Forme fruste choledochal cyst

Four patients had the characteristic features of choledochal cyst except for the cystic component. All patients had stenosis of the distal common bile duct, a "long common channel" secondary to a proximal junction of the common bile and pancreatic ducts, cholecystitis and the classic pathological microscopic features of choledochal cyst in the wall of the common bile duct. Three children had coexisting intrahepatic duct cysts and/or stenosis and one had intrahepatic choledocholithiasis. The clinical presentations were cholangitis (2), pancreatitis (1) and biliary obstruction (1). In all cases the common bile duct was resected and biliary reconstruction was carried out by choledochojejunostomy (Roux-en-Y). Morbidity was minor except in one patient with ductal disease extending far into the intrahepatic ducts. This child developed an anastomotic stricture requiring revision of the anastomosis and long-term "U" tube stenting. Forme fruste choledochal cyst appears to be another variation in the spectrum of pancreaticobiliary malformations of choledochal cyst. Treatment is identical, that is, excision of all malformed ductal tissue.