Biliary Atresia

What is Biliary Atresia?

Biliary atresia is a condition occurring in infants in which the bile ducts both outside and inside the liver are scarred, blocked, or destroyed. The bile ducts are tubes that carry bile (fluid that aids in digestion) to the gallbladder. When the bile ducts are blocked, bile builds up in the liver and then damages it. This can lead to other health problems, including scarring, loss of liver tissue and function, and cirrhosis. Biliary atresia occurs in about 1 in 10,000 to 15,000 births in the U.S. and is slightly more likely to affect females than males. Babies with this condition may appear normal at birth, but the first symptoms often start to appear by the second or third week of life.

What are the symptoms of biliary atresia?

• Yellow skin and whites of the eyes (jaundice) that appears within the first 2 to 6 weeks of life
• Pale yellow, white, or gray stool
• Dark urine
• Swollen stomach
• Abnormally enlarged liver
• By age 6 to 10 weeks: irritability, poor weight gain, increase in blood pressure
• Scarring of the liver
• Liver failure
• Less common symptoms: intense itching, heart malformations, kidney malformations, absence of the spleen, more than one spleen, and/or other anatomical abnormalities

What causes biliary atresia?

The exact cause of this condition is unknown. It is not inherited, but there are many potential contributing factors that are still being researched. Research suggests that the bile ducts are normal at first, but something causes them to be damaged in the womb or shortly after birth.

How is biliary atresia diagnosed?

Biliary atresia is diagnosed using the following procedures:

• Recording of medical and family history
• Physical exam
• Blood tests
• Ultrasound
• Hepatobiliary scan
• Liver biopsy
• Abdominal surgery to confirm diagnosis

What are the treatments for biliary atresia?

• Surgery called the Kasai procedure, which removes obstructions and allows bile to flow again
• Liver transplant
• Antibiotics to treat bile duct infections
• Special dieting plans and supplements
• Genetic counseling

Where can I find out more about biliary atresia?

• National Organization for Rare Disorders (NORD)
• MedlinePlus
• American Liver Foundation
• NIH