Congenital choledochal dilatation: Classification, clinical features, and long-term results

Choledochal cyst is generally accompanied by pancreatobiliary malunion. Pancreatic juice usually refluxes into the bile duct via the malunion, and bile juice occasionally regurgitates into the pancreatic duct. As a result, various pathological conditions occur in the biliary tract, pancreas, and liver. We have revised our classification of choledochal cyst on the basis of malunion. Abdominal mass, pain, and jaundice are regarded as the triad of choledochal cysts, but symptoms are quite non-specific. In neonates and infants less than 12 months of age, choledochal cyst is usually huge and no cylindrical dilatation is observed. Vomiting and jaundice with acholic stool are often observed. No hyperamylasemia is seen, despite the presence of a malunion. Whereas children over 13 months of age tend to show diffuse dilatation, and an abdominal mass is rarely palpable. Epigastralgia with hyperamylasemia, fever, vomiting, and slight jaundice are often observed. Cyst excision is the treatment of choice to prevent the development of ascending cholangitis and cancer in the cyst. Late complications of excisional surgery include recurrent cholangitis, pancreatitis, and rarely, biliary malignancy. These complications usually develop 5 years or more after initial surgery and become the object of reoperation. Complete removal of the extrahepatic bile duct from the hepatic hilum to the intrapancreatic duct and a hilar hepaticoenterostomy with wide anastomosis after ductoplasty, including the coexisting primary stricture, are essential at the primary surgery. Provided a large anastomosis, favorable results can be obtained with either duodenostomy or Roux-Y jejunostomy. Surgical enlargement of the hilar ducts and careful lifelong follow-up should be routine in all patients who have undergone cyst excision. When ductal stricture with intrahepatic gallstones is confirmed, reoperation at an early stage is indicated to obtain a good quality of life with short hospitalization.