Factors influencing jaundice-free survival with the native liver in post-portoenterostomy biliary atresia patients: results from a single institution

J Pediatr Surg. 2013 Dec;48(12):2368-72. doi: 10.1016/j.jpedsurg.2013.08.007.


Purpose: We reviewed post-portoenterostomy (PE) biliary atresia (BA) patients who became "jaundice-free" (JF; total bilirubin (T-bil) ≤1.2 mg/dL) to determine factors associated with survival with the native liver (SNL).

Methods: We reviewed 76 BA patients treated by PE at our institute between 1989, when liver transplantation (LTx) became available in Japan, and 2012, prospectively. Of these, 60 who became JF and remained JF were divided into two groups, SNL (n=44) and LTx (n=16). Age and weight at PE, pre- and post-PE T-bil, AST, γ-GT, time taken to become JF, corticosteroid requirements, incidence of cholangitis, and micro-bile duct size were compared between the two groups.

Results: The SNL patients became JF significantly earlier: 58 vs. 115 days (p<.05). Corticosteroid requirement, cholangitis, and postoperative AST/γ-GT were significantly lower in the SNL patients (p<.05). SNL was significantly higher if patients became JF ≤60 days post-PE (p<.01). LTx was performed from 0.5 to 11 years post-PE (mean=3.4). All patients who had cholangitis within 3 months of PE eventually required LTx (p<.05).

Conclusions: Becoming JF ≤60 days post-PE would appear to be a factor associated with SNL, while cholangitis within 3 months of PE would appear to be associated with LTx. Elevation of AST and γ-GT would also appear to be early indicators of risk for LTx during follow-up of JF patients after successful PE.

Keywords: Biliary atresia; Jaundice-free; Liver transplantation; Portoenterostomy; Survival with native liver.

Publication types

  • Evaluation Study

MeSH terms

  • Biliary Atresia / complications
  • Biliary Atresia / surgery*
  • Cholangitis / etiology
  • Female
  • Follow-Up Studies
  • Humans
  • Jaundice / etiology*
  • Kaplan-Meier Estimate
  • Liver Failure / etiology
  • Liver Failure / surgery
  • Liver Transplantation
  • Male
  • Portoenterostomy, Hepatic*
  • Postoperative Complications
  • Reoperation
  • Treatment Outcome