Congenital choledochal dilatation: Classification, clinical
features, and long-term results
Takuji Todani
J Hep Bil Pancr Surg 4: 276-282, 1997
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.
Return