Skip to main content

Advertisement

Log in

Choledochal Cyst Disease in a Western Center: A 30-Year Experience

  • Original Article
  • Published:
Journal of Gastrointestinal Surgery Aims and scope

Abstract

Background

The aim of this study was to report a Western experience in the diagnosis and management of choledochal cyst disease.

Results

Sixty-seven patients were identified including 15 children and 52 adults; 76.1 % were females. The median age at diagnosis was 3 [inter-quartile range (IQR) = 6.0–0.7] years for children, and 46 [IQR = 55.6–34.3] years for adults. Forty-eight patients (72 %) were symptomatic. Types of choledochal cyst included: I (n = 49, 73.1 %), II (n = 1, 1.5 %), IV (n = 9, 13.4 %), and V (n = 8, 12 %). The median diameter of the type I choledochal cyst was 35 [IQR = 47–25] mm. All 48 patients underwent excision of cyst with Roux-en-Y hepaticojejunostomy, and eight underwent resection with hepaticoduodenostomy. Six patients underwent liver resection, and five patients underwent orthotopic liver transplantation. Malignancy was concomitant in five adult patients, being identified on preoperative imaging in three cases; and atypia was seen in three additional patients. Early morbidity included Clavien–Dindo classification grades III (n = 7) and II (n = 5), while long-term complications consisted of Clavien–Dindo grades V (n = 5), IV (n = 2), III (n = 18), and II (n = 1).

Conclusions

Presentation and management of choledochal cyst is varied. Malignant transformation is often detected incidentally, and so should be the driving source for resection when a choledochal cyst is diagnosed.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6

Similar content being viewed by others

References

  1. Soreide K, Korner H, Havnen J, Soreide JA. Bile duct cysts in adults. Br J Surg 2004;91:1538-48.

    Article  CAS  PubMed  Google Scholar 

  2. Vater A, Ezler C. Dissertatio de Scirrhis viserum occasione sections viri tymponite defunte. Wittenburgae Pamphlets 1723;4:22.

    Google Scholar 

  3. Yamaguchi M. Congenital choledochal cyst. Analysis of 1,433 patients in the Japanese literature. Am J Surg 1980;140:653-7.

    Article  CAS  PubMed  Google Scholar 

  4. Lee SE, Jang JY, Lee YJ, Choi DW, Lee WJ, Cho BH, Kim SW. Choledochal cyst and associated malignant tumors in adults: a multicenter survey in South Korea. Arch Surg 2011;146:1178-84.

    Article  PubMed  Google Scholar 

  5. Miyano T, Yamataka A. Choledochal cysts. Curr Opin Pediatr 1997;9: 283-8.

    Article  CAS  PubMed  Google Scholar 

  6. Edil BH, Cameron JL, Reddy S, Lum Y, Lipsett PA, Nathan H, Pawlik TM, Choti MA, Wolfgang CL, Schulick RD. Choledochal cyst disease in children and adults: a 30-year single-institution experience. J Am Coll Surg. 2008;206:1000-5.

    Article  PubMed  Google Scholar 

  7. Komi N, Tamura T, Miyoshi Y, Kunitomo K, Udaka H, Takehara H. Nationwide survey of cases of choledochal cyst. Analysis of coexistent anomalies, complications and surgical treatment in 645 cases. Surg Gastroenterol 1984;3:69-73.

    CAS  PubMed  Google Scholar 

  8. Lipsett PA, Pitt HA, Colombani PM, Boitnott JK, Cameron JL. Choledochal cyst disease. A changing pattern of presentation. Ann Surg 1994;220:644-52.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  9. Dhupar R, Gulack B, Geller DA, Marsh JW, Gamblin TC. The changing presentation of choledochal cyst disease: an incidental diagnosis. HPB Surg 2009;2009:103739.

    Article  PubMed  PubMed Central  Google Scholar 

  10. Todani T, Watanabe Y, Narusue M, Tabuchi K, Okajima K. Congenital bile duct cysts: classification, operative procedures, and review of thirtyseven cases including cancer arising from choledochal cyst. Am J Surg 1977;134:263-9.

    Article  CAS  PubMed  Google Scholar 

  11. 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 1959;108:1–30.

    CAS  PubMed  Google Scholar 

  12. Khandelwal C, Anand U, Kumar B, Priyadarshi RN. Diagnosis and management of choledochal cysts. Indian J Surg 2012;74:401-6.

    Article  PubMed  PubMed Central  Google Scholar 

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

    CAS  Google Scholar 

  14. Kamisawa T, Ando H, Hamada Y, Fujii H, Koshinaga T, Urushihara N, Itoi T, Shimada H. Diagnostic criteria for pancreaticobiliary maljunction 2013. J Hepatobiliary Pancreat Sci 2014;21:159-61.

    Article  PubMed  Google Scholar 

  15. Kim Y, Hyun JJ, Lee JM, Lee HS, Kim CD. Anomalous union of the pancreaticobiliary duct without choledochal cyst: is cholecystectomy alone sufficient? Langenbecks Arch Surg 2014;399:1071-6.

    Article  PubMed  Google Scholar 

  16. Mori K, Nagakawa T, Ohta T, Nakano T, Kayahara M, Akiyama T, Kanno M, Ueno K, Konishi I, Izumi R, Konishi K, Miyazaki I. Pancreatitis and anomalous union of the pancreaticobiliary ductal system in childhood. J Pediatr Surg 1993;28:67-71.

    Article  CAS  PubMed  Google Scholar 

  17. Ziegler KM, Pitt HA, Zyromski NJ, Chauhan A, Sherman S, Moffatt D, Lehman GA, Lillemoe KD, Rescorla FJ, West KW, Grosfeld JL. Choledochoceles: are they choledochal cysts. Ann Surg 2010;252:683-90.

    PubMed  Google Scholar 

  18. Imazu M, Iwai N, Tokiwa K, Shimotake T, Kimura O, Ono S. Factors of biliary carcinogenesis in choledochal cysts. Eur J Pediatr Surg 2001;11:24-7.

    Article  CAS  PubMed  Google Scholar 

  19. Liu Y, Yao X, Li S, Liu W, Liu L, Liu J. Comparison of therapeutic effects of laparoscopic and open operation for congenital choledochal cysts in adults. Gastroenterol Res Pract 2014;2014:670260.

    PubMed  PubMed Central  Google Scholar 

  20. Dindo D, Demartines N, Clavien PA. Classification of surgical complications. A new proposal with evaluation in a cohort of 6336 patients and results of a survey. Ann Surg 2004; 240:205-13.

    Article  PubMed  PubMed Central  Google Scholar 

  21. Hung MH, Lin LH, Chen DF, Huang CS. Choledochal cysts in infants and children: experiences over a 20-year period at a single institution. Eur J Pediatr 2011;170:1179-85.

    Article  PubMed  Google Scholar 

  22. Yamaguchi M. Congenital choledochal cyst. Analysis of 1,433 patients in the Japanese literature. Am J Surg 1980;140:653-7.

    Article  CAS  PubMed  Google Scholar 

  23. Soares KC, Arnaoutakis DJ, Kamel I, Rastegar N, Anders R, Maithel S, Pawlik TM. Choledochal cysts: presentation, clinical differentiation, and management. J Am Coll Surg 2014;219:1167-80.

    Article  PubMed  PubMed Central  Google Scholar 

  24. Soares KC, Kim Y, Spolverato G, Maithel S, Bauer TW, Marques H, Sobral M, Knoblich M, Tran T, Aldrighetti L, Jabbour N, Poultsides GA, Gamblin TC, Pawlik TM. Presentation and Clinical Outcomes of Choledochal Cysts in Children and Adults: A Multi-institutional Analysis. JAMA Surg 2015;150:577-84.

    Article  PubMed  Google Scholar 

  25. Saluja SS, Nayeem M, Sharma BC, Bora G, Mishra PK. Management of choledochal cysts and their complications. Am Surg 2012;78:284-90.

    PubMed  Google Scholar 

  26. Khandelwal C, Anand U, Kumar B, Priyadarshi RN. Diagnosis and management of choledochal cysts. Indian J Surg 2012;74:401-6.

    Article  PubMed  PubMed Central  Google Scholar 

  27. Shah OJ, Shera AH, Zargar SA, Shah P, Robbani I, Dhar S, Khan AB. Choledochal cysts in children and adults with contrasting profiles: 11-year experience at a tertiary care center in Kashmir. World J Surg 2009;33:2403-11.

    Article  PubMed  Google Scholar 

  28. Bismuth H, Krissat J. Choledochal cystic malignancies. Ann Oncol 1999;10:94-8.

    Article  PubMed  Google Scholar 

  29. Kumamoto T, Tanaka K, Takeda K, Nojiri K, Mori R, Taniguchi K, Matsuyama R, Ueda M, Sugita M, Ichikawa Y, Nagashima Y, Endo I. Intrahepatic cholangiocarcinoma arising 28 years after excision of a type IV-A congenital choledochal cyst: report of a case. Surg Today 2014;44:354-8.

    Article  PubMed  Google Scholar 

  30. Zheng X, Gu W, Xia H, Huang X, Liang B, Yang T, Yang S, Zeng J, Dong J. Surgical treatment of type IV-A choledochal cyst in a single institution: children vs. adults. J Pediatr Surg 2013;48:2061-6.

    Article  PubMed  Google Scholar 

  31. Liu YB, Wang JW, Devkota KR, Ji ZL, Li JT, Wang XA, Ma XM, Cai WL, Kong Y, Cao LP, Peng SY. Congenital choledochal cysts in adults: twenty-five-year experience. Chin Med J (Engl) 2007;120:1404-7.

    Google Scholar 

  32. Jan YY, Chen HM, Chen MF. Malignancy in choledochal cysts. Hepatogastroenterology 2000;47:337-40.

    CAS  PubMed  Google Scholar 

  33. Chijiiwa K, Kimura H, Tanaka M. Malignant potential of the gallbladder in patients with anomalous pancreaticobiliary ductal junction. The difference in risk between patients with and without choledochal cyst. Int Surg 1995;80:61-4.

    CAS  PubMed  Google Scholar 

  34. Watanabe Y, Toki A, Todani T. Bile duct cancer developed after cyst excision for choledochal cyst. J Hepatobiliary Pancreat Surg 1999;6:207-12.

    Article  CAS  PubMed  Google Scholar 

  35. Ng WT. Bile duct carcinogenesis after excision of extrahepatic bile ducts in pancreaticobiliary maljunction. Surgery 2000;128:492-4.

    Article  CAS  PubMed  Google Scholar 

  36. Ohashi T, Wakai T, Kubota M, Matsuda Y, Arai Y, Ohyama T, Nakaya K, Okuyama N, Sakata J, Shirai Y, Ajioka Y. Risk of subsequent biliary malignancy in patients undergoing cyst excision for congenital choledochal cysts. J Gastroenterol Hepatol 2013;28:243-7.

    Article  PubMed  PubMed Central  Google Scholar 

  37. Gunay-Aygun M. Liver and Kidney Disease in Ciliopathies. Am J Med Genet C Semin Med Genet 2009;151C:296-306.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  38. MacWorter GL. Congenital cystic dilatatin of the common bile duct. Ann Surg 1924;8:604-25.

    Google Scholar 

  39. Suita S, Shono K, Kinugasa Y, Kubota M, Matsuo S. Influence of age on the presentation and outcome of choledochal cyst. J Pediatr Surg 1999;34:1765-8.

    Article  CAS  PubMed  Google Scholar 

  40. Diao M, Li L, Cheng W. Timing of surgery for prenatally diagnosed asymptomatic choledochal cysts: a prospective randomized study. J Pediatr Surg 2012;47:506-12.

    Article  PubMed  Google Scholar 

  41. Xia HT, Dong JH, Yang T, Zeng JP, Liang B. Extrahepatic cyst excision and partial hepatectomy for Todani type IV-A cysts. Dig Liver Dis 2014;46:1025-30.

    Article  PubMed  Google Scholar 

  42. Ando H, Kaneko K, Ito T, Watanabe Y, Seo T, Harada T, Ito F, Nagaya M, Sugito T. Complete excision of the intrapancreatic portion of choledochal cysts. J Am Coll Surg. 1996;183:317-21.

    CAS  PubMed  Google Scholar 

  43. Cosentino CM, Luck SR, Raffensperger JG, Reynolds M. Choledochal duct cyst: resection with physiologic reconstruction. Surgery 1992;112:740-7.

    CAS  PubMed  Google Scholar 

  44. Edil BH, Olino K, Cameron JL. The current management of choledochal cysts. Adv Surg 2009;43:221-32.

    Article  PubMed  Google Scholar 

  45. Narayanan SK, Chen Y, Narasimhan KL, Cohen RC. Hepaticoduodenostomy versus hepaticojejunostomy after resection of choledochal cyst: a systematic review and meta-analysis. J Pediatr Surg 2013;48:2336-42.

    Article  PubMed  Google Scholar 

  46. Okada A, Higaki J, Nakamura T, Kamata S. Roux-en-Y versus interposition biliary reconstruction. Surg Gynecol Obstet 1992;174:313-6.

    CAS  PubMed  Google Scholar 

  47. Law R, Topazian M. Diagnosis and treatment of choledochoceles. Clin Gastroenterol Hepatol. 2014;12:196-203.

    Article  PubMed  Google Scholar 

  48. Elton E, Hanson BL, Biber BP, Howell DA. Dilated common channel syndrome: endoscopic diagnosis, treatment, and relationship to choledochocele formation. Gastrointest Endosc 1998;47:471-8.

    Article  CAS  PubMed  Google Scholar 

  49. Samavedy R, Sherman S, Lehman GA. Endoscopic therapy in anomalous pancreatobiliary duct junction. Gastrointest Endosc 1999;50:623-7.

    Article  CAS  PubMed  Google Scholar 

  50. Visser BC, Suh I, Way LW, Kang SM. Congenital choledochal cysts in adults. Arch Surg 2004;139:855-60.

    Article  PubMed  Google Scholar 

  51. Cerwenka H. Bile duct cyst in adults: interventional treatment, resection, or transplantation. World J Gastroenterol 2013;19:5207-11.

    Article  PubMed  PubMed Central  Google Scholar 

  52. Mabrut JY, Partensky C, Jaeck D, Oussoultzoglou E, Baulieux J, Boillot O, Lerut J, de Ville de Goyet J, Hubert C, Otte JB, Audet M, Ducerf C, Gigot JF. Congenital intrahepatic bile duct dilatation is a potentially curable disease. Long-term results of a multi-institutional study. Ann Surg 2007;246:236-45.

    Article  PubMed  PubMed Central  Google Scholar 

  53. Bockhorn M, Malagó M, Lang H, Nadalin S, Paul A, Saner F, Frilling A, Broelsch CE. The role of surgery in Caroli’s disease. J Am Coll Surg 2006;202:928-32.

    Article  PubMed  Google Scholar 

  54. Kassahun WT, Kahn T, Wittekind C, Mössner J, Caca K, Hauss J, Lamesch P. Caroli’s disease: liver resection and liver transplantation. Experience in 33 patients. Surgery 2005;138: 888-98.

    Article  PubMed  Google Scholar 

  55. Espinoza R, San Martín S, Court F, Vera E, Ferreira R, Croxatto H. Hepatic resection in localized Caroli disease. Rev Med Chil 2003;131:183-9.

    PubMed  Google Scholar 

  56. Moslim MA, Gunasekaran G, Vogt D, Cruise M, Morris-Stiff G. Surgical Management of Caroli’s Disease: Single Center Experience and Review of the Literature. J Gastrointest Surg 2015;19:2019-27.

    Article  PubMed  Google Scholar 

  57. Alizai NK, Dawrant MJ, Najmaldin AS. Robot-assisted resection of choledochal cysts and hepaticojejunostomy in children. Pediatr Surg Int 2014;30:291-4.

    Article  PubMed  Google Scholar 

  58. Farello GA, Cerofolini A, Rebonato M, Bergamaschi G, Ferrari C, Chiappetta A. Congenital choledochal cyst: video-guided laparoscopic treatment. Surg Laparosc Endosc 1995;5:354-8.

    CAS  PubMed  Google Scholar 

  59. Duan X, Mao X, Jiang B, Wu J. Totally Laparoscopic Cyst Excision and Roux-en-Y Hepaticojejunostomy for Choledochal Cyst in Adults: A Single-institute Experience of 5 Years. Surg Laparosc Endosc Percutan Tech 2015;25:e65-8.

    Article  PubMed  Google Scholar 

  60. Qiao G, Li L, Li S, Tang S, Wang B, Xi H, Gao Z, Sun Q. Laparoscopic cyst excision and Roux-Y hepaticojejunostomy for children with choledochal cysts in China: a multicenter study. Surg Endosc 2015;29:140-4.

    Article  PubMed  Google Scholar 

  61. Diao M, Li L, Cheng W. Laparoscopic versus Open Roux-en-Y hepatojejunostomy for children with choledochal cysts: intermediate-term follow-up results. Surg Endosc 2011;25:1567-73.

    Article  PubMed  Google Scholar 

  62. Dawrant MJ, Najmaldin AS, Alizai NK. Robot-assisted resection of choledochal cysts and hepaticojejunostomy in children less than 10 kg. J Pediatr Surg 2010;45:2364-8.

    Article  PubMed  Google Scholar 

  63. Meehan JJ, Elliott S, Sandler A. The robotic approach to complex hepatobiliary anomalies in children: preliminary report. J Pediatr Surg 2007;42:2110-4.

    Article  PubMed  Google Scholar 

  64. Margonis GA, Spolverato G, Kim Y, Marques H, Poultsides G, Maithel S, Aldrighetti L, Bauer TW, Jabbour N, Gamblin TC, Soares K, Pawlik TM. Minimally invasive resection of choledochal cyst: a feasible and safe surgical option. J Gastrointest Surg 2015;19:858-65.

    Article  PubMed  Google Scholar 

  65. Yamataka A, Ohshiro K, Okada Y, Hosoda Y, Fujiwara T, Kohno S, Sunagawa M, Futagawa S, Sakakibara N, Miyano T. Complications after cyst excision with hepaticoenterostomy for choledochal cysts and their surgical management in children versus adults. J Pediatr Surg 1997;32:1097-102.

    Article  CAS  PubMed  Google Scholar 

  66. Woon CY, Tan YM, Oei CL, Chung AY, Chow PK, Ooi LL. Adult choledochal cysts: an audit of surgical management. ANZ J Surg 2006 ;76:981-6.

    Article  PubMed  Google Scholar 

  67. Takeshita N, Ota T, Yamamoto M. Forty-year experience with flow-diversion surgery for patients with congenital choledochal cysts with pancreaticobiliary maljunction at a single institution. Ann Surg 2011;254:1050-3.

    Article  PubMed  Google Scholar 

  68. Lenriot JP, Gigot JF, Ségol P, Fagniez PL, Fingerhut A, Adloff M. Bile duct cysts in adults: a multi-institutional retrospective study. French Associations for Surgical Research. Ann Surg 1998;228:159–66.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  69. Kim JH, Choi TY, Han JH, Yoo BM, Kim JH, Hong J, Kim MW, Kim WH. Risk factors of postoperative anastomotic stricture after excision of choldochal cysts with hepaticojejunostomy. J Gastrointest Surg 2008;12:822-8.

    Article  PubMed  Google Scholar 

  70. Urushihara N, Fukumoto K, Fukuzawa H, Mitsunaga M, Watanabe K, Aoba T, Yamoto M, Miyake H. Long-term outcomes after excision of choledochal cysts in a single institution: operative procedures and late complications. J Pediatr Surg 2012;47:2169-74.

    Article  PubMed  Google Scholar 

  71. Kobayashi S, Asano T, Yamasaki M, Kenmochi T, Nakagohri T, Ochiai T. Risk of bile duct carcinogenesis after excision of extrahepatic bile ducts in pancreaticobiliary maljunction. Surgery 1999;126:939-44.

    Article  CAS  PubMed  Google Scholar 

Download references

Acknowledgments

We thank Dave Schumick for his assistance with the medical illustrations.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Maitham A. Moslim.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Moslim, M.A., Takahashi, H., Seifarth, F.G. et al. Choledochal Cyst Disease in a Western Center: A 30-Year Experience. J Gastrointest Surg 20, 1453–1463 (2016). https://doi.org/10.1007/s11605-016-3181-4

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11605-016-3181-4

Keywords

Navigation