biliary atresia
Pronunciation: BIL-ee-air-ee uh-TREE-zhuh
A neonatal cholestatic liver disease characterised by progressive obliteration or absence of the extrahepatic bile ducts, leading to cirrhosis if untreated.
Full Definition
Biliary atresia is the most common cause of cholestatic jaundice requiring surgical intervention in neonates and the leading indication for pediatric liver transplantation worldwide. The condition involves inflammatory fibrosclerotic destruction of the extrahepatic biliary tree, with the intrahepatic ducts often secondarily involved. The Kasai portoenterostomy (hepatic portoenterostomy) is the initial surgical procedure aimed at restoring bile flow; its success depends critically on age at operation. Histological features include bile duct proliferation, portal fibrosis, and intracanalicular bile plugs. Editors must not confuse 'biliary atresia' with 'neonatal hepatitis', which is a distinct entity despite overlapping clinical presentation.
Usage
Usage note: Do not abbreviate to 'BA' without prior definition in formal documents; 'EHBA' (extrahepatic biliary atresia) is the preferred abbreviation in some specialist texts.
In Context
- "The Kasai portoenterostomy was performed at 52 days of life for confirmed biliary atresia." — Surgical operative note
- "Liver biopsy at six weeks demonstrated florid bile duct proliferation and portal tract expansion, supportive of biliary atresia." — Pediatric pathology report