Biliary atresia and biliary cysts

The authors present a review of the classification, aetiology, presentation, treatment and long-term outcome of children and adults with biliary atresia and choledochal cyst disease. Biliary atresia should be suspected in any infant with jaundice beyond the second week of life. Although the aetiology and pathogenesis remain unclear, early management with portoenterostomy has significantly improved the course of this disease. Recent advances in immunosuppression have made liver transplantation a valuable and necessary adjunct to biliary bypass. With choledochal cyst disease, adults, unlike children, often present with acute biliary tract symptoms or pancreatitis. The treatment of choice remains extrahepatic cyst excision and biliary bypass. This treatment has excellent long-term results that minimize the development of malignancy.