The Kasai portoenterostomy (KPE) changed the care paradigm for children afflicted with biliary atresia (BA). If successful, a KPE precludes orthotopic liver transplantation (OLTxp) in the first few years of life. Prior to the widespread availability of pediatric OLTxp, revision of a non-draining KPE represented a viable if not ideal alternative. This approach has been abandoned as the efficacy of revision KPE following an initial unsuccessful KPE was poor.1 Successful drainage rates, defined as a direct bilirubin less the 2mg/dl at 90 days post-KPE, range from 50 to 80 %. At variable times post KPE, children may experience cholangitis that can precipitate cholestasis and KPE failure. First line therapy for cholangitis includes intravenous antibiotics and steroids. If medical therapy fails, KPE revision can be considered because, without restoration of biliary drainage, chronic cholestasis will lead to end stage liver disease (ESLD), and liver transplantation will be required.2 As such, the role of KPE revision is nuanced (Table 1). Below, we provide an overview of KPE revision as well as a discussion of its utility in current treatment algorithms. Even with a successful KPE, virtually all patients afflicted with BA will eventually need liver transplantation.3
Comments (0)