Skip to main content

Advertisement

Log in

Japanese clinical practice guidelines for pancreaticobiliary maljunction

Journal of Gastroenterology Aims and scope Submit manuscript

Abstract

There have been no clinical guidelines for the management of pancreaticobiliary maljunction (PBM). The Japanese Study Group on Pancreaticobiliary Maljunction (JSPBM) has proposed to establish clinical practice guidelines on how to deal with PBM, with the support of the Japan Biliary Association (JBA). Because the body of evidence-based literature is relatively small, we decided to create guidelines based on the consensus of experts, using the medical literature for reference. A total of 46 clinical questions (CQs) were considered by the members of the editorial committee responsible for the guidelines. The CQs covered distinct aspects of PBM: (1) Concepts and Pathophysiology (10 CQs); (2) Diagnosis (10 CQs); (3) Pancreatobiliary complications (9 CQs); and (4) Treatments and prognosis (17 CQs). Statements and comments for each CQ were prepared by the guidelines committee members and collaborating partners. The CQs were completed after review by members of the editorial committee, meetings of this committee, public comments on the homepages of the JSPBM and the JBA, public hearings, and assessment and approval by the guidelines evaluation board. PBM includes cases where the bile duct is dilated (PBM with biliary dilatation) and those in which it is not (PBM without biliary dilatation). In these guidelines, PBM with biliary dilatation is defined as being identical to congenital biliary dilatation of Todani type I (except for type Ib) and type IV-A, both of which are accompanied by PBM in almost all cases. These guidelines are created to provide assistance in the clinical practice of PBM management; their contents focus on clinical utility, and they include general information on PBM to make this disease more widely recognized.

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.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6
Fig. 7
Fig. 8
Fig. 9
Fig. 10
Fig. 11
Fig. 12

References

  1. The Committee of Japanese Study Group on Pancreaticobiliary Maljunction (JSPBM) for diagnostic criteria: diagnostic criteria of pancreaticobiliary maljunction. J Hepatobiliary Pancreat Surg. 1994;1:219–21.

    Google Scholar 

  2. Kamisawa T, Okamoto A. Biliopancreatic and pancreatobiliary refluxes in cases with and without pancreaticobiliary maljunction: diagnosis and clinical implications. Digestion. 2006;73:228–36.

    PubMed  Google Scholar 

  3. Kamisawa T, Takuma K, Anjiki H, Egawa N, Kurata M, Honda G, et al. Pancreaticobiliary maljunction. Clin Gastroenterol Hepatol. 2009;7:S84–8.

    PubMed  Google Scholar 

  4. Odgers PNB. Some observations on the development of the ventral pancreas in man. J Anat. 1930;65:1–7.

    PubMed  CAS  Google Scholar 

  5. Oi I, Ohashi M. Embryological study of pancreaticobiliary maljunction. Tan to Sui. 1982;3:463–76 (in Japanese).

    Google Scholar 

  6. Oi I, Toki F, Nishino T, Oyama H. A developmental study of cystic biliary dilatation of pancreaticobiliary maljunction based on three cases with cystic dilatation of choledochus and cystic duct but hepatic duct. J Japan Biliary Assoc. 2007;21:39–44 (in Japanese with English abstract).

    Google Scholar 

  7. Ando H, Kaneko K, Ito F, Seo T, Harada T, Watanabe Y. Embryogenesis of pancreaticobiliary maljunction inferred from development of duodenal atresia. J Hepatobiliary Pancreat Surg. 1999;6:50–4.

    PubMed  CAS  Google Scholar 

  8. Matsumoto Y, Fujii H, Itakura J, Mogaki M, Matsuda M, Morozumi A, et al. Pancreaticobiliary maljunction: etiologic concepts based on radiologic aspects. Gastrointest Endosc. 2001;53:614–9.

    PubMed  CAS  Google Scholar 

  9. Tanaka T. Pathogenesis of choledochal cyst with anomalous pancreatico-biliary ductal union. Am J Gastroenterol. 1995;90:685.

    PubMed  CAS  Google Scholar 

  10. Komi N. Our studies on choledochal cysts and review of the literature: with special reference to pancreaticobiliary maljunction. In: Koyanagi Y, Aoki T, editors. Pancreaticobiliary maljunction. Tokyo: Igaku Tosho; 2002. p. 1–16.

    Google Scholar 

  11. Komi N, Udaka H, Ikeda N, Kashiwagi Y. Congenital dilatation of the biliary tract; new classification and study with particular reference to anomalous arrangement of the pancreaticobiliary ducts. Gastroenterol Jpn. 1977;12:293–304.

    PubMed  CAS  Google Scholar 

  12. 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.

    PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  14. Todani T. Congenital choledochal dilatation: classification, clinical features, and long-term results. J Hepatobiliary Pancreat Surg. 1997;4:276–82.

    Google Scholar 

  15. Tashiro S, Imaizumi T, Ohkawa H, Okuda A, Katoh T, Kawarada Y, et al. Overall report on the registration study of the Japanese study group on pancreaticobiliary maljunction for the past 10 years. In: Koyanagi Y, Aoki T, editors. Pancreaticobiliary maljunction. Tokyo: Igaku Tosho; 2002. p. 401–10.

    Google Scholar 

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

    PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  18. Kim MH, Lim BC, Park HJ, Lee SK, Kim CD, Roe IH, et al. A study on normal structures, variations, and anomalies of the Korean pancreaticobiliary ducts: cooperative multicenter study. Korean J Gastrointest Endosc. 2000;21:624–32.

    Google Scholar 

  19. Olbourne NA. Choledochal cysts: a review of the cystic anomalies of the biliary tree. Ann R Coll Surg. 1975;56:26–32.

    CAS  Google Scholar 

  20. Howell CG, Templeton JM, Weiner S, Glassman M, Betts JM, et al. Antenatal diagnosis and early surgery for choledochal cyst. J Pediatr Surg. 1983;18:387–93.

    PubMed  CAS  Google Scholar 

  21. Lenriot JP, Gigot JF, Segol P. Bile duct cysts in adults: a multi-institutional retrospective study. French Association for Surgical Research. Ann Surg. 1998;228:159–66.

    PubMed  CAS  Google Scholar 

  22. Suda K, Miyano T, Hashimoto K. The choledocho-pancreatico-ductal junction in infantile obstructive jaundice disease. Acta Pathol Jpn. 1980;30:187–94.

    PubMed  CAS  Google Scholar 

  23. Boyden EA. The anatomy of the choledochoduodenal junction in man. Surg Gynecol Obstet. 1957;104:642–52.

    Google Scholar 

  24. Fujii H, Yang Y, Tang R, Kunitomo K, Itakura J, Mogaki M, et al. Epithelial cell proliferation activity of the biliary ductal system with congenital biliary malformations. J Hepatobiliary Pancreat Surg. 1999;6:294–302.

    PubMed  CAS  Google Scholar 

  25. Hanada K, Itoh M, Fujii K, Tsuchida A, Ooishi H, Kajiyama G. K-ras and p53 mutations in stage I gallbladder carcinoma with anomalous junction of the pancreatobiliary duct. Cancer. 1996;77:452–8.

    PubMed  CAS  Google Scholar 

  26. Motosugi U, Ichikawa T, Araki T, Kitahara F, Sato T, Itakura J, et al. Secretin-stimulating MRCP in patients with pancreatobiliary maljunction and occult pancreatobiliary reflux: direct demonstration of pancreatobiliary reflux. Eur Radiol. 2007;17:2262–7.

    PubMed  Google Scholar 

  27. Fujii H, Matsumoto Y, Yamamoto M, Miura K, Matsuda M, Sugahara K. Bile flow analysis by hepatobiliary scintigraphy in the terminal bile duct in patients with congenital malformations of the pancreaticobiliary ductal system. Gastroenterol Jpn. 1991;26:201–8.

    PubMed  CAS  Google Scholar 

  28. Tanaka M, Ikeda S, Kawakami K, Nakayama F. The pressure of a positive pressure gradient from pancreatic duct to choledochal cyst demonstrated by duodenoscopic microtransducer manometry: clue to pancreaticobiliary reflux. Endoscopy. 1982;14:45–7.

    PubMed  CAS  Google Scholar 

  29. Tashiro S, Imaizumi T, Ohkawa H, Okada A, Katoh T, Kawaharada Y, et al. Pancreaticobiliary maljunction: retrospective and nationwide survey in Japan. J Hepatobiliary Pancreat Surg. 2003;10:345–51.

    PubMed  Google Scholar 

  30. Matsufuji H, Araki Y, Nakamura A, Ohigashi S, Watanabe F. Dynamic study of pancreaticobiliary reflux using secretin-stimulated magnetic resonance cholangiopancreatography in patients with choledochal cysts. J Pediatr Surg. 2006;41:1652–6.

    PubMed  Google Scholar 

  31. Armstrong CP, Taylor TV. Pancreatic duct reflux and acute gallstone pancreatitis. Ann Surg. 1986;204:59–64.

    PubMed  CAS  Google Scholar 

  32. Heloury Y, Leborgne J, Rogez JM, Robert R, Lehur PA, Pannier M, et al. Radiologic anatomy of the bile ducts based on intraoperative investigation in 250 cases. Anat Clin. 1985;7:93–102.

    PubMed  CAS  Google Scholar 

  33. Fujisaki S, Tomita R, Koshinaga T, Fukuzawa M. Analysis of pancreaticobiliary ductal union based on intraoperative cholangiography in patients undergoing laparoscopic cholecystectomy. Scand J Gastroenterol. 2002;37:956–9.

    PubMed  CAS  Google Scholar 

  34. Fumino S, Tokiwa K, Katoh T, Ono S, Iwai N. New insight into bile flow dynamics in anomalous arrangement of the pancreaticobiliary duct. Br J Surg. 2002;89:865–9.

    PubMed  CAS  Google Scholar 

  35. Dowdy GS, Waldron GW, Brown WG. Surgical anatomy of the pancreaticobiliary ductal system. Arch Surg. 1962;84:229–46.

    PubMed  Google Scholar 

  36. Kamisawa T, Amemiya K, Tu Y, Egawa N, Sakaki N, Tsuruta K, et al. Clinical significance of a long common channel. Pancreatology. 2002;2:122–8.

    PubMed  Google Scholar 

  37. Kamisawa T, Funata N, Hayashi Y, Egawa N, Nakajima H, Tsuruta K, et al. Pathologic changes in the non-carcinomatous epithelium of the gallbladder in patients with a relatively long common channel. Gastrointest Endosc. 2004;60:56–60.

    PubMed  Google Scholar 

  38. Kamisawa T, Go K, Chen PY, Tu Y, Fujiwara T, Endoh J, et al. Lesions with a high risk of carcinogenesis in the gallbladder of patients with a long common channel. Dig Endosc. 2006;18:192–5.

    Google Scholar 

  39. Kamisawa T, Suyama M, Fujita N, Maguchi H, Hanada K, Ikeda S, et al. Pancreatobiliary reflux and the length of a common channel. J Hepatobiliary Pancreat Sci. 2010;17:865–70.

    PubMed  Google Scholar 

  40. Sai JK, Suyama M, Kubokawa Y, Tadokoro H, Sato N, Maehara T, et al. Occult pancreatobiliary reflux in patients with a normal pancreaticobiliary junction. Gastrointest Endosc. 2003;57:364–8.

    PubMed  Google Scholar 

  41. Japanese Study Group on Pancreaticobiliary Maljunction (JSPBM): Registration study of pancreaticobiliary maljunction cases In: Funabiki T, editor. Pancreaticobiliary maljunction—consensus and controversy. Tokyo: Igaku Tosho; 1997. p. 409–25 (in Japanese).

  42. Sugai M, Ishido K, Endoh M, Hada R, Munakata H. Sonographic demonstration of wall thickness of the gallbladder in pediatric patients with pancreatico-biliary maljunction. J Hepatobiliary Pancreat Sci. 2010;17:345–8.

    PubMed  Google Scholar 

  43. Hanada K, Itoh M, Fujii K, Tsuchida A, Hirata M, Ishimaru S, et al. Pathology and cellular kinetics of gallbladder with an anomalous junction of the pancreaticobiliary duct. Am J Gastroenterol. 1996;91:1007–11.

    PubMed  CAS  Google Scholar 

  44. Yamamoto M, Nakajo S, Tahara E, Ito M, Taniyama K, Shimamoto F, et al. Mucosal changes of the gallbladder in anomalous union with the pancreatico-biliary duct system. Pathol Res Pract. 1991;187:241–6.

    PubMed  CAS  Google Scholar 

  45. Itokawa F, Itoi T, Nakamura K, Sofuni A, Kakimi K, Moriyasu F, et al. Assessment of occult pancreatobiliary reflux in patients with pancreaticobiliary disease by ERCP. J Gastroenterol. 2004;39:988–94.

    PubMed  Google Scholar 

  46. Sugiyama M, Atomi Y. Anomalous pancreaticobiliary junction without congenital choledochal cyst. Br J Surg. 1998;85:911–6.

    PubMed  CAS  Google Scholar 

  47. Irie H, Honda H, Jimi M, Yokohata K, Chijiiwa K, Kuroiwa T, et al. Value of MR cholangiopancreatography in evaluating choledochal cysts. AJR Am J Roentgenol. 1998;171:1381–5.

    PubMed  CAS  Google Scholar 

  48. Hirohashi S, Hirohashi R, Uchida H, Akira M, Itoh T, Haku E, et al. Pancreatitis: evaluation with MR cholangiopancreatography in children. Radiology. 1997;203:411–5.

    PubMed  CAS  Google Scholar 

  49. Sugiyama M, Baba M, Atomi Y, Hanaoka H, Mizutani Y, Hachiya J. Diagnosis of anomalous pancreaticobiliary junction: value of magnetic resonance cholangiopancreatography. Surgery. 1998;123:391–7.

    PubMed  CAS  Google Scholar 

  50. Matos C, Nicaise N, Devière J, Cassart M, Metens T, Struyven J, et al. Choledochal cysts: comparison of findings at MR cholangiopancreatography and endoscopic retrograde cholangiopancreatography in eight patients. Radiology. 1998;209:443–8.

    PubMed  CAS  Google Scholar 

  51. Kim MJ, Han SJ, Yoon CS, Kim JH, Oh JT, Chung KS, et al. Using MR cholangiopancreatography to reveal anomalous pancreaticobiliary ductal union in infants and children with choledochal cysts. AJR Am J Roentgenol. 2002;179:209–14.

    PubMed  Google Scholar 

  52. Kamisawa T, Tu Y, Egawa N, Tsuruta K, Okamoto A, Kamata N. MRCP of congenital pancreaticobiliary malformation. Abdom Imaging. 2007;32:129–33.

    PubMed  CAS  Google Scholar 

  53. Lam WW, Lam TP, Saing H, Chan FL, Chan KL. MR cholangiography and CT cholangiography of pediatric patients with choledochal cysts. AJR Am J Roentgenol. 1999;173:401–5.

    PubMed  CAS  Google Scholar 

  54. Mitake M, Nakazawa S, Naitoh Y, Kimoto E, Tsukamoto Y, Yamao K, et al. Value of endoscopic ultrasonography in the detection of anomalous connection of the pancreatobiliary duct. Endoscopy. 1991;23:117–20.

    PubMed  CAS  Google Scholar 

  55. Sugiyama M, Atomi Y. Endoscopic ultrasonography for diagnosing anomalous pancreaticobiliary junction. Gastrointest Endosc. 1997;45:261–7.

    PubMed  CAS  Google Scholar 

  56. Yusuf TE, Bhutani MS. Role of endoscopic ultrasonography in diseases of the extrahepatic biliary system. J Gastroenterol Hepatol. 2004;19:243–50.

    PubMed  Google Scholar 

  57. Tanno S, Obara T, Maguchi H, Mizukami Y, Shudo R, Fujii T, et al. Thickened inner hypoechoic layer of the gallbladder wall in the diagnosis of anomalous pancreaticobiliary ductal union with endosonography. Gastrointest Endosc. 1997;46:520–6.

    PubMed  CAS  Google Scholar 

  58. Sakamoto H, Mutoh H, Ido K, Satoh S, Kumagai M, Hayakawa H, et al. Intestinal metaplasia in gallbladder correlates with high amylase levels in bile in patients with a morphologically normal pancreaticobiliary duct. Hum Pathol. 2009;40:1762–7.

    PubMed  CAS  Google Scholar 

  59. Horaguchi J, Fujita N, Noda Y, Kobayashi G, Ito K, Takasawa O, et al. Amylase levels in bile in patients with a morphologically normal pancreaticobiliary ductal arrangement. J Gastroenterol. 2008;43:305–11.

    PubMed  CAS  Google Scholar 

  60. 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.

    PubMed  CAS  Google Scholar 

  61. Kitagawa Y, Yamaguchi Ai, Isogai M, Hori A, Kaneoka Y, Igami T, et al. Clinicopathological studies on cases with high amylase concentration of gallbladder bile. J Japan Biliary Assoc. 1999;13:39–44 (in Japanese with English abstract).

  62. Morine Y, Mori H, Utsunomiya T, Imura S, Ikemoto T, Shimada M. Epidemiology and clinical features of pancreaticobiliary maljunction. J Japan Biliary Assoc. 2011;25:133–40 (in Japanese with English abstract).

    Google Scholar 

  63. Jeong IH, Jung YS, Kim H, Kim BW, Kim JW, Hong J, et al. Amylase level in extrahepatic bile duct in adult patients with choledochal cyst plus anomalous pancreatico-biliary ductal union. World J Gastroenterol. 2005;11:1965–70.

    PubMed  CAS  Google Scholar 

  64. Sugiyama Y, Kobori H, Hakamada K, Seito D, Sasaki M. Altered bile composition in the gallbladder and common bile duct of patients with anomalous pancreaticobiliary ductal junction. World J Surg. 2000;24:17–21.

    PubMed  CAS  Google Scholar 

  65. Matsuda M, Watanabe G, Hashimoto M, Udagawa H. Evaluation of pancreaticobiliary maljunction and low bile amylase levels. J Japan Biliary Assoc. 2007;21:119–24 (in Japanese with English abstract).

    Google Scholar 

  66. Dewbury KC, Aluwihare AP, Birch SJ, Freeman NV. Prenatal ultrasound demonstration of a choledochal cyst. Br J Radiol. 1980;53:906–7.

    PubMed  CAS  Google Scholar 

  67. Kawashima S, Urushihara N, Fukumoto K, Suzuki K, Matsuoka T, Fukuzawa H, et al. The management of prenatally diagnosed choledochal cyst. J Jpn Soc Pediatr Surg. 2009;45:699–705 (in Japanese with English abstract).

    Google Scholar 

  68. Schooeder D, Smith L, Prain C. Antenatal diagnosis of choledochal cyst at 15 weeks’ gestation: etiologic implications and management. J Pediatr Surg. 1989;24:936–8.

    Google Scholar 

  69. Redkar R, Davenport M, Howard ER. Antenatal diagnosis of congenital anomalies of the biliary tract. J Pediatr Surg. 1988;33:700–4.

    Google Scholar 

  70. Lugo-Vicente HL. Prenatally diagnosed choledochal cysts: observation or early surgery? J Pediatr Surg. 1995;30:1288–90.

    PubMed  CAS  Google Scholar 

  71. Sherwood W, Boyd P, Lakhoo K. Postnatal outcome of antenatally diagnosed intra-abdominal cysts. Pediatr Surg Int. 2008;24:763–5.

    PubMed  CAS  Google Scholar 

  72. Uchimura M. Anomalous arrangement of pancreaticobiliary ductal system and cholelithiasis. In: Komi N, editor. Pancreaticobiliary maljunction. Tokyo: Herusu; 1987. p. 105–16 (in Japanese).

  73. Kusano T, Takao T, Tachibana K, Tanaka Y, Kamachi M, Ikematsu Y, et al. Whether or not prophylactic excision of the extrahepatic bile duct is appropriate for patients with pancreaticobiliary maljunction without bile duct dilatation. Hepatogastroenterology. 2005;52:1649–53.

    PubMed  CAS  Google Scholar 

  74. Terui K, Yoshida H, Kouchi K, Hishiki T, Saito T, Mitsunaga T, et al. Endoscopic sphincterotomy is a useful preoperative management for refractory pancreatitis associated with pancreaticobiliary maljunction. J Pediatr Surg. 2008;43:495–9.

    PubMed  Google Scholar 

  75. Sugiyama M, Atomi Y, Kuroda A. Pancreatic disorders associated with anomalous pancreaticobiliary junction. Surgery. 1999;126:492–7.

    PubMed  CAS  Google Scholar 

  76. Kamisawa T, Tu Y, Nakajima H, Egawa N, Tsuruta K, Okamoto A, et al. Acute pancreatitis and a long common channel. Abdom Imaging. 2007;32:365–9.

    PubMed  Google Scholar 

  77. Ohuchida J, Chijiiwa K, Hiyoshi M, Kobayashi K, Konomi H, Tanaka M. Long-term results of treatment for pancreaticobiliary maljunction without bile duct dilatation. Arch Surg. 2006;141:1066–70.

    PubMed  Google Scholar 

  78. Toki F, Nishino T, Yoshida K, Cho M, Kozu T, Takeuchi T, et al. Anomaly of the pancreatic and biliary duct system—its association with pancreatitis. Tan to sui. 1991;12:1085–93 (in Japanese).

  79. Kamisawa T, Matsukawa M, Amemiya K, Tu Y, Egawa N, et al. Pancreatitis associated with pancreaticobiliary maljunction. Hepatogastroenterology. 2003;50:1665–8.

    PubMed  Google Scholar 

  80. Opie EL. Etiology of acute hemorrhagic pancreatitis. Bull Johns Hopkins Hosp. 1901;12:182–8.

    Google Scholar 

  81. Shimada K, Yanagisawa J, Nakayama F. Increased lysophosphatidylcholine and pancreatic enzyme content in bile of patients with anomalous pancreaticobiliary ductal junction. Hepatology. 1991;13:438–44.

    PubMed  CAS  Google Scholar 

  82. Kaneko K, Ando H, Ito T, Watanabe Y, Seo T, Harada T, et al. Protein plugs cause symptoms in patients with choledochal cysts. Am J Gastroenterol. 1997;92:1018–21.

    PubMed  CAS  Google Scholar 

  83. Todani T, Urushihara N, Watanabe Y, Toki A, Uemura S, Sato Y, et al. Pseudopancreatitis in choledochal cyst in children: intraoperative study of amylase levels in the serum. J Pediatr Surg. 1990;25:303–6.

    PubMed  CAS  Google Scholar 

  84. Rossi RL, Silverman ML, Braasch JW, Munson JL, ReMine SG. Carcinomas arising in cystic conditions of the bile ducts. A clinical and pathologic study. Ann Surg. 1987;205:377–84.

    PubMed  CAS  Google Scholar 

  85. Hasumi A, Matsui H, Sugioka A, Uyama I, Komori Y, Fujita J, et al. Precancerous conditions of biliary tract cancer in patients with pancreaticobiliary maljunction: reappraisal of nationwide survey in Japan. J Hepatobiliary Pancreat Surg. 2000;7:551–5.

    PubMed  CAS  Google Scholar 

  86. Ogawa A, Sugo H, Takamori S, Kojima K, Fukasawa M, Beppu T, et al. Double cancers in the common bile duct: molecular genetic findings with an analysis of LOH. J Hepatobiliary Pancreat Surg. 2001;8:374–8.

    PubMed  CAS  Google Scholar 

  87. Okamoto A, Tsuruta K, Matsumoto G, Takahashi T, Kamisawa T, Egawa N, et al. Papillary carcinoma of the extrahepatic bile duct: characteristic features and implications in surgical treatment. J Am Coll Surg. 2003;196:394–401.

    PubMed  Google Scholar 

  88. Hori H, Ajiki T, Fujita T, Okazaki T, Suzuki Y, Kuroda Y, et al. Double cancer of gall bladder and bile duct not associated with anomalous junction of the pancreaticobiliary duct system. Jpn J Clin Oncol. 2006;36:638–42.

    PubMed  Google Scholar 

  89. Itoh T, Fuji N, Taniguchi H, Yasukawa S, Yasuda H, Wakabayashi N, et al. Double cancer of the cystic duct and gallbladder associated with low junction of the cystic duct. J Hepatobiliary Pancreat Surg. 2008;15:338–43.

    PubMed  Google Scholar 

  90. Fujii T, Kaneko T, Sugimoto H, Okochi O, Inoue S, Takeda S, et al. Metachronous double cancer of the gallbladder and common bile duct. J Hepatobiliary Pancreat Surg. 2004;11:280–5.

    PubMed  Google Scholar 

  91. Sheth S, Bedford A, Chopra S. Primary gallbladder cancer: recognition of risk factors and the role of prophylactic cholecystectomy. Am J Gastroenterol. 2000;95:1402–10.

    PubMed  CAS  Google Scholar 

  92. Hsing AW, Bai Y, Andreotti G, Rashid A, Deng J, Chen J, et al. Family history of gallstones and the risk of biliary tract cancer and gallstones: a population-based study in Shanghai, China. Int J Cancer. 2007;121:832–8.

    PubMed  CAS  Google Scholar 

  93. Tsuchida A, Itoi T. Carcinogenesis and chemoprevention of biliary tract cancer in pancreaticobiliary maljunction. World J Gastrointest Oncol. 2010;2:130–5.

    PubMed  Google Scholar 

  94. Kozuka S, Tsubone N, Yasui A, Hachisuka K. Relation of adenoma to carcinoma in the gallbladder. Cancer. 1982;50:2226–34.

    PubMed  CAS  Google Scholar 

  95. Watanabe H, Date K, Itoi T, Matsubayashi H, Yokoyama N, Yamano M, et al. Histological and genetic changes in malignant transformation of gallbladder adenoma. Ann Oncol. 1999;10:136–9.

    PubMed  Google Scholar 

  96. Aoki T, Tsuchida A, Kasuya K, Aoki T, Saito H, Endo M, et al. Carcinogenesis in pancreaticobiliary maljunction. In: Koyanagi Y, Aoki T, editors. Pancreaticobiliary maljunction. Tokyo: Igaku Tosho; 2002. p. 295–302.

    Google Scholar 

  97. Sai JK, Suyama M, Nobukawa B, Kubokawa Y, Yokomizo K, Sato N. Precancerous mucosal changes in the gallbladder of patients with occult pancreatobiliary reflux. Gastrointest Endosc. 2005;61:264–8.

    PubMed  Google Scholar 

  98. Beltrán MA, Vracko J, Cumsille MA, Cruces KS, Almonacid J, Danilova T. Occult pancreaticobiliary reflux in gallbladder cancer and benign gallbladder diseases. J Surg Oncol. 2007;96:26–31.

    PubMed  Google Scholar 

  99. Sugiyama M, Abe N, Tokuhara M, Masaki T, Mori T, Atomi Y. Pancreatic carcinoma associated with anomalous pancreaticobiliary junction. Hepatogastroenterology. 2001;48:1767–9.

    PubMed  CAS  Google Scholar 

  100. Mizutani M, Fuse A, Kimura W. A case of carcinoma of the pancreas associated with anomalous junction of the pancreaticobiliary tracts. Suizo. 2003;18:42–7 (in Japanese with English abstract).

    Google Scholar 

  101. Minami Y, Hasuike Y, Takeda Y, Tsujinaka T. Metachronous double cancer of the gallbladder and pancreas associated with pancreaticobiliary maljunction. J Hepatobiliary Pancreat Surg. 2008;15:330–3.

    PubMed  Google Scholar 

  102. Funabiki T, Matsubara T, Miyakawa S, Ishihara S. Pancreaticobiliary maljunction and carcinogenesis to biliary and pancreatic malignancy. Langenbecks Arch Surg. 2009;394:159–69.

    PubMed  Google Scholar 

  103. Adachi T, Tajima Y, Kuroki T, Mishima T, Kitasato A, Fukuda K, et al. Bile-reflux into the pancreatic ducts is associated with the development of intraductal papillary carcinoma in hamsters. J Surg Res. 2006;136:106–11.

    PubMed  Google Scholar 

  104. Saikusa N, Naito S, Iinuma Y, Ohtani T, Yokoyama N, Nitta K. Invasive cholangiocarcinoma identified in congenital biliary dilatation in a 3-year-old boy. J Pediatr Surg. 2009;44:2202–5.

    PubMed  Google Scholar 

  105. Okada T, Sasaki F, Ueki S, Hirokata G, Okuyama K, Cho K, et al. Postnatal management for prenatally diagnosed choledochal cysts. J Pediatr Surg. 2004;39:1055–8.

    PubMed  Google Scholar 

  106. KoyanagiY, Nagae I, Ito S, et al. Differences between pediatric cases and adult cases (in Japanese). In: Funabiki T, editor. Pancreaticobiliary maljunction—consensus and controversy. Tokyo: Igaku Tosho; 1997. p. 109–14.

  107. Takamatsu H, Yano T, Noguti H, et al. Pathophysiology of congenital biliary dilatation (in Japanese). In: Funabiki T, editor. Pancreaticobiliary maljunction—consensus and controversy. Tokyo: Igaku Tosho; 1997. p. 96–100.

  108. Jeong JB, Whang JH, Ryu JK, Yoon YB, Kim YT. Risk factors for pancreatitis in patients with anomalous union of pancreatobiliary duct. Hepatogastroenterology. 2004;51:1187–90.

    PubMed  CAS  Google Scholar 

  109. Ando H, Kaneko K, Ito F, Seo T, Harada T, Watanabe Y, et al. Surgical removal of protein plugs complicating choledochal cysts: primary repair after adequate opening of the pancreatic duct. J Pediatr Surg. 1998;33:1265–7.

    PubMed  CAS  Google Scholar 

  110. Diao M, Li L, Zhang JS, Cheng W. Laparoscopic-assisted clearance of protein plugs in the common channel in children with choledochal cysts. J Pediatr Surg. 2010;45:2099–102.

    PubMed  Google Scholar 

  111. Ando H, Kaneko K, Ito T, Watanabe Y, Seo T, Harada T, et al. Complete excision of the intrapancreatic portion of choledochal cysts. J Am Coll Surg. 1996;183:317–21.

    PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  113. Todani T, Watanabe Y, Urushihara, Noda T, Morotomi Y. Biliary complications after excisional procedure for choledochal cyst. J Pediatr Surg. 1995;30:478–81.

  114. Todani T, Watanabe Y, Toki A, Urushihara N, Sato Y. Reoperation for congenital choledochal cyst. Ann Surg. 1988;207:142–7.

    PubMed  CAS  Google Scholar 

  115. Ando H, Ito T, Nagaya M, Watanabe Y, Seo T, Kaneko K. Pancreaticobiliary maljunction without choledochal cysts in infants and children: clinical features and surgical therapy. J Pediatr Surg. 1995;30:1658–62.

    PubMed  CAS  Google Scholar 

  116. Ando H, Ito T, Kaneko K, Seo T. Congenital stenosis of the intrahepatic bile duct associated with choledochal cysts. J Am Coll Surg. 1995;181:426–30.

    PubMed  CAS  Google Scholar 

  117. Koshinaga T, Inoue M, Ohashi K, Sugito K, Ikeda T, Hagiwara N, et al. Persistent biliary dilatation and stenosis in postoperative congenital choledochal cyst. J Hepatobiliary Pancreat Sci. 2011;18:47–52.

    PubMed  Google Scholar 

  118. Ando H, Ito T, Kaneko K, Seo T, Ito F. Intrahepatic bile duct stenoses causing intrahepatic calculi formation following excision of a choledochal cyst. J Am Coll Surg. 1996;183:56–60.

    PubMed  CAS  Google Scholar 

  119. Ando H, Kaneko K, Ito F, Seo T, Ito T. Operative treatment of congenital stenoses of the intrahepatic bile ducts in patients with choledochal cysts. Am J Surg. 1997;173:491–4.

    PubMed  CAS  Google Scholar 

  120. Todani T, Watanabe Y, Mizuguchi T, Fujii T, Toki A. Hepaticoduodenostomy at the hepatic hilum after excision of choledochal cyst. Am J Surg. 1981;142:584–7.

    PubMed  CAS  Google Scholar 

  121. Lilly JR. Total excision of choledochal cyst. Surg Gynecol Obstet. 1978;146:254–6.

    PubMed  CAS  Google Scholar 

  122. Todani T, Watanabe Y, Toki A, Hara H. Hilar duct carcinoma developed after cyst excision followed by hepaticoduodenostomy. In: Koyanagi Y, Aoki T, editors. Pancreaticobiliary maljunction. Tokyo: Igaku Tosho; 2002. p. 17–21.

    Google Scholar 

  123. Takada K, Hamada Y, Watanabe K, Tanano A, Tokuhara K, Kamiyama Y. Duodenogastric reflux following biliary reconstruction after excision of choledochal cyst. Pediatr Surg Int. 2005;21:1–4.

    PubMed  CAS  Google Scholar 

  124. Shimotakahara A, Yamataka A, Yanai T, Kobayashi H, Okazaki T, Lane GJ, et al. Roux-en-Y hepaticojejunostomy or hepaticoduodenostomy for biliary reconstruction during the surgical treatment of choledochal cyst: which is better? Pediatr Surg Int. 2005;21:5–7.

    PubMed  Google Scholar 

  125. Diao M, Li L, Zhang JZ, Cheng W. A shorter loop in Roux-Y hepaticojejunostomy reconstruction for choledochal cysts is equally effective: preliminary results of a prospective randomized study. J Pediatr Surg. 2010;45:845–7.

    PubMed  Google Scholar 

  126. Ando K, Miyano T, Kohno S, Takamizawa S, Lane G. Spontaneous perforation of choledochal cyst: a study of 13 cases. Eur J Pediatr Surg. 1998;8:23–5.

    PubMed  CAS  Google Scholar 

  127. Todani T, Tabuchi K, Watanabe Y, Nabeyama A, Emoto T, Munetomo Y. Perforated choledochal cyst in children. Z Kinderchirurgie. 1978;23:280–6.

    Google Scholar 

  128. Franga DL, Howell CG, Mellinger JD, Hatley RM. Single-stage reconstruction of perforated choledochal cyst: case report and review of the literature. Am Surg. 2005;7:398–401.

    Google Scholar 

  129. Nishimura T, Fumino S, Iwabuchi T, Shimadera S, Ono S, Deguchi E, et al. Ruptured choledocal cyst in 11-months-old girl. J Jpn Soc Pediatr Surg. 2006;42:497–501 (in Japanese with English abstract).

    Google Scholar 

  130. Kamisawa T, Tu Y, Kuwata G, Egawa N, Nakajima H, Tsuruta K, et al. Biliary carcinoma risk in patients with pancreaticobiliary maljunction and the degree of extrahepatic bile duct dilatation. Hepatogastroenterology. 2006;53:816–8.

    PubMed  Google Scholar 

  131. Tsuchida A, Itoi T, Endo M, Kitamura K, Mukaide M, Itokawa F, et al. Pathological features and surgical outcome of pancreaticobiliary maljunction without dilatation of the extrahepatic bile duct. Oncol Rep. 2004;11:269–76.

    PubMed  Google Scholar 

  132. Matsubara T, Sakurai Y, Zhi LZ, Miura H, Ochiai M, Funabiki T. K-ras and p53 gene mutations in noncancerous biliary lesions of patients with pancreaticobiliary maljunction. J Hepatobiliary Pancreat Surg. 2002;9:312–21.

    PubMed  Google Scholar 

  133. Ishida M, Niguma T, Yukawa T, Mimura T, Tsutsui M. A case of lower bile duct cancer associated with pancreaticobiliary maljunction without bile duct dilatation after operation of a gallbladder cancer. Jpn J Gastroenterol Surg. 2007;40:1623–29 (in Japanese with English abstract).

    Google Scholar 

  134. Todani T, Watanabe Y, Toki A, Ogura K, Wang ZQ. Co-existing biliary anomalies and anatomical variants in choledochal cyst. Br J Surg. 1998;85:760–3.

    PubMed  CAS  Google Scholar 

  135. Miyano T, Yamataka A, Kato Y, Kohno S, Fujiwara T. Choledochal cyst: special emphasis on the usefulness of intraoperative endoscopy. J Pediatr Surg. 1995;30:482–4.

    PubMed  CAS  Google Scholar 

  136. Sugiyama M, Abe N, Yamaguchi Y, Yamato T, Koyama H, Tokuhara M, et al. Endoscopic pancreatic stent insertion for treatment of pseudocyst after distal pancreatectomy. Gastrointest Endosc. 2001;53:538–9.

    PubMed  CAS  Google Scholar 

  137. Vos PM, van Beek EJ, Smits NJ, Rauws EA, Gouma DJ, Reeders JW. Percutaneous balloon dilatation for benign hepaticojejunostomy strictures. Abdom Imaging. 2000;25:134–8.

    PubMed  CAS  Google Scholar 

  138. Shimizu H, Takeuchi D, Ito H, Kimura F, Togawa A, Miyazaki M. A case of surgical treatment for occlusion of biliary expandable metallic stent placed for benign stricture of choledochojejunostomy after extrahepatic bile duct resection. J Japan Surg Assoc. 2003;64:2257–61.

    Google Scholar 

  139. Chiba K, Kamisawa T, Egawa N. Relapsing acute pancreatitis caused by protein plugs in a remnant choledochal cyst. J Hepatobiliary Pancreat Sci. 2010;17:729–30.

    PubMed  Google Scholar 

  140. Koshinaga T, Hoshino M, Inoue M, Gotoh H, Sugito K, Ikeda T, et al. Pancreatitis complicated with dilated choledochal remnant after congenital choledochal cyst excision. Pediatr Surg Int. 2005;21:936–8.

    PubMed  Google Scholar 

  141. Nakano K, Mizuta A, Oohashi S, Kuroki S, Yamaguchi K, Tanaka M, et al. Protein stone formation in an intrapancreatic remnant cyst after resection of a choledochal cyst. Pancreas. 2003;26:405–7.

    PubMed  Google Scholar 

  142. Jalleh RP, Williamson RCN. Choledochal cyst and chronic pancreatitis treated by proximal pancreatectomy. HPB Surg. 1991;4:245–50.

    PubMed  CAS  Google Scholar 

  143. Chijiiwa K, Tanaka M. Late complications after excisional operation in patients with choledochal cyst. J Am Coll Surg. 1994;179:139–44.

    PubMed  CAS  Google Scholar 

  144. Saing H, Han H, Chan KL, Lam W, Chan FL, Cheng W, et al. Early and late results of excision of choledochal cysts. J Pediatr Surg. 1999;32:1563–6.

    Google Scholar 

  145. Tsuchida Y, Takahashi A, Suzuki N, Kuroiwa M, Murai H, Toki F, et al. Development of intrahepatic biliary stones after excision of choledochal cysts. J Pediatr Surg. 2002;37:165–7.

    PubMed  Google Scholar 

  146. 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.

    PubMed  CAS  Google Scholar 

  147. Shiozawa S, Kim DH, Usui T, Inose S, Aizu M, Tsutiya R, et al. Therapeutic results of pancreaticobiliary maljunction without biliary dilatation and potential problems. Nihon Gekakei Rengo Gakkaishi. 2009;34:1005–11 (in Japanese with English abstract).

    Google Scholar 

  148. Sakaguchi T, Suzuki S, Suzuki A, Fukumoto K, Jindo O, Ota S, et al. Late postoperative complications in patients with pancreaticobiliary maljunction. Hepatogastroenterology. 2007;54:585–9.

    PubMed  Google Scholar 

  149. Tsuchida A, Kasuya K, Endo M, Saito H, Inoue K, Nagae I, et al. High risk of bile duct carcinogenesis after primary resection of a congenital biliary dilatation. Oncol Rep. 2003;10:1183–7.

    PubMed  Google Scholar 

  150. Todani T, Watanabe Y, Toki A, Urushihara N. Carcinoma related to choledochal cysts with internal drainage operation. Surg Gynecol Obstet. 1987;164:61–4.

    PubMed  CAS  Google Scholar 

  151. Noda T, Oyama T. Carcinogenesis after surgery in pancreaticobiliary maljunction. Tan to Sui. 2010;31:1325–30 (in Japanese).

    Google Scholar 

  152. Sekido H, Sano A, Ichimanda M, Asano F, Matsumoto C, Shimizu T, et al. A case of bile duct carcinoma developed in pancreaticobiliary maljunction without bile duct dilatation, which has history of cholecystectomy more than forty years ago. Tan to Sui. 2010;31:329–32 (in Japanese).

    Google Scholar 

Download references

Conflict of interest

The authors declare that they have no conflict of interest.

Author information

Authors and Affiliations

Authors

Consortia

Corresponding author

Correspondence to Terumi Kamisawa.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Kamisawa, T., Ando, H., Suyama, M. et al. Japanese clinical practice guidelines for pancreaticobiliary maljunction. J Gastroenterol 47, 731–759 (2012). https://doi.org/10.1007/s00535-012-0611-2

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00535-012-0611-2

Keywords

Navigation