Biliary atresia Q&A series

  1. Apart from biliary atresia, can all other diseases that cause jaundice be cured by medication?  Many parents ask, “If we rule out biliary atresia, can we assume that our child can be cured with medication?  No, it does not. In addition to biliary atresia, there are other jaundice disorders for which effective medical treatment is lacking. For example, familial cholestatic syndrome/intrahepatic biliary dysplasia and some specific metabolic diseases, medication does not work and liver transplantation is required to cure them. In addition, some jaundice is caused by abnormal intrahepatic portal vein shunts, which cannot be cured by pharmacological treatment. Ultrasonography can visually detect such portal shunts.  2.What are the treatments for biliary atresia?  The pathogenesis of biliary atresia is the occlusion of the extrahepatic bile duct, which leads to the inability of intrahepatic bile to be secreted through the bile duct and causes a series of metabolic disorders in the intestine. To use an inappropriate analogy, the extrahepatic biliary tract is like a river, and bile is the river water. When the river is blocked, it must be opened to prevent the river water from spilling out of the riverbed. In biliary atresia surgery, the way to open the river is a hilar-intestinal anastomosis, in which the atretic bile duct (i.e., fibrous mass) is removed from the hilar part of the liver and then the intestine is anastomosed to the hilar parenchyma. Some people may ask why the bile duct is not anastomosed directly to the intestine, as is done in adults with choledo-enteric anastomosis. This is because the intrahepatic bile ducts in children with biliary atresia are also often poorly developed and too slender to make an anastomosis. This procedure was first invented by the Japanese surgeon Kasai, so it is also known as Kasai’s technique, and in Chinese it is called Kasai’s technique.  It is important to note that Kasai is a palliative surgery. The radical cure for biliary atresia is liver transplantation. In Western countries, the most common cause of liver transplantation in children is biliary atresia. In recent years, more and more liver transplants for biliary atresia have been performed in China. I have seen many parents bring their children to Tianjin, Beijing and even Taiwan for liver transplantation.  3.What is the efficacy of Geschichte (hepatic hilar anastomosis)?  There is a 1/3 theory about the efficacy of Geschi operation. In other words, 1/3 of all children who underwent Geschi’s surgery are ineffective, i.e. they cannot establish effective bile drainage and cannot slow down the progression of cirrhosis; 1/3 can establish effective bile drainage but cannot slow down the progression of cirrhosis; and 1/3 are effective, i.e. they can establish effective bile drainage and can survive with their own liver for a longer period of time.  Why is this the case? This may be related to the complexity of the etiology of biliary atresia. First, the current premise of the effective treatment with Geschi operation assumes that the intrahepatic bile duct is patent, but in fact biliary atresia can be a simultaneous occlusion of different segments of the intrahepatic and extrahepatic bile duct. Secondly, some scholars believe that the persistent inflammatory response of the biliary tract is an important cause of the progression of cirrhosis after Geschi operation.  4.How to choose the treatment?  If you have good economic conditions and can find a suitable liver source, liver transplantation is the first choice. Nowadays, the technology of liver transplantation for children is very mature and the success rate is also very high. I have seen several children who went to Beijing and Tianjin for surgery, and a large part of them had pro-liver transplantation (the mother or father donated the left outer lobe to the child), all of which were very successful.  For those who are not financially well-off enough to afford liver transplantation, it is recommended to opt for Gexi surgery. Later, when conditions are ripe, liver transplantation will be performed. Studies have shown that the more the average number of surgeries per year, the better the surgical outcome of the center with the same Gussy procedure. Therefore, the Gussy procedure should be performed in hospitals in China where this procedure is performed earlier and more often.