Biliary atresia

It’s entirely natural that you might be concerned right now about your child’s health; a diagnosis of biliary atresia can be frightening. But you can rest assured that at Children’s Hospital Boston, your child is in good hands. Our physicians are bright, compassionate and committed to focusing on the whole child, not just his condition—that’s one reason we’re frequently ranked as a top pediatric hospital in the United States.

After the diagnosis is made, your child’s team will work with you to optimize his health, including making a plan to foster nutrition and growth and give him regular liver tests to monitor the progression of the disease.

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.

Coping and support

We understand that a diagnosis of biliary atresia can be frightening for your whole family. From your first visit, you’ll work with a team of professionals who are committed to supporting you.

Patient education: Our nurses will be on hand to walk you through your child’s treatment and help answer any questions you may have —What symptoms might my child have? What do we do next? They will also reach out to you by phone, continuing the care and support you received while at Children’s.

Parent to parent: Want to talk with someone whose child has been treated for biliary atresia? We may be able to put you in touch with other families who can share their experience.

Faith-based support: If you are in need of spiritual support, we will help connect you with the Children’s chaplaincy. Our program includes nearly a dozen clergy representing Episcopal, Jewish, Lutheran, Muslim, Roman Catholic, Unitarian and United Church of Christ traditions who will listen to you, pray with you and help you observe your own faith practices during your hospital experience.

Social work and mental health professionals: Our social workers and mental health clinicians have helped many other families in your situation. We can offer counseling and assistance with issues such as coping with your child’s diagnosis, stresses relating to coping with illness and dealing with financial difficulties.

On our For Patients and Families site, you can read all you need to know about:

• getting to Children’s
• accommodations
• navigating the hospital experience
• resources that are available for your family