Is the Gussy procedure effective in all children with biliary atresia?

  Biliary atresia, a disease in which the bile ducts inside and outside the liver become blocked and can lead to cirrhosis or even raw liver failure. If the child is not treated within 3 months of birth, irreversible cirrhosis can occur and a liver transplant is required or the child will lose his or her life around 1 year of age. Graciectomy is currently the treatment of choice for biliary atresia, but is Graciectomy effective in all children with biliary atresia?  The cause of biliary atresia is still not clear. Some studies have shown that biliary atresia is related to cytomegalovirus infection and the immune inflammatory response of the body. There is controversy in the medical community as to whether the condition is congenital, so it is currently referred to as biliary atresia.  Can a child with biliary atresia be detected during a maternity checkup? In response to such questions, experts say that it is difficult to detect the presence of biliary atresia in a fetus through maternity testing. The fetal bile duct is very thin, only 1-2mm, and ultrasound cannot distinguish between a normal biliary structure or an atretic biliary structure, so it is difficult to determine biliary atresia through maternal examination.  The development of the surgical plan for biliary atresia is based on the typing of biliary atresia. Professor Wang introduced that biliary atresia is divided into 6 types according to the site of biliary obstruction (see the figure below), of which types I, II and III are called non-treatable, and types IV, V and VI are treatable. The difference is that types I, II and III are intrahepatic biliary atresia, while types IV, V and VI are extrahepatic biliary atresia.  If the child has extrahepatic biliary atresia (types IV, V and VI), the results are very good by removing the atretic bile duct and bile duct reconstruction surgery.  If the child has intrahepatic biliary atresia, it is best to have a Gussy procedure within 60 days. In fact, the Gracie procedure is a palliative procedure that provides only partial relief, not a cure, and is only effective in a small percentage of children with biliary atresia. In these children with biliary atresia, although there is no normal biliary structure in the liver, there are some capillary bile ducts in the liver in some children. The jaundice will subside as soon as possible. Such a child can live on his or her own liver for the rest of his or her life without the need for a liver transplant. The efficiency of this type of Gracie procedure is about 30%.  In children with biliary atresia, the jaundice will slowly subside, but the liver function indicators are not normal, indicating that the effect of the Gracie procedure is not very satisfactory in these children. For this group of children, liver transplantation is an issue that must be considered sooner or later. In the remaining about 1/3 of children with biliary atresia, the Gussy procedure is completely ineffective and the jaundice does not subside at all after surgery. For such children liver transplantation is needed early, usually 6-8 months after birth, preferably within one year of age, otherwise it is life threatening.