Prognosis of Biliary Atresia After 2-year Survival With Native Liver:A Nationwide Cohort Analysis

OBJECTIVES: To determine the prognosis of patients with biliary atresia (BA) after two years of native liver survival (NLS) and to identify prognostic factors for continued NLS after two years of age.METHODS: We retrospectively analyzed perioperative, laboratory and outcome parameters of all BA patients in The Netherlands between January 1987-June 2015 with NLS of at least two years. We compared parameters between patients who continued to have their native liver (NLS+) to those who did not, either by transplant or death (NLS-).RESULTS: We included 100 patients. Upon a median follow-up of 16.4 years, NLS ended in 37% by liver transplantation (LTx) and in 6% by (pre-transplant) mortality. NLS rates at 5, 10, 15, 18 years of age were 89, 72, 60, 54%, respectively. Corresponding overall survival rates were 98, 90, 87, 87%, respectively. Six months post-Kasai, NLS+ patients had higher clearance of jaundice (COJ) rate, significantly lower total and direct serum bilirubin, aspartate-aminotransferase and alkaline phosphatase levels, compared with NLS- patients (each P < .05). Cox regression could only assess a significant effect of COJ on continued NLS. Main indications for LTx after the age of two were irreversible jaundice and portal hypertension.CONCLUSIONS: Eighty-seven percent of patients with two-year NLS reach adult age, and more than 50% with their native liver. However, a pre-transplant mortality of 6% exists among patients who reach the age of two years with their native livers. Early life parameters, other than COJ, did not have a significant effect on continued NLS after two years of age.