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Choledochal Cysts: Differential Diagnoses & Workup

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
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: Nov 10, 2009

Overview
Differential Diagnoses & Workup
Treatment & Medication
Follow-up
Multimedia

Differential Diagnoses

Bile Duct Tumors
Biliary Obstruction
Cholangiocarcinoma
Pancreatitis, Acute

Workup

Laboratory Studies

CBC count: No laboratory studies are specific for the diagnosis of a choledochal cyst. An elevated white blood cell count with increased numbers of neutrophils and immature neutrophil forms may be observed in the presence of cholangitis.
Liver function tests may be useful in narrowing the differential diagnosis. Hepatocellular enzyme and alkaline phosphatase levels may be elevated. None of these tests are specific for the diagnosis of a choledochal cyst.
Serum amylase and lipase concentrations may be increased in the presence of pancreatitis. Serum amylase concentrations also may be elevated in biliary obstruction and cholangitis.
Results of serum chemistry may be abnormal if the patient is vomiting. One might expect to see a hypochloremic, hypokalemic metabolic alkalosis in this clinical picture.

Imaging Studies

Abdominal ultrasonography is the test of choice for the diagnosis of a choledochal cyst. Ultrasound is useful in the antenatal period as well, according to Chen and coworkers, and can demonstrate a choledochal cyst in a fetus as early as the beginning of the second trimester.⁶Caroli disease has also been detected antenatally with ultrasound by Sgro and colleagues.⁷(See image below.)
Diagnostic ultrasonogram demonstrating type I choledochal cyst in a 4-month-old child presenting with hyperbilirubinemia and transaminase elevations.
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Diagnostic ultrasonogram demonstrating type I choledochal cyst in a 4-month-old child presenting with hyperbilirubinemia and transaminase elevations.
Abdominal CT scan and MRI help to delineate the anatomy of the lesion and the surrounding structures. These tests also can assist in defining the presence and extent of intrahepatic ductal involvement. Yu and associates published a series of 64 patients in whom magnetic resonance cholangiopancreatography (MRCP) was particularly valuable in defining anomalous pancreaticobiliary junctions.⁸(See images below.)
Computed tomography (CT) scan of choledochal cyst demonstrating intrahepatic extension involving the main left hepatic duct.
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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.
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Computed tomography (CT) scan of choledochal cyst involving the common hepatic duct.
Computed tomography (CT) scan demonstrating large choledochal cyst and adjacent gall bladder.
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Computed tomography (CT) scan demonstrating large choledochal cyst and adjacent gall bladder.
Computed tomography (CT) scan of large, saccular type I choledochal cyst.
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Computed tomography (CT) scan of large, saccular type I choledochal cyst.
Fitoz and colleagues described the use of MRCP in 17 children with pancreatobiliary anomalies.⁹The reported diagnostic accuracy of MRCP in choledochal cysts in this series was 100%.
Invasive diagnostic studies: When noninvasive measures (eg, ultrasound, CT scan, MRI) fail to sufficiently delineate the anatomy, they should be supplemented by the addition of percutaneous transhepatic cholangiography (PTC) or endoscopic retrograde cholangiopancreatography (ERCP). As reported by Nagi and coworkers, these studies are particularly helpful in demonstrating the presence of an anomalous pancreatobiliary junction and in delineating associated extrahepatic or intrahepatic strictures and stones.¹⁰

Histologic Findings

Evidence of chronic inflammation is typically observed in the cyst wall. The cyst wall is thin, fibrous, and frequently devoid of a true epithelial surface, although it can be lined by a low columnar epithelium. Note that infants can develop complete obstruction of the distal common bile duct secondary to acute and chronic inflammatory changes. In the liver, ductal fibrosis and portal edema may be present. Changes consistent with biliary cirrhosis may be observed in adults with long-standing disease. The most feared histologic abnormality is the presence of cholangiocarcinoma.

More on Choledochal Cysts

Overview: Choledochal Cysts

Differential Diagnoses & Workup: Choledochal Cysts

Treatment & Medication: Choledochal Cysts

Follow-up: Choledochal Cysts

Multimedia: Choledochal Cysts

References

Douglas AH. Case of dilatation of the common bile duct. Monthly J M Sci (London). 1852;14:97.
Shah OJ, Shera AH, Zargar SA, Shah P, Robbani I, Dhar S, et al. Choledochal cysts in children and adults with contrasting profiles: 11-year experience at a tertiary care center in Kashmir. World J Surg. Nov 2009;33(11):2403-11. [Medline].
Singham J, Yoshida EM, Scudamore CH. Choledochal cysts: part 1 of 3: classification and pathogenesis. Can J Surg. Oct 2009;52(5):434-40. [Medline]. [Full Text].
Miyano T, Yamataka A. Choledochal cysts. Curr Opin Pediatr. Jun 1997;9(3):283-8. [Medline].
Wu GS, Zou SQ, Luo XW, Wu JH, Liu ZR. Proliferative activity of bile from congenital choledochal cyst patients. World J Gastroenterol. Jan 2003;9(1):184-7. [Medline].
Chen CP, Cheng SJ, Chang TY, Yeh LF, Lin YH, Wang W. Prenatal diagnosis of choledochal cyst using ultrasound and magnetic resonance imaging. Ultrasound Obstet Gynecol. Jan 2004;23(1):93-4. [Medline].
Sgro M, Rossetti S, Barozzino T, Toi A, Langer J, Harris PC, et al. Caroli's disease: prenatal diagnosis, postnatal outcome and genetic analysis. Ultrasound Obstet Gynecol. Jan 2004;23(1):73-6. [Medline].
Yu ZL, Zhang LJ, Fu JZ, Li J, Zhang QY, Chen FL. Anomalous pancreaticobiliary junction: image analysis and treatment principles. Hepatobiliary Pancreat Dis Int. Feb 2004;3(1):136-9. [Medline].
Fitoz S, Erden A, Boruban S. Magnetic resonance cholangiopancreatography of biliary system abnormalities in children. Clin Imaging. Mar-Apr 2007;31(2):93-101. [Medline].
Nagi B, Kochhar R, Bhasin D, Singh K. Endoscopic retrograde cholangiopancreatography in the evaluation of anomalous junction of the pancreaticobiliary duct and related disorders. Abdom Imaging. Nov-Dec 2003;28(6):847-52. [Medline].
Jordan PH Jr, Goss JA Jr, Rosenberg WR, Woods KL. Some considerations for management of choledochal cysts. Am J Surg. Jun 2004;187(6):790-5. [Medline].
Edil BH, Olino K, Cameron JL. The current management of choledochal cysts. Adv Surg. 2009;43:221-32. [Medline].
Visser BC, Suh I, Way LW, Kang SM. Congenital choledochal cysts in adults. Arch Surg. Aug 2004;139(8):855-60; discussion 860-2. [Medline].
Shimotakahara A, Yamataka A, Yanai T, Kobayashi H, Okazaki T, Lane GJ, et al. Roux-en-Y hepaticojejunostomy or hepaticoduodenostomy for biliary reconstruction during the surgical treatment of choledochal cyst: which is better?. Pediatr Surg Int. Jan 2005;5-7.:21(1). [Medline].
Lee H, Hirose S, Bratton B, Farmer D. Initial experience with complex laparoscopic biliary surgery in children: biliary atresia and choledochal cyst. J Pediatr Surg. Jun 2004;39(6):804-7; discussion 804-7. [Medline].
Jang JY, Kim SW, Han HS, Yoon YS, Han SS, Park YH. Totally laparoscopic management of choledochal cysts using a four-hole method. Surg Endosc. Nov 2006;20(11):1762-5.
Woo R, Le D, Albanese CT, Kim SS. Robot-assisted laparoscopic resection of a type I choledochal cyst in a child. J Laparoendosc Adv Surg Tech A. Apr 2006;16(2):179-83. [Medline].
Lee SC, Kim HY, Jung SE, Park KW, Kim WK. Is excision of a choledochal cyst in the neonatal period necessary?. J Pediatr Surg. Dec 2006;41(12):1984-6. [Medline].
Woon CY, Tan YM, Oei CL, Chung AY, Chow PK, Ooi LL. Adult choledochal cysts: an audit of surgical management. ANZ J Surg. Nov 2006;76(11):981-6. [Medline].
Ahn SM, Jun JY, Lee WJ, Oh JT, Han SJ, Choi SH, et al. Laparoscopic total intracorporeal correction of choledochal cyst in pediatric population. J Laparoendosc Adv Surg Tech A. Oct 2009;19(5):683-6. [Medline].
Chijiiwa K, Koga A. Surgical management and long-term follow-up of patients with choledochal cysts. Am J Surg. Feb 1993;165(2):238-42. [Medline].
Dundas SE, Robinson-Bridgewater LA, Duncan ND. Antenatal diagnosis of a choledochal cyst. Case management and literature review. West Indian Med J. Sep 2002;51(3):184-7. [Medline].
Fieber SS, Nance FC. Choledochal cyst and neoplasm: a comprehensive review of 106 cases and presentation of two original cases. Am Surg. Nov 1997;63(11):982-7. [Medline].
Gallivan EK, Crombleholme TM, D'Alton ME. Early prenatal diagnosis of choledochal cyst. Prenat Diagn. Oct 1996;16(10):934-7. [Medline].
Hernandez Bartolome MA, Fuerte Ruiz S, Manzanedo Romero I, Ramos Lojo B, Rodriguez Prieto I, Gimenez Alvira L, et al. Biliary cystadenoma. World J Gastroenterol. Jul 28 2009;15(28):3573-5. [Medline].
Hewitt PM, Krige JE, Bornman PC, Terblanche J. Choledochal cysts in adults. Br J Surg. Mar 1995;82(3):382-5. [Medline].
Ishibashi T, Kasahara K, Yasuda Y, Nagai H, Makino S, Kanazawa K. Malignant change in the biliary tract after excision of choledochal cyst. Br J Surg. Dec 1997;84(12):1687-91. [Medline].
Kubota H, Eckelman WC, Poulose KP, Reba RC. Technetium-99m-pyridoxylideneglutamate, a new agent for gallbladder imaging: comparison with 131I-rose bengal. J Nucl Med. Jan 1976;17(1):36-9. [Medline].
Lindberg CG, Hammarstrom LE, Holmin T, Lundstedt C. Cholangiographic appearance of bile-duct cysts. Abdom Imaging. Nov-Dec 1998;23(6):611-5. [Medline].
Rabie ME, Al-Humayed SM, Hosni MH, Katwah RA, Dewan M. Choledochocele: the disputed origin. Int Surg. Oct-Dec 2002;87(4):221-5. [Medline].
Rha SY, Stovroff MC, Glick PL, Allen JE, Ricketts RR. Choledochal cysts: a ten year experience. Am Surg. Jan 1996;62(1):30-4. [Medline].
Saing H, Han H, Chan KL, Lam W, Chan FL, Cheng W, et al. Early and late results of excision of choledochal cysts. J Pediatr Surg. Nov 1997;32(11):1563-6. [Medline].
Schultz RM. The potential role of cytokines in cancer therapy. Prog Drug Res. 1992;39:219-50. [Medline].
Stringer MD, Dhawan A, Davenport M, Mieli-Vergani G, Mowat AP, Howard ER. Choledochal cysts: lessons from a 20 year experience. Arch Dis Child. Dec 1995;73(6):528-31. [Medline].
Weyant MJ, Maluccio MA, Bertagnolli MM, Daly JM. Choledochal cysts in adults: a report of two cases and review of the literature. Am J Gastroenterol. Dec 1998;93(12):2580-3. [Medline].
Yamataka A, Ohshiro K, Okada Y, Hosoda Y, Fujiwara T, Kohno S, et al. Complications after cyst excision with hepaticoenterostomy for choledochal cysts and their surgical management in children versus adults. J Pediatr Surg. Jul 1997;32(7):1097-102. [Medline].

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Keywords

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

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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.

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: eMedicine Salary Employment

Managing 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, and American Society for Gastrointestinal Endoscopy
Disclosure: Nothing to disclose.

CME Editor

Alex J Mechaber, MD, FACP, 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; 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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