Objective:To report the clinical presentation and long-term results of surgical treatment of congenital bile duct cysts in The First Affiliated Hospital of Zhejiang University.Background:Congenital bile duct cysts is a congenital disorder predisposing to intrahepatic or extrahepatic bile duct stones,cholangitis,pancreatitis and cholangiocarcinoma. Excision of the cysts avoids the risk of cancer.The management remains difficult and confusion persists regarding the appropriate proximal and distal extent of cysts excision.Methods:Seventy-two patients which treated for congenital bile duct cysts at The First Affiliated Hospital Zhejiang University From January 2000 to March 2009 were retrospectively enrolled for demographic and clinical information.The preoperative data included patient’s age at diagnosis,clinical manifesting symptoms,and the results of laboratory test.Preoperative radiographic imaging findings were reviewed to verify the morphologic condition of the biliary abnormalities.The type of congenital bile duct cysts according to Todani et al classification.Details of operation were determined.The diagnosis was confirmed by pathology report postoperatively. Long-term results of the treatment were followed-up. Results:Seventy-two adults,range in 13 to 71 years old,with mean(SD) age at diagnosis was 42 years.There are all Chinese,26 male(36%) and 46 female(64%).The primary presenting symptom in 61 patients(84,7%) was abdominal pain,15 patients (20,8%) with jaundice or impaired liver function,9 patients(12,5%) had fever,7 patients(9,7%) complained weight loss,and 3 patients(4,2%) asymptomatic.Seven patients(9,7%) had episode of cholangitis,2 patients(2,8%) had pancreatitis.Accuracy rate of imaging studies USG,CT scan,MRCP in diagnosis congenital bile duct cysts were 84%,89%,and 98%,respectively.According to Todani et al classification;44 patients(61,1%) were classified as typeⅠ;3 patients(4,2%) typeⅡ; 1 patient(1,4%) typeⅢ;19 patients(26,4%) typeⅣa;and 5 patients(6,9%) typeⅤ(Caroli’s disease,Guntz type 1).Endoscopic sphinterectomy(EST) procedure was performed for 1 patient(typeⅢ),cyst(diverticulum) excision for 2 patients(typeⅡ),cyst excision plus Roux-en-Y hepaticojejunostomy for 54 patients(44 typeⅠand 10 typeⅣa),cyst excision and liver resection plus Roux-en-Y hepaticojejunostomy for 7 patients(typeⅣa),and liver resection(left hepatectomy) for 3 patients(typeⅤ,unilobar).Two patients(typeⅤ,bilobar) went to liver transplantation.One asymptomatic patient(typeⅡ) and 2 patients(typeⅣa) who refused surgery were managed conservatively.During operation,biliary stones and cholangiocarcinoma were present in 19 patients(26,4%) and 6 patients(8,3%),respectively.Five of 7 patients reporting weight loss were found to have cholangiocarcinoma within the abnormal biliary tree.During a median follow-up of 43 months,we found 3 patients which had T3 cholangiocarcinoma were died after 6-15 months post operation.One patient who had T2 cholangiocarcinoma previously,was found to have recurrent cholangiocarcinoma. Other findings were 1 patient(typeⅣa) who refused surgery and 1 patient(typeⅡ) had cyst(diverticulum) excision suffered from cholangiocarcinoma after 2 years discharged from our hospital.There were no cholangitis and bile duct cysts recurrence findings in all post operative patients. Conclusion:All patients with congenital bile duct cysts require surgical intervention.Early surgery after diagnosis is strongly recommended to prevent type changing and the risk of malignancy development.Complete cysts removal is the goal of treatment.The surgical treatment of patients varies according to the type of diagnosis.For typeⅠ,Ⅱ, andⅣ,the treatment of choice is cyst excision(extrahepatic biliary tract excision) plus Roux-en-Y hepaticojejunostomy to re-establish drainage of the proximal bile ducts.For typeⅢ,endoscopic sphincterectomy and cyst unroofing become the treatment of choice besides transduodenal cyst excision or sphincteroplasty.For typeⅤ(typeⅠGuntz et al subclassification) and also other types of congenital intrahepatic duct dilatation(typeⅡand typeⅢGuntz et al subclassification),liver resection is recommended for patients with disease confined to one lobe and liver transplantation will be necessary for bilobar disease.Long-term follow-up post operatively has showed the dominant advantage of the surgical treatment which resulted curable condition for congenital bile duct cysts patients without repeated cholangitis and cysts recurrence.The surgical treatment could prevent the development of cholangiocarcinoma in congenital bile duct cysts definitely.