Congenital bile duct atresia presenting with the 1st 2-3 weeks of life; marked by progressive jaundice, hepatosplenomegaly, and liver failure. Condition is caused by canalization of hepatic ducts (intrahepatic and/or extrahepatic); it is possibly related to intrauterine infection with Listeria monocytogenes. Treatment is Kasai procedure and (in liver failure) transplantation.