先天性膽管囊腫(潛在可治愈疾病)：外科治療長期隨訪結(jié)果.pdf

1、Background: Congenital bile duct cysts is a congenital disorder predisposing to intrahepatic orextrahepatic bilestones, cholangitis, pancreatitis and cholangiocarcinoma.Excision of the cysts avoids the risk of cancer

2、. The management remains difficult andconfusion persists regarding the appropriate proximal and distal extent of cystsexcision. Objective: To report the clinical presentation and long-term results of surgical tre

3、atment ofcongenital bile duct cysts in The First Affiliated Hospital of Zhejiang University. Methods: Seventy-two patients which treated for congenital bile duct cysts at The FirstAffiliated Hospital Zhejiang Uni

4、versity From January 2000 to March 2009 wereretrospectively enrolled for demographic and clinical information. The preoperativedata included patient's age at diagnosis, clinical manifesting symptoms, and theresults of la

5、boratory test. Preoperative radiographic imaging findings were reviewedto verify the morphologic condition of the biliary abnormalities. The type ofcongenital bile duct cysts according to Todani. et al classification. De

6、tails of operationwere determined. The diagnosis was confirmed by pathology report postoperatively.Long-term results of the treatment were followed-up. Conclusion: All patients with congenital bile duct cysts req

7、uire surgical intervention. Earlysurgery after diagnosis is strongly recommended to prevent type changing and the riskof malignancy development. Complete cysts removal is the goal of treatment. Thesurgical treatment of

8、patients varies according to the type of diagnosis. For type I, II,and IV, the treatment of choice is cyst excision (extrahepatic biliary tract excision)plus Roux-en-Y hepaticojejunostomy to re-establish drainage of the

9、 proximal bileducts. For type III, endoscopic sphincterectomy and cyst unroofing become thetreatment of choice besides transduodenal cyst excision or sphincteroplasty. For typeV (type I Guntz et al subclassification) and

10、 also other types of congenitalintrahepatic duct dilatation (type II and type III Guntz et al subclassification), liverresection is recommended for patients with disease confined to one lobe and livertransplantation wi

11、ll be necessary for bilobar disease. Long-term follow-up post operatively has showed the dominant advantage of thesurgical treatment which resulted curable condition for congenital bile duct cystspatients without rep