Biliary atresia is a rare neonatal disease of unknown etiology, where obstruction of the biliary tree causes severe cholestasis, leading to biliary cirrhosis and death in the first years of life, if the condition is left untreated. Biliary atresia is the most frequent surgical cause of cholestatic jaundice in neonates and should be evoked whenever this clinical sign is associated with pale stools and hepatomegaly. The treatment of biliary atresia is surgical and currently recommended as a sequence of, eventually, two interventions. During the first months of life a hepatoportoenterostomy (a “Kasai,” modifications of which are discussed in this paper) should be performed, in order to restore the biliary flow to the intestine and lessen further damage to the liver. If this fails and/or the disease progresses towards biliary cirrhosis and life-threatening complications, then liver transplantation is indicated, for which biliary atresia represents the most frequent pediatric indication. Of importance, the earlier the Kasai is performed, the later a liver transplantation is usually needed. This warrants a great degree of awareness of biliary atresia, and the implementation of systematic screening for this life-threatening pathology. 1. Introduction to Biliary Atresia Biliary atresia (BA) is a rare neonatal disease usually manifesting in the first months of life, when ascending obstruction of the biliary tree causes severe cholestasis and rapidly progressing biliary cirrhosis. 1.1. Classification and Anatomy BA occurs most often (~90%) isolated without the presence of other malformations, but can also be part of a syndrome [1]. Syndromic BA can be associated with various congenital anomalies such as polysplenia or asplenia (100%), situs inversus (50%), preduodenal portal vein (60%), absence of retrohepatic inferior vena cava (40%), or cardiac anomalies (50%) [2]. BA is classified on anatomical bases, referring to the level and severity of the obstruction. The more commonly used Japanese and Anglo-Saxon classification describes 3 main types. In type I, atresia is limited to the common bile duct, and the gallbladder and hepatic ducts are patent (i.e., “distal” BA). In type II, atresia affects the hepatic duct, but the proximal intrahepatic ducts are patent (i.e., “proximal” BA). Type II is subgrouped in type IIa, where a patent gallbladder and patent common bile duct are present (sometimes with a cyst in the hilum, i.e., “cystic BA”), and in type IIb, where the gallbladder as well as the cystic duct and common bile duct are also obliterated. In type III, there
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