HOME
Return to Table of Contents
BILIARY ATRESIA IN CANADA: THE IMPACT OF CENTRE CASELOAD EXPERIENCE ON OUTCOME
R Schreiber, C Barker, EA Roberts, SR Martin, the Canadian Pediatric Hepatology Research Group
INTRODUCTION: Biliary atresia (BA) is a uniquely pediatric liver disease characterized by a progressive fibrosclerosing obliteration of large bile ducts. The current standards of care for BA are surgical and sequential with an initial Kasai hepato-portoenterostomy (HPE) in the first months of life followed by liver transplantation (LT) for those cases with end stage liver disease. Recent European studies have suggested that those centres managing a limited caseload per year demonstrate significantly worse outcomes. The aim of this study is to determine the role of centre experience on the outcome of Canadian children with BA.
METHODS: A retrospective review of a Canadian database of BA patients born between Jan 1,1992 and December 31, 2002 who were followed at one of 12 Canadian university based pediatric hospitals. The care centres were categorized into small, medium or large respectively, if <1, 1-3, or >3 HPE operations were performed annually. Outcome measures were analyzed using T or Chi square tests and Kaplan-Meier analysis.
RESULTS: 230 patients were identified. Of the study centres, 6 were small, 4 medium and 2 large, managing a total of 36, 74 and 120 cases respectively. There was no difference in the rate of HPE among the centres (mean=90%). The median age at Kasai was significantly lower in the medium sized centres (56 days) compared with the others (66 days) (p=0.05). The overall 4 yr patient survival was 84% with the 4 yr rates being 81%, 88% and 82% in the small, medium and large centres respectively (NS).The 4 yr post HPE native liver survival was 39% without any significant difference between the centres. Of the patients undergoing liver transplantation, 4 yr survival rates between the centres were comparable, with an overall 84% 4 yr survival.
CONCLUSION: In Canada, BA patient outcomes appear not to be influenced by centre caseload experience. These results contrast the findings in other countries, but they may be explained in part by our national policies for universal access to health care as well as the quasi-centralized pediatric tertiary system of care already existing in Canada. Outcomes for Canadian children with BA are comparable to those in Europe and elsewhere.