KidsMD Health Topics

Biliary atresia

  • Bile is an important fluid produced by your child’s liver and stored in her gallbladder. In a healthy system, it flows through a tube called the common bile duct to the small intestine, where it helps to digest food.

    If your child has biliary atresia, her common bile duct is blocked or damaged, so that it’s impossible for bile to flow through it, just as it’s hard for water to pass through a clogged pipe. This is called “cholestasis,” or poor bile flow, and quickly leads to malnutrition and liver damage.

    Biliary atresia is a chronic, progressive liver problem, and fatal if left untreated. This condition:

    • Is the most common cause of chronic liver disease in newborns
    • occurs once in every 30,000 births
    • is the most common reason for liver transplants in children
    •  affects children of all races and ethnicities, but may be more common in Asians

     

    The Boston Children’s Hospital approach

    At Boston Children’s, biliary atresia is treated by a small number of doctors with specific expertise within the large, multidisciplinary Divisions of  Gastroenterology and  Pediatric Surgery. We’re known for our individualized and science-driven approach. We’re home to the most extensive research enterprise located in a pediatric hospital in the world, and we partner with a number of top biotech and health care organizations—but our physicians never forget that your child is a child, and not just a patient.

    Children’s is also home to New England’s largest pediatric Liver Transplant Program. If your child has biliary atresia, our team will work with you from the beginning to identify the best treatment options for each stage of her condition. Call 617-355-6058 for an appointment.

  • Why is biliary atresia a concern?

    There are two main reasons why biliary atresia is a problem:

    1. It makes it harder for bile to get to your child’s small intestines. Without enough bile in the small intestines, two problems happen:

    • It’s harder for your child’s body to digest fat. This is a concern because:
      • Fat is a major sources of calories (energy) for children.
         
      • Some vitamins, such as A, D, E and K, need to bond with fat, or else they can’t be absorbed.
         
      • Fat plays a vital role in maintaining healthy skin and hair, insulating body organs against shock, maintaining body temperature and promoting healthy cell function.
         
      • Not only is it harder for your child’s body to get the energy she needs, living with biliary atresia – or any chronic condition – means that she needs more energy to begin with, putting her at even more risk of malnutrition.
         
    • It’s harder for your child’s body to get rid of certain toxins that need bile to help them exit the body.

    2. It causes liver damage. This may happen in two ways:

    • As the biliary atresia progresses, inflammation and scarring damage the liver more and more, eventually resulting in cirrhosis of the liver.
       
    • If bile can’t leave the liver, it accumulates. This can result in a buildup of toxins that causes even more liver damage and raises your child’s cholesterol level.

    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.

    Causes

    What causes biliary atresia? 

    We know that biliary atresia is caused by inflammation and scarring of the bile ducts, but what causes the inflammation and scarring is still unknown.

    Biliary atresia does not seem to be linked to anything the mother did during pregnancy, such as any medications she might have taken.

    There are two types of biliary atresia:

    1. embryonal biliary atresia

    • probably present at birth
    • signs and symptoms appear earlier
    • often associated with other malformations, including:
      • intestinal malrotation (the intestine is not twisted in the usual position within the abdomen)
      • polysplenia (multiple spleens)
      • abnormal veins
      • congenital heart defects

    2. perinatal biliary atresia

    • 85 percent of biliary atresia cases are thought to develop after birth, within the first weeks of life.
    • Some researchers believe this type may be caused by exposure to infections or toxic substances.

    Signs and symptoms

    What are the symptoms of biliary atresia? 

    Infants with biliary atresia may be born with jaundice, but usually appear healthy at birth. Most often, you or your child’s pediatrician will notice signs within the first two weeks to two months of your child’s life. These may include:

    • jaundice
    • dark urine
    • light-colored stools

    Signs that may appear later include:

    • distended (swollen) abdomen
    • weight loss

    These are later signs, not usually in the first months of life.

    Long-term outlook

    What’s the long-term outlook for my child?

    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.

    FAQ

    Q: My baby was born jaundiced. Should I be concerned?

    A: Not necessarily – many babies have jaundice (yellow discoloration of the skin and whites of the eyes) in the first week or two of life. This is a different kind of jaundice from biliary atresia, and does not mean there is a liver problem.

    But if the jaundice goes away and then returns, or lasts longer than 2 weeks, that's often a sign that something is wrong, and you should talk to your child’s pediatrician.

    Q: What’s the long-term outlook if my child has biliary atresia?

    A: Unfortunately, there are no medications that can unblock your child’s bile ducts or encourage new ducts to grow. Early surgery (Kasai portoenterostomy) and good medical care can help your child to live longer and have a better quality of life. If your child’s liver starts to fail, a liver transplant can be life saving.

    Up to 66 percent of children with biliary atresia, even those who undergo the Kasai portoenterostomy, will eventually need to have a liver transplant.

    Q: Can biliary atresia be detected before birth?

    A: No, there is no prenatal test that can detect biliary atresia.

    Q: If my child has biliary atresia, does that mean that I’m more likely to have another child with the condition?

    A: No, researchers believe that biliary atresia isn’t genetic, and the condition doesn’t recur in families with any frequency. Even in identical twins, one infant may have biliary atresia and the other may not.

    Q: What’s the difference between bile and bilirubin?

    A: Bile is a golden yellow fluid made by your child’s liver. It’s stored in the gallbladder and passed through the common bile duct into the intestine, where it helps digest fat.

    Bilirubin is a yellowish pigment that is found in bile. It is a chemical formed by breakdown of red blood cells.

    Q: What causes jaundice?

    A: Jaundice occurs when there is too much bilirubin in your child’s blood (hyperbilirubinemia). Because bilirubin is yellow, it causes a yellowing of the baby's skin and tissues.

    Not all yellowing of the skin is jaundice, however. Some babies eat a lot of orange vegetables, such as carrots, and their skin may appear yellow or orange. In that case, the whites of the child’s eyes remain white, and blood tests show that the child’s bilirubin levels are normal.

    Q: Why would there be too much bilirubin in my child’s blood?

    A: The problem starts as your child’s old red blood cells are broken down. As this occurs, hemoglobin (the protein molecule in red blood cells) is changed into bilirubin and, in a healthy system, is removed by the liver. If the liver can’t remove the bilirubin, it gets backed up in the blood.

     

  • The first step in treating your child is forming an accurate and complete diagnosis. If your child has biliary atresia, it’s important that she be diagnosed quickly, since surgery must be performed early in order to be effective.

    Usually, the diagnostic process begins when you or your child’s pediatrician notices that your child appears jaundiced. The doctor will do one or more blood tests to determine whether it may be because of a liver problem.

    These blood tests may include:

    • bilirubin level test
    • liver enzymes and bile duct enzymes tests
    • blood cultures – can show bacterial infections that may affect the liver
    • viral tests – some viruses can cause liver damage
    • tests for some metabolic diseases (such as enzyme deficiencies) that can cause early jaundice and liver test abnormalities
    • tests for cystic fibrosis, such as a sweat test

    The doctor may also want to check your child’s urine and stool.

    He or she may also order diagnostic tests to view your child’s biliary system, including:

    • abdominal ultrasound to see if there is a normal gallbladder or abnormalities that may explain the jaundice
       
    • biliary excretion or “HIDA” scan – a special chemical is injected into your baby’s vein, then pictures are taken to see if it is excreted normally by the liver, just as bile would be.

    Finally, if the diagnosis is still not definitive, we may need to perform a diagnostic surgical procedure. These may include:

    • liver biopsy – a tissue sample is taken from your child's liver and examined for abnormalities
       
    • operative cholangiogram – a small incision is made in your child’s abdomen, and a contrast agent is injected into her bile duct. This agent can be seen on a special monitor, helping surgeons to see any blockages in the bile ducts.

    After we complete all necessary tests, our experts meet to review and discuss what they have learned about your child's condition. Then we’ll meet with you and your family to discuss the results and outline the best treatment options.


  • 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 Boston Children's Hospital, 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.

  • Boston Children’s Hospital is home to the world’s most extensive research enterprise at a pediatric hospital.

    We also have many partnerships with the top research, biotech and health care organizations, and we work together to find innovative ways to improve kids’ health.

    Pioneering research

    Faculty members of Boston Children's Liver Transplant Program are conducting clinical studies to better understand why children’s livers fail and how we can improve liver transplants. We are constantly striving to translate our research findings into tangible treatments.

    We’re also researching non-invasive tests to measure liver scarring in children with chronic liver disease, with the hope that these tests can help prevent the need for liver biopsies or other invasive tests.

    Research by Maureen M. Jonas, MD, medical director of the Liver Transplant Program, has focused on liver disease in children with emphasis on viral hepatitis. Her recent work includes investigations into the treatment of hepatitis B and C viruses in children.

    For more information about our research, click here.

    Biliary atresia: Reviewed by Maureen Jonas, MD
    © Boston Children’s Hospital, 2010

  • Emily's story

    Emily was born with a condition called biliary atresia. The bile ducts that were supposed to drain bile from her liver were closed. At two months old, she had a surgery called a “Kasai” where they took a loop of her intestine and attached it to the liver to attempt to drain bile. This procedure is only successful about a third of the time and in Emily's case it was not. Of course, I did not want to hear that, so I would just say things like, “nah, it takes time,” while inside I knew that she would eventually require a liver transplant. I just couldn't believe it. This was like the kind of thing you read about, not something that could happen to you or someone you love, right?

    We were referred to Children's in March 2009. Emily was six months old at that time. She was extremely jaundiced by this point. The whites of her eyes were so yellow that they almost looked green. She was seen by Dr. Heung Bae Kim and the transplant team and Dr. Maureen Jonas and the GI team. The staff was great. The team made sure we understood everything that was going on as well as what lay ahead.

    By Emily's second visit they gave us “the beeper” so they could get in touch with us anytime. She was officially on the list for a liver on April 6, 2009, and had her transplant 18 days later. Those days were so tough and I realize now that she was very lucky because there are children who wait months, even years. As awful as it may sound, we knew we were waiting for someone to die so that our baby might live. The day we got the call — aside from the worry we felt — we felt awful that on the other end of this miracle someone had just suffered such tragedy.

    Other than that, the morning we received the call could not have been more perfect. We received a call from Dr. Kim at 6:18 a.m. I worked third shift at the time and had just had a night off so I was home. Daddy hadn't even gotten up for work yet. And big brother Christian was on school vacation so we were all home. Perfect timing for a beautiful day.

    Emily went into surgery around two p.m. and was out around 9:30 p.m. We were finally able to see her at about midnight. The transplant went well and she went home the day after Mother's Day.

    I have absolutely nothing but good things to say about Boston Children’s Hospital, from the doctors to the directions to the hospital that were sent in our initial packet. The nurses in the Intensive Care Unit were great even though we were only there for two days. The nurses on the transplant inpatient unit were just so knowledgeable and caring. Great patient care as well as a little something extra: It wasn't just a job/daily grind to them. They truly seemed to love what they do. Such simple things can really make someone's day and when your child is in the hospital, it is a very trying, stressful time.

    One nurse, Crystal, told us to leave for a little while and get out. Little did I know they were planning something for us while we were out. When we came back, Crystal had made a Mother's Day card for me from Emily, with her footprints as flowers. Katie would time vitals around Emily's schedule as best as she could at night so she wouldn't constantly get woken up. All the nurses were great. Before Emily was discharged, they made sure we knew how to pour her medications, signs to look for in the event of a complication, phone numbers we needed to know, etc. 

    Today, Emily continues to do well. She is walking all over now and is continuing to meet her milestones on time. I'm very happy to know that we have one of the greatest hospitals in our country right in our backyard.

    - Jessica Raposo

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