0
We're unable to sign you in at this time. Please try again in a few minutes.
Retry
We were able to sign you in, but your subscription(s) could not be found. Please try again in a few minutes.
Retry
There may be a problem with your account. Please contact the AMA Service Center to resolve this issue.
Contact the AMA Service Center:
Telephone: 1 (800) 262-2350 or 1 (312) 670-7827  *   Email: subscriptions@jamanetwork.com
Error Message ......
Special Feature |

Image of the Month—Diagnosis FREE

Arch Surg. 2011;146(10):1213. doi:10.1001/archsurg.2011.263-b.
Text Size: A A A
Published online

Choledochocele is a rare type of choledochal cyst (type III according to Todani classifications1) characterized by cystic dilatation of the bile duct terminal and protrusion of the papilla of Vater into the duodenal lumen.2 This anomaly has a prevalence of 0.3% to 0.4% among endoscopic retrograde cholangiopancreatography recipients and 1% to 5% among choledochal cysts.3

Horaguchi et al2 have proposed a practical classification of choledochoceles into 2 types. In type I, the bile duct terminal forms a choledochocele and opens to the duodenum independently from the pancreatic duct or forms a very short common channel with the pancreatic duct. In type II, the pancreatic duct opens to the choledochocele. Type II occurs in 10% of choledochoceles and represents an anomalous arrangement of the pancreatobiliary ductal systems.2

Choledochocele is an extremely rare entity in the medical literature. Edil et al,4 in the largest series of the western world for choledochal cysts, reported 4 cases of choledochocele in 92 patients. Singham et al,5 from Canada, in the second largest series reported 1 case in 51 patients. Choledochoceles usually remain asymptomatic or may cause symptoms similar to those of choledocholithiasis, such as intermittent upper abdominal pain and jaundice. The diagnosis is difficult and in asymptomatic cases can be missed even at cholecystectomy.6 Stones in the choledochocele or the bile duct may induce acute pancreatitis and cholangitis.

Preoperative diagnosis of choledochocele is mainly based on endoscopic ultrasonography, magnetic resonance cholangiopancreatography, and endoscopic retrograde cholangiopancreatography. In upper gastrointestinal endoscopy, choledochocele may appear similar to duodenal duplication cyst, papillitis, papillary neoplasm, or mucosal elevation because of inadvertent submucosal injection of water or contrast material during endoscopic retrograde cholangiopancreatography.6,7 Impacted gallstones at the papilla of Vater or cystic changes at the bile duct terminal after endoscopic sphincterectomy for lithiasis may mimic choledochocele.

Choledochocele can be accompanied by lithiasis due to endocystic cholestasis. In a collection of 85 cases from the world literature, Mascetti et al8 found lithiasis in 43%. Coexistent biliary malignancy in choledochocele, as described in the literature, is rare and occurs in 2.5% to 4% of cases. Choledochocele entails usually little risk of bile duct malignancy compared with the other types of choledochal cysts, as it is not associated with abnormal junction between the pancreatic and common bile ducts and the mucosal line is usually duodenal and not biliary.9 However, certain authors report higher rates of concomitant biliary malignancy. Horaguchi et al2 reported 14.3% prevalence of coexisting malignancy in 21 patients.

The surgical management of type III cysts is less clear and controversial.2,4 In asymptomatic patients, close observation for complications of lithiasis with or without ductal obstruction, pancreatitis, and possible malignancy is recommended. Endoscopic sphincteroplasty or unroofing of the cyst is usually an adequate treatment.4 Radical surgery, such as pancreaticoduodenectomy, is seldom necessary, only if there is a high suspicion for biliary malignancy.4 In symptomatic cases, with stones entrapped within the sac of choledochocele, as occurred in our patient, surgical exploration and stone extraction is recommended.4,6

Our patient had a type I choledochocele, according to the Horaguchi et al classification,2 as there was a very short common pancreatobiliary channel. She was symptomatic with lithiasis and inflammation and the surgical treatment was very effective.

Return to Quiz Case.

Correspondence: Evangelos P. Misiakos, MD, Third Department of Surgery, University of Athens School of Medicine, Rimini 1, Chaidari, Athens 124 62, Greece (misiakos@med.uoa.gr).

Accepted for Publication: June 30, 2010.

Author Contributions:Study concept and design: A. Machairas and Misiakos. Acquisition of data: N. Machairas. Analysis and interpretation of data: Petropoulos, N. Machairas, Charalabopoulos, and Misiakos. Drafting of the manuscript: Petropoulos, N. Machairas, and Charalabopoulos. Critical revision of the manuscript for important intellectual content: A. Machairas. Study supervision: A. Machairas and Misiakos.

Financial Disclosure: None reported.

Todani T, Watanabe Y, Narusue M, Tabuchi K, Okajima 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):263-269
PubMed   |  Link to Article
Horaguchi J, Fujita N, Noda Y,  et al.  Choledochocele associated with superficial spreading cancer with cholesterolosis of the bile duct.  J Gastroenterol. 2007;42(4):318-324
PubMed   |  Link to Article
Horaguchi J, Fujita N, Kobayashi G, Noda Y, Ito K, Takasawa O. Clinical study of choledochocele: is it a risk factor for biliary malignancies?  J Gastroenterol. 2005;40(4):396-401
PubMed   |  Link to Article
Edil BH, Cameron JL, Reddy S,  et al.  Choledochal cyst disease in children and adults: a 30-year single-institution experience.  J Am Coll Surg. 2008;206(5):1000-1008
PubMed   |  Link to Article
Singham J, Schaeffer D, Yoshida E, Scudamore C. Choledochal cysts: analysis of disease pattern and optimal treatment in adult and paediatric patients.  HPB (Oxford). 2007;9(5):383-387
PubMed   |  Link to Article
Becker CD, Nagy AG, Gibney RG, Burhenne HJ. Diagnosis and treatment of choledochocele complicated by choledocholithiasis (case report).  Gastrointest Radiol. 1987;12(4):322-324
PubMed   |  Link to Article
Adamek HE, Schilling D, Weitz M, Riemann JF. Choledochocele imaged with magnetic resonance cholangiography.  Am J Gastroenterol. 2000;95(4):1082-1083
PubMed   |  Link to Article
Masetti R, Antinori A, Coppola R,  et al.  Choledochocele: changing trends in diagnosis and management.  Surg Today. 1996;26(4):281-285
PubMed   |  Link to Article
Benjamin IS. Biliary cystic disease: the risk of cancer.  J Hepatobiliary Pancreat Surg. 2003;10(5):335-339
PubMed   |  Link to Article

Figures

Tables

References

Todani T, Watanabe Y, Narusue M, Tabuchi K, Okajima 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):263-269
PubMed   |  Link to Article
Horaguchi J, Fujita N, Noda Y,  et al.  Choledochocele associated with superficial spreading cancer with cholesterolosis of the bile duct.  J Gastroenterol. 2007;42(4):318-324
PubMed   |  Link to Article
Horaguchi J, Fujita N, Kobayashi G, Noda Y, Ito K, Takasawa O. Clinical study of choledochocele: is it a risk factor for biliary malignancies?  J Gastroenterol. 2005;40(4):396-401
PubMed   |  Link to Article
Edil BH, Cameron JL, Reddy S,  et al.  Choledochal cyst disease in children and adults: a 30-year single-institution experience.  J Am Coll Surg. 2008;206(5):1000-1008
PubMed   |  Link to Article
Singham J, Schaeffer D, Yoshida E, Scudamore C. Choledochal cysts: analysis of disease pattern and optimal treatment in adult and paediatric patients.  HPB (Oxford). 2007;9(5):383-387
PubMed   |  Link to Article
Becker CD, Nagy AG, Gibney RG, Burhenne HJ. Diagnosis and treatment of choledochocele complicated by choledocholithiasis (case report).  Gastrointest Radiol. 1987;12(4):322-324
PubMed   |  Link to Article
Adamek HE, Schilling D, Weitz M, Riemann JF. Choledochocele imaged with magnetic resonance cholangiography.  Am J Gastroenterol. 2000;95(4):1082-1083
PubMed   |  Link to Article
Masetti R, Antinori A, Coppola R,  et al.  Choledochocele: changing trends in diagnosis and management.  Surg Today. 1996;26(4):281-285
PubMed   |  Link to Article
Benjamin IS. Biliary cystic disease: the risk of cancer.  J Hepatobiliary Pancreat Surg. 2003;10(5):335-339
PubMed   |  Link to Article

Correspondence

CME
Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.
NOTE:
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).
Submit a Comment

Multimedia

Some tools below are only available to our subscribers or users with an online account.

Related Content

Customize your page view by dragging & repositioning the boxes below.

Articles Related By Topic
Related Topics
PubMed Articles
JAMAevidence.com

The Rational Clinical Examination
Make the Diagnosis: Cancer, Family History

The Rational Clinical Examination
Original Article: Does This Patient Have a Family History of Cancer?