Bile lakes in patients with biliary atresia who presented with jaundice-free native liver survival indicating the risk of subsequent liver transplantation due to various factors

Pediatr Surg Int. 2024 Jul 17;40(1):197. doi: 10.1007/s00383-024-05786-x.

Abstract

Purpose: The prognostic factors of subsequent liver transplantation (LT) in patients with biliary atresia (BA) who presented with jaundice-free native liver survival were investigated.

Methods: This study retrospectively reviewed patients who underwent portoenterostomy (PE) for BA. Patients with jaundice-free native liver survival at 1 year postoperatively were divided into the autologous liver survivor and liver transplant recipient groups. Peri- and postoperative data were compared between the two groups.

Results: Among 97 patients with BA, 29 who received LT within 1 year after PE were excluded from the analysis. Further, 48 patients currently living with native liver and 20 who received LT after 1 year postoperatively were compared. Bile lake (BL) was the strongest risk factor of LT. The risk score was 2.38 B L s c o r e + 0.00466 T B A , and the area under the receiver operating characteristic curve was 0.83. Patients with BL and those without significantly differed in terms of the native liver survival rate. Patients with BL who presented with not only cholangitis but also gastrointestinal hemorrhage and hepatopulmonary syndrome received LT.

Conclusion: BL can cause different pathologies. Moreover, it is an evident risk factor of subsequent LT in patients with BA who are living with native liver at 1 year after PE.

Keywords: Bile lake; Biliary atresia; Intrahepatic cystic lesion; Liver transplantation; Risk score.

MeSH terms

  • Bile
  • Biliary Atresia* / complications
  • Biliary Atresia* / mortality
  • Biliary Atresia* / surgery
  • Child, Preschool
  • Female
  • Humans
  • Infant
  • Jaundice / etiology
  • Liver
  • Liver Transplantation*
  • Male
  • Portoenterostomy, Hepatic* / methods
  • Prognosis
  • Retrospective Studies
  • Risk Factors
  • Survival Rate / trends