What is congenital biliary atresia? Atresia or dysplasia can occur in the intrahepatic bile duct, hepatic duct, or common bile duct, as well as atresia or dysplasia of the gallbladder. What causes congenital biliary atresia? The etiology is unknown. It is generally thought to be related to intrauterine viral infection, secondary obstruction by inflammation of the intrahepatic hepatic ducts, and congenital malformation of the biliary tract development. Atresia or dysplasia of the intrahepatic bile duct, hepatic duct, or common bile duct can occur, as can atresia or dysplasia of the gallbladder. What are the symptoms of congenital biliary atresia? Jaundice often appears 1 to 3 weeks after birth, persists, and worsens progressively. The feces may be dark green, but soon after birth, grayish white stools are passed. In severe cases, bilirubin may be leaked from the intestinal mucosa epithelium, causing the gray feces to turn light yellow in appearance. The liver is progressively enlarged and gradually becomes hard. Most of them have splenomegaly, and ascites occurs in the late stage. In the early stages, the appetite is still good and the nutritional status is mostly good; in the late stages, due to impaired absorption of fat and fat-soluble vitamins, the body is gradually weakened and vitamin A, D and K deficiency may occur, causing dry eye disease, rickets and bleeding tendency. What tests are needed for congenital biliary atresia? Laboratory tests, serum conjugated bilirubin and alkaline phosphatase are continuously increased. Normal liver function in the early stage with abnormal changes in the late stage. Urine bilirubin is positive and urinary bilirubin is negative. Nuclear 99mTc-IDA (acetanilide diacetate) imaging can be done when available. No radioactive concentration in the intestine within 6 hours of static tracer injection supports the diagnosis of the disease. How to treat? Congenital biliary atresia should be treated surgically within the first 3 months of life. Currently, the most successful surgery is the hepatic hilar jejunostomy. However, the possibility and outcome of surgery varies depending on the type of malformation, and surgery is difficult in cases of intrahepatic obstruction.