Biliary Atresia

Biliary AtresiaBile, a fluid that is produced in the liver, affects many important processes in the body, including the removal of the chemical substance, bilirubin, from the liver and the whole system (see neonatal jaundice).

Bile travels through a series of tiny ducts in the liver, and part of the bile is stored in the gallbladder. When exiting from the gallbladder, it connects to the bile that passes through larger ducts outside the liver. All the ducts eventually connect and form the common bile duct which leads to the duodenum (part of the small intestine).

In rare cases, a baby who is missing parts of the ducts can be born, and that is why a bile stays in the liver. The bilirubin can not be removed, and it is accumulated in the bloodstream. This causes a prolonged jaundice that usually begins in the second week of life. A newborn may also have a pale stool and dark urine.

What to do?

The doctor must examine the newborn with the symptoms described. If the baby has atresia, the surgeon will do an operation called laparotomy when the baby is about 2 months old. The inner part of the abdomen is examined and, in the case of a atresia outside the liver, it is possible (in some cases) to perform the operation of connecting the gallbladder to the duodenum. This allows flow of bile from the liver directly into the intestine. If the operation fails, liver cirrhosis will gradually develop and almost certainly lead to death in early childhood.

If the bile duct outside the liver is normal, a part of the liver is taken for examination (biopsy). Microscopic examination of this part will be shown whether the newborn has an atresia in the liver. Unfortunately, there is no successful treatment of this condition, and the child usually suffers from liver cirrhosis after a one to two years. Perhaps, in the future, this disease may be cured with liver transplants.