Biliary atresia: what it is and how it manifests itself

By Cristiano Antonino On Dec 8, 2022

Biliary atresia is a severe disease affecting infants that causes inflammation and obstruction of the bile ducts (the channels responsible for transporting bile from the liver to the intestine)

When bile cannot flow normally, it flows back into the liver (this situation is called ‘biliary stasis’), causing jaundice (yellowing of the skin and sclerae) and cirrhosis.

Cirrhosis is a condition that occurs when healthy liver cells are destroyed and then replaced by fibrous tissue.

The fibrosis interferes with bile flow passing through the liver, causing further cell damage and further fibrosis and thus restarting the cycle of liver damage.

Aetiology and symptoms of biliary atresia

The cause of biliary atresia has not yet been discovered.

The disease affects approximately one in 20,000 infants, with a preference for the female sex over the male, but without discriminating by race or ethnicity.

It is not a hereditary condition although, in very rare cases, more than one infant within a family may be affected by the disease.

The symptoms of biliary atresia usually become manifest between two and six weeks after birth.

The newborn presents jaundice, an enlarged and harder liver and a swollen abdomen; stools are usually clear and urine dark.

Some infants may present with intense itching, a condition that makes them extremely impatient and irritable.

The cause of the itching is not yet known, but researchers have discovered a connection between itching and the return of bile.

Diagnosis of biliary atresia

There are many liver diseases that cause symptoms similar to those of biliary atresia. This is why, before making a diagnosis of biliary atresia, it is advisable to carry out various laboratory tests (blood and urine tests, liver function tests and tests for clot function) to be sure that other liver diseases can be ruled out.

In these cases, a (non-painful) examination using ultrasound (ECHO) is often performed to analyse the liver and determine the size of the bile ducts and gallbladder.

Other tests are based on specific X-ray or radioactive scanning of the liver, techniques that may prove useful in focusing on the real problem.

Treatment of biliary atresia

The most successful treatment of biliary atresia, so far, is surgical.

The operation creates a drainage of bile from the liver when the ducts are completely blocked.

This operation is called ‘Kasai’s operation’ (or, technically, hepatoportoenterostomy), named after Dr Morio Kasai, the Japanese surgeon who developed it.

During the operation, the surgeon removes the damaged extrahepatic ducts and replaces them with a piece of intestine, taken from the child itself, which acts as a new duct.

The purpose of this operation is to allow the passage of bile from the liver into the intestine through the new duct.

The operation is successful in about 50% of cases

In babies who respond well to the operation, jaundice usually disappears after a few weeks.

In the remaining 50% of cases, in which Kasai’s operation is unsuccessful, the failure is caused by the fact that the obstructed bile ducts are ‘intrahepatic’, i.e. they are located inside the liver.

After the operation, an attempt is made to encourage the family and the child to grow and develop normally.

If the bile flow is good, the child is given a regular diet; if the bile flow is low, a low-fat diet is recommended, as the bile, which is used by the body in the absorption of fats and vitamins, does not perform its task well.

Multiple vitamins, the B vitamin complex and vitamins E, D and K can be administered as an additional aid.

Unfortunately, despite the reactivated biliary flow, Kasai’s surgery is not the definitive cure for biliary atresia: for reasons still unknown, liver damage often continues its course and eventually leads to cirrhosis (with all its complications).

Possible complications of biliary atresia

Patients with cirrhosis experience changes in the blood flow through the liver, which in turn can produce dysfunctions, such as rashes, nosebleeds, fluid retention and varices in the stomach and oesophagus.

The high pressure produced within these veins can cause them to bleed.

In some cases, a procedure in which a sclerosing agent is injected into the varices may be necessary.

As the disease progresses, however, other complications may occur.

While all children tend to be sleepy after eating, those with biliary atresia may be excessively sleepy after eating protein-rich foods due to increased nitrogen products in the blood stream.

Children with biliary atresia may also be easier prey for infections.

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Life perspectives for patients with biliary atresia

The extent and type of liver damage differs from child to child.

Some respond well to Kasai’s surgery, others do not.

If the bile continues to flow, long-term survival is possible.

However, it is currently impossible for the doctor to determine in advance which children will respond to treatment and which will not.

In any case, there can be no definitive cure for biliary atresia until its cause is discovered, so hopes for a definitive solution all rest on scientific research.

Liver transplantation

Liver transplantation is increasingly the only or last viable solution for those suffering from certain liver diseases.

Survival rates for transplant recipients have increased dramatically with improved surgical techniques and the development of new drugs that help overcome the problem of organ rejection.

In children with biliary atresia, liver transplantation is generally not attempted until Kasai’s surgery has been performed first.

If this operation is unsuccessful, and before the resulting complications of cirrhosis become severe and endanger the child’s life, liver transplantation may be attempted, which has been successful in many cases.

However, as with all organ transplants, a successful outcome depends largely on the timely availability of compatible organs for donation, the time factor (a donated liver must be re-implanted within 16 hours for the procedure to be successful) and other factors that are still being researched.

The practice of living transplants, given the small size of the organ needed (often the right half of an adult liver is enough for a child) is greatly improving the time and availability of compatible donors.

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