Antenatal diagnosis of biliary atresia: A narrative review

Abstract

Biliary atresia (BA) is a rare obliterative disease of the extra- and intrahepatic biliary tree that represents the leading indication for liver transplantation in childhood, accounting for about 50% of transplants in children and about 10% of transplants at any age. This disease usually presents as persistent jaundice and acholic stools beyond the first few weeks of life. Nevertheless, 10-15% of patients present a developmental and truly innate disease in origin, showing either congenital anomalies (such as those in BA splenic malformation syndrome, BASMs) or cystic dilatation of the extrahepatic biliary tract that takes origin in early embryogenic life. The evidence on antenatal diagnosis of BA is poor and only few case reports and case series reported the impact of prenatal assessment on post-surgical outcome. Here we provide a narrative review of antenatal diagnosis of BA, focusing on the ultrasonographic findings of non-visualization of the gallbladder (GB), cystic dilatation of the extrahepatic biliary tract and congenital anomalies associated with BA. Antenatal suspicion of BA can target the post-natal assessment of a cholestatic neonate, promoting centralization of the family in order to avoid diagnostic and surgical delay, as the early diagnosis of BA is associated with a better outcome after portoenterostomy.