eMedicine Specialties > Gastroenterology > Biliary

Choledochal Cysts

Author: Michael AJ Sawyer, MD, Director, Videoendoscopic Surgical Institute of Oklahoma, Consulting Staff, Department of Surgery, Comanche County Memorial Hospital; Consulting Staff, Great Plains Surgical Clinic, Lawton, Oklahoma
Coauthor(s): Tarak H Patel, MD, Consulting Surgeon, Department of Surgery, Reynolds Army Medical Center, Fort Sill; Thomas F Murphy, MD, Chief of Abdominal Imaging Section, Department of Radiology, Tripler Army Medical Center; 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
Contributor Information and Disclosures

Updated: Jun 8, 2007

Introduction

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.

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.

Pathophysiology

No strong unifying etiologic theory exists for choledochal cysts. The pathogenesis is probably multifactorial. 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.2 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 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 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 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 III choledochal cysts arise from the intraduodenal portion of the common bile duct and are described alternately by the term choledochocele.
  • Type IVA cysts 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 V (Caroli disease) consists of multiple dilatations limited to the intrahepatic bile ducts.

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).2

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 recently exposed cells from a cholangiocarcinoma cell line to bile from patients with choledochal cysts and from controls with structurally normal biliary systems.3 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.

Clinical

History

The history varies according to the age at presentation. Choledochal cysts can present dramatically in infancy. The clinical manifestations in older children and adults are more protean.

  • Infants
    • Infants frequently present with jaundice and acholic stools. In early infancy, this may prompt a workup for biliary atresia.
    • In addition, infants with choledochal cysts often have a palpable mass in the right upper quadrant of the abdomen, accompanied with hepatomegaly.
  • Children
    • Children diagnosed after infancy typically have a clinical picture of intermittent biliary obstruction or recurrent bouts of pancreatitis.
    • Those with a biliary obstructive pattern can still present with a palpable right upper quadrant mass and jaundice.
    • Children whose primary manifestation is pancreatitis may pose some difficulty in arriving at the correct diagnosis. These patients frequently have only intermittent attacks of colicky abdominal pain. Biochemical testing reveals elevated amylase and lipase concentrations, which lead to the proper diagnostic workup.
  • Adults
    • Adults with choledochal cysts can present with one or more severe complications.
    • Frequently, adults with choledochal cysts complain of vague epigastric or right upper quadrant pain and can develop jaundice or cholangitis.
    • The most common symptom in adults is abdominal pain.
    • A classic triad of abdominal pain, jaundice, and a palpable right upper quadrant abdominal mass has been described in adults with choledochal cysts but is found in only 10-20% of patients.

Physical

A right upper quadrant mass may be palpable. This is observed more frequently in infancy and early childhood. Patients who develop pancreatitis present with nonspecific midepigastric or diffuse abdominal pain.

Causes

No unifying etiologic theory exists for choledochal cysts. The pathogenesis probably is multifactorial.

More on Choledochal Cysts

Overview: Choledochal Cysts
Differential Diagnoses & Workup: Choledochal Cysts
Treatment & Medication: Choledochal Cysts
Follow-up: Choledochal Cysts
Multimedia: Choledochal Cysts
References
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References

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Further Reading

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Keywords

bile duct cysts, congenital bile duct anomalies, biliary tree, extrahepatic biliary radicles, intrahepatic biliary radicles, upper abdominal mass, jaundice, Caroli disease, choledochocele, acholic stools

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Contributor Information and Disclosures

Author

Michael AJ Sawyer, MD, Director, Videoendoscopic Surgical Institute of Oklahoma, Consulting Staff, Department of Surgery, Comanche County Memorial Hospital; Consulting Staff, Great Plains Surgical Clinic, Lawton, Oklahoma
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.

Medical Editor

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.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

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

CME Editor

Alex J Mechaber, MD, FACP, Assistant Dean for Medical Curriculum, Associate Professor of Medicine, Division of General Internal 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; Consulting Staff, Department of Medicine, Section of Gastroenterology and Hepatology, Hospital of the Medical College of Pennsylvania
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 and Ethics, American Trauma Society, Association of American Medical Colleges, and Physicians for Social Responsibility
Disclosure: Nothing to disclose.

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