Biliary Atresia | Treatments

What are the treatment options for biliary atresia?

While there's no medical cure for biliary atresia, two different operations can allow your child to live longer and have a better quality of life – a Kasai portoenterostomy and a liver transplant.

Kasai portoenterostomy

During a Kasai portoenterostomy, your child's surgeon replaces her damaged bile ducts with a piece of her intestine. This allows bile to drain directly from the small bile ducts at the edge of your child's liver straight into her intestine. Following the surgery, your child may stay in the hospital between seven and 10 days. If done within the first two to three months of your child's life, a Kasai portoenterostomy can allow her to grow and remain in fairly good health for several years.

In about 10 to 20 percent of cases, even after a successful portoenterostomy, jaundice may not go away, bile won't flow, and these children will need a liver transplant usually by the age of 2.

Along with the Kasai portoenterostomy, your child's doctors will do a liver biopsy to determine the extent of your child's liver damage. It may also help predict how her condition will progress in the coming months.

After surgery, your child will need ongoing medical care and monitoring, including tests. Your child's doctor can explain to you what the tests mean, and what the signs are that your baby's liver is doing well or getting worse. The doctor will also talk to you about the complications of biliary atresia that may require a liver transplant.

Liver transplant

A liver transplant is an operation in which the transplant surgeon removes your child's damaged liver and replaces it with a new liver.

The new liver can be the whole liver from a deceased donor, or a segment from a deceased donor or a living family member (if he or she is a suitable candidate). In the case of a living donor, the donor's liver will grow back to more or less its full size just a few weeks after the operation.

After surgery, the new liver begins functioning and the child's health often improves quickly, although transplants carry complications of their own.

Nutrition and biliary atresia

Children with biliary atresia have special nutritional needs. Our registered pediatric dieticians can make recommendations including:

  • feeding with special formulas that contain a kind of fat that’s more easily digestible
  • adding specific vitamins to your child's diet, as directed by your child's physician
  • adding MCT (medium-chain triglyceride) oil for extra calories to the diet. MCTs are more easily digested without bile than other types of fats. MCT oil can be added to foods and liquids that your child eats.

If your baby can’t take in the amount of calories needed to maintain a normal growth rate, we may recommend liquid feedings. These are given through a tube called a nasogastric tube (NG) that is guided into the nose, down the esophagus and into her stomach.

What is the long-term outlook for biliary atresia?

While there’s no cure for the condition, there are ways for your child to have a longer and healthier life. The first step is surgery, called the Kasai portoenterostomy, in which the blocked bile duct is bypassed using a piece of your child’s intestine. Afterwards, special formulas and vitamin preparations, as well as other medications, can help your child grow and help to preserve her liver function.

Nonetheless, many children who have Kasai portoenterostomies early in life will need a liver transplant at some point.

Current national statistics confirm that about 85 percent of children who have liver transplants survive the first year, and most children who survive the first year go on to live into adulthood.