Biliary Artresia

Also known as: BA, extrahepatic ductopenia, progressive obliterative cholangiopathy

What is biliary artresia?

The biliary system refers to the ducts and channels that allow the fluid produced in the liver (bile) to drain into the intestines. Bile helps with the digestion of food (and carries waste products from the liver to the intestines for removal). In biliary atresia the bile duct system is blocked. When the bile can't leave the liver, it causes liver damage which can affect many other parts of the body.

What causes biliary artresia?

Biliary atresia is quite rare and seems to affect girls more often than boys. The causes of biliary atresia are not exactly clear. In some children the ducts don't form normally while the baby is in the womb.In others it may be related to a viral infection that occurs soon after the baby is born. Some infants with biliary atresia are also more likely to have other birth defects, such as problems with the heart, spleen, intestines or blood vessels.

What are the symptoms of biliary artresia?

Most babies with biliary atresia appear normal after birth (some may be jaundiced- yellow eyes and skin- as newborns), with symptoms of jaundice developing 2-8 weeks later. In addition, infants with biliary atresia may have dark urine, clay-colored stools, irritability, and weight loss.

What are biliary artresia care options?

A surgical procedure known as a Kasai procedure is often effective at treating biliary atresia. It involves redirecting the flow of bile around the blocked portions of the ducts. In cases where the procedure fails, a liver transplant may be necessary. Nutritional support to improve nutrition is important.

Reviewed by: Jack Wolfsdorf

This page was last updated on: March 20, 2019 04:06 PM