Biliary atresia

Is a rare condition in newborn infants in which the common bile duct between the
liver and the small intestine is blocked or absent If unrecognized, the condition
leads to liver failure. The condition is congenital, which means it is present from
birth.

Causes, incidence, and risk factors

Biliary atresia occurs when the bile ducts inside or outside the liver do not develop
normally. It is not known why the biliary system fails to develop normally.

The bile ducts help remove waste from the liver and carry salts that help the small
intestine break down (digest) fat.

In babies with biliary atresia, bile flow from the liver to the gallbladder is blocked.
This can lead to liver damage and cirrhosis of the liver, which is deadly if not
treated.

Symptoms

Newborns with this condition may appear normal at birth. However, jaundice (a
yellow color to the skin and mucous membranes) develops by the second or third
week of life. The infant may gain weight normally for the first month, but then will
lose weight and become irritable, and have worsening jaundice.

Other symptoms may include:

 Dark urine
 Enlarged spleen
 Floating stools
 Foul-smelling stools
  Pale or clay-colored stools
 Slow growth
 Slow or no weight gain

Treatment

An operation called the Kasai procedure is done to connect the liver to the small
intestine, going around the abnormal ducts. It is most successful if done before
the baby is 8 weeks old. However, a liver transplant may still be needed.

The Kasai Procedure

The Kasai procedure can restore bile flow and correct many of the problems of
biliary atresia. This operation is usually not a cure for the condition, although it can
have an excellent outcome. Without this surgery, a child with biliary atresia is
unlikely to live beyond the age of 2. The operation works best if done before the
infant is 90 days old and results are usually better in younger children.

The improved results of the surgery make the early diagnosis of biliary atresia
very important, preferably before the infant is several months old and has
suffered permanent liver damage. Some infants with biliary atresia who undergo a
successful Kasai operation are restored to good health and can lead a normal life
without jaundice or major liver problems.

Unfortunately, the Kasai procedure is not always successful. If bile flow is not
restored, the child will likely develop worsening liver disease and cirrhosis and
require liver transplantation within the first 1 to 2 years of life. In addition, the
Kasai operation, even when initially successful, may not totally restore normal liver
development and function. A child with biliary atresia may slowly develop cirrhosis
and related complications and require a liver transplant later in childhood.
While the Kasai procedure has been a great advance in the management of biliary
atresia, improvements in the operation and clinical management of children who
undergo it are needed to improve the outcomes of children with this disease.