Biliary Atresia
Progressive obliteration of bile ducts in newborns, requiring urgent surgical intervention to prevent liver failure.
Full Definition
Biliary atresia is a rare pediatric condition characterized by progressive inflammation and obliteration of the extrahepatic and sometimes intrahepatic bile ducts, occurring in approximately 1 in 18,000 live births. The condition typically presents in the first few weeks of life with jaundice, acholic stools, and hepatomegaly. Without surgical intervention via the Kasai procedure (hepatoportoenterostomy), progressive liver fibrosis and cirrhosis develop rapidly. Even with timely surgery, many patients eventually require liver transplantation, making this condition the leading indication for pediatric liver transplantation worldwide.
Usage
Usage note: Often abbreviated as BA in pediatric surgical literature.
In Context
- "The Kasai procedure was performed at 6 weeks of age for suspected biliary atresia." — operative report
- "Liver biopsy confirmed the diagnosis of biliary atresia with advanced fibrosis." — pathology report