Developmental Abnormalities of the Bile Duct and Foregut

Extrahepatic biliary atresia, paucity of intrahepatic bile ducts, viral hepatitis, and malformations of bile ducts are the main causes of neonatal cholestasis. Biliary atresia occurs due to inflammation and destruction of extrahepatic bile duct systems in utero and during the perinatal period. Malformations of bile ducts are associated with abnormal remodeling of embryonic bile duct plate and fibrocystic lesions are developed, and they include congenital hepatic fibrosis [1], Caroli’s disease [2], microhamartoma [3], and choledochal cyst, and they carry an increased risk of bile duct carcinoma. Differing from other cystic lesions, ciliated hepatic cyst arising from embryonic foregut is detected incidentally in adults and should be differentiated from bile duct lesions [4]. Infantile and adult types of hereditary polycystic disease cause renal failure, and hepatic failure is rare [5, 6].