Role of Liver Biopsy in Biliary Atresia: Impact of Histological Fibrosis on Kasai Portoenterostomy Outcomes and Decision Making.

PURPOSE: This study aimed to evaluate the effect of histological fibrosis on liver biopsy at presentation on the decision-making and outcomes of Kasai portoenterostomy (KPE), as well as the subsequent timing of liver transplantation.

METHODS

A retrospective, cross-sectional study of children <13 years old with biliary atresia (BA) seen at the Royal Hospital between January 2007 and December 2021.

RESULTS

Forty children were diagnosed with BA, of whom 31 (77.5%) underwent a liver biopsy, and 26 (65.0%) underwent KPE. Thirty children (96.8%) had either complete or impending cirrhosis. Eleven children (42.3%) who underwent KPE received a liver transplantation at a median age of 26 months (interquartile range [IQR]=33-34). Four children did not undergo KPE, and seven (50.0%) of these underwent liver transplantation at a median age of 22 months (IQR=13-25). The age of liver transplantation did not differ statistically between the KPE group and the primary liver transplantation group (p=0.311). Three children (11.5%) were alive with their native liver following KPE at 5 years. The mortality rate was similar in the KPE and non-KPE groups (p=0.171).

CONCLUSION

Of the 40 children with biliary atresia, 31 had a liver biopsy, with 30 (96.8%) exhibiting either complete or impending cirrhosis. Hepatic fibrosis did not impact the decision to perform KPE. The age of liver transplantation did not differ significantly between the KPE and non-KPE groups (median ages of 26 vs. 22 months, p=0.311).