Choledochal Cyst

Chapter

Abstract

A choledochal cyst (congenital dilatation of the bile duct) shows extra and/or intrahepatic dilatation of the bile duct, and mainly cystic dilatation of the common bile duct. Although the etiology is unknown, it might be congenital. In 1936, Yotsuyanagi suggested a new etiological theory based on unequal epithelial proliferation at the stage of the physiological occlusion of the primitive choledochus. Thereafter, Babbit proposed an abnormal relationship between the common bile duct and pancreatic duct. However, Rustad and Lilly suggested that this abnormal relationship was simply a malformation associated with a choledochal cyst. Therefore, the etiology has not yet been established.

Keywords

Congenital disorders biliary tract Choledochal cyst Surgery Outcomes 

References

  1. 1.
    Yotsuyanagi S. Contribution to the etiology and pathology of idiopathic cystic dilatation of the common bile duct, with report of three cases. Gann. 1936;30:601–53.Google Scholar
  2. 2.
    Babbitt DP. Congenital choledochal cyst: new etiological concept based on an anomalous relationship on the common bile duct and pancreatic bulb. Ann Radiol. 1968;12:231–40.Google Scholar
  3. 3.
    Rustad DG, Lilly JR. Letter to the editor. Surgery. 1987;101:250.Google Scholar
  4. 4.
    Lu S. Biliary cysts and strictures. In: Kaplowitz N, editor. Liver and biliary diseases. Baltimore: Williams & Wilkins, 1996. p. 739–53.Google Scholar
  5. 5.
    Alonso-Lej RWB, Passagno DJ. Congenital choledochal cyst, with a report of two and an analysis of 94 cases. Int Abstr Surg. 1959;108:1–30.Google Scholar
  6. 6.
    Todani T, Watanabe Y, Naruse M, et al. Congenital bile duct cyst. Classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst. Am J Surg. 1977;134:263–9.CrossRefGoogle Scholar
  7. 7.
    Caroli J, Soupault R, Kossakowski J, et al. La dilatation polykystique congenital des voices biliares intrahepatiques. Semin Hop. 1958;34:488–95.Google Scholar
  8. 8.
    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. 1996;31:1417–21.CrossRefGoogle Scholar
  9. 9.
    She WH, Chung HY, Lan LC, et al. Management of choledochal cyst: 30-year experience and results in a single center. J Pediatr Surg. 2009;44:2307–11.CrossRefGoogle Scholar
  10. 10.
    Shi LB, Peng SY, Meng XK, et al. Diagnosis and treatment of congenital choledochal cyst: 20 year’s experience in China. World J Gastroenterol. 2001;7:732–4.CrossRefGoogle Scholar
  11. 11.
    Iwai N, Deguchi E, Yanagihara J, et al. Cancer arising in a choledochal cyst in a 12-year-old girl. J Pediatr Surg. 1990;25:1261–3.CrossRefGoogle Scholar
  12. 12.
    Hori T, Oike F, Ogura Y, et al. Liver transplantation for congenital biliary dilatation: a single-center experience. Dig Surg. 2010;27:492–501.CrossRefGoogle Scholar
  13. 13.
    Ruiz-Elizalde AR, Cowels RA. A practical algorithm for accurate diagnosis and treatment of perinatally identified biliary ductal dilatation: three cases that underscore the importance of an individualized approach. J Matern Fetal Neonatal Med. 2009;22:622–8.CrossRefGoogle Scholar
  14. 14.
    Mackenzie TC, Rowel LJ, Flake AW, et al. Management of prenatally diagnosed choledochal cysts. J Pediatr Surg. 2001;36:1241–3.CrossRefGoogle Scholar
  15. 15.
    Iwai N, Deguchi E, Sasaki Y, et al. Antenatal diagnosis of biliary atresia (noncorrectable cyst type). Eur J Pediatr Surg. 1999;9:340–2.CrossRefGoogle Scholar
  16. 16.
    Howell CG, Templeton JM, Weiner S, et al. Antenatal diagnosis and early surgery for choledochal cyst. J Pediatr Surg. 1983;18:387–93.CrossRefGoogle Scholar
  17. 17.
    Lee SC, Kim HY, Se J, et al. Is excision of choledochal cyst in the neonatal period necessary ? J Pediatr Surg. 2006;41:1984–6.CrossRefGoogle Scholar
  18. 18.
    Foo DC, Wong KK, Lon LC, et al. Impact of prenatal diagnosis on choledochal cysts and the benefits of early excision. J Pediatr Surg. 2009;45:28–30.Google Scholar
  19. 19.
    Lugo-Vicente HI. Prenatally diagnosed choledochal cysts: observation or early surgery? J Pediatr Surg. 1995;30:1288–90.CrossRefGoogle Scholar
  20. 20.
    Kasai M. Advances in treatment of biliary atresia. Jpn J Surg. 1983;13:265–76.CrossRefGoogle Scholar
  21. 21.
    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. 1990;171:291–8.Google Scholar
  22. 22.
    Todani T, Urushihara N, Morotomi Y, et al. Characteritics of choledochal cysts in neonates and early infants. Eur J Pediatr Surg. 1995;5:143–5.CrossRefGoogle Scholar
  23. 23.
    Davenport M, Stringer MD, Howard ER. Biliary amylase and congenital choledochal dilatation. J Pediatr Surg. 1995;30:474–7.CrossRefGoogle Scholar
  24. 24.
    Gubernick JA, Rosenberg HK, Ilaslan H, et al. US approach to jaundice in infants and children. Radiographics. 2000;20:173–95.CrossRefGoogle Scholar
  25. 25.
    Kim JE, Lee JK, Lee KT, et al. The clinical significance of common bile-duct dilatation in patients without biliary symptoms or causative lesions on ultrasonography. Endoscopy. 2001;33:495–500.CrossRefGoogle Scholar
  26. 26.
    Fumino S, Ono S, lwai N, et al. Diagnostic impact of computed tomography cholangiography and magnetic resonance cholangiopancreatography on pancreaticobiliary maljunction. J Pediatr Surg. 2011;46:1373–8.CrossRefGoogle Scholar
  27. 27.
    Otto AK, Neal MD, Slivka AN, et al. An appraisal of endoscopic retrograde cholangiopancreatography (ERCP) for pancreaticobiliary disease in children: our institutional experience in 231 cases. Surg Endosc. 2011;25:2536–40.CrossRefGoogle Scholar
  28. 28.
    Paris C, Bejjani J, Beaunoyer M, et al. Endoscopic retrograde cholangiopancreatography is useful and safe in children. J Pediatr Surg. 2010;45:938–42.CrossRefGoogle Scholar
  29. 29.
    Sharma AK, Wakhlu A, Sharma SS. The role of endoscopic retrograde cholangiopancreatography in the management of choledochal cyst in children. J Pediatr Surg. 1995;30:65–7.CrossRefGoogle Scholar
  30. 30.
    Chavhan GB, Babyn PS, Manson D, et al. Pediatric MR cholangiopancreatography: principles, technique, and clinical applications. Radiographics. 2008;28:1951–62.CrossRefGoogle Scholar
  31. 31.
    Karjoo M, Bishop HG, Borns P, et al. Choledochal cyst presenting as recurrent pancreatitis. Pediatrics. 1973;51:289–91.Google Scholar
  32. 32.
    Matsumoto Y, Uchida K, Nakase A, et al. Congenital cystic dilatation of the common bile duct as a cause of primary bile stone. Am J Surg. 1977;134:346–52.CrossRefGoogle Scholar
  33. 33.
    Tsuchiya R, Harada N, Ito T, et al. Malignant tumors in choledochal cysts. Ann Surg. 1977;186:22–8.CrossRefGoogle Scholar
  34. 34.
    Kasai M, Asakura Y, Taira Y. Surgical treatment of choledochal cyst. Ann Surg. 1970;172:844–51.CrossRefGoogle Scholar
  35. 35.
    Ishida M, Tsuchida Y, Saito S, et al. Primary excision of choledochal cysts. Surgery. 1970;68:884–8.Google Scholar
  36. 36.
    Lilly JR. Total excision of choledochal cyst. Surg Gynecol Obstet. 1978;146:254–6.Google Scholar
  37. 37.
    Iwai N, Yanagihara J, Tokiwa K, et al. Congenital choledochal dilatation with emphasis on pathophysiology of the biliary tract. Ann Surg. 1992;215:27–30.CrossRefGoogle Scholar
  38. 38.
    Todani T, Watanabe Y, Urushihara N, et al. Biliary complication after excisional procedure for choledochal cyst. J Pediatr Surg. 1995;30:478–81.CrossRefGoogle Scholar
  39. 39.
    Okada A, Nakamura T, Okumura K, et al. Surgical treatment of congenital dilatation of bile duct (choledochal cyst) with technical consideration. Surgery. 1987;101:238–43.PubMedPubMedCentralGoogle Scholar
  40. 40.
    Stringer MD, Dhawan A, Davenport M, et al. Choledochal cysts: lessons from a 20 year experience. Arch Dis Child. 1995;73:528–31.CrossRefGoogle Scholar
  41. 41.
    Shimotakahara A, Yamataka A, Yanai T, et al. Roux-en-Y hepaticojejuno stomy or hepaticoduodenostomy for biliary reconstruction during the surgical treatment of choledochal cyst: which is better? Pediatr Surg Int. 2005;21:5–7.CrossRefGoogle Scholar
  42. 42.
    Todani T, Watanabe Y, Mizuguchi T, et al. Hepaticoduodenostomy at the hepatic hilum after excision of choledochal cyst. Am J Surg. 1981;142:584–7.CrossRefGoogle Scholar
  43. 43.
    Santore MT, Behar BJ, Blinman TA, et al. Hepaticoduodenostomy vs hepaticojejunostomy for reconstruction after resection of choledochal cyst. J Pediatr Surg. 2011;46:209–13.CrossRefGoogle Scholar
  44. 44.
    Okada A, Hasegawa T, Oguchi Y, et al. Recent advances in patho physiology and surgical treatment of congenital dilatation of the bile duct. J Hepatobiliary Pancreat Surg. 2002;9:342–51.CrossRefGoogle Scholar
  45. 45.
    Chiang L, Chui CH, Low Y, et al. Perforation: a rare complication of choledochal cysts in children. Pediatr Surg Int. 2011;27:823–7.CrossRefGoogle Scholar
  46. 46.
    Ando K, Miyano T, Kohno S, et al. Spontaneous perforation of choledochal cyst: a study of 13 cases. Eur J Pediatr Surg. 1998;8:23–5.CrossRefGoogle Scholar
  47. 47.
    Fumino S, Iwai N, Deguchi E, et al. Spontaneous rupture of choledochal cyst with pseudocyst formation-report on 2 cases and literature review. J Pediatr Surg. 2006;41:e19–21.CrossRefGoogle Scholar
  48. 48.
    Madjov R, Chervenkov P, Madjova V, et al. Caroli’s disease. Report of 5 cases and review of literature. Hepatogastroenterology. 2005;52:606–9.Google Scholar
  49. 49.
    Yonem O, Bayraktar Y. Clinical characteristics of Caroli’s syndrome. World J Gastroenterol. 2007;13:1934–7.CrossRefGoogle Scholar
  50. 50.
    Kamisawa T, Okamoto A, Tsuruta K, et al. Carcinoma arising in congenital choledochal cysts. Hepatogastroenterology. 2008;55:329–32.Google Scholar
  51. 51.
    Ono S, Fumino S, Iwai N. Diagnosis and treatment of pancreaticobiliary maljunction in children. Surg Today. 2011;41:601–5.CrossRefGoogle Scholar
  52. 52.
    Imazu M, Iwai N, Tokiwa K, et al. Factors of biliary carcinogenesis in choledochal cysts. Eur J Pediatr Surg. 2001;11:24–7.CrossRefGoogle Scholar
  53. 53.
    Ono S, Sakai K, Iwai N, et al. Development of bile duct cancer in a 26-year-old man after resection of infantile choledochal cyst. J Pediatr Surg. 2008;43:E17–9.CrossRefGoogle Scholar
  54. 54.
    Watanabe Y, Toki A, Todani T. Bile duct cancer developed after cyst excision for choledochal cyst. J Hepaticobiliary Pancreat Surg. 1999;6:207–12.CrossRefGoogle Scholar
  55. 55.
    Ohta T, Nakagawa T, Ueno K, et al. Clinical experience of biliary tract carcinomas associated with anomalous union of the pancreaticobiliary ductal system. Jpn J Surg. 1990;20:36–43.CrossRefGoogle Scholar
  56. 56.
    Todani T, Watanabe Y, Urushihara N, et al. Choledochal cyst, pancreatobiliary malunion and cancer. J Hepatobilary Pancreat Surg. 1994;1:247–51.CrossRefGoogle Scholar
  57. 57.
    Witcombe JB, Cremin BJ. The width of the common bile duct in childhood. Pediatr Radiol. 1978;7:147–9.CrossRefGoogle Scholar
  58. 58.
    Miyano T, Ando K, Yamataka A, et al. Pancreatobiliary maljunction associated with nondilatation or minimal dilatation of the common bile duct in children: diagnosis and treatment. Eur J Pediatr Surg. 1996;6:334–7.CrossRefGoogle Scholar
  59. 59.
    Iwai N, Fumino S, Tsuda T, et al. Surgical treatment for anomalous arrangement of the pancreaticbiliary duct with nondilatation of the common bile duct. J Pediatr Surg. 2004;39:1794–6.CrossRefGoogle Scholar
  60. 60.
    Yamauchi S, Koga A, Matsumoto S, et al. Anomalous junction of pancreaticobiliary duct without congenital choledochal cyst: a possible risk factor for gallbladder cancer. Am J Gastroenterol. 1987;82:20–4.Google Scholar
  61. 61.
    Tanaka K, Nishimura A, Yamada K, et al. Cancer of the gallbladder associated with anomalous junction of the pancreaticobiliary duct system without bile duct dilatation. Br J Surg. 1993;80:622–4.CrossRefGoogle Scholar
  62. 62.
    Ando H, Ito T, Nagaya M, et al. Pancreaticobiliary maljunction without choledochal cysts in infants and children: clinical features and surgical therapy. J Pediatr Surg. 1995;30:1658–62.CrossRefGoogle Scholar
  63. 63.
    Ono S, Fumino S, lwai N, et al. Long-term outcomes after hepatico jejunostomy for choledochal cyst: a 10- to 27-year follow-up. J Pediatr Surg. 2010;45:376–8.CrossRefGoogle Scholar
  64. 64.
    Saing H, Han H, Chan KL, et al. Early and late results of excision of choledochal cysts. J Pediatr Surg. 1997;32:1563–8.CrossRefGoogle Scholar
  65. 65.
    Takeshita N, Ota T, Yamamoto M. Forty-year experience with flow diversion surgery for patients with congenital choledochal cyst with pancreaticobiliary maljunction at a single institution. Ann Surg. 2011;254:1050–3.CrossRefGoogle Scholar
  66. 66.
    Ure B, Schier F, Schmidt AL, et al. Laparoscopic resection of congenital choledochal cyst, choledochojejunostomy, and extraabdominal Roux-en-Y anastomosis. Surg Endosc. 2005;19:1055–7.CrossRefGoogle Scholar
  67. 67.
    Palanivelu C, Rangarajun M, Parthasarathi R, et al. Laparoscopic management of choledochal cysts: technique and outcomes- a retrospective study of 35 patients from a tertiary center. J Am Coll Surg. 2008;207:839–46.CrossRefGoogle Scholar
  68. 68.
    Urushihara N, Fukuzawa H, Fukumoto K, et al. Totally laparoscopic management of choledochal cyst: Roux-en-Y jejunostomy and wide hepaticojejunostomy with hilar ductoplasty. J Laparoendosc Adv Surg Tech A. 2011;21:361–6.CrossRefGoogle Scholar
  69. 69.
    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. 2010;25:1587–73.CrossRefGoogle Scholar
  70. 70.
    Nguyen Thanh L, Hien PD, Dung le A, et al. Laparoscopic repair for choledochal cyst: lessons from 190 cases. J Pediatr Surg. 2010;45:540–4.CrossRefGoogle Scholar

Copyright information

© Springer-Verlag London Ltd., part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of SurgeryMeiji University of Integrative MedicineKyotoJapan

Personalised recommendations