Biliary atresia is an obstructive cholangiopathy of unknown etiology involving both the intrahepatic and extrahepatic bile ducts. It presents in the neonatal period with persistent jaundice, clay-colored stools, and hepatomegaly. It is fatal if left untreated with a reported survival of less than 10% at 3 years of age.
The earliest reported reference of the condition was in 1817 by Dr. John Burns, who described it as an incurable state of the biliary apparatus. Later on, the first surgical success was achieved by Dr. William Ladds in 1920, but biliary atresia continued to have a dismal outcome until the 1950s when Dr. Morio Kasai first described the Kasai porto-enterostomy by dissecting the proximal obstructed biliary tract and creating a Roux-en-Y loop. It is now considered the standard procedure and is offered to all children undergoing surgical correction. With the progress and advancement of transplantation surgery, liver transplantation is an option available to children who have either failed to restore the bile flow in the initial Kasai surgery or have developed advanced liver cirrhosis.