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Biliary atresia

Q:What is Biliary atresia?

Biliary atresia is a rare condition in new born infants in which there is a blockage in the tubes(common bile ducts), which carry bile between the liver and the small intestine.

Q: What are the types of biliary atresia?

Blockage may be inside the liver (intra-hepatic) or outside the liver (extrahepatic). Generally, the extra-hepatic type are easier to treat surgically.

Another system called as Kasai classification divides it into 3 types depending on the location of blockage. In type I, the common bile duct is blocked but the hepatic duct is open. In type II, the hepatic duct is blocked. Type III atresia refers to blocked right and the left hepatic ducts, and is most difficult to treat surgically, but is also the most common type, accounting for more than 90% of cases.

Q:How common is the problem? Who is more likely to be affected?

About one in 10,000 to 20,000 new born babies are affected every year, girls slightly more often than boys. Within the same family, it is common for only one child in a pair of twins or only one child within the same family to have it.

Q:Why does it happen? How does it progress?

Biliary atresia occurs when the bile ducts inside or outside the liver do not develop normally, the reason for which is not known. The bile ducts may have be damaged by the body's immune system in response to a viral infection acquired after birth. If unrecognized, the condition leads to liver damage and cirrhosis of liver.

Q:What are the signs and symptoms of biliary atresia?

Babies with biliary atresia usually appear healthy when they are born. Symptoms of the disease typically appear within the first two weeks to two months of life. Those symptoms include:

10 - 15 % of infants with biliary atresia may be born with other problems in the heart, spleen (polysplenia), blood vessels (inferior vena caval anomalies, pre-duodenal portal vein) or intestines (situs-inversus or malrotation).

Q:Which tests and examinations are required to diagnose / evaluate this disease?

Jaundice may be present because of other liver disorders, therefore, several tests are needed may be required to establish the correct diagnosis.

Q:What is the best treatment for biliary atresia?

Biliary atresia cannot be treated with medication.

A Kasai procedure or Hepatoportoenterostomy, where the damaged ducts outside of the liver (extrahepatic ducts) are removed joined to the baby's own intestine. This allows bile to pass from the liver directly into the intestine.

If this procedure is unsuccessful, it is usually because the blocked bile ducts are inside the liver (intrahepatic), as well as outside the liver (extrahepatic). These children develop cirrhosis of liver, which can be treated by replacing the entire liver i.e. liver transplantation.

Q:What is the expected prognosis (Outlook) after various treatments?

Long-term outcome after the Kasai procedure is influenced by progressive liver disease (cirrhosis) and the development of portal hypertension (high blood pressure in the portal vein that carries blood to the liver).

Nearly one-half of all infants who have had a Kasai procedure require liver transplantation before 5 years of age. 65 - 85% of children with biliary atresia will need to have a liver transplant before 20 years of age.