Outcome for biliary atresia patients treated at a low-volume centre

Abstract

Objectives: The importance of case load for treatment of extrahepatic biliary atresia (BA) is debated. The aim of this study was to register results of BA treatment in a small volume centre. Methods: Retrospective chart review study of patients with BA treated from 2000 to 2017. The institutional review board approved the study. Results: 45 babies were identified of which 42 (93%) are alive. 41 patients had a Kasai portoenterostomy (PE), two a hepaticojejunostomy and two a primary liver transplantation. The age at PE/hepaticojejunostomy was median 63 (4-145 days). Seven surgeons performed the operations, and the median duration of the diagnostic work-up was 8 (3-24) days. Clearance of jaundice was achieved in 23/43 (53%) babies, and 3- and 5-year native liver survival was 47% and 40% respectively. Clearance of jaundice post PE/hepaticojejunostomy was a strong predictor of native liver survival (adjusted OR: 0.027; 95%; P=0.009). Plasma level of conjugated bilirubin at time of referral was also a significant predictor of native liver survival (adjusted OR: 1.053; P=0.017). Conclusion: A small volume centre may achieve satisfactory results for BA patients. The study has, however, identified factors that may further improve results; earlier referral, optimizing diagnostic work-up, and establishing one dedicated surgical team.