Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Biliary cysts

Mark Topazian, MD
Section Editors
Sanjiv Chopra, MD, MACP
Elizabeth B Rand, MD
Deputy Editor
Shilpa Grover, MD, MPH, AGAF


Biliary cysts are cystic dilations that may occur singly or in multiples throughout the biliary tree. They were originally termed choledochal cysts due to their involvement of the extrahepatic bile duct. However, the original clinical classification [1] was revised in 1977 to include intrahepatic cysts [2]. Biliary cysts are associated with significant complications such as ductal strictures, stone formation, cholangitis, rupture, and secondary biliary cirrhosis. In addition, certain types of biliary cysts have a high risk of malignancy.

This topic will review the pathogenesis, clinical manifestations, diagnosis, and management of biliary cysts. Cholangiocarcinoma, gallbladder cancer, and a detailed discussion of type V biliary cysts (Caroli disease) are discussed elsewhere. (See "Epidemiology, pathogenesis, and classification of cholangiocarcinoma" and "Pathology of malignant liver tumors" and "Clinical manifestations and diagnosis of cholangiocarcinoma" and "Treatment of localized cholangiocarcinoma: Adjuvant and neoadjuvant therapy and prognosis" and "Gallbladder cancer: Epidemiology, risk factors, clinical features, and diagnosis" and "Caroli disease".)


A classification scheme for cysts of the extrahepatic bile ducts (choledochal cysts) was proposed initially in 1959 [1]. It was expanded in 1977 to include intrahepatic cysts [2] and further refined in 2003 to incorporate the presence of an abnormal pancreaticobiliary junction (APBJ) [3] (See 'Abnormal pancreatobiliary junction' below.)

The classification scheme defines six types of biliary cyst (figure 1) [3-5]:

Type I cysts (50 to 85 percent of cysts) – Type I cysts are characterized by cystic or fusiform dilation of the common bile duct (image 1) [2,6]. Type I cysts do not involve the intrahepatic bile ducts. Type I cysts are further subcategorized as [3]:

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Nov 2017. | This topic last updated: Dec 15, 2016.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
  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 1959; 108:1.
  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 1977; 134:263.
  3. Todani T, Watanabe Y, Toki A, Morotomi Y. Classification of congenital biliary cystic disease: special reference to type Ic and IVA cysts with primary ductal stricture. J Hepatobiliary Pancreat Surg 2003; 10:340.
  4. Cha SW, Park MS, Kim KW, et al. Choledochal cyst and anomalous pancreaticobiliary ductal union in adults: radiological spectrum and complications. J Comput Assist Tomogr 2008; 32:17.
  5. Singham J, Yoshida EM, Scudamore CH. Choledochal cysts: part 1 of 3: classification and pathogenesis. Can J Surg 2009; 52:434.
  6. Lipsett PA, Pitt HA, Colombani PM, et al. Choledochal cyst disease. A changing pattern of presentation. Ann Surg 1994; 220:644.
  7. Kagiyama S, Okazaki K, Yamamoto Y, Yamamoto Y. Anatomic variants of choledochocele and manometric measurements of pressure in the cele and the orifice zone. Am J Gastroenterol 1987; 82:641.
  8. Tanaka T. Pathogenesis of choledochocele. Am J Gastroenterol 1993; 88:1140.
  9. Sarris GE, Tsang D. Choledochocele: case report, literature review, and a proposed classification. Surgery 1989; 105:408.
  10. Scholz FJ, Carrera GF, Larsen CR. The choledochocele: correlation of radiological, clinical and pathological findings. Radiology 1976; 118:25.
  11. Law R, Topazian M. Diagnosis and treatment of choledochoceles. Clin Gastroenterol Hepatol 2014; 12:196.
  12. O'Neill JA Jr. Choledochal cyst. Curr Probl Surg 1992; 29:361.
  13. Hukkinen M, Koivusalo A, Lindahl H, et al. Increasing occurrence of choledochal malformations in children: a single-center 37-year experience from Finland. Scand J Gastroenterol 2014; 49:1255.
  14. Komi N, Takehara H, Kunitomo K, et al. Does the type of anomalous arrangement of pancreaticobiliary ducts influence the surgery and prognosis of choledochal cyst? J Pediatr Surg 1992; 27:728.
  15. Howell CG, Templeton JM, Weiner S, et al. Antenatal diagnosis and early surgery for choledochal cyst. J Pediatr Surg 1983; 18:387.
  16. Han SJ, Hwang EH, Chung KS, et al. Acquired choledochal cyst from anomalous pancreatobiliary duct union. J Pediatr Surg 1997; 32:1735.
  17. Iwata F, Uchida A, Miyaki T, et al. Familial occurrence of congenital bile duct cysts. J Gastroenterol Hepatol 1998; 13:316.
  18. Lu, SC. Biliary cysts. In: Textbook of gastroenterology, Yamada, T (Eds), Lippincott Williams and Williams, Philadelphia 1999. p.2292.
  19. Yotuyangi S. Contribution to etiology and pathology of idiopathic cystic dilatation of the common bile duct, with a report of three cases. Gann (Tokyo) 1936; 30:601.
  20. Tyler KL, Sokol RJ, Oberhaus SM, et al. Detection of reovirus RNA in hepatobiliary tissues from patients with extrahepatic biliary atresia and choledochal cysts. Hepatology 1998; 27:1475.
  21. Spitz L. Experimental production of cystic dilatation of the common bile duct in neonatal lambs. J Pediatr Surg 1977; 12:39.
  22. Yamao K, Mizutani S, Nakazawa S, et al. Prospective study of the detection of anomalous connections of pancreatobiliary ducts during routine medical examinations. Hepatogastroenterology 1996; 43:1238.
  23. Jabłońska B. Biliary cysts: etiology, diagnosis and management. World J Gastroenterol 2012; 18:4801.
  24. Ragot E, Mabrut JY, Ouaïssi M, et al. Pancreaticobiliary Maljunctions in European Patients with Bile Duct Cysts: Results of the Multicenter Study of the French Surgical Association (AFC). World J Surg 2017; 41:538.
  25. Song HK, Kim MH, Myung SJ, et al. Choledochal cyst associated the with anomalous union of pancreaticobiliary duct (AUPBD) has a more grave clinical course than choledochal cyst alone. Korean J Intern Med 1999; 14:1.
  26. Funabiki T, Matsubara T, Miyakawa S, Ishihara S. Pancreaticobiliary maljunction and carcinogenesis to biliary and pancreatic malignancy. Langenbecks Arch Surg 2009; 394:159.
  27. Misra SP, Gulati P, Thorat VK, et al. Pancreaticobiliary ductal union in biliary diseases. An endoscopic retrograde cholangiopancreatographic study. Gastroenterology 1989; 96:907.
  28. Li L, Yamataka A, Yian-Xia W, et al. Ectopic distal location of the papilla of vater in congenital biliary dilatation: Implications for pathogenesis. J Pediatr Surg 2001; 36:1617.
  29. Matsumoto S, Tanaka M, Ikeda S, Yoshimoto H. Sphincter of Oddi motor activity in patients with anomalous pancreaticobiliary junction. Am J Gastroenterol 1991; 86:831.
  30. Kato T, Hebiguchi T, Matsuda K, Yoshino H. Action of pancreatic juice on the bile duct: pathogenesis of congenital choledochal cyst. J Pediatr Surg 1981; 16:146.
  31. Iwai N, Tokiwa K, Tsuto T, et al. Biliary manometry in choledochal cyst with abnormal choledochopancreatico ductal junction. J Pediatr Surg 1986; 21:873.
  32. Craig AG, Chen LD, Saccone GT, et al. Sphincter of Oddi dysfunction associated with choledochal cyst. J Gastroenterol Hepatol 2001; 16:230.
  33. Xia HT, Wang J, Yang T, et al. Sphincter of Oddi Dysfunction and the Formation of Adult Choledochal Cyst Following Cholecystectomy: A Retrospective Cohort Study. Medicine (Baltimore) 2015; 94:e2088.
  34. 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 1998; 93:2580.
  35. Voyles CR, Smadja C, Shands WC, Blumgart LH. Carcinoma in choledochal cysts. Age-related incidence. Arch Surg 1983; 118:986.
  36. Katabi N, Pillarisetty VG, DeMatteo R, Klimstra DS. Choledochal cysts: a clinicopathologic study of 36 cases with emphasis on the morphologic and the immunohistochemical features of premalignant and malignant alterations. Hum Pathol 2014; 45:2107.
  37. Singham J, Yoshida EM, Scudamore CH. Choledochal cysts: part 2 of 3: Diagnosis. Can J Surg 2009; 52:506.
  38. Shah OJ, Shera AH, Zargar SA, et al. 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.
  39. Singham J, Schaeffer D, Yoshida E, Scudamore C. Choledochal cysts: analysis of disease pattern and optimal treatment in adult and paediatric patients. HPB (Oxford) 2007; 9:383.
  40. Søreide K, Søreide JA. Bile duct cyst as precursor to biliary tract cancer. Ann Surg Oncol 2007; 14:1200.
  41. Lee SE, Jang JY, Lee YJ, et al. Choledochal cyst and associated malignant tumors in adults: a multicenter survey in South Korea. Arch Surg 2011; 146:1178.
  42. Todani T, Watanabe Y, Toki A, Urushihara N. Carcinoma related to choledochal cysts with internal drainage operations. Surg Gynecol Obstet 1987; 164:61.
  43. Kobayashi S, Asano T, Yamasaki M, et al. Risk of bile duct carcinogenesis after excision of extrahepatic bile ducts in pancreaticobiliary maljunction. Surgery 1999; 126:939.
  44. Watanabe Y, Toki A, Todani T. Bile duct cancer developed after cyst excision for choledochal cyst. J Hepatobiliary Pancreat Surg 1999; 6:207.
  45. Sastry AV, Abbadessa B, Wayne MG, et al. What is the incidence of biliary carcinoma in choledochal cysts, when do they develop, and how should it affect management? World J Surg 2015; 39:487.
  46. Todani T, Tabuchi K, Watanabe Y, Kobayashi T. Carcinoma arising in the wall of congenital bile duct cysts. Cancer 1979; 44:1134.
  47. Ohtsuka T, Inoue K, Ohuchida J, et al. Carcinoma arising in choledochocele. Endoscopy 2001; 33:614.
  48. Dayton MT, Longmire WP Jr, Tompkins RK. Caroli's Disease: a premalignant condition? Am J Surg 1983; 145:41.
  49. Elnemr A, Ohta T, Kayahara M, et al. Anomalous pancreaticobiliary ductal junction without bile duct dilatation in gallbladder cancer. Hepatogastroenterology 2001; 48:382.
  50. Sugiyama M, Abe N, Tokuhara M, et al. Pancreatic carcinoma associated with anomalous pancreaticobiliary junction. Hepatogastroenterology 2001; 48:1767.
  51. Sugiyama M, Atomi Y. Anomalous pancreaticobiliary junction without congenital choledochal cyst. Br J Surg 1998; 85:911.
  52. Funabiki T, Matsubara T, Ochiai M, et al. Surgical strategy for patients with pancreaticobiliary maljunction without choledocal dilatation. Keio J Med 1997; 46:169.
  53. Hidaka E, Yanagisawa A, Seki M, et al. High frequency of K-ras mutations in biliary duct carcinomas of cases with a long common channel in the papilla of Vater. Cancer Res 2000; 60:522.
  54. Kim Y, Hyun JJ, Lee JM, et al. Anomalous union of the pancreaticobiliary duct without choledochal cyst: is cholecystectomy alone sufficient? Langenbecks Arch Surg 2014; 399:1071.
  55. Son YJ, Lee MJ, Koh H, Kim S. Asymptomatic Bile Duct Dilatation in Children: Is It a Disease? Pediatr Gastroenterol Hepatol Nutr 2015; 18:180.
  56. Oduyebo I, Law JK, Zaheer A, et al. Choledochal or pancreatic cyst? Role of endoscopic ultrasound as an adjunct for diagnosis: a case series. Surg Endosc 2015; 29:2832.
  57. Fulcher AS, Turner MA, Sanyal AJ. Case 38: Caroli disease and renal tubular ectasia. Radiology 2001; 220:720.
  58. Masetti R, Antinori A, Coppola R, et al. Choledochocele: changing trends in diagnosis and management. Surg Today 1996; 26:281.
  59. Akhan O, Demirkazik FB, Ozmen MN, Ariyürek M. Choledochal cysts: ultrasonographic findings and correlation with other imaging modalities. Abdom Imaging 1994; 19:243.
  60. Lam WW, Lam TP, Saing H, et al. MR cholangiography and CT cholangiography of pediatric patients with choledochal cysts. AJR Am J Roentgenol 1999; 173:401.
  61. Park DH, Kim MH, Lee SK, et al. Can MRCP replace the diagnostic role of ERCP for patients with choledochal cysts? Gastrointest Endosc 2005; 62:360.
  62. Kim MJ, Han SJ, Yoon CS, et al. Using MR cholangiopancreatography to reveal anomalous pancreaticobiliary ductal union in infants and children with choledochal cysts. AJR Am J Roentgenol 2002; 179:209.
  63. Kim SH, Lim JH, Yoon HK, et al. Choledochal cyst: comparison of MR and conventional cholangiography. Clin Radiol 2000; 55:378.
  64. Keil R, Snajdauf J, Rygl M, et al. Diagnostic efficacy of ERCP in cholestatic infants and neonates--a retrospective study on a large series. Endoscopy 2010; 42:121.
  65. Sugiyama M, Haradome H, Takahara T, et al. Anomalous pancreaticobiliary junction shown on multidetector CT. AJR Am J Roentgenol 2003; 180:173.
  66. Wiedmeyer DA, Stewart ET, Dodds WJ, et al. Choledochal cyst: findings on cholangiopancreatography with emphasis on ectasia of the common channel. AJR Am J Roentgenol 1989; 153:969.
  67. Metreweli C, So NM, Chu WC, Lam WW. Magnetic resonance cholangiography in children. Br J Radiol 2004; 77:1059.
  68. Yazumi S, Takahashi R, Tojo M, et al. Intraductal US aids detection of carcinoma in situ in a patient with a choledochal cyst. Gastrointest Endosc 2001; 53:233.
  69. Lambie H, Cook AM, Scarsbrook AF, et al. Tc99m-hepatobiliary iminodiacetic acid (HIDA) scintigraphy in clinical practice. Clin Radiol 2011; 66:1094.
  70. Sood A, Senthilnathan MS, Deswal S, et al. Spontaneous rupture of a choledochal cyst and the role of hepatobiliary scintigraphy. Clin Nucl Med 2004; 29:392.
  71. Aggarwal S, Kumar A, Roy S, Bandhu S. Massive dilatation of the common bile duct resembling a choledochal cyst. Trop Gastroenterol 2001; 22:219.
  72. Ouaissi M, Kianmanesh R, Ragot E, et al. Impact of previous cyst-enterostomy on patients' outcome following resection of bile duct cysts. World J Gastrointest Surg 2016; 8:427.
  73. Nakano K, Mizuta A, Oohashi S, et al. Protein stone formation in an intrapancreatic remnant cyst after resection of a choledochal cyst. Pancreas 2003; 26:405.
  74. Stain SC, Guthrie CR, Yellin AE, Donovan AJ. Choledochal cyst in the adult. Ann Surg 1995; 222:128.
  75. Wei MF, Qi BQ, Xia GL, et al. Use of the appendix to replace the choledochus. Pediatr Surg Int 1998; 13:494.
  76. Delarue A, Chappuis JP, Esposito C, et al. Is the appendix graft suitable for routine biliary surgery in children? J Pediatr Surg 2000; 35:1312.
  77. Ando H, Kaneko K, Ito T, et al. Complete excision of the intrapancreatic portion of choledochal cysts. J Am Coll Surg 1996; 183:317.
  78. Mizuguchi Y, Nakamura Y, Uchida E. Subsequent biliary cancer originating from remnant intrapancreatic bile ducts after cyst excision: a literature review. Surg Today 2017; 47:660.
  79. Röthlin MA, Löpfe M, Schlumpf R, Largiadèr F. Long-term results of hepaticojejunostomy for benign lesions of the bile ducts. Am J Surg 1998; 175:22.
  80. Zheng X, Gu W, Xia H, et al. Surgical treatment of type IV-A choledochal cyst in a single institution: children vs. adults. J Pediatr Surg 2013; 48:2061.
  81. Singham J, Yoshida EM, Scudamore CH. Choledochal cysts. Part 3 of 3: management. Can J Surg 2010; 53:51.
  82. Xia HT, Dong JH, Yang T, et al. Extrahepatic cyst excision and partial hepatectomy for Todani type IV-A cysts. Dig Liver Dis 2014; 46:1025.
  83. Saing H, Han H, Chan KL, et al. Early and late results of excision of choledochal cysts. J Pediatr Surg 1997; 32:1563.
  84. Yamada T, Furukawa K, Yokoi K, et al. Liver cyst with biliary communication successfully treated with laparoscopic deroofing: a case report. J Nippon Med Sch 2009; 76:103.
  85. Ouaïssi M, Kianmanesh R, Belghiti J, et al. Todani Type II Congenital Bile Duct Cyst: European Multicenter Study of the French Surgical Association and Literature Review. Ann Surg 2015; 262:130.
  86. Chatila R, Andersen DK, Topazian M. Endoscopic resection of a choledochocele. Gastrointest Endosc 1999; 50:578.
  87. Antaki F, Tringali A, Deprez P, et al. A case series of symptomatic intraluminal duodenal duplication cysts: presentation, endoscopic therapy, and long-term outcome (with video). Gastrointest Endosc 2008; 67:163.
  88. Tao KS, Lu YG, Wang T, Dou KF. Procedures for congenital choledochal cysts and curative effect analysis in adults. Hepatobiliary Pancreat Dis Int 2002; 1:442.
  89. Ono S, Fumino S, Shimadera S, Iwai N. Long-term outcomes after hepaticojejunostomy for choledochal cyst: a 10- to 27-year follow-up. J Pediatr Surg 2010; 45:376.
  90. Cho MJ, Hwang S, Lee YJ, et al. Surgical experience of 204 cases of adult choledochal cyst disease over 14 years. World J Surg 2011; 35:1094.
  91. Eriguchi N, Aoyagi S, Okuda K, et al. Carcinoma arising in the pancreas 17 years after primary excision of a choledochal cysts: report of a case. Surg Today 2001; 31:534.
  92. Kurokawa Y, Hasuike Y, Tsujinaka T, et al. Carcinoma of the head of the pancreas after excision of a choledochal cyst. Hepatogastroenterology 2001; 48:578.
  93. Tsuchida A, Kasuya K, Endo M, et al. High risk of bile duct carcinogenesis after primary resection of a congenital biliary dilatation. Oncol Rep 2003; 10:1183.