Are there any other problems linked with biliary atresia?
Biliary atresia is a rare disease of the liver and bile ducts. It presents very early in life and requires urgent treatment.
In newborns with biliary atresia, bile ducts are missing, incomplete, blocked or damaged. This stops the flow of bile from the liver to the gallbladder and small intestine. Bile backs up and gets trapped inside the liver.
The information on this page is for:
- Parents of babies and children with biliary atresia or suspected biliary atresia.
- Young people with biliary atresia.
- Family, friends, carers, and healthcare professionals.
Some babies with biliary atresia will have additional problems linked with the condition. They may include:
On this page:
Problems with their spleen
Some babies with biliary atresia have problems with their spleen. They may have no spleen (asplenia) or multiple small spleens (polysplenia). This can affect the way the immune system works to fight off infections.
The spleen is a small organ which sits in the upper left abdomen next to the stomach. It helps fight infection and filter the blood.
Problems with their heart
Some babies with biliary atresia have problems with their heart. We don’t know the exact number of babies affected, but it is thought to be around 7% of babies with biliary atresia in the UK.
Having heart problems alongside biliary atresia can make the condition more serious for your baby. The medical team will do extra tests, such as heart scans to diagnose any problems. Your baby will be referred to the cardiology team to explain the findings and what treatment is needed. If your baby needs surgery to treat heart problems, this may be done before the Kasai procedure, where possible, as it may help improve your baby’s outcome.
Problems with their blood vessels
Some babies with biliary atresia have problems with their blood vessels, particularly those around the liver and spleen. This can make the Kasai procedure more difficult for the surgeon.
Problems with their intestines and digestive system
Babies with biliary atresia may have problems with the intestines or other parts of the digestive system. Some problems can be corrected at the same time as the Kasai procedure.
Biliary atresia splenic malformation syndrome (BASM)
If some or all of these complications occur, it is a condition known as biliary atresia splenic malformation syndrome (BASM). This condition does not affect how a baby responds to the Kasai procedure. However, the link with heart problems can affect the outcome of surgery.
Babies with BASM may need additional tests. They may also need to take daily antibiotics to reduce their risk of infection.
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This content was last reviewed: July 2026
Our expert reviewers:
We would like to thank everyone who helped with creating and reviewing this page. Including Professor Mark Davenport, Consultant Paediatric Surgeon, King’s College Hospital and Professor Deirdre Kelly, Professor of Paediatric Hepatology. And all our patient reviewers.
Find out how we make our patient information.
Everyone’s experience of liver disease will be different. Always talk to your specialist medical team for personal advice.
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