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.