Biliary atresia is a common disease that poses a serious health risk to newborns, and 95% of patients are “untreatable”. Since the introduction of the Kasai procedure, which originated in Japan, cases that were previously “untreatable” have become treatable, changing the previous situation of 97.5% mortality rate. Currently, Kasai surgery is the mainstay of treatment, and liver transplantation is also a major treatment option, but the high cost and stringent requirements for the procedure, as well as the problems associated with the long-term use of immunosuppressive drugs, have severely limited its use. Previous surgeries and treatments of an exploratory nature have been eliminated. In the past 5 years, the Department of Pediatric Surgery of the First Affiliated Hospital of Zhengzhou University has achieved good results with an overall efficiency of more than 90%, but the number of children who can survive for a long time (no death due to the development of the disease) is about 50%, and the number of children who can survive well (all physiological indicators are normal or basically normal and do not affect the quality of survival, such as no liver sclerosis, normal liver function, etc.) is about 1/3. The outcome of the child is related to many factors, mainly to the time of consultation (better results before 12 weeks), the rate of hepatic steatosis, the level of surgery, and the complications after surgery (mainly postoperative cholangitis). The problem of post-operative cholangitis is another major issue that affects the overall outcome. Addressing this problem can greatly improve the cure rate. Without surgical treatment, only 1% of biliary atresia survives to 4 years of age. But undergoing surgery also requires a great deal of commitment and has far-reaching consequences for both the infant and the family. Undergoing surgery can undoubtedly prolong survival. Long-term survival is based on: (1) surgery before 10-12 weeks of life; (2) a large bile duct (>150 μm) in the hilar region; and (3) a blood bilirubin concentration <8.8 mg/dl 3 months after surgery. For many years, it was believed that Kasai surgery for biliary atresia could be the first treatment option for biliary atresia and, if necessary, liver transplantation could be performed after the infant had grown to achieve a permanent cure. Biliary atresia occurs as a result of progressive fibrosis of the common hepatic duct leading to obstruction of the biliary system. Most biliary atresia manifests clinically after the first few weeks of life, also after extrahepatic biliary tract infection and fibrosis. It is rarely found in the postnatal or intrauterine period. Although specific viral infections have been reported, the true source of infection has not been identified. Neonatal hepatitis syndrome (cytomegalic hepatitis) is usually idiopathic and the infection is caused by cytomegalovirus, hepatitis B virus, and deficiency of alpha-antitrypsin. Biliary atresia and neonatal hepatitis syndrome represent a persistent pathological process more than a specific pathology. Both diseases usually present in the first 2 weeks of life with hyperbilirubin jaundice, white clay stools, and hepatomegaly. There is no clear conclusion on the cause of the disease, but it was early thought to be a congenital abnormality of bile duct development, related to the arrest or disturbance of the development of the biliary system in the 4th to 10th week of embryonic life. However, autopsies of the biliary system in a large number of aborted or premature infants have not revealed biliary atresia, and instead recent studies have provided more evidence to support an acquired form of the disease. The fact that some of these children were born with normal yellow stools and only developed grayish stools and jaundice a few weeks later also suggests that biliary obstruction occurred after birth in these children. In addition, pathological examination revealed inflammatory changes in the liver tissue with inflammatory cell infiltration around the hilum and bile ducts, microscopic foci of pus or limited necrosis in the hepatic lobules, and granulation tissue formation at the bile duct occlusion. A comparative pathological study of extrahepatic biliary atresia and de novo hepatitis revealed similar hepatic histopathology, differing only in degree. Extrahepatic biliary atresia is characterized by biliary thrombosis and inflammatory lesions, whereas hepatocellular necrosis is more prominent in infantile hepatitis. Therefore, it is now thought that biliary atresia may be an acquired disease with a similar pathological process to that of infantile hepatitis. Biliary atresia seen after birth is the final stage and outcome of an inflammatory process that results in fibrous scarring and occlusion of the bile ducts due to inflammatory destruction. The cause of inflammation is mainly viral infection, such as hepatitis B virus, cytomegalovirus, but also rubella virus, hepatitis A virus or herpes virus. It has been proposed that abnormalities in the confluence site of the pancreaticobiliary ducts may also be a congenital factor in the occurrence of biliary atresia. Although the etiology of this disease is multifactorial, the end result is obstruction of the biliary excretory pathway and the development of obstructive jaundice. Recent studies have shown that the development of the intrahepatic and extrahepatic bile ducts are of two sources, thus explaining the fact that the ducts below the gallbladder can be patent in biliary atresia, while the ductal lumen above the hepatobiliary duct is fibrotic resulting in atresia.