The Outcome of a Centralization Program in Biliary Atresia: Twenty Years and Beyond

Abstract

Objective: Biliary atresia is a rare disease and reported outcomes of surgical management, typically a Kasai portoenterostomy (KPE), vary considerably across the world. Centralization has been proposed to improve this.

Background: A national centralization program was started in January 1999, involving 3 English units with colocated liver transplant facilities. As the program has now reached the 20-year point, the main aim was to update outcome statistics and identify trends.

Methods: Prospective registry and database. The main measures of outcome were (1) time to KPE, (2) clearance of jaundice (CoJ), defined as reaching a bilirubin value of <20 µmol/L (≈1.5 mg/dL), and (3) actuarial native liver survival and overall survival (OS). Data are quoted as median (interquartile range) and nonparametric statistical comparison used with P <0.05 regarded as statistically significant.

Results: A total of 867 infants were born with biliary atresia and managed between January 1999 and December 2019. Death occurred without intervention (n = 10, 1.1%) or were subject to primary transplant (n = 26, 3.0%); leaving 831 (95.9%) infants who underwent KPE at a median age of 51 (interquartile range: 39-64) days. Age at KPE reduced over the period ( P = 0.0001) becoming 48 (35-57) days in the last 5-year era. CoJ was achieved in 505/831 (60.6%), also increasing over the period ( P = 0.002). Forty-two (5.0%) died post-KPE and 384 were transplanted, leaving 405 alive with their native livers at the last follow-up. Of the 412 children transplanted, there were 23 (5.6%) deaths, leaving 387 alive. Five-year and 10-year native liver survivals were 51.3% (95% CI: 54.8-47.8) and 46.5% (95% CI: 50.1-42.9) and OSs were 91.5% (95% CI 93.2-89.4) and 90.5% (95% CI 92.3-88.2%), respectively.

Conclusions: There have been continued improvements in efficiency over the period of centralization with a significant reduction in time to KPE and improved CoJ after KPE. OS in this disease remains >90%.