What is the treatment of Biliary Atresia?

Surgery. If biliary atresia appears to be the cause of the jaundice in the newborn, the next step is surgery. At the time of surgery the bile ducts can be examined and the diagnosis confirmed. For this procedure, the infant is sedated. While the infant is asleep, the surgeon makes an incision in the abdomen to directly examine the liver and bile ducts. If the surgeon confirms that biliary atresia is the problem, a Kasai procedure will usually be performed on the spot. Kasai procedure (hepato-portoenterostomy). If biliary atresia is the diagnosis, the surgeon generally goes ahead and performs an operation called the “Kasai procedure,” named after the Japanese surgeon who developed this operation. In the Kasai procedure, the bile ducts are removed and a loop of intestine is brought up to replace the bile ducts and drain the liver. As a result, bile flows from the small bile ducts straight into the intestine, bypassing the need for the larger bile ducts completely. Liver transplant. If the Kasai procedure is not successful, the infant usually will need a liver transplant within the first 1 to 2 years of life. Children with the fetal form of biliary atresia are more likely to need liver transplants—and usually sooner—than infants with the typical perinatal form. The pattern of the bile ducts affected and the extent of damage can also influence how soon a child will need a liver transplant.   The Kasai ProcedureThe Kasai procedure can restore bile flow and correct many of the problems of biliary atresia. This operation is usually not a cure for the condition, although it can have an excellent outcome. Without this surgery, a child with biliary atresia is unlikely to live beyond the age of 2. The operation works best if done before the infant is 90 days old and results are usually better in younger children.The improved results of the surgery make the early diagnosis of biliary atresia very important, preferably before the infant is several months old and has suffered permanent liver damage. Some infants with biliary atresia who undergo a successful Kasai operation are restored to good health and can lead a normal life without jaundice or major liver problems. Unfortunately, the Kasai procedure is not always successful. If bile flow is not restored, the child will likely develop worsening liver disease and cirrhosis and require liver transplantation within the first 1 to 2 years of life. In addition, the Kasai operation, even when initially successful, may not totally restore normal liver development and function. A child with biliary atresia may slowly develop cirrhosis and related complications and require a liver transplant later in childhood.While the Kasai procedure has been a great advance in the management of biliary atresia, improvements in the operation and clinical management of children who undergo it are needed to improve the outcomes of children with this disease.Liver TransplantationLiver transplantation is a highly successful treatment for biliary atresia and the survival rate after surgery has increased dramatically in recent years. Children with biliary atresia are now living into adulthood, some even having children of their own. Because biliary atresia is not an inherited disease, the children of survivors of biliary atresia do not have an increased risk of having it themselves.Improvements in transplant surgery have also led to a greater availability of livers for transplantation in children with biliary atresia. In the past, only livers from small children co...