Biliary atresia: service delivery and outcomes

Biliary atresia is a complex disorder dependent on multidisciplinary management. A series of comprehensive national audits in the United Kingdom and France exposed a clear relationship between center volume and clinical outcomes. Different models were adopted in each country in an attempt to improve results. In the United Kingdom, the management of biliary atresia was centralized to three specialist units in 1999, whereas in France, a strategy of decentralized management with closer inter-unit cooperation was adopted in 1997. Both policy changes led to improved outcomes for infants with biliary atresia, but only centralization improved the overall results of Kasai portoenterostomy. Other countries have adopted alternative systems of audit based on voluntary registries, but the impact of these on clinical outcomes at a national level remains unknown. The utility of monitoring tools in assessing performance in biliary atresia, the importance of risk stratification, and the need for standardized definitions of outcome are highlighted.

Keywords: Biliary atresia, Kasai portoenterostomy, Outcomes analysis