The Kasai Procedure
The 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 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...