Cystic Bile Duct Dilatations and Caroli’s Disease

  • Felice GiulianteEmail author
  • Agostino Maria De Rose
  • Gennaro Nuzzo


Bile duct dilatations (BDD) are rare congenital anomalies of the biliary tree characterized by cystic dilatation of the common bile duct (CBD) and/or intrahepatic bile ducts. Despite the etiology is not fully understood, the presence of an anomalous biliopancreatic junction (ABPJ) is the most widely accepted etiopathogenic hypothesis. BDD are associated with cholangitis, biliary lithiasis, and risk of cholangiocarcinoma. Cholangiography in magnetic resonance is to date the most accurate preoperative imaging technique to classify the disease according to the Todani classification. Complete cyst excision with cholecystectomy followed by Roux-en-Y hepaticojejunostomy represents the gold standard treatment for the CBD component of the disease (type I and IV BDD). A cysto-enteric anastomosis is currently thought to be a dangerous and incomplete treatment. The treatment of intrahepatic bile duct dilatations remains controversial (type IVA and V BDD). A liver resection should be considered for patients in whom the impaired intrahepatic bile ducts can be completely resected after a careful evaluation of presence and severity of underlying chronic liver and kidney disease. Liver transplantation is recommended in patients with diffuse bilobar forms of the disease complicated by repeated sepsis or to patients with cirrhosis or portal hypertension. For type II BDD a limited cyst excision is recommended; CBD resection is not routinely indicated. For type III BDD the transduodenal excision is currently replaced by conservative endoscopic sphincterotomy. The risk of development of cholangiocarcinoma that can occur even after complete cyst excision justifies a careful long-term follow-up.


Choledochal cyst Caroli’s disease Surgery Cholangiocarcinoma Cholangitis 


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Copyright information

© Springer International Publishing AG Switzerland 2015

Authors and Affiliations

  • Felice Giuliante
    • 1
    Email author
  • Agostino Maria De Rose
    • 1
  • Gennaro Nuzzo
    • 1
  1. 1.Hepatobiliary Surgery UnitCatholic University of the Sacred Heart – A. Gemelli HospitalRomeItaly

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