A jaundiced 6 week old infant has biliary atresia. Which of the following statements are true?

a. Portoenterostomy is the initial procedure of choice

c. Approximately two-thirds of patients managed with portoenterostomy will develop chronic liver disease sufficient to indicate liver transplantation

Biliary atresia is an idiopathic process in which the extrahepatic biliary ducts are replaced in whole or in part with dense fibrous tissue containing evidence of both acute and chronic inflammation. There is an intrahepatic component as well. Although antiinflammatory therapy is of some theoretical interest, there are no data to suggest that antiinflammatory pharmocologic therapy will influence the natural history of liver disease associated with biliary atresia.

The approach for the usual infant in whom biliary atresia is discovered within the first 90 days of life is to confirm the suspected diagnosis by operative cholangiogram at laparotomy and then proceed with portoenterostomy. In general terms, one-third of these infants do well on a long-term basis, one-third have prompt failure, and the remainder have chronic liver disease that becomes problematic more slowly. Therefore, approximately two-thirds of these patients develop chronic liver disease for which liver transplantation is a reasonable alternative. Hepatic transplantation is best considered a necessary and complementary approach to portoenterostomy for infants with biliary atresia. Data support its use in infants with failed portoenterostomy or in older infants with established cirrhosis at the time of presentation. Growth failure, hepatic synthetic failure and sequelae of portal hypertension are indications to proceed with transplantation.