Long-term outcomes after hepaticojejunostomy for choledochal cyst: a 10- to 27-year follow-up.
BACKGROUND
Choledochal cyst (CC) is closely associated with anomalous arrangement of the pancreaticobiliary duct, which is considered a high-risk factor for biliary tract malignancy. Early diagnosis and early treatment for CC could lead to a good prognosis. This study investigated late complications and long-term outcomes after surgery for CC.


METHODS
Fifty-six patients with CC and over 10 years of postoperative follow-up were analyzed retrospectively. All patients had undergone total resection of the extrahepatic bile duct and hepaticojejunostomy.


RESULTS
Six patients showed liver dysfunction manifested in the first 10 years after surgery, but all returned to normal thereafter. Dilatation of intrahepatic bile ducts persisted in 6 postoperatively, and in 3, this was still apparent more than 10 years after. Recurrent abdominal pain was encountered in 3, 1 had pancreas divisum with a pancreatic stone, and 1 had adhesive small bowel obstruction. Two patients developed biliary tract malignancy. A 14-year-old girl died of recurrent common bile duct cancer 2 years after the initial resection of CC with adenocarcinoma. A 26-year-old man with repeated cholangitis owing to multiple intrahepatic bile stones developed cholangiocarcinoma 26 years after the initial resection of CC. Event-free survival rate and overall survival rate were 89% (50/56) and 96% (54/56), respectively.


CONCLUSIONS
Choledochal cyst generally has an excellent prognosis with early total resection and reconstruction. Long-term surveillance for the development of malignancy is still essential, especially if there is ongoing dilatation of the intrahepatic bile duct or biliary stones.
Department of Pediatric Surgery, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto 602-8566, Japan. shige@koto.kpu-m.ac.jp
Shigeru
S
Ono
Shigehisa
S
Fumino
Shinichi
S
Shimadera
Naomi
N
Iwai
D000293:Adolescent; D000328:Adult; D001650:Bile Duct Neoplasms / Q000601:surgery; D001653:Bile Ducts, Intrahepatic / Q000601:surgery; D001662:Biliary Tract Surgical Procedures / Q000379:methods; D018281:Cholangiocarcinoma / Q000601:surgery; D015529:Choledochal Cyst / Q000601:surgery*; D003135:Common Bile Duct / Q000601:surgery; D004108:Dilatation, Pathologic / Q000601:surgery; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D016896:Treatment Outcome