Diseases of bile duct in children

Abstract

Several diseases originate from bile duct pathology. Despite studies on these diseases, certain etiologies of some of them still cannot be concluded. The most common disease of the bile duct in newborns is biliary atresia, whose prognosis varies according to the age of surgical correction. Other diseases such as Alagille syndrome, inspissated bile duct syndrome, and choledochal cysts are also time-sensitive because they can cause severe liver damage due to obstruction. The majority of these diseases present with cholestatic jaundice in the newborn or infant period, which is quite difficult to differentiate regarding clinical acumen and initial investigations. Intraoperative cholangiography is potentially necessary to make an accurate diagnosis, and further treatment will be performed synchronously or planned as findings suggest. This article provides a concise review of bile duct diseases, with interesting cases.

Keywords: Bile duct, Cholestasis, Biliary atresia, Biliary hypoplasia, Biliary imaging, Inspissated bile syndrome, Choledochal cyst

Core Tip: Bile duct diseases are rare conditions in children but are mostly pathologic and require timely investigations and management. Biliary atresia (BA) is a common cause of biliary cirrhosis, and affected children with delayed diagnosis require liver transplantation. Early detection of BA using stool color charts or proposed biomarkers has been integrated into the management of infants with cholestasis in many countries. Extrahepatic biliary obstruction caused by stones and choledochal cysts can be easily diagnosed by abdominal ultrasonography and treated by surgical resection. Nowadays, genetic analysis plays a vital role in many bile duct diseases, such as Alagille syndrome. In addition, advanced endoscopic management can improve treatment outcomes and is less invasive than conventional surgical therapy.