30 -Year Outcomes with Biliary Atresia

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M Fanna et al. JPGN 2019; 69: 416-24. In this retrospective 30-year study (1986-2015) from France, patients were examined in 4 time cohorts: 1986-96, 1997-2002, 2003-9, and 2010-5.

  • Age at Kasai operation remained stable throughout the study period -median 59 days.
  • Early Kasai was associated with a reduced need for liver transplantation. 25-year survival with native liver was 38%, 27%, 22%, and 19% for patients operated in first, second, third months or later respectively.
  • Clearance of jaundice (total bilirubin ≤20 micromol/L) after Kasai did not change appreciably in the time cohorts and was 38.8%.
  • 753 (of 1428 in cohort) patients underwent liver transplantation.
  • Overall survival of entire cohort was 87% (including all levels of followup).
  • Survival after LT was 79% at 28 years.
  • Five-year patient survival after LT was 76%, 91%, 88%, and 92% in the cohorts, indicating better survival more recently.
  • 22% of patients reached age 30 years without transplantation.

The authors note that better outcomes were noted in a long-term study from Japan where there are lower rates of LT needed for biliary atresia. IN Japan 20-year survival with native liver and overall patient survival was 48% and 89%, compared to 26% and 76% in France.

My take: This study indicates that the majority of patients with BA will require liver transplantation and that earlier Kasai operation is associated with a better chance of survival with native liver.  It is likely that data in the U.S. would be more similar to France than Japan based on prior publications.

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