Table of Contents
Biliary Atresia Definition
Biliary Atresia – absence or malformation of the bile ducts, also called neonatal cholestasis. Biliary atresia is nearly always congenital (present at birth). In some infants biliary atresia appears to develop as a consequence of an inflammatory process that occurs shortly after birth, destroying the bile ducts. Biliary atresia prevents the flow of bile from the liver, causing toxins to accumulate in the liver.
Symptoms of Biliary Atresia
Symptoms depend on the extent of the atresia and may become apparent within days of birth or manifest slowly over the first six months of life. Symptoms include
- Jaundice, a yellowish discoloration of the skin resulting from the liver’s inability to break down bilirubin into components the body can excrete as waste
- Stools that are pale in color, the consistency of clay, and unusually foul smelling
- Dark urine
- Failure to grow or gain weight
- Abdominal distention resulting from enlarged spleen
- Fussiness and irritability
Neonatal jaundice is fairly common, affecting about 50 percent of full-term and 80 percent of preterm (premature) newborns. It points to biliary atresia or other liver conditions only when it is apparent within the first 24 hours after birth or persists despite treatment.
The diagnostic path includes blood tests to measure the amounts of bilirubin in the blood and percutaneous liver biopsy to determine whether the hepatocytes, the cells that process bilirubin, are normal. Normal hepatocytes strongly suggest biliary atresia. Other diagnostic procedures may include ultrasound and intraoperative cholangiography (injecting dye into the bile ducts to visualize them using fluoroscopy or other imaging technologies).
Biliary Atresia Treatment
The only treatments for biliary atresia are surgical procedures to help restore the flow of bile. The first of these procedures is hepatic portoenterostomy, in which the surgeon creates an opening between the jejunum (middle segment of the small intestine) and the bile duct structures that exist outside the liver. This procedure allows bile to drain directly from the liver into the small intestine and can achieve adequate liver function for up to several years.
However, it does not correct the structural defects of the bile transport network within the liver, and damage to the liver (fibrosis and cirrhosis) continues. Nearly all infants who have biliary atresia require liver transplantation, the second surgical procedure to treat the condition, before they are three years old. Long-term success of liver transplantation depends on numerous variables.
Page last reviewed: