Surgical treatment of choledochal cysts

Biliary cystic disease is uncommon in Asia and very rare in Europe and the Americas. Patients with biliary cysts may present as infants, children, or adults. When patients present as adults, they are more likely to have stones in the gallbladder, common duct, or intrahepatic ducts and to present with biliary colic, acute cholecystitis, cholangitis, or gallstone pancreatitis. With increasing age at presentation, the risks of intrahepatic strictures and stones, segmented hepatic atrophy/hypertrophy, secondary biliary cirrhosis, portal hypertension, and biliary malignancy all increase significantly. Factors to be considered when performing surgery on patients with biliary cystic disease include: (1) age, (2) presenting symptoms, (3) cyst type, (4) associated biliary stones, (5) prior biliary surgery, (6) intrahepatic strictures, (7) hepatic atrophy/hypertrophy, (8) biliary cirrhosis, (9) portal hypertension, and (10) associated biliary malignancy. In general, regardless of age, presenting symptoms, biliary stones, prior surgery or other secondary problems, surgery should include cholecystectomy and excision of extrahepatic cyst(s). With respect to the distal bile duct, the surgical principle should be excision of a portion of the intrapancreatic bile duct with care to not injure the pancreatic duct or a long common channel. Resection of the pancreatic head should be reserved for patients with an established malignancy. With respect to the intrahepatic ducts, surgery should be individualized depending on whether (1) both lobes are involved, (2) strictures and stones are present, (3) cirrhosis has developed, or (4) an associated malignancy is localized or metastatic. When the liver is not cirrhotic, hepatic parenchyma should be preserved even when strictures and stones are present. If cirrhosis is advanced, hepatic transplantation may be indicated, but this sequence of events is unusual. If a malignancy has developed, oncologic principles should be followed. Whenever possible, resection of a localized tumor including adjacent hepatic parenchyma and regional lymph nodes should be performed.