Abstract
Congenital biliary dilatation (CBD) is a congenital malformation involving both local dilatation of the extrahepatic bile duct, including the common bile duct, and pancreaticobiliary maljunction (PBM) that is thought to develop as a misarrangement of the embryonic connections in the pancreaticobiliary ductal system. Patients can be diagnosed with CBD at any age, but more than two-thirds of cases are diagnosed in children younger than 10 years of age. The major clinical symptoms are recurrent abdominal pain, nausea and vomiting, and mild jaundice. The occurrence of signs and symptoms is explained by the bile and pancreatic flow being disturbed by protein plugs. In PBM, the sphincters of the pancreatic duct and the bile duct cannot work, and this long common channel permits reflux of pancreatic juice freely into the biliary tract. This free reflux may be a key factor in the pathogenesis of malignant changes in the bile duct. According to a nationwide survey performed in Japan, cancer of the biliary tract was found in 21.6% of 997 patients with CBD/PBM diagnosed in adulthood and was developed biliary tract cancer 15–20 years earlier than individuals without PBM. Immediate extrahepatic bile duct resection is recommended once a definitive diagnosis is established because juvenile patients with CBD can develop cholangitis and/or cancer even if asymptomatic. However, reports of intrahepatic stones and/or bile duct carcinoma after surgery are gradually increasing. Therefore, careful long-term follow-up is very important.
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References
Hamada Y, Ando H, Kamisawa T, et al. Diagnostic criteria for congenital biliary dilatation 2015. J Hepatobiliary Pancreat Sci. 2016;23:342–6.
Kamisawa T, Ando H, Suyama M, et al. Japanese clinical practice guidelines for pancreraticobiliary maljunction. J Gastroenterl. 2012;47:731–59.
Vater A. Dissertation in auguralis media, poes diss. Qua Scirris viscerum dissert, c.s. ezlerus, vol. 70. Edinburgh: University Library; 1723. p. 19.
McConnell AA. Cyst of the common bile duct. Br J Surg. 1919;7:520–4.
Arnolds. Eine manneskopfgroβen Retentionszyste des Choledochus. Dtsch Med Wochenschr. 1906;32:1804.
Kozumi I, Kodama T. A case report and the etiology of choledochal cystic dilatation (in Japanese). J Tokyo Med Assoc. 1916;30:1413–23.
Irwin ST, Morison JE. Congenital cyst of common bile duct containing stones and undergoing cancerous change. Br J Surg. 1944;32:319–21.
Alonso-Lej F, Rever EB Jr, Pessagno DJ. Congenital choledochal cyst, with a report of 2, and an analysis of 94, cases. Int Abstr Surg. 1959;108:1–30.
Todani T. Congenital choledochal dilatation: classification, clinical features, and long-term results. J Hepatobiliary Pancreat Surg. 1997;4:276–82.
Davenport M, Basu R. Under pressure: choledochal malformation manometry. J Pediatr Surg. 2005;40:331–5.
Yamaguchi M. Congenital choledochal cyst. Analysis of 1,433 patients in the Japanese literature. Am J Surg. 1980;140:653–7.
Ishibashi H, Shimada M, Kamisawa T, et al. Japanese clinical practice guidelines for congenital biliary dilatation. J Hepatobiliary Pancreat Sci. 2017;24:1–16.
Yotuyanagi S. Contributions to the aetiology and pathogeny of idiopathic cystic dilatation of the common bile-duct with report of 3 cases: new aetiological theory based on supposed unequal epithelial proliferation at stage of physiological epithelial occlusion of primitive choledochus. Gann. 1936;30:601–50.
Suda K, Matsumoto Y, Miyano T. Narrow duct segment distal to choledochal cyst. Am J Gastroenterol. 1991;86:1259–63.
Ando H, Kaneko K, Ito F, Seo T, et al. Embryogenesis of pancreaticobiliary maljunction inferred from development of duodenal atresia. J Hepatobiliary Pancreat Surg. 1999;6:50–4.
Kamisawa T, Kuruma S, Tabata T, et al. Pancreaticobiliary maljunction and biliary cancer. J Gastroenterol. 2015;50:273–9.
Kaneko K, Ando H, Ito T, et al. Protein plugs cause symptoms in patients with choledochal cysts. Am J Gastroenterol. 1997;92:1018–21.
Tashiro S, Imaizumi T, Ohkawa H, Committee for Registration of the Japanese Study Group on Pancreaticobiliary Maljunction, et al. Pancreaticobiliary maljunction: retrospective and nationwide survey in Japan. J Hepatobiliary Pancreat Surg. 2003;10:345–51.
Morine Y, Shimada M, Takamatsu H, et al. Clinical features of pancreaticobiliary maljunction: update analysis of 2nd Japan-nationwide survey. J Hepatobiliary Pancreat Sci. 2013;20:472–80.
Kamisawa T, Kaneko K, Itoi T, Ando H. Pancreaticobiliary maljunction and congenital biliary dilatation. Lancet Gastroenterol Hepatol. 2017;2:610–8.
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Ando, H. (2018). Outline of Congenital Biliary Dilatation and Pancreaticobiliary Maljunction. In: Kamisawa, T., Ando, H. (eds) Pancreaticobiliary Maljunction and Congenital Biliary Dilatation. Springer, Singapore. https://doi.org/10.1007/978-981-10-8654-0_1
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DOI: https://doi.org/10.1007/978-981-10-8654-0_1
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