Treatment for Biliary Atresia

How do doctors treat biliary atresia?

Doctors treat biliary atresia with a surgery called the Kasai procedure and eventually, in most cases, a liver transplant. Thanks to advances in treatment, more than 80 to 90 percent of infants with biliary atresia survive to adulthood.5,6

The Kasai procedure

The Kasai procedure is usually the first treatment for biliary atresia. The Kasai procedure does not cure biliary atresia. However, if the procedure is successful, it may slow liver damage and delay or prevent complications and the need for a liver transplant. The earlier the procedure is done, the more effective it may be.

During the procedure, a surgeon removes the damaged bile ducts outside the liver. The surgeon uses a loop of the infant’s own small intestine to replace the damaged bile ducts. If the surgery is successful, bile will flow directly from the liver to the small intestine. Within 3 months of the procedure, one has an idea of whether the surgery has worked or not. After a successful surgery, most infants no longer have jaundice and have a reduced risk of developing complications of advancing liver disease.

Complications. After the procedure, a common complication is infection of the liver, called cholangitis. Doctors may prescribe antibiotics after surgery to help prevent this infection. If cholangitis occurs, doctors treat it with antibiotics, usually intravenous (IV) antibiotics given in the hospital.

If the procedure is not successful, the flow of bile will remain blocked. After an unsuccessful procedure, infants will develop complications of biliary atresia and will usually need a liver transplant by age 2.5

Even after a successful surgery, most children will slowly develop complications of biliary atresia, over years or decades, and will eventually need a liver transplant. In some cases, after a successful procedure, children never need a liver transplant.

Liver transplant

If biliary atresia leads to serious complications, the infant or child will need a liver transplant. A liver transplant is surgery to remove a diseased or injured liver and replace it with a healthy liver from another person, called a donor.

Most children with biliary atresia eventually need a liver transplant, even after a successful Kasai procedure.

References

September 2017
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This content is provided as a service of the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), part of the National Institutes of Health. The NIDDK translates and disseminates research findings through its clearinghouses and education programs to increase knowledge and understanding about health and disease among patients, health professionals, and the public. Content produced by the NIDDK is carefully reviewed by NIDDK scientists and other experts.

The NIDDK would like to thank:
Benjamin L. Shneider, M.D., Baylor College of Medicine, Texas Children’s Hospital