Biliary atresia

Abstract

Abstract Neonatal cholestasis syndrome includes a wide spectrum of diseases with varying clinical phenotypes all of which present with cholestasis in infancy. Biliary atresia is one of the causes of cholestasis and mandates early diagnosis and corrective surgery. Biliary atresia is also the commonest cause for liver transplantation in children. The disease predominantly affects the extrahepatic biliary tree. A sequential treatment strategy of a Kasai portoenterostomy followed by liver transplantation in unsuccessful cases has resulted in a survival rate of about 90% in these children. It is important to differentiate biliary atresia from the other diseases in the neonatal cholestasis spectrum. The diagnostic algorithms have evolved to include more non-invasive procedures with high diagnostic accuracy. The outcome of the Kasai operation depends on several factors and optimizing the outcome results in jaundice clearance in approximately half of the children. Understanding the multifactorial aetiology of biliary atresia and the consequent pathogenic pathways involved will enable the development of novel therapeutic measures which will help reduce the disease burden.