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Choledochal Cysts Clinical Presentation

  • Author: Michael AJ Sawyer, MD; Chief Editor: BS Anand, MD  more...
 
Updated: Feb 10, 2016
 

History

The patient 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.

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Physical Examination

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. The images below depict nuclear medicine scans of choledochal cysts.

Nuclear medicine scan of choledochal cyst. Nuclear medicine scan of choledochal cyst.
Nuclear medicine scan of choledochal cyst. Nuclear medicine scan of choledochal cyst.
Nuclear medicine scan of choledochal cyst. Nuclear medicine scan of choledochal cyst.
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Contributor Information and Disclosures
Author

Michael AJ Sawyer, MD Consulting Staff, Department of Surgery, Comanche County Memorial Hospital; Medical Director, Lawton Bariatrics

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

Disclosure: Nothing to disclose.

Coauthor(s)

Fernando V Ona, MD Associate Clinical Professor, University of Hawaii, John A Burns School of Medicine; Professor, St Luke's College of Medicine and University of Santo Tomas Faculty of Medicine and Surgery; Chief, Center for Digestive and Liver Diseases and Nutrition, VAPIHCS

Fernando V Ona, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American Gastroenterological Association

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

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, American Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Acknowledgements

Mounzer Al Al Samman, MD Assistant Professor, Department of Internal Medicine, Division of Gastroenterology, 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.

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

Disclosure: Nothing to disclose.

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

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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