Quick Summary

• Biliary atresia is a rare disease of the liver and bile ducts. It is a serious condition that a baby is born with. It presents very early in life and requires urgent treatment.
• Biliary atresia happens in around 1 in 10,000 to 1 in 20,000 births in the UK.
• There are several different types of biliary atresia. The type is based on the location of the blockage and the amount of damage to the bile ducts.
• We still don’t know exactly why biliary atresia happens, but doctors think it could be caused by a mixture of things. There is no way that biliary atresia can be prevented.
• Babies with biliary atresia usually appear well and healthy when they are first born. But signs such as prolonged jaundice, dark urine and pale stools (poo) usually start to appear during the first few weeks of life.
• Tests for biliary atresia include liver blood tests, an ultrasound scan and sometimes a liver biopsy.
• There is no medical cure for biliary atresia. But a surgical operation called a Kasai procedure can create a new path for bile to flow from the liver. This is best done in the first 2 to 3 months of life.
• If a baby does not have a Kasai procedure in the first 2 to 3 months of life, the success rate of the operation is low. But even if a Kasai procedure is performed early, it may not always be successful.
• Complications following a Kasai procedure may include cholangitis, itching, ongoing jaundice and poor growth. Sadly, in some children, liver disease can get worse and may lead to more severe complications and liver failure.
• Thanks to improved medical support and treatments, more babies with biliary atresia are growing to reach adulthood without needing a liver transplant. But it is likely that they will need a liver transplant at some point in their adult life. 

Find out more about biliary atresia using the links below.

Useful words

Bile – a green/yellow liquid that helps digest fat.

Biliary – anything to do with bile or the bile ducts.

Bilirubin – a yellow substance that can cause jaundice.

Jaundice – a condition where the skin or eyes turn yellow.

Kasai portoenterostomy – an operation to get bile flowing from the liver to the intestine.