What is biliary atresia?
Bile is a liquid which passes into the gut through small tubes called bile ducts and is necessary for the proper digestion of fat within the diet.
Biliary atresia is a condition in which inflammation develops within the bile ducts around the time of birth. The inflammation can occur in any of the bile ducts both inside and outside the liver. This leads to bile duct damage, reducing the flow of bile, which causes scarring of the liver.
What causes biliary atresia?
The cause of biliary atresia is currently unknown. Research is being carried out, but it has not yet provided any answers.
What are the signs and symptoms of biliary atresia?
In the first weeks of life babies with biliary atresia often seem well, apart from being jaundiced (yellowing of the skin and whites of the eyes). Jaundice is very common in babies and mostly clears within the first two weeks of birth.
In addition to prolonged jaundice, other signs to look out for are:
- Yellow coloured urine
- Pale stools
- Bleeding from the umbilicus or frequent nosebleeds may be an indication of liver disease
CLDF’s Yellow Alert campaign highlights the signs and symptoms for parents, parents to be and healthcare professionals. Visit CLDF’s dedicated Yellow Alert website to find out more.
How is biliary atresia diagnosed?
The signs above are non-specific so there will need to be a number of investigations. Tests need to be carried out in hospital and include blood tests, scans and liver biopsy. Once other causes of liver disease have been excluded and investigations indicate biliary atresia your baby will be scheduled for surgery.
Treatment options for biliary atresia
Initial treatment is an operation called the ‘Kasai Procedure’ after the Japanese surgeon who pioneered the operation. The aim of the Kasai procedure is to allow bile to drain from the liver into the gut.
Babies with biliary atresia will often need a special milk formula so they can absorb nutrients more fully. The hospital dietitian will give advice. The doctor may prescribe extra vitamins.
Are there any other problems to be aware of?
Even after a successful Kasai operation a number of problems may occur:
- Cholangitis (an infection of the bile ducts in the liver, resulting in inflammation)
- Ascites (an abnormal collection of fluid in the abdomen)
- Portal hypertension (increased blood vessel pressure caused by scarring of the liver, which may cause blood being vomited or passed in the stools)
- Pruritus (itching)
- Failure to thrive
The future for biliary atresia
A child or young person’s growth and development will be followed up by periodic visits to the hospital. Blood tests will be needed to monitor liver function and periodically an ultrasound will be performed to assess the size of the liver and other structures in the abdomen. Routine endoscopy may be undertaken to monitor the development of oesophageal varices.
Want to know more about biliary atresia?
More information on biliary atresia, the Kasai operation and post-operative treatment can be found in CLDF’s leaflet on biliary atresia. You can also find articles written by biliary atresia families in our blog section including Fraser's story and Amelia's.
The information on this site is for guidance only. It is not a substitute for professional medical care by a qualified doctor or other health care professional. ALWAYS check with your medical team if you have any concerns about your condition or treatment. CLDF is not responsible or liable, directly or indirectly, for any form of damages resulting from the use (or misuse) of information contained in or implied by the information on this site.