Biliary Atresia

Key points

• Biliary atresia is a rare condition in babies
• The first sign of biliary atresia is usually yellowing (jaundice) of the skin at about 2 to 6 weeks of age
• It happens when the bile ducts inside or outside the liver are blocked by scar tissue
• Bile builds up in the liver and damages it
• This stops the liver from working properly and leads to scarring and cirrhosis
• There is no cure for biliary atresia, but it can be treated with surgery or a liver transplant
• Children with biliary atresia must usually follow a special diet all their life

What is biliary atresia?

Biliary atresia is a condition that affects the bile ducts. These are tubes that that carry bile out of the liver. Bile is a liquid that the liver puts out to carry away waste and help the body digest fats and vitamins.

If your baby has biliary atresia, they may have been born with no bile ducts, or the bile ducts are blocked or inflamed.

Soon after birth, the bile starts to build up in the liver. Here the bile quickly starts to destroy liver cells. This causes scarring of the liver and cirrhosis.

What are the symptoms of biliary atresia?

The symptoms of biliary atresia normally come on around 2 to 6 weeks after birth.

The first sign is often jaundice, or yellowing of the skin and whites of the eyes. This is caused by too much of the substance bilirubin in the blood. It’s a sign the liver isn’t working properly.

Jaundice is common in newborn babies. But if it first starts between 2 and 6 weeks of age, and doesn’t clear up in 1 to 2 weeks, it might be a sign of biliary atresia.

Other signs and symptoms of biliary atresia are:

• a swollen belly
• the liver feels hard
• pale grey poos (that look like clay)
• dark yellow or brown urine
• feeding more often

How is biliary atresia diagnosed?

There are lots of conditions that can cause the symptoms above. To confirm biliary atresia, your doctor will order a range of tests. These tests are often done in a children’s hospital.

Tests your baby may need include:

• blood tests to check how well the liver is working
• urine tests
• an ultrasound to look at the shape and size of the liver
• tests to see how the bile is flowing through the liver (nuclear medicine scan or cholangiogram)
• a liver biopsy, when small piece of liver tissue is removed with a needle and then looked at under a microscope

Why does my baby have biliary atresia?

Biliary atresia is only found in newborn babies. It is very rare. Worldwide, only about 1 in every 15,000 to 18,000 babies is born with biliary atresia.

We don’t fully understand why some babies are born this way. There is some evidence biliary atresia may be caused by a virus or an autoimmune condition (when the body’s immune system goes into overdrive). It doesn’t seem to be caused by genetics and you can’t catch it from someone else.

Babies born with biliary atresia often also have abnormalities in other organs like the heart, kidneys or spleen.

How is biliary atresia treated?

There is no cure for biliary atresia.

The main treatment is surgery. The surgeon removes the gall bladder and the damaged bile duct outside the liver. They connect the liver straight to the small intestine to drain the bile from the remaining bile ducts in the liver. This operation means the bile can flow out of the liver again and the baby’s jaundice and other symptoms disappear in a few weeks.

The surgery is called the Kasai procedure or a portoenterostomy. It needs to be done as soon as possible, and before 3 months of age.

The surgery is completely successful in about 1 in 4 cases. In half of cases, the bile will flow but not very well.

In the other 1 in 4 cases, there will be no bile flow even after surgery. Sometimes the problem is with bile ducts inside the liver itself. In these cases, the only possible treatment is a liver transplant.

If your baby needs a liver transplant, it’s important that it’s done as quickly as possible – before too much damage has been done to the liver.

Caring for a baby with biliary atresia

You may be able to breastfeed your baby, but many babies with biliary atresia also need a special milk formula that contains fats that are easier to digest and help them gain weight.

If the surgery is completely successful and the bile is flowing well, your baby can grow up normally. They will be able to eat a normal diet.

If your child can’t absorb fats or vitamins properly, they will need to eat a special low-fat diet and take vitamin supplements. The most common supplements are vitamins A, D, E and K.

If your child has a special eating plan and still doesn’t grow properly, they may need a feeding tube (naso-gastric tube) to take a high calorie supplement straight into the stomach. Sometimes children with biliary atresia need to be feed through a drip (intravenous line).

There are some complications or biliary atresia to watch for:

• Cholangitis: Cholangitis is an infection of the bile ducts caused by bacteria. This is a common problem after surgery and can be life threatening. Seek medical help straight away if your baby has had surgery for biliary atresia and develops a fever, the jaundice gets worse, they’re not eating, or they’re more irritable than normal.
• Cirrhosis: Biliary atresia can cause long-term damage to the liver, called cirrhosis. This stops the liver from working properly and can affect the growth and physical development of the child.
• Failure to thrive: If the bile isn’t getting to the small intestine, the child can’t absorb their food properly. This can lead to poor growth or vitamin deficiencies.
• Portal hypertension: This is pressure on the veins travelling through the liver. It is caused by the scarring. Increased pressure on the portal vein can cause an enlarged spleen, ascites or varices.
• Ascites: This is a build-up of fluid, causing the child’s tummy to swell up.
• Varices: Varices are enlarged veins in the food pipe or stomach that can burst and cause internal bleeding. The signs of bleeding might be blood in the vomit or very dark or black poos. If you notice these symptoms, take your child to hospital straight away.

What is the outlook for a baby with biliary atresia?

The chances of long-term survival from biliary atresia are excellent in Australia. But if it’s not treated, biliary atresia usually leads to death by age 2.

If the surgery is successful, children may go on to lead a normal, healthy life. But in most cases, the liver still gets gradually damaged over time. Children born with biliary atresia often go on to need a liver transplant later in childhood or as a young adult. Biliary atresia is one of the main reasons children have liver transplants in Australia.

All babies born with biliary atresia will need to be monitored closely throughout their lives. Their growth and nutrition will be regularly checked and the specialist team will look out for possible complications.

Additional information and support

• Rare Voices Australia
• Australian Clinical Trials
• Children’s Liver Disease Foundation (UK)
• National Organization for Rare Disorders

What next?

• Read more about caring for a child with liver disease
• Read more about living well

Reviewed November 2022