Biliary atresia in babies: symptoms, Kasai operation and outlook

Liver UK describes biliary atresia as a rare disease of the liver and bile ducts that presents very early in life and requires urgent treatment. In affected newborns the bile ducts are missing, incomplete, blocked or damaged, which stops the flow of bile from the liver to the gallbladder and small intestine. Bile backs up and gets trapped inside the liver, and the condition can cause serious liver damage.

Liver UK says there are several types depending on the location of the blockage and the amount of damage, that doctors think it could be caused by a mixture of things without knowing exactly why, and that there is no way to prevent it.

Liver UK says babies with biliary atresia usually appear well and healthy when first born, but prolonged jaundice, dark urine and pale stools (poo) usually start to appear during the first few weeks of life. The UK National Screening Committee describes early symptoms as jaundice and pale stools.

Newborn jaundice is very common and usually harmless. The NHS says for most babies with jaundice the yellow is most noticeable at around 3 days old and it usually takes around 2 weeks for the jaundice to go.

The NHS says to ask for an urgent GP or midwife appointment or call NHS 111 if your baby is over 24 hours old and you think they have jaundice, if jaundice has been diagnosed and is not getting better or is getting worse, or if their pee is dark yellow or brown or their poo is a pale creamy colour. It suggests keeping a sample of the poo to show a midwife or doctor.

The NHS says to call 999 or go to A&E if a baby with jaundice is sleepier than normal or difficult to wake, is not feeding, is floppy or stiff or has jerking or twitching movements, has a high or low temperature, has not been producing wet nappies, has difficulty breathing, or is less than 24 hours old.

Liver UK says tests include liver blood tests, an ultrasound scan and sometimes a liver biopsy. King's College Hospital says a diagnosis is made following blood tests and imaging, and that all suspected cases should be referred to one of the three national centres, which are King's College Hospital, Leeds Children's Hospital and Birmingham Children's Hospital.

Liver UK says there is no medical cure for biliary atresia but a surgical operation called the 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 that time, the success rate of the operation is low, and even if it is performed early it may not always be successful.

King's College Hospital says that in most cases bile flow is restored and jaundice reduced after surgery, and these babies can be managed with outpatient follow-up, but some need further inpatient care and possibly a liver transplant. Liver UK lists complications following the Kasai procedure that may include cholangitis (infection of the bile ducts), itching, ongoing jaundice and poor growth.

Liver UK says that in some children liver disease can get worse and may lead to more severe complications and liver failure. It says that thanks to improved medical support and treatments, more babies with biliary atresia are growing to reach adulthood without needing a liver transplant, but that it is likely they will need a liver transplant at some point in their adult life.

NHS Blood and Transplant describes a liver transplant as a major operation in which a diseased liver is replaced by a donor liver, with assessment, a period on the waiting list if suitable (which may take several months), then the operation. King's College Hospital says that if a transplant is needed it is carried out within the same specialist service.

The UK National Screening Committee does not recommend screening newborns for biliary atresia, because there is inconsistent evidence on the accuracy of stool colour cards as a screening test and no evidence that screened babies would be able to have surgery at a younger age than unscreened babies. It last reviewed the condition in June 2026, with the next review estimated for 2029 to 2030. This means parents and clinicians are the main safety net: check your baby's poo colour and act on prolonged jaundice.

Biliary atresia is managed in only three centres in the UK, so care is organised through the NHS even when a baby is first seen in a local hospital. The registry of babies born with biliary atresia in England and Wales is used to assess national outcomes. If you would like a second opinion, ask the team looking after your baby how to arrange it and make sure records are shared with the specialist centre.