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Biliary Atresia

This page contains a summary of biliary atresia. For more detailed information, download or order a copy of CLDF's biliary atresia leaflet.

What is biliary atresia?

Biliary atresia is a medical condition which affects the liver. It can occur either:

  • Before birth due to the bile ducts developing abnormally
  • Shortly after birth due to the bile ducts becoming inflamed or obstructed

In most babies with biliary atresia, bile ducts on the inside and outside of the liver are affected, eventually leading to a complete blockage of bile flow from the liver. This in turn causes scarring (fibrosis) in the liver.

What causes biliary atresia?

The cause of biliary atresia is currently unknown. Research is being carried out, but it has not provided any definite answers yet. Parents often feel guilty that they may have, in some way, contributed to their child having the condition. These feelings are understandable but there is no evidence to suggest that biliary atresia is hereditary (can be passed on to children by their parents) or due to anything that parents might have done or taken. Children with biliary atresia will not pass the condition on to their own children.

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 (yellow discolouration of the skin and whites of the eyes) and in some cases feeding more often.

Prolonged jaundice (jaundice lasting more that two weeks in a full term baby or three weeks in a pre-term baby) should be explored further and can be a key sign of biliary atresia.

Other key signs are:

 

  • Pale stools - Normally the stools of a baby are green or yellow. If the stools are grey, white, fawn or pale then this can be an indication of liver disease. CLDF have a stool chart which includes the colours of health and potentially unhealthy stools. You can use this to examine your baby's stool. You can download a stool chart or order a hard copy here.
  • Urine colour - Normally the urine of a newborn baby is colourless. If a baby's urine is persistently yellow or dark in colour this can 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?

If a baby has the signs and symptoms above then they may be admitted to hospital for further investigation.

A number of investigations will need to be carried out in order to make a diagnosis. These may include:

  • Blood tests
  • Urine test
  • An ultrasound
  • Liver excretion test
  • Some children will need a liver biopsy and/or an operation to confirm or rule out a diagnosis.

Treatment options for biliary atresia

If the tests show that there is a strong indication that your baby has biliary atresia, he/she will require surgery. This will involve carrying out an investigation called an operative cholangiogram under a general anaesthetic to confirm the diagnosis.

If biliary atresia is confirmed then this will be followed by a "Kasai procedure", usually done under the same general anaesthetic at the same time.

Further details of the operation can be found in our biliary atresia leaflet which can be downloaded here.

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)
  • Pruritus (itching)

What happens if the biliary atresia is not treated or treatment is unsuccessful?

If a baby has not had a Kasai procedure in the first two to three months of life the success rate of the operation is very low. If biliary atresia is not treated or the treatment isn't successful the bile produced in the liver builds up causing damage to the cells of the liver, eventually resulting in severe scarring (cirrhosis). If this continues liver function deteriorates, complications occur and general health suffers. Liver transplantation is often the accepted form of treatment. Transplant is a huge step and is only considered when other treatments are ineffective.

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.