Abstract
Background
Pancreaticobiliary maljunctions (PBMs) are congenital anomalies of the junction between pancreatic and bile ducts, frequently associated with bile duct cyst (BDC). BDC is congenital biliary tree diseases that are characterized by distinctive dilatation types of the extra- and/or intrahepatic bile ducts. Todani’s types I and IVa, in which dilatation involves principally the main bile duct, are the most frequent. PBM induces pancreatic juice reflux into the biliary tract that is supposed to be one of the main factors of biliary cancer degeneration, although the diagnostic criteria of PBM that can be either morphological and/or functional are not well defined especially in Western series.
Objective
The aim of this study was to assess the relative prevalence of PBM in BDC in a large European multicenter study, to analyze the characteristics of PBM and try to propose diagnostic criteria of PBMs based on morphological and/or functional criteria and define the positive, negative predictive values, sensibility and specificity of either criteria.
Results
From 1975 to 2012, 263 patients with BDC were analyzed. Among them, 190 (72.2 %) were considered to present PBM. Types I and IVa had a similar rate of PBM association. According to the “AFC classification,” 57.2 % had a C–P type, 34.5 % a P–C type and 8.3 % a complex type (“anse-de-seau”). The median length of the common channel in patients with PBM was 15.8 ± 6.8 mm (range 5–40 mm). The median intrabiliary amylase and lipase levels were 65,249 and 172,104 UI/L, respectively. For the diagnostic of PBM, a common channel length of more than 8 mm and an intrabiliary amylase level superior to 8000 UI/L were associated with a predictive positive value and a specificity of more than 90 %. Synchronous biliary cancer had an incidence of 8.7 % in all patients with BDC and PBM 11.1 % in adults. Compared to type IV, the type I BDC was associated with statistically more cancer patients in the presence of PBM.
Conclusions
Characteristics of PBM associated with BDC in Western population are quite close to reported Eastern series. The results suggest considering both the intrabiliary value of amylase >8000 UI/L and a length of a common channel >8 mm as appropriate values for positive diagnosis of PBM.
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Abbreviations
- AFC:
-
Association Française de Chirurgie
- ASA:
-
American Society of Anesthesiologists
- BDC:
-
Bile duct cyst
- C–P:
-
Choledochal to pancreatic
- ERCP:
-
Endoscopic retrograde cholangiopancreatography
- HBP:
-
Hepato-biliary and pancreatic
- MRCP:
-
Magnetic resonance cholangiopancreatography
- NPV:
-
Negative predictive value
- P–C:
-
Pancreatic to choledochal
- PBJ:
-
Pancreaticobiliary junction
- PBM:
-
Pancreaticobiliary maljunctions
- PPV:
-
Positive predictive value
References
Makin E, Davenport M (2012) Understanding choledochal malformation. Arch Dis Child 97(1):69–72
Todani T, Watanabe Y, Narusue M, Tabuchi K, Okajima K (1977) Congenital bile duct cysts: classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst. Am J Surg 134(2):263–269
Lee SE, Jang JY, Lee YJ et al (2011) Choledochal cyst and associated malignant tumors in adults: a multicenter survey in South Korea. Arch Surg 146(10):1178–1184
Cho MJ, Hwang S, Lee YJ et al (2011) Surgical experience of 204 cases of adult choledochal cyst disease over 14 years. World J Surg 35(5):1094–1102. doi:10.1007/s00268-011-1009-7
Todani T, Watanabe Y, Fujii T et al (1984) Anomalous arrangement of the pancreatobiliary ductal system in patients with a choledochal cyst. Am J Surg 147(5):672–676
Kimura K, Ohto M, Ono T, Tsuchiya Y, Saisho H, Kawamura K, Yogi Y, Karasawa E, Okuda K (1977) Congenital cystic dilatation of the common bile duct: relationship to anomalous pancreaticobiliary ductal union. AJR Am J Roentgenol 128(4):571–577
Baumann R, Uettwiller H, Duclos B, Jouin H, Kerschen A, Adloff M, Weill JP (1987) Congenital cystic dilatation of the common bile duct, anomaly of the biliopancreatic junction and cancer of the bile ducts. Gastroenterol Clin Biol 11(12):849–855
Tashiro S, Imaizumi T, Ohkawa H, Okada A, Katoh T, Kawaharada Y, Shimada H, Takamatsu H, Miyake H, Todani T (2003) Pancreaticobiliary maljunction: retrospective and nationwide survey in Japan. J Hepato Biliary Pancreatic Surg 10(5):345–351
Kamisawa T, Ando H, Suyama M, Shimada M, Morine Y, Shimada H, Working Committee of Clinical Practice Guidelines for Pancreaticobiliary Maljunction, Japanese Study Group on Pancreaticobiliary Maljunction (2012) Japanese clinical practice guidelines for pancreaticobiliary maljunction. J Gastroenterol 7(7):731–759. doi:10.1007/s00535-012-0611-2
Komi N, Udaka H, Ikeda N, Kashiwagi Y (1977) Congenital dilatation of the biliary tract; new classification and study with particular reference to anomalous arrangement of the pancreaticobiliary ducts. Gastroenterologia Japonica 12(4):293–304
Mabrut JY, Kianmanesh R, Nuzzo G, Castaing D, Boudjema K, Létoublon C, Adham M, Ducerf C, Pruvot FR, Meurisse N, Cherqui D, Azoulay D, Capussotti L, Lerut J, Reding R, Mentha G, Roux A, Gigot JF (2013) Surgical management of congenital intrahepatic bile duct dilatation, Caroli’s disease and syndrome: long-term results of the French Association of Surgery Multicenter Study. Ann Surg 258(5):713–721 (discussion 721)
Morine Y, Shimada M, Takamatsu H, Araida T, Endo I, Kubota M, Toki A, Noda T, Matsumura T, Miyakawa S, Ishibashi H, Kamisawa T, Shimada H (2013) Clinical features of pancreaticobiliary maljunction: update analysis of 2nd Japan-nationwide survey. J Hepatobiliary Pancreat Sci 20(5):472–480
Lilly JR, Stellin GP, Karrer FM (1985) Forme fruste choledochal cyst. J Pediatr Surg 20(4):449–451
Okada A (1986) Forme fruste choledochal cyst. J Pediatr Surg 21(4):383
Shimotakahara A, Yamataka A, Kobayashi H, Okada Y, Yanai T, Lane GJ, Miyano T (2003) Forme fruste choledochal cyst: long-term follow-up with special reference to surgical technique. J Pediatr Surg 38(12):1833–1836
Irie H, Honda H, Jimi M, Yokohata K, Chijiiwa K, Kuroiwa T et al (1998) Value of MR cholangiopancreatography in evaluating choledochal cysts. AJR Am J Roentgenol 171:1381–1385
Huang CT, Lee HC, Chen WT, Jiang CB, Shih SL, Yeung CY (2011) Usefulness of magnetic resonance cholangiopancreatography in pancreatobiliary abnormalities in pediatric patients. Pediatr Neonatol 52(6):332–336
Misra SP, Dwivedi M (1990) Pancreaticobiliary ductal union. Gut 31(10):1144–1149
Kianmanesh R, Regimbeau JM, Belghiti J (2001) Pancreato-biliary maljunctions and congenital cystic dilatation of the bile ducts in adults. J Chir 138(4):196–204
Kamisawa T, Anjiki H, Egawa N, Kurata M, Honda G, Tsuruta K (2008) Diagnosis and clinical implications of pancreatobiliary reflux. World J Gastroenterol 14(43):6622–6626
Kamisawa T, Suyama M, Fujita N, Maguchi H, Hanada K, Ikeda S, Igarashi Y, Itoi T, Kida M, Honda G, Sai J, Horaguchi J, Takahashi K, Sasaki T, Takuma K, Itokawa F, Ando H, Takehara H, Committee of Diagnostic Criteria of The Japanese Study Group on Pancreaticobiliary (2010) Pancreatobiliary reflux and the length of a common channel. J Hepatobiliary Pancreat Sci 17(6):865–870. doi:10.1007/s00534-010-0282-4
Matsumoto Y, Fujii H, Itakura J, Matsuda M, Nobukawa B, Suda K (2002) Recent advances in pancreaticobiliary maljunction. J Hepato Biliary Pancreatic Surg 9(1):45–54
Hasumi A, Matsui H, Sugioka A, Uyama I, Komori Y, Fujita J, Aoki H (2000) Precancerous conditions of biliary tract cancer in patients with pancreaticobiliary maljunction: reappraisal of nationwide survey in Japan. J Hepato Biliary Pancreatic Surg 7(6):551–555
Todani T, Watanabe Y, Toki A et al (1987) Carcinoma related to choledochal cysts with internal drainage operations. Surg Gynecol Obstet 164:61–64
Flanigan DP (1977) Biliary carcinoma associated with biliary cysts. Cancer 40:880–883
Acknowledgments
The authors would like to thank Adham Mustapha—Hôpital Edouard Herriot, Lyon, France; Doussot Alexandre—Hôpital Universitaire Dijon, France; Branchereau Sophie—Hôpital Universitaire de Necker, Paris, France; Ayav Ahmet—Hôpital Universitaire de Nancy, France; Balladur Pierre—Hôpital Saint Antoine, Paris, France; Adam René, Paul Brousse—CHB, Villejuif, France; Troisi Roberto I.-University Hospital Gent, Gent, Belgium; Regimbeau Jean Marc-Amiens—Hôpital Universitaire d’Amiens, France; Majno Pietro—Hôpital Universitaire de Genève, Geneva, Switzerland; Sommacale Daniele—Hôpital Universitaire Robert Debré, Reims, France; Bouzid Chafik—Pierre et Marie Curie Hospital, Alger, Algeria; and Falconi Massimo—Verona hospital, Verona, Italy.
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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 41, 538–545 (2017). https://doi.org/10.1007/s00268-016-3684-x
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DOI: https://doi.org/10.1007/s00268-016-3684-x