[Biliary atresia].

Extrahepatic biliary atresia (EHBA) is an inflammatory fibrosing process affecting the extrahepatic and intrahepatic biliary tree resulting in fibrous obliteration of the extrahepatic biliary tract, ductopenia of intrahepatic bile ducts, and biliary cirrhosis. EHBA is divided in a fetal, prenatal or embryonic, and a more common, perinatal form. The symptoms of the fetal form start shortly after birth and there is frequently an association with a variety of congenital anomalies. Children with the perinatal form become jaundiced several weeks after birth; no associated congenital anomalies are present. Morphologically, an inflammatory and fibrosing process of the extrahepatic biliary tree leads to complete luminal obliteration. The liver is characterized by a nonspecific giant cell transformation, and portal expansion by fibrous connective tissue with marked ductular proliferation. The differential diagnosis with other conditions with similar microscopic patterns such as alpha-1 antitrypsin deficiency, total parental nutrition, obstruction by a choledochal cyst, arteriohepatic dysplasia, familial progressive intrahepatic cholestasis a. s. o. is discussed. Different etiologies have been postulated in the perinatal form of EHBA: genetic susceptibility, vascular factors, toxins, and infections. EHBA is a heterogenous disease, resulting from a combination of genetic factors, insults, and immunologic pathways. The treatment of EHBA is surgical, whit anastomosis between the biliary tree and the intestine in the “correctable” type and a hepatic portoenterostomy (HPE) for “noncorrectable” group. HPE is a temporizing treatment allowing the infant to develop and grow, followed in the majority of the patients by liver transplantation.