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Biliary atresiaDisclaimer: This fact sheet is for educational use only. Please consult your doctor or other health professional to make sure this information is right for your child. Key points to remember
 Back to TopWhat is biliary atresia?Biliary atresia is a rare liver disorder found in babies. It affects the tubes (bile ducts) that carry bile from within the liver to the gut (intestine). The bile ducts are damaged or missing in biliary atresia.
Bile is a yellow-green liquid made in the liver. It contains bile salts that help digestion. Bile also contains bilirubin (see What is bilirubin? in the jaundice fact sheet) and other substances that the body needs to get rid of. These substances build up in the body if the bile ducts do not drain properly. Some of these substances cause liver damage.
If bilirubin cannot drain away through the bile ducts into the intestine, it causes jaundice. Jaundice is the medical word for yellowing of the skin and the whites of the eyes.
Once bilirubin reaches the intestine, it is digested and changes colour. It gives poo (bowel motions, stools) its normal brown colour. Pale, white or light beige-coloured poo is one sign of a complete blockage or lack of connection between the liver and the intestine. See the Stool colour chart1 at the UK Children's Liver Disease Foundation website.
Biliary atresia affects about one in 10,000 babies. The cause is unknown.
Back to TopWhat are the early signs of biliary atresia?The first sign of biliary atresia is jaundice. Jaundice affects many babies, but it usually goes away by two weeks of age (see Jaundice in babies). The jaundice of biliary atresia does not go away. Jaundice may be the only sign of early biliary atresia in babies. These babies may initially gain weight and otherwise seem well.
If your baby has biliary atresia, they may also have:
Back to TopWhen should I seek help?You should see your GP (general practitioner) or midwife if your baby:
Back to TopHow is biliary atresia diagnosed?There are many reasons why your baby might be jaundiced after the age of two weeks. Your GP (general practitioner) or midwife should check your baby. Your GP should ask for a blood test to help find the reason for the jaundice.
The blood test will usually show another cause for the jaundice. Biliary atresia is a very rare cause.
If biliary atresia is suspected, your GP will refer your baby to see a paediatrician (a specialist in children’s health).
Your baby may have:
Back to TopHow is biliary atresia treated?The treatment for biliary atresia is a surgical operation called the Kasai procedure (named after the surgeon who developed it). The operation involves connecting part of the intestine directly to the liver so that bile is able to flow from the liver to the intestine. This operation works best if it is done when your baby is young (less than two months old). The operation does not always cure biliary atresia, even when it is done early.
If the operation does not help, then over time your child’s liver will become more and more damaged. This leads to liver failure. The only cure for liver failure is a liver transplant.
Back to TopWhat are the complications of biliary atresia?Biliary atresia can cause a number of problems.
Growth problems: Your baby will not be able to digest the fats from milk feeds because bile (which is required for fat digestion) is not getting into the intestine. This leads to poor growth. Extra feeds or supplements given through a feeding tube might be needed to help your baby grow.
Vitamin deficiencies: Vitamins A, D, E and K dissolve in fat so they cannot be absorbed properly into the body in biliary atresia. Your baby will be given extra medication to provide these vitamins.
Later, if the liver starts to fail, there may be other problems.
Fluid collects in the tummy (abdomen). This is called ascites. It causes your child’s tummy to swell. It is treated with drugs that increase urine output (diuretics).
Infection: Fluid in the abdomen (ascites) can become infected. Symptoms include fever, poor feeding and irritability. The sooner intensive antibiotic treatment is started, the better the results will be.
Bleeding into the stomach: Liver damage can cause enlarged veins (varices) in the wall of the food pipe (oesophagus). These can leak a lot of blood. You should see a doctor straight away:
Back to TopAn important complication to watch for after surgery for biliary atresiaAfter a Kasai operation, the risk of infection of the bile ducts increases. This is called cholangitis.
Symptoms include fever, poor feeding and irritability. Cholangitis is usually recognised first by a fever, followed a day or two later by pale poo (bowel motions, stools). Do not wait. You should see a doctor straight away if your child develops any of these symptoms.
The sooner intensive antibiotic treatment is started, the better the results will be.
Back to TopHow can biliary atresia be prevented?The cause of biliary atresia is not known. There is no way to prevent it happening.
Back to TopWhere to go for more informationOn this website
Cincinnati Children’s Hospital Medical Centre
Cincinnati Children’s Hospital has a detailed section on biliary atresia.
Back to TopLinks (these are the web addresses for the numbered links in the text above) Back to TopYour notesEndorsement
This fact sheet was endorsed by PSNZ - 25/06/2008
CopyrightFact sheets are subject to copyright. In the interests of information sharing they may be copied but acknowledgement must be given to PSNZ and Starship Foundation. © The Paediatric Society of New Zealand and Starship Foundation 2005 - 2010
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