Absence or malfunctioning of the bile ducts that carry the secretion called bile away from the liver. Causes for the disorder are unclear, but some bile duct damage may be caused by viral infection near birth. Symptoms of biliary atresia generally appear two to six weeks after birth and include jaundice and a swollen abdomen. Ultrasound, X-rays, and other radioactive scanning devices, along with blood tests, urinalysis, and liver function tests, help doctors distinguish biliary atresia from other disorders, including neonatal hepatitis. Untreated, biliary atresia will lead to cirrhosis of the liver and often to death, generally in infancy. About half of the cases of biliary atresia are treatable by surgery; the Kasai procedure, or hepatoportoenterostomy, involves removing damaged bile ducts and creating artificial bypass ducts from the baby’s intestine. For some children with biliary atresia, however, such as those whose damaged ducts are inside the liver, liver transplants at present offer the only hope for survival.
Congenital absence of major bile ducts; also called neonatal hepatitis.
A potentially fatal disorder in newborns, in which the bile ducts inside or outside the liver fail to develop or develop abnormally. Biliary atresia causes jaundice (a yellowing of the skin and the whites of the eyes) and cirrhosis (a severe liver disease). The symptoms of biliary atresia appear from 2 to 6 weeks after birth. Jaundice is the primary symptom. An infant may also experience pale stools, dark urine, an enlarged and hardened liver, and a swollen abdomen. Some babies develop an intense, uncomfortable itching that can make them very irritable. The cause of biliary atresia is not known.
Closure or absence of some or all of the major bile ducts.
The condition characterized by the incomplete development or total absence of the ducts responsible for transporting bile.
A rare inborn condition arises when certain or all of the bile ducts fail to form or develop in an abnormal manner. Consequently, the flow of bile from the liver becomes obstructed, leading to a condition known as cholestasis. Without appropriate treatment for the atresia, a potentially life-threatening condition called secondary biliary cirrhosis can ensue. Symptoms of this disorder include the onset of jaundice, typically occurring approximately one week after birth, as well as the excretion of dark urine and pale stools. The primary treatment approach involves surgical intervention to create an alternative passage for the bile ducts. However, if this procedure proves unsuccessful or if jaundice recurs, a liver transplant becomes imperative.