and 13 received LT without prior Kasai operation. Twenty-seven patients received LT after Kasai operation. Survival with native liver was 39 % at 10 years. With the application of LT, overall 10-year survival for patients with BA was 94.9 %. Among patients alive with native livers after Kasai operation, 14 patients (58.3 %) have at least one complication associated with biliary cirrhosis and portal hypertension. Age at which Kasai operation was performed (60 days) and postoperative normalization of bilirubin were independent risk factors for survival with the native liver, according to multivariate analysis (HR 2.90, p = 0.033 and HR 9.89, p = 0.002).

Conclusions: Survival of BA patients has greatly increased in the era of LT. However, many patients surviving with native livers after Kasai operation continue to have signs of biliary cirrhosis and abnormal liver function.

MeSH terms

  • Biliary Atresia / complications
  • Biliary Atresia / mortality*
  • Biliary Atresia / surgery*
  • Female
  • Follow-Up Studies
  • Humans
  • Hypertension, Portal / complications
  • Infant
  • Liver Cirrhosis, Biliary / complications
  • Liver Transplantation / methods*
  • Liver Transplantation / statistics & numerical data
  • Male
  • Portoenterostomy, Hepatic / methods*
  • Portoenterostomy, Hepatic / mortality*
  • Portoenterostomy, Hepatic / statistics & numerical data
  • Postoperative Complications
  • Republic of Korea
  • Retrospective Studies
  • Risk Factors
  • Survival Analysis
  • Treatment Outcome