Choledochal Cysts 

  • Author: Michael AJ Sawyer, MD; Chief Editor: Julian Katz, MD   more...
 
Updated: Jan 4, 2012
 

Background

Choledochal cysts are congenital bile duct anomalies. These cystic dilatations of the biliary tree can involve the extrahepatic biliary radicles, the intrahepatic biliary radicles, or both. In 1723, Vater and Ezler published the anatomical description of a choledochal cyst. Douglas wrote the clinical report involving a 17-year-old girl presenting with jaundice, fever, intermittent abdominal pain, and an abdominal mass.[1] The patient died a month after an attempt at percutaneous drainage of the mass. (See image below.)

Operative specimen of type I choledochal cyst. Operative specimen of type I choledochal cyst.

In 1959, Alonzo-Lej produced a systematic analysis of choledochal cysts, reporting on 96 cases. He devised a classification system, dividing choledochal cysts into 3 categories, and outlined therapeutic strategies. Todani has since refined this classification system to include 5 categories. This article reviews the incidence, pathophysiology, diagnosis, and management of choledochal cysts.

Recent research

In a retrospective analysis of 32 children and 47 adults with choledochal cysts, Shah et al investigated the differences between these 2 groups with regard to the presentation, management, and histopathology of, as well as the outcomes related to, these lesions.[2] The following were among the authors' findings:

  • A history of biliary surgery, pancreatitis, cholangitis, early postoperative complications, and late postoperative complications occurred, respectively, 5.1, 5.4, 6.4, 2.0, and 3.3 times more frequently in adults than they did in children.
  • The classic triad of abdominal pain, jaundice, and a palpable right upper quadrant abdominal mass occurred 6.7 times more frequently in children than in adults.
  • Fibrosis of the cyst wall was peculiar to children.
  • Signs of inflammation and hyperplasia were primarily seen in adults.
  • Long-term complications occurred in 29.7% of adults but in only 9.3% of children.

The authors concluded that because of such differences, choledochal cysts in children should be considered separate entities from those in adults.

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Pathophysiology

No strong unifying etiologic theory exists for choledochal cysts. The pathogenesis is probably multifactorial.[3] In many patients with choledochal cysts, an anomalous junction between the common bile duct and the pancreatic duct can be demonstrated. This occurs when the pancreatic duct empties into the common bile duct more than 1 cm proximal to the ampulla.

Some series, such as the one published by Miyano and Yamataka in 1997, have documented such anomalous junctions in 90-100% of patients with choledochal cysts.[4] This abnormal union allows pancreatic secretions to reflux into the common bile duct, where the pancreatic proenzymes become activated, damaging and weakening the bile duct wall. Defects in epithelialization and recanalization of the developing bile ducts and congenital weakness of the ductal wall also have been implicated. The result is formation of a choledochal cyst.

These anomalies are classified according to the system published by Todani and coworkers. Five major classes of choledochal cysts exist (ie, types I-V), with subclassifications for types I and IV (ie, types IA, IB, IC; types IVA, IVB).

  • Type I cysts (see image below) are the most common and represent 80-90% of choledochal cysts. They consist of saccular or fusiform dilatations of the common bile duct, which involve either a segment of the duct or the entire duct. Type I choledochal cyst. Type I choledochal cyst.
  • Type IA is saccular in configuration and involves either the entire extrahepatic bile duct or the majority of it.
  • Type IB is saccular and involves a limited segment of the bile duct.
  • Type IC is more fusiform in configuration and involves most or all of the extrahepatic bile duct.
  • Type II choledochal cysts (see image below) appear as an isolated diverticulum protruding from the wall of the common bile duct. The cyst may be joined to the common bile duct by a narrow stalk. Type II choledochal cyst. Type II choledochal cyst.
  • Type III choledochal cysts (see image below) arise from the intraduodenal portion of the common bile duct and are described alternately by the term choledochocele. Type III choledochal cyst (choledochocele). Type III choledochal cyst (choledochocele).
  • Type IVA cysts (see image below) consist of multiple dilatations of the intrahepatic and extrahepatic bile ducts. Type IVB choledochal cysts are multiple dilatations involving only the extrahepatic bile ducts. Type IV choledochal cyst (extrahepatic and intraheType IV choledochal cyst (extrahepatic and intrahepatic disease).
  • Type V (Caroli disease) cysts (see image below) consist of multiple dilatations limited to the intrahepatic bile ducts.[4] Type V choledochal cyst (intrahepatic, Caroli diseType V choledochal cyst (intrahepatic, Caroli disease).
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Epidemiology

Frequency

United States

Choledochal cysts are relatively rare in Western countries. Reported frequency rates range from 1 case per 100,000-150,000 to 1 case per 2 million live births.

International

Choledochal cysts are much more prevalent in Asia than in Western countries. Approximately 33-50% of reported cases come from Japan, where the frequency in some series approaches 1 case per 1000 population (as described by Miyano and Yamataka).[4]

Mortality/Morbidity

The morbidities associated with choledochal cysts depend on the age of the patient at the time of presentation. Infants and children may develop pancreatitis, cholangitis, and histologic evidence of hepatocellular damage. Adults in whom subclinical ductal inflammation and biliary stasis may have been present for years may present with one or more severe complications, such as hepatic abscesses, cirrhosis, portal hypertension, recurrent pancreatitis, and cholelithiasis.

Cholangiocarcinoma is the most feared complication of choledochal cysts, with a reported incidence of 9-28%. Wu and colleagues exposed cells from a cholangiocarcinoma cell line to bile from patients with choledochal cysts and from controls with structurally normal biliary systems.[5] The bile from the patients with choledochal cysts produced significantly more mitogenic activity in the cancer cell line than the bile from the controls.

Sex

Choledochal cysts are more prevalent in females than males, with a female-to-male ratio in the range of 3:1 to 4:1.

Age

Most patients with choledochal cysts are diagnosed during infancy or childhood, although the condition may be discovered at any age. Approximately 67% of patients present with signs or symptoms referable to the cyst before the age of 10 years.[2]

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Contributor Information and Disclosures
Author

Michael AJ Sawyer, MD  Consulting Staff, Department of Surgery, Southwestern Medical Center; Consulting Staff, Department of Surgery, Comanche County Memorial Hospital; Consulting Staff, Great Plains Surgical Clinic, Inc

Michael AJ Sawyer, MD is a member of the following medical societies: American College of Surgeons, Society for Surgery of the Alimentary Tract, Society of American Gastrointestinal and Endoscopic Surgeons, and Society of Laparoendoscopic Surgeons

Disclosure: Nothing to disclose.

Coauthor(s)

Tarak H Patel, MD  Consulting Surgeon, Department of Surgery, Reynolds Army Medical Center, Fort Sill

Disclosure: Nothing to disclose.

Thomas F Murphy, MD  Chief of Abdominal Imaging Section, Department of Radiology, Tripler Army Medical Center

Disclosure: Nothing to disclose.

Fernando V Ona, MD  Associate Professor, John A Burns School of Medicine, Phillipines; Chief, Center for Digestive and Liver Diseases, Nutrition, University of Hawaii; Professor, St Luke's College of Medicine, Veterans Administration Medical Center

Fernando V Ona, MD is a member of the following medical societies: American Gastroenterological Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Mounzer Al Al Samman, MD  Department of Internal Medicine, Division of Gastroenterology, Assistant Professor, Texas Tech University School of Medicine

Mounzer Al Al Samman, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, and American Gastroenterological Association

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

BS Anand, MD  Professor, Department of Internal Medicine, Division of Gastroenterology, Baylor College of Medicine

BS Anand, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American Gastroenterological Association, and American Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Alex J Mechaber, MD, FACP  Senior Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine

Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine

Disclosure: Nothing to disclose.

Chief Editor

Julian Katz, MD  Clinical Professor of Medicine, Drexel University College of Medicine

Julian Katz, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Geriatrics Society, American Medical Association, American Society for Gastrointestinal Endoscopy, American Society of Law, Medicine & Ethics, American Trauma Society, Association of American Medical Colleges, and Physicians for Social Responsibility

Disclosure: Nothing to disclose.

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Operative specimen of type I choledochal cyst.
Type I choledochal cyst.
Type II choledochal cyst.
Type III choledochal cyst (choledochocele).
Type IV choledochal cyst (extrahepatic and intrahepatic disease).
Type V choledochal cyst (intrahepatic, Caroli disease).
Nuclear medicine scan of choledochal cyst.
Nuclear medicine scan of choledochal cyst.
Nuclear medicine scan of choledochal cyst.
Computed tomography (CT) scan of choledochal cyst demonstrating intrahepatic extension involving the main left hepatic duct.
Computed tomography (CT) scan of choledochal cyst involving the common hepatic duct.
Computed tomography (CT) scan demonstrating large choledochal cyst and adjacent gall bladder.
Computed tomography (CT) scan of large, saccular type I choledochal cyst.
Diagnostic ultrasonogram demonstrating type I choledochal cyst in a 4-month-old child presenting with hyperbilirubinemia and transaminase elevations.
Intraoperative cholangiogram of type I choledochal cyst.
Roux-en-Y hepaticojejunostomy to restore biliary-enteric continuity following resection of choledochal cyst.
 
 
 
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