Management of Biliary Atresia in France 1986-2015: Long Term Results.

OBJECTIVES This study analyses the prognosis of Biliary Atresia (BA) in France since 1986, when both Kasai operation (KOp) and Liver Transplantation (LT) became widely available. METHODS The charts of all patients diagnosed with BA born between 1986 and 2015 and living in France were reviewed. RESULTS 1428 patients were included; 1340 (94%) underwent KOp. Total clearance of jaundice (total bilirubin ≤20 μmol/l) was documented in 516 patients (39%). Age at KOp (median 59 days, range 6-199) was stable over time. Survival with Native Liver (SNL) after KOp was 41%, 35%, 26% and 22% at 5, 10, 20 and 30 years, stable in the 4 cohorts. 25-year SNL was 38%, 27%, 22%, 19% in patients operated in the 1, 2, 3 month of life or later, respectively (p = 0.0001). Center caseloads had a significant impact on results in the 1986-96 cohort only.16%, 7%, 7%, 8% of patients died without LT in the 4 cohorts (p = 0.0001).753 patients (55%) underwent LT. Patient survival after LT was 79% at 28 years. 5-year patient survival after LT was 76%, 91%, 88%, and 92% in cohorts 1 to 4, respectively (p < 0.0001),Actual BA patient survival (from diagnosis) was 81%. 5-year BA patient survival was 72%, 88%, 87%, 87% in cohorts 1986-96, 1997-2002, 2003-09, 2010-15, respectively (p < 0.0001). CONCLUSIONS In France, 87% of BA patients survive nowadays and 22% reach the age of 30 years without transplantation. Improvement of BA prognosis is mainly due to reduced mortality before LT and better outcomes after LT.