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36 - Choledochal cyst

from Part IV - Abdomen

Published online by Cambridge University Press:  08 January 2010

By
Takeshi Miyano
Edited by
Mark D. Stringer ,
Keith T. Oldham  and
Pierre D. E. Mouriquand
Takeshi Miyano
Affiliation:
Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine, Bunkyo-ku, Tokyo, Japan
Mark D. Stringer
Affiliation:
University of Otago, New Zealand
Keith T. Oldham
Affiliation:
Children's Hospital of Wisconsin
Pierre D. E. Mouriquand
Affiliation:
Debrousse Hospital, Lyon
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Summary

Introduction

Choledochal cyst is a congenital dilatation of the common bile duct and has the potential to become malignant. It has a hereditary predisposition, which may explain the higher incidence seen in Asia, particularly in Japan, and its familial occurrence in siblings and twins. It was first reported by Douglas in 1852 and may or may not be associated with congenital dilatation of the intrahepatic bile ducts (Fig. 36.1). Choledochal cyst is almost always associated with an abnormal junction between the pancreatic and common bile duct, i.e., pancreaticobiliary malunion. This allows pancreatic secretions to reflux into the biliary tree, and bile to flow into the pancreatic duct, causing various pathologic changes in the biliary tract, pancreas, and liver.

Because concurrent abnormalities of the pancreatic duct and intrahepatic bile ducts are almost always present, the importance of cholangiography in the planning of surgical management cannot be overemphasized. If these anomalies go unnoticed, aberrant pancreatic anatomy might be damaged, causing serious postoperative morbidity. Internal drainage of the choledochal cyst, a procedure popular in the past, has been abandoned for more than a decade now because of a prohibitive incidence of postoperative complications such as recurrent cholangitis, cholelithiasis, and biliary duct cancer. Primary cyst excision with biliary reconstruction to avoid two-way reflux of bile and pancreatic secretions is now the standard surgical treatment of choice.

Surgery for choledochal cyst is generally successful and a satisfactory surgical outcome with low morbidity is expected in the short term.

Type
Chapter
Information
Pediatric Surgery and Urology
Long-Term Outcomes
 , pp. 465 - 479
Publisher: Cambridge University Press
Print publication year: 2006

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References

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