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.
Similar content being viewed by others
References
Soreide K, Korner H, Havnen J, Soreide JA. Bile duct cysts in adults. Br J Surg 2004;91:1538-48.
Vater A, Ezler C. Dissertatio de Scirrhis viserum occasione sections viri tymponite defunte. Wittenburgae Pamphlets 1723;4:22.
Yamaguchi M. Congenital choledochal cyst. Analysis of 1,433 patients in the Japanese literature. Am J Surg 1980;140:653-7.
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.
Miyano T, Yamataka A. Choledochal cysts. Curr Opin Pediatr 1997;9: 283-8.
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.
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.
Lipsett PA, Pitt HA, Colombani PM, Boitnott JK, Cameron JL. Choledochal cyst disease. A changing pattern of presentation. Ann Surg 1994;220:644-52.
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.
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.
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.
Khandelwal C, Anand U, Kumar B, Priyadarshi RN. Diagnosis and management of choledochal cysts. Indian J Surg 2012;74:401-6.
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.
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.
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.
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.
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.
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.
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.
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.
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.
Yamaguchi M. Congenital choledochal cyst. Analysis of 1,433 patients in the Japanese literature. Am J Surg 1980;140:653-7.
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.
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.
Saluja SS, Nayeem M, Sharma BC, Bora G, Mishra PK. Management of choledochal cysts and their complications. Am Surg 2012;78:284-90.
Khandelwal C, Anand U, Kumar B, Priyadarshi RN. Diagnosis and management of choledochal cysts. Indian J Surg 2012;74:401-6.
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.
Bismuth H, Krissat J. Choledochal cystic malignancies. Ann Oncol 1999;10:94-8.
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.
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.
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.
Jan YY, Chen HM, Chen MF. Malignancy in choledochal cysts. Hepatogastroenterology 2000;47:337-40.
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.
Watanabe Y, Toki A, Todani T. Bile duct cancer developed after cyst excision for choledochal cyst. J Hepatobiliary Pancreat Surg 1999;6:207-12.
Ng WT. Bile duct carcinogenesis after excision of extrahepatic bile ducts in pancreaticobiliary maljunction. Surgery 2000;128:492-4.
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.
Gunay-Aygun M. Liver and Kidney Disease in Ciliopathies. Am J Med Genet C Semin Med Genet 2009;151C:296-306.
MacWorter GL. Congenital cystic dilatatin of the common bile duct. Ann Surg 1924;8:604-25.
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.
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.
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.
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.
Cosentino CM, Luck SR, Raffensperger JG, Reynolds M. Choledochal duct cyst: resection with physiologic reconstruction. Surgery 1992;112:740-7.
Edil BH, Olino K, Cameron JL. The current management of choledochal cysts. Adv Surg 2009;43:221-32.
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.
Okada A, Higaki J, Nakamura T, Kamata S. Roux-en-Y versus interposition biliary reconstruction. Surg Gynecol Obstet 1992;174:313-6.
Law R, Topazian M. Diagnosis and treatment of choledochoceles. Clin Gastroenterol Hepatol. 2014;12:196-203.
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.
Samavedy R, Sherman S, Lehman GA. Endoscopic therapy in anomalous pancreatobiliary duct junction. Gastrointest Endosc 1999;50:623-7.
Visser BC, Suh I, Way LW, Kang SM. Congenital choledochal cysts in adults. Arch Surg 2004;139:855-60.
Cerwenka H. Bile duct cyst in adults: interventional treatment, resection, or transplantation. World J Gastroenterol 2013;19:5207-11.
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.
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.
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.
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.
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.
Alizai NK, Dawrant MJ, Najmaldin AS. Robot-assisted resection of choledochal cysts and hepaticojejunostomy in children. Pediatr Surg Int 2014;30:291-4.
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.
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.
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.
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.
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.
Meehan JJ, Elliott S, Sandler A. The robotic approach to complex hepatobiliary anomalies in children: preliminary report. J Pediatr Surg 2007;42:2110-4.
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.
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.
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.
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.
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.
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.
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.
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.
Acknowledgments
We thank Dave Schumick for his assistance with the medical illustrations.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
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
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s11605-016-3181-4