eMedicine Specialties > Pediatrics: Surgery > General Surgery

Choledochal Cyst: Surgical Perspective: Workup

Author: Gail E Besner, MD, Professor of Surgery and Pediatrics, Department of Surgery, Ohio State University College of Medicine and Public Health; Director, Pediatric Surgical Research, Department of Surgery, Children's Hospital
Coauthor(s): Heather N Paddock, MD, Pediatric Surgery Fellow, Department of Pediatric Surgery, University of Florida; Luong Tuyen Nguyen, MD, Associate Professor, Department of Surgery, Department of Pediatric General Surgery, McGill University; Consulting Surgeon, Montreal Children's Hospital; Saundra M Kay, MD, FRCSC, Consulting Staff, Rocky Mountain Pediatric Surgery
Contributor Information and Disclosures

Updated: Sep 30, 2008

Workup

Laboratory Studies

  • Laboratory studies that may be useful for the diagnosis and preoperative evaluation of a patient with a choledochal cyst include direct bilirubin, alkaline phosphatase, serum aspartate aminotransferase (AST), serum alanine aminotransferase (ALT), gamma-glutamyl transferase (GGT), and coagulation profiles. A CBC count should also be obtained to exclude any associated or underlying anemia prior to surgery.

Imaging Studies

  • Imaging studies are the cornerstone of diagnosis of choledochal cysts. They serve not only to confirm the diagnosis but also to outline the anatomy of the anomaly in preparation for surgical intervention.
  • Ultrasonography is the best initial study.4 In neonates, it may be the only test needed. Ultrasonography can demonstrate changes in the bile ducts as well as in the liver (see Media file 5).
  • Endoscopic retrograde cholangiopancreatography (ERCP) remains the criterion standard diagnostic study. In expert hands, ERCP can be performed with a high rate of success, even in small infants. When successful, ERCP clearly shows the anatomy of the pancreaticobiliary junction.5,22,2,17 With forceful injection of contrast even small choledochoceles can be imaged, presumably by distension of the cyst wall.
  • Magnetic resonance cholangiopancreatography (MRCP) has largely supplanted ERCP as the diagnostic test of choice for choledochal cysts because it offers high resolution detailed images of relevant anatomy, is noninvasive, and does not suffer from complications such as postprocedure pancreatitis (see Media file 6).44 MRCP detects most choledochal cysts with sensitivities from 90-100% and specificities from 73-100%, with the exception of small choledochoceles and minor ductal anomalies.45 MRCP has been shown to be effective in neonates,46 children,47 adults,48 and fetuses.49
  • CT scanning may also be useful to delineate the cyst and its relationship to surrounding structures. In older patients, especially adults, CT scanning combined with cholangiography may be useful (see Media file 7).
  • Upper GI imaging and cholangiography with oral or intravenous contrast enhancement are of limited value in the setting of hyperbilirubinemia and are generally outdated.
  • Scintigraphy with technitium-99m diisopropyl iminodiacetic acid (DISIDA) may show complete obstruction of the distal bile duct without any drainage to the intestine.8,7

More on Choledochal Cyst: Surgical Perspective

Overview: Choledochal Cyst: Surgical Perspective
Workup: Choledochal Cyst: Surgical Perspective
Treatment: Choledochal Cyst: Surgical Perspective
Multimedia: Choledochal Cyst: Surgical Perspective
References
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References

  1. Alonso-Lej F, Rever WB Jr, Pessagno DJ. Congenital choledochal cyst, with a report of 2, and an analysis of 94, cases. Int Abstr Surg. Jan 1959;108(1):1-30. [Medline].

  2. Todani T, Watanabe Y, Narusue M, et al. Congenital bile duct cysts: Classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst. Am J Surg. Aug 1977;134(2):263-9. [Medline].

  3. Flanigan PD. Biliary cysts. Ann Surg. Nov 1975;182(5):635-43. [Medline].

  4. Yamaguchi M. Congenital choledochal cyst. Analysis of 1,433 patients in the Japanese literature. Am J Surg. Nov 1980;140(5):653-7. [Medline].

  5. Kasai M, Asakura Y, Taira Y. Surgical treatment of choledochal cyst. Ann Surg. Nov 1970;172(5):844-51. [Medline].

  6. Katyal D, Lees GM. Choledochal cysts: a retrospective review of 28 patients and a review of the literature. Can J Surg. Dec 1992;35(6):584-8. [Medline].

  7. O'Neill JA Jr. Choledochal cyst. Curr Probl Surg. Jun 1992;29(6):361-410. [Medline].

  8. Lipsett PA, Pitt HA, Colombani PM, et al. Choledochal cyst disease. A changing pattern of presentation. Ann Surg. Nov 1994;220(5):644-52. [Medline].

  9. Okada A, Nakamura T, Higaki J, et al. Congenital dilatation of the bile duct in 100 instances and its relationship with anomalous junction. Surg Gynecol Obstet. Oct 1990;171(4):291-8. [Medline].

  10. Saing H, Tam PK, Lee JM, Pe-Nyun. Surgical management of choledochal cysts: a review of 60 cases. J Pediatr Surg. Aug 1985;20(4):443-8. [Medline].

  11. Babbitt DP, Starshak RJ, Clemett AR. Choledochal cyst: a concept of etiology. Am J Roentgenol Radium Ther Nucl Med. Sep 1973;119(1):57-62. [Medline].

  12. Bismuth H, Krissat J. Choledochal cystic malignancies. Ann Oncol. 1999;10 Suppl 4:94-8. [Medline].

  13. Spitz L. Experimental production of cystic dilatation of the common bile duct in neonatal lambs. J Pediatr Surg. Feb 1977;12(1):39-42. [Medline].

  14. Kato T, Asakura Y, Kasai M. An attempt to produce choledochal cyst in puppies. J Pediatr Surg. Aug 1974;9(4):509-13. [Medline].

  15. Kato T, Hebiguchi T, Matsuda K, Yoshino H. Action of pancreatic juice on the bile duct: pathogenesis of congenital choledochal cyst. J Pediatr Surg. Apr 1981;16(2):146-51. [Medline].

  16. Miyano T, Suruga K, Suda K. Abnormal choledocho-pancreatico ductal junction related to the etiology of infantile obstructive jaundice diseases. J Pediatr Surg. Feb 1979;14(1):16-26. [Medline].

  17. Todani T, Watanabe Y, Fujii T, Uemura S. Anomalous arrangement of the pancreatobiliary ductal system in patients with a choledochal cyst. Am J Surg. May 1984;147(5):672-6. [Medline].

  18. Yamashiro Y, Miyano T, Suruga K, et al. Experimental study of the pathogenesis of choledochal cyst and pancreatitis, with special reference to the role of bile acids and pancreatic enzymes in the anomalous choledocho-pancreatico ductal junction. J Pediatr Gastroenterol Nutr. Nov 1984;3(5):721-7. [Medline].

  19. Babbitt DP. [Congenital choledochal cysts: new etiological concept based on anomalous relationships of the common bile duct and pancreatic bulb]. Ann Radiol (Paris). 1969;12(3):231-40. [Medline].

  20. Miyano T, Suruga K, Suda K. Choledocho-pancreatic end to side anastomosis in dog as an experimental model of choledocho-pancreatic long common channel disorders. Jpn J Pediatr Surg. 1981;13:525-31.

  21. Todani T, Watanabe Y, Fujii T, et al. Congenital choledochal cyst with intrahepatic involvement. Arch Surg. Sep 1984;119(9):1038-43. [Medline].

  22. Jona JZ, Babbitt DP, Starshak RJ, et al. Anatomic observations and etiologic and surgical considerations in choledochal cyst. J Pediatr Surg. Jun 1979;14(3):315-20. [Medline].

  23. Oguchi Y, Okada A, Nakamura T, et al. Histopathologic studies of congenital dilatation of the bile duct as related to an anomalous junction of the pancreaticobiliary ductal system: clinical and experimental studies. Surgery. Feb 1988;103(2):168-73. [Medline].

  24. Miyano T, Yamataka A, Kato Y, et al. Hepaticoenterostomy after excision of choledochal cyst in children: a 30-year experience with 180 cases. J Pediatr Surg. Oct 1996;31(10):1417-21. [Medline].

  25. Yamaguchi M, Sakurai M, Takeuchi S, Awazu S. Observation of cystic dilatation of the common bile duct by ultrasonography. J Pediatr Surg. Apr 1980;15(2):207-10. [Medline].

  26. Postema RR, Hazebroek FW. Choledochal cysts in children: a review of 28 years of treatment in a Dutch children's hospital. Eur J Surg. Dec 1999;165(12):1159-61. [Medline].

  27. Todani T, Watanabe Y, Urushihara N, et al. Biliary complications after excisional procedure for choledochal cyst. J Pediatr Surg. Mar 1995;30(3):478-81. [Medline].

  28. Raffensperger JG. Swenson's Pediatric Surgery. 5th ed. Norwalk, Conn: Appleton & Lange; 1990:665.

  29. Suita S, Shono K, Kinugasa Y, et al. Influence of age on the presentation and outcome of choledochal cyst. J Pediatr Surg. Dec 1999;34(12):1765-8. [Medline].

  30. Kamisawa T, Okamoto A, Tsuruta K, Tu Y, Egawa N. Carcinoma arising in congenital choledochal cysts. Hepatogastroenterology. Mar-Apr 2008;55(82-83):329-32. [Medline].

  31. Imazu M, Iwai N, Tokiwa K, et al. Factors of biliary carcinogenesis in choledochal cysts. Eur J Pediatr Surg. Feb 2001;11(1):24-7. [Medline].

  32. Iwai N, Deguchi E, Yanagihara J, et al. Cancer arising in a choledochal cyst in a 12-year-old girl. J Pediatr Surg. Dec 1990;25(12):1261-3. [Medline].

  33. Todani T, Tabuchi K, Watanabe Y, Kobayashi T. Carcinoma arising in the wall of congenital bile duct cysts. Cancer. Sep 1979;44(3):1134-41. [Medline].

  34. Todani T, Watanabe Y, Toki A, Urushihara N. Carcinoma related to choledochal cysts with internal drainage operations. Surg Gynecol Obstet. Jan 1987;164(1):61-4. [Medline].

  35. Watanabe Y, Toki A, Todani T. Bile duct cancer developed after cyst excision for choledochal cyst. J Hepatobiliary Pancreat Surg. 1999;6(3):207-12. [Medline].

  36. Hamada Y, Tanano A, Sato M, et al. Rapid enlargement of a choledochal cyst: antenatal diagnosis and delayed primary excision. Pediatr Surg Int. Jul 1998;13(5-6):419-21. [Medline].

  37. Howell CG, Templeton JM, Weiner S, et al. Antenatal diagnosis and early surgery for choledochal cyst. J Pediatr Surg. Aug 1983;18(4):387-93. [Medline].

  38. Mackenzie TC, Howell LJ, Flake AW, Adzick NS. The management of prenatally diagnosed choledochal cysts. J Pediatr Surg. Aug 2001;36(8):1241-3. [Medline].

  39. Marchildon MB. Antenatal diagnosis of choledochal cyst: the first four cases. Pediatr Surg Int. 1988;3:431-6.

  40. Shamberger RC, Lund DP, Lillehei CW, Hendren WH 3rd. Interposed jejunal segment with nipple valve to prevent reflux in biliary reconstruction. J Am Coll Surg. Jan 1995;180(1):10-5. [Medline].

  41. Komuro H, Makino S, Tahara K. Choledochal cyst associated with duodenal obstruction. J Pediatr Surg. Aug 2000;35(8):1259-62. [Medline].

  42. Ono S, Tokiwa K, Aoi S, et al. A bleeding tendency as the first symptom of a choledochal cyst. Pediatr Surg Int. 2000;16(1-2):111-2. [Medline].

  43. Patel S, Sterkin L, Donahue PE, Young S. Congenital cyst of common bile duct: an unusual cause of obstructive jaundice. Surgery. Mar 1991;109(3 Pt 1):333-5. [Medline].

  44. Kim SH, Lim JH, Yoon HK, et al. Choledochal cyst: comparison of MR and conventional cholangiography. Clin Radiol. May 2000;55(5):378-83. [Medline].

  45. Park DH, Kim MH, Lee SK, et al. Can MRCP replace the diagnostic role of ERCP for patients with choledochal cysts?. Gastrointest Endosc. Sep 2005;62(3):360-6. [Medline].

  46. Lin JTH YH, Chen YH, Ni HS, et al. Magnetic resonance cholangiopancreatography diagnosed pancreatitis associated choledochal cyst: report of one case. Acta Paediatr Taiwan. Nov-Dec 2001;42(6):363-6. [Medline].

  47. Yamataka A, Kuwatsuru R, Shima H, et al. Initial experience with non-breath-hold magnetic resonance cholangiopancreatography: a new noninvasive technique for the diagnosis of choledochal cyst in children. J Pediatr Surg. Nov 1997;32(11):1560-2. [Medline].

  48. Zhong L, Yao QY, Li L, Xu JR. Imaging diagnosis of pancreato-biliary diseases: a control study. World J Gastroenterol. Dec 2003;9(12):2824-7. [Medline].

  49. Wong AM, Cheung YC, Liu YH, et al. Prenatal diagnosis of choledochal cyst using magnetic resonance imaging: a case report. World J Gastroenterol. Aug 28 2005;11(32):5082-3. [Medline].

  50. Fu M, Wang Y, Zhang J. Evolution in the treatment of choledochus cyst. J Pediatr Surg. Sep 2000;35(9):1344-7. [Medline].

  51. Joseph VT. Surgical techniques and long-term results in the treatment of choledochal cyst. J Pediatr Surg. Jul 1990;25(7):782-7. [Medline].

  52. 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].

  53. Chijiiwa K, Tanaka M. Late complications after excisional operation in patients with choledochal cyst. J Am Coll Surg. Aug 1994;179(2):139-44. [Medline].

  54. Shimotakahara A, Yamataka A, Yanai T, 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;21(1):5-7. [Medline].

  55. Lilly JR. Total excision of choledochal cyst. Surg Gynecol Obstet. Feb 1978;146(2):254-6. [Medline].

  56. Li L, Feng W, Jing-Bo F, et al. Laparoscopic-assisted total cyst excision of choledochal cyst and Roux-en-Y hepatoenterostomy. J Pediatr Surg. Nov 2004;39(11):1663-6. [Medline].

  57. Martinez-Ferro M, Esteves E, Laje P. Laparoscopic treatment of biliary atresia and choledochal cyst. Semin Pediatr Surg. Nov 2005;14(4):206-15. [Medline].

  58. Hata Y, Sasaki F, Takahashi H, et al. Surgical treatment of congenital biliary dilatation associated with pancreaticobiliary maljunction. Surg Gynecol Obstet. Jun 1993;176(6):581-7. [Medline].

  59. Cosentino CM, Luck SR, Raffensperger JG, Reynolds M. Choledochal duct cyst: resection with physiologic reconstruction. Surgery. Oct 1992;112(4):740-7; discussion 747-8. [Medline].

  60. Reynolds M, Luck SR, Raffensperger JG. The valved conduit prevents ascending cholangitis: a follow-up. J Pediatr Surg. Dec 1985;20(6):696-702. [Medline].

  61. Liu DC, Rodriguez JA, Meric F, Geiger JL. Laparoscopic excision of a rare type II choledochal cyst: case report and review of the literature. J Pediatr Surg. Jul 2000;35(7):1117-9. [Medline].

  62. Maning P, Polley T, Oldam K. Choledochocele: an unusual form of choledochal cyst. Pediatr Surg Int. 1990;5:22-6.

  63. Ohi R, Yaoita S, Kamiyama T, et al. Surgical treatment of congenital dilatation of the bile duct with special reference to late complications after total excisional operation. J Pediatr Surg. Jun 1990;25(6):613-7. [Medline].

  64. Ohi R, Koike N, Matsumoto Y, et al. Changes of intrahepatic bile duct dilatation after surgery for congenital dilatation of the bile duct. J Pediatr Surg. Apr 1985;20(2):138-42. [Medline].

  65. Todani T, Narusue M, Watanabe Y, et al. Management of congenital choledochal cyst with intrahepatic involvement. Ann Surg. Mar 1978;187(3):272-80. [Medline].

  66. Yamataka A, Ohshiro K, Okada Y, 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].

  67. Miyano T, Yamataka A, Kato Y. Choledochal cysts: special emphasis on the usefulness of intraoperative endoscopy. J Pediatr Surg. Mar 1995;30(3):482-4. [Medline].

  68. Yamataka A, Segawa O, Kobayashi H, et al. Intraoperative pancreatoscopy for pancreatic duct stone debris distal to the common channel in choledochal cyst. J Pediatr Surg. Jan 2000;35(1):1-4. [Medline].

  69. Todani T, Urushihara N, Morotomi Y, et al. Characteristics of choledochal cysts in neonates and early infants. Eur J Pediatr Surg. Jun 1995;5(3):143-5. [Medline].

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Further Reading

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Keywords

choledochal cyst, cystic dilatation of the common bile duct, cystic dilatation of the CBD, cystic dilatation, biliary tract anomaly, cystic disease of the biliary tract, distal CBD obstruction, distal common bile duct obstruction, anomaly of the pancreaticobiliary junction, fibrosis, cirrhosis, portal hypertension, adenosquamous carcinoma, small cell carcinoma, cancer, biliary tract malignancy, jaundice, hepatomegaly, choledocholithiasis, cyst rupture, biliary carcinoma, Caroli disease

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Contributor Information and Disclosures

Author

Gail E Besner, MD, Professor of Surgery and Pediatrics, Department of Surgery, Ohio State University College of Medicine and Public Health; Director, Pediatric Surgical Research, Department of Surgery, Children's Hospital
Gail E Besner, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Burn Association, American College of Surgeons, American Gastroenterological Association, American Medical Association, American Medical Women's Association, American Pediatric Surgical Association, Association for Academic Surgery, Federation of American Societies for Experimental Biology, Society of Critical Care Medicine, Society of Surgical Oncology, and Society of University Surgeons
Disclosure: Trillium Therapeutics, Inc. Consulting fee Consulting; Trillium Therapeutics, Inc. Grant/research funds Other

Coauthor(s)

Heather N Paddock, MD, Pediatric Surgery Fellow, Department of Pediatric Surgery, University of Florida
Disclosure: Nothing to disclose.

Luong Tuyen Nguyen, MD, Associate Professor, Department of Surgery, Department of Pediatric General Surgery, McGill University; Consulting Surgeon, Montreal Children's Hospital
Luong Tuyen Nguyen, MD is a member of the following medical societies: Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

Saundra M Kay, MD, FRCSC, Consulting Staff, Rocky Mountain Pediatric Surgery
Saundra M Kay, MD, FRCSC is a member of the following medical societies: American College of Surgeons, American Pediatric Surgical Association, Canadian Association of Pediatric Surgeons, International Pediatric Endosurgery Group, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

Medical Editor

Rebeccah Brown, MD, Associate Director of Trauma Services, Associate Professor, Department of Clinical Surgery and Pediatrics, Cincinnati Children's Hospital Medical Center and University of Cincinnati Hospital
Rebeccah Brown, MD is a member of the following medical societies: American College of Surgeons, American Medical Association, and American Medical Women's Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Deborah F Billmire, MD, Associate Professor, Department of Surgery, Indiana University Medical Center
Deborah F Billmire, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, Phi Beta Kappa, and Society of Critical Care Medicine
Disclosure: Nothing to disclose.

CME Editor

H Biemann Othersen Jr, MD, Professor of Surgery and Pediatrics, Emeritus Head, Division of Pediatric Surgery, Medical University of South Carolina
H Biemann Othersen Jr, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Association for the Surgery of Trauma, American Burn Association, American Cancer Society, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, American Society for Parenteral and Enteral Nutrition, American Surgical Association, American Thoracic Society, British Association of Paediatric Surgeons, Society for Surgery of the Alimentary Tract, Society of Critical Care Medicine, South Carolina Medical Association, Southeastern Surgical Congress, Southern Medical Association, Southern Society for Pediatric Research, and Southern Thoracic Surgical Association
Disclosure: Nothing to disclose.

Chief Editor

Harsh Grewal, MD, FACS, FAAP, Professor of Surgery and Pediatrics, Temple University School of Medicine; Chief, Section of Pediatric Surgery, Temple University Children's Medical Center
Harsh Grewal, MD, FACS, FAAP is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, Association for Surgical Education, Children's Oncology Group, Eastern Association for the Surgery of Trauma, International Pediatric Endosurgery Group, Society of American Gastrointestinal and Endoscopic Surgeons, Society of Laparoendoscopic Surgeons, and Southwestern Surgical Congress
Disclosure: Nothing to disclose.

 
 
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