What is biliary atresia?

Biliary atresia is a liver disease of infants. Bile is produced in liver and transported to intestine via biliary system (bile ducts and gall bladder). It is required for digestion of food in intestine. In biliary atresia the biliary ducts become inflamed and eventually scarred resulting in bile pooling in the liver. The ongoing bile pooling in liver will induce liver failure.

What are the symptoms of biliary atresia?

Jaundice and clay colored stools are cardinal signs of biliary atresia. Initially, the babies feed and grow well. As the disease progresses the jaundice deepens, urine becomes very dark and abdomen becomes distended. Eventually, signs of liver failure (poor overall health, huge abdomen, blood in vomit etc.) become apparent.

Any newborn child with jaundice beyond first two weeks of life should be investigated by a doctor.

How common is biliary atresia?

Biliary atresia is an uncommon disease found in 1 out of 5000 infants. It is the most frequent cause of liver related death in children.

What are the tests?

Blood investigations will include blood counts and liver function tests. Several other blood tests will be required to rule out other conditions that may also cause jaundice and liver damage such as infections and genetic problems.

Sonography of abdomen will show a contracted gall bladder even in fasting condition.

HIDA scan will show absence of tracer in intestine and will warrant further investigations to confirm the diagnosis of biliary atresia. The baby should be given oral phenobarbital for five days before performing HIDA scan.

Liver biopsy is taken during or before the surgery. It will show bile pooling, proliferation of tiny bile ductules and changes of liver cirrhosis. Expert histopathologist is required to confirm the diagnosis of biliary atresia on a tru-cut biopsy.

What is operative cholangiogram?

Cholangiogram is a special x-ray of biliary system. When cholangiogram is done by an operation it is called operative cholangiogram. A special dye is injected in the biliary system and x-rays are taken. If the dye is going in small intestine and in liver, biliary atresia is ruled out. Otherwise, biliary atresia is confirmed and the Kasai procedure is performed.

What is the treatment of biliary atresia?

Surgery is the only curative treatment.

  1. Kasai procedure; Biliary tree outside liver is removed and small intestine is joined to the part of liver from which the bile ducts exit. This procedure gives good results when surgery is performed within first two months of life and liver is in good condition.

    Sometimes, even after the Kasai operation, the damage to the liver is bad enough that the liver eventually fails and a liver transplant is needed. In these cases, the Kasai procedure allows the child to grow for several months or years. Obtaining a donor liver for transplant, the transplant procedure becomes easier because the child is bigger.

  2. Liver transplant; when the child is older and liver is not in good condition, a primary liver transplant should be performed. A successful liver transplant will cure the child of the disease.

Medical treatment for correction of coagulopathy (bleeding tendency) and vitamin deficiency (especially vitamin A,D,E and K) is mandatory. Antibiotics and steroids are also required after Kasai procedure.

What are the risks?

The risks of both Kasai procedure and liver transplant are bleeding, infection, leak from intestinal suture lines, liver failure and death.

What is the long term outcome?

In general, one-third of infants undergoing Kasai procedure avoid transplant, one-third have liver failure in the first year, and one-third progress to liver failure later in childhood.

The long-term survival of BA infants with their native liver, without liver transplant, depends largely on the condition of the liver at the time of the Kasai procedure. A successful surgery restores flow of bile from the liver to the small intestine and lessens further damage to the liver.

Infants with a shorter duration of jaundice have a better response to the Kasai procedure. This is because the longer the liver does not drain bile, the more injured and scarred it becomes, and the Kasai procedure cannot reverse the damage already done.

After a Kasai procedure, babies can have an infection of the bile ducts, spreading up into the liver (cholangitis). This presents with fevers, chills, yellow skin, yellow eyes and light colored stools. This is a very serious infection and requires immediate medical attention. If the baby has repeated episodes of cholangitis, it can scar the liver more and may lead to overall worsening of the liver status.