Biliary atresia

An important function of the liver is make a substance called bile. Bile is a liquid which passes into the gut through small tubes called bile ducts and is stored in the gall bladder. Bile contains waste products and other products needed to digest food.

 

What is biliary atresia?

Biliary atresia is a medical condition. 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 unknown. Research is being carried out but it hasn’t 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 parents might have done or taken. People with biliary atresia will not pass the condition on to their own children.

 

What are the signs 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 of the whites of the eyes) and in some cases feeding more often.

Prolonged jaundice (jaundice lasting more than 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 important signs are:

  • Pale poo – normally the stools of a baby are green or yellow. If the stools of a baby are grey, white, fawn or pale then this can be an indication of liver disease.
  • 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.

Read more on prolonged jaundice in babies

 

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 a small operation to confirm or rule out a diagnosis.

These investigations are completed over the course of a few days. Find out more about routine liver investigations.

 

How can biliary atresia be treated?

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. The aim of the procedure is to help bile drain from the liver into the gut.

Find out more about the Kasai operation

 

Are there any other complications of biliary atresia?

Between 10 – 20 babies out of 100 with biliary atresia may have additional problems found during surgery. These may include the following:

  • Some children with biliary atresia can have multiple small spleens (polysplenia) or the spleen can be absent (asplenia).
  • The intestine may not have properly curled up into the abdomen during development (intestinal malrotation). This will need to be corrected at the time the surgeon operates for biliary atresia.
  • Some abnormalities of the veins in the abdomen
  • Heart defects

If some or all of these complications occur it is a condition known as “biliary atresia – splenic malformation syndrome (BASM)”. This group of children with biliary atresia may be less likely to clear their jaundice after the operation. They also need additional tests such as a heart scan to check for problems.

It may also be necessary for them to take daily antibiotics to reduce their risk of infection.

 

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.

If liver transplantation is an option for your child this will be discussed with you by your child’s medical team. The aim of liver transplantation is to restore your child to good health.

Find out more about liver transplantation.

 

What other problems might occur?

In any baby who undergoes a Kasai procedure, even if they clear their jaundice completely, 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 will happen in the future?

Your baby/child’s growth and development will be followed up by periodic visits to the hospital. How often you will need to go to the hospital will vary for each child. Blood tests will be done to measure your child’s bilirubin level and liver function. Periodically an ultrasound will be performed to assess the size of the liver and other structures in the abdomen (e.g. the spleen).

Download CLDF’s leaflet on biliary atresia

 

This website provides general information but does not replace medical advice. It is important to contact your/your child’s medical team if you have any worries or concerns