Biliary atresia liver histopathological determinants of early post-Kasai outcome

J Pediatr Surg. 2021 Jul;56(7):1169-1173. doi: 10.1016/j.jpedsurg.2021.03.039. Epub 2021 Mar 26.

Abstract

Background: A retrospective chart review of liver histologies in Kasai biliary atresia BA patients operated 1/2017- 7/2019 at our institution was conducted to identify histologic prognostic factors for biliary outcome.

Methods: Patients with wedge liver biopsies and portal plate biopsies (n = 85) were categorized into unfavorable and favorable outcome, based on a 3-month serum total bilirubin level of <34 μM or mortality. Hepatocellular histologies, presence of ductal plate malformation (DPM) and of large bile duct of ≥ 150 μm diameter size at the portal plate were evaluated.

Results: Total Bilirubin levels> 34 μM correlates with worse 1-year survival. Age at surgery, histologic fibrosis or inflammation does not predict outcome. Potential adverse predictors are severe hepatocellular swelling, severe cholestasis, presence of DPM (n = 24), and portal plate bile duct size < 150 µm (n = 28). In multivariate analyses adjusting for age at Kasai and postop cholangitis, bile duct size and severe hepatocellular swelling remain independent histologic prognosticators (OR 3.25, p = 0.039 and OR 3.26, p = 0.006 respectively), but not DPM.

Conclusion: Advanced histologic findings of portal plate bile duct size of <150 µm and severe hepatocellular damage predict poor post-Kasai jaundice clearance and short-term survival outcome, irrespective of Kasai timing.

Level of evidence: Level III.

Keywords: Bile duct size; Biliary atresia; Cholestasis; Ductal plate malformation; Kasai procedure; Prognostic factor.

MeSH terms

  • Biliary Atresia* / surgery
  • Cholestasis*
  • Humans
  • Infant
  • Liver / surgery
  • Portoenterostomy, Hepatic
  • Retrospective Studies
  • Treatment Outcome