Biliary Atresia

Biliary atresia is a progressive fibro-obliterative cholangiopathy of infancy. The extrahepatic bile ducts—and often portions of the intrahepatic ducts—become inflamed, scarred, and obstructed. Bile cannot reach the intestine, so conjugated bilirubin accumulates in the blood and progressive liver injury develops. Untreated disease advances to biliary cirrhosis and is fatal; biliary atresia is also the leading indication for liver transplantation in children. The classic infant is jaundiced at or after 2 weeks of age, has pale or acholic stools, dark urine, and hepatomegaly. The infant may otherwise look well and feed normally. That apparent wellness is a trap: serious obstructive liver disease can be evolving before the infant looks acutely ill. Physiologic newborn jaundice should be resolving during the first 1–2 weeks. Breast-milk jaundice is generally unconjugated and can persist longer, but it must not be assumed until conjugated hyperbilirubinemia has been excluded. Pale, clay-coloured stools are never a normal explanation for prolonged jaundice. A three-week-old infant with yellow skin, dark urine, and a nearly white stool needs fractionated bilirubin testing and prompt escalation—not reassurance that the infant is simply breastfed.