Biliary atresia is a chronic, progressive liver problem that becomes evident shortly after birth. Tubes inside and outside the liver, called bile ducts, normally allow a liquid produced by the liver called bile to drain into the intestines and kidneys. Bile aids in digestion and carries waste products from the liver to the intestine and kidneys for excretion. In biliary atresia, bile ducts that are located inside or outside the liver are blocked. When the bile is unable to leave the liver through the bile ducts, the liver becomes damaged and many vital body functions are affected.
Biliary atresia causes liver damage and affects numerous important processes that allow the body to function normally. It is a life-threatening disease and is fatal without treatment.
Biliary atresia is the most common cause of chronic liver disease in neonates. It occurs once in every 10,000 to 20,000 births. Asian populations most frequently affected; African Americans are affected approximately twice as much as Caucasians.