In plain words
Biliary atresia is a rare condition in which the tubes that carry bile from the liver to the intestine are blocked or missing. Bile cannot drain, so the liver can be damaged and builds up in the body. Babies with it are often born looking healthy. The first sign is usually that does not go away, often with pale or clay-colored stools and dark urine. Stool color is one reason clinicians ask families to watch the diaper. The condition is found in roughly one in 8,000 to 18,000 births, with differences by region. Surgery performed early gives the best chance of draining bile. Most babies with jaundice do not have this condition. Clinicians check jaundice that lasts past about two weeks for this reason.
The deeper version
Biliary atresia is an obliterative cholangiopathy of the extrahepatic bile ducts, with onset in fetal or early neonatal life, and it is the leading indication for liver transplantation in children. Its incidence is commonly estimated at about 1 in 8,000 to 18,000 live births, varying by region and population (Hartley, Davenport, and Kelly, Lancet, 2009). The diagnostic pathway combines a rising conjugated bilirubin with ultrasound, hepatobiliary scintigraphy, and, when needed, liver biopsy and intraoperative cholangiography. Earlier portoenterostomy, ideally in the first weeks of life, is associated with better bile drainage and survival with the native liver. Screening for prolonged jaundice is a recognized step in evaluation. The condition is distinct from and from physiologic jaundice, which typically resolve on their own.
Where it comes up
- Newborn jaundiceBaby Basics
- Diaper counts and what newborn poop looks likeBaby Basics
- Feeding a baby with jaundiceFeeding Your Baby
- When to call the pediatrician (and when to go in)Baby Basics
- Pediatrician visits in the first weeksBaby Basics
What mothers here say about biliary atresia
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Sources
- Hartley JL, Davenport M, Kelly DA — Biliary atresia. The Lancet, 2009
- Kemper AR and colleagues, American Academy of Pediatrics — Clinical Practice Guideline Revision: Management of Hyperbilirubinemia in the Newborn Infant, 2022