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Special Feature
October 1, 2007

Image of the Month—Diagnosis

Arch Surg. 2007;142(10):1008. doi:10.1001/archsurg.142.10.1008
Answer: Choledochal Cyst, Todani Type I—Diagnosis

Bile duct cysts (BDCs) are rare (1 in 1300 to 1 in 2 million live births), constituting less than 1% of benign biliary diseases.1,2Typically, affected children exhibit intermittent jaundice along with a palpable and painful abdominal mass. Twenty percent of patients are adults with right upper abdominal discomfort.3Surgery is performed in an effort to reduce short- and long-term cyst-associated complications such as cholecystitis, cholangitis, pancreatitis, intrahepatic abscesses or liver cirrhosis, portal hypertension, and malignant changes, all of which occur more frequently in adults.4In our patient, the cyst was completely resected, followed by classic Roux-en-Y anastomosis to the common hepatic duct. Noninvasive diagnostic imaging includes ultrasonography, computed tomography, or magnetic resonance cholangiopancreatography, with endoscopic retrograde cholangiopancreatography as an invasive option. The new standard criterion seems to be magnetic resonance cholangiopancreatography because its sensitivity is similar to that of endoscopic retrograde cholangiopancreatography and it is associated with less risk than endoscopic retrograde cholangiopancreatography.

The cause of BDC remains unclear because any potentially important observations are based on fewer than 4000 documented cases.2One accepted hypothesis relies on the long common channel theory.5An anomaly of the pancreaticobiliary junction leading to a common channel longer than 15 mm may cause reflux of pancreatic juices into the biliary system, resulting in inflammation, ectasia, and, finally, cystic dilatations of the biliary ducts.5Not all patients with an anomalous pancreaticobiliary junction have BDCs. Dysfunction of the sphincter of Oddi or oligoganglionosis, leading to Hirschsprung disease of the bile duct, may also contribute to BDC formation.

Because cyst location determines the type of surgery required, most authors opt for the 1977 classification of Todani et al.3Type I cysts show cystic dilatations of the extrahepatic bile ducts; type II, a diverticulum of the extrahepatic bile duct; and type III, cystic dilatation or diverticulum of the intraduodenal portion of the choledochal duct. Type IV-A cysts consist of multiple intrahepatic and extrahepatic cysts; type IV-B exhibit only multiple extrahepatic cysts. Type V cysts, also known as Caroli disease, are solitary or multiple purely intrahepatic cysts. The most common cyst is type I (79%), followed by type IV (13%), type III (4%), type II (2.6%), and type V (<1%).6

As many as 80% of patients with BDCs have complications, with calculi in as many as 72%.7Cholecystitis is also common, and pancreatitis occurs in 30% to 70% of patients.6-8The lifetime risk of malignant changes is 10% to 30%, and cholangiocarcinomas are as much as 20 times more common in patients with BDCs compared with the general population.4,9,10Tumors arise within the cyst, the pancreaticobiliary system, or the liver parenchyma, sometimes even after cyst removal,7,10with the greatest risk associated with type I and type IV cysts.10

Before the 1980s, the favored treatment of BDCs was enterocystostomy. Owing to higher morbidity and increased risk of malignant changes of the gallbladder, the remaining cyst, and the bile ducts, this is no longer an option.4,9,10Type I cysts require resection of the intrapancreatic portions of the bile duct; pancreaticoduodenectomy is reserved for existing malignant changes. Proximal resection margins should extend up to the hepatic bifurcation to minimize the risk of postoperative strictures. Cyst resection and cholecystectomy are required for type II cysts; usually, small type III cysts (choledochoceles) are removed by transduodenal excision or endoscopic sphincterotomy. Type IV-A and IV-B cysts require complete resection of the extrahepatic bile ducts. Liver transplantation is the ultimate treatment option for bilobar cystic lesions or Caroli disease with cirrhosis or portal hypertension.

Correspondence:Juerg Metzger, MD, Department for Visceral Surgery, Cantonal Hospital Lucerne, Spitalstrasse 6000, Lucerne 16, Switzerland (juerg.metzger@ksl.ch).

Accepted for Publication:October 2, 2006.

Author Contributions:Study concept and design: Banz, Mahler, Treumann, and Metzger. Acquisition of data: Banz, Mahler, and Metzger. Analysis and interpretation of data: Banz, Mahler, and Metzger. Drafting of the manuscript: Banz and Mahler. Critical revision of the manuscript for important intellectual content: Banz, Mahler, and Treumann. Study supervision: Treumann.

Financial Disclosure:None reported.

Saxena  RPradeep  RChander  J  et al.  Benign disease of the common bile duct.  Br J Surg 1988;75 (8) 803- 806PubMedGoogle ScholarCrossref
Yamaguchi  M Congenital choledochal cyst: analysis of 1,433 patients in the Japanese literature.  Am J Surg 1980;140 (5) 653- 657PubMedGoogle ScholarCrossref
Todani  TWatanabe  YNarusue  MTabuchi  KOkajima  K Congenital bile duct cysts: classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst.  Am J Surg 1977;134 (2) 263- 269PubMedGoogle ScholarCrossref
Hewitt  PMKrige  JEBornman  PCTerblanche  J Choledochal cysts in adults.  Br J Surg 1995;82 (3) 382- 385PubMedGoogle ScholarCrossref
Babbitt  DP Congenital choledochal cysts: new etiological concept based on anomalous relationships of the common bile duct and pancreatic bulb [article in multiple languages].  Ann Radiol (Paris) 1969;12 (3) 231- 240PubMedGoogle Scholar
Nagorney  DM Bile duct cysts in adults. Blumgart  LHFong  Y Surgery of the Liver and Biliary Tract. Vol 2. Philadelphia, Pa WB Saunders Co2000;1229- 1244Google Scholar
Nagorney  DMMcIlrath  DCAdson  MA Choledochal cysts in adults: clinical management.  Surgery 1984;96 (4) 656- 663PubMedGoogle Scholar
Swisher  SGCates  JAHunt  KK  et al.  Pancreatitis associated with adult choledochal cysts.  Pancreas 1994;9 (5) 633- 637PubMedGoogle ScholarCrossref
Liu  CLFan  STLo  CMLam  CMPoon  RTWong  J Choledochal cysts in adults.  Arch Surg 2002;137 (4) 465- 468PubMedGoogle ScholarCrossref
Ishibashi  TKasahara  KYasuda  YNagai  HMakino  SKanazawa  K Malignant change in the biliary tract after excision of choledochal cyst.  Br J Surg 1997;84 (12) 1687- 1691PubMedGoogle ScholarCrossref