Advertisement

Choledochal Malformations and Pancreaticobiliary Maljunction: A European Perspective

  • Filippo Parolini
  • Mark Davenport
Chapter

Abstract

Choledochal malformation (CM) may be defined as morphological abnormality of the biliary tract characterised by dilatation in the absence of acute mechanical obstruction. Bile duct maljunction with the incoming pancreatic duct and formation of a long common channel is a recognised element of CM. This allows free intermixing of pancreatic juice and bile, although the how dynamic this relationship is remains unknown. We have established that there is an inverse relationship of choledochal pressure and bile amylase (as a surrogate of reflux) and that the key component of damage to the biliary epithelium is pressure generated.

This chapter explores choledochal malformation from the European perspective where much less has been published.

Keywords

Congenital choledochal malformation Biliary amylase Intrabiliary pressure 

References

  1. 1.
    Soares KC, Arnaoutakis DJ, Kamel I, et al. Choledochal cysts: presentation, clinical differentiation, and management. J Am Coll Surg. 2014;219(6):1167–80.CrossRefPubMedPubMedCentralGoogle Scholar
  2. 2.
    Atkinson JJ, Davenport M. Controversies in choledochal malformation. S Afr Med J. 2014;104(11 Pt 2):816–9.CrossRefPubMedGoogle Scholar
  3. 3.
    Takeshita N, Ota T, Yamamoto M. Forty-year experience with flow-diversion surgery for patients with congenital choledochal cysts with pancreaticobiliary maljunction at a single institution. Ann Surg. 2011;254(6):1050–3.CrossRefPubMedGoogle Scholar
  4. 4.
    Turowski C, Knisely AS, Davenport M. Role of pressure and pancreatic reflux in the aetiology of choledochal malformation. Br J Surg. 2011;98:1319–26.CrossRefPubMedGoogle Scholar
  5. 5.
    Davenport M, Basu R. Under pressure: choledochal malformation manometry. J Pediatr Surg. 2005;40(2):331–5.CrossRefPubMedGoogle Scholar
  6. 6.
    Davenport M, Stringer MD, Howard ER. Biliary amylase and congenital choledochal dilatation. J Pediatr Surg. 1995;30(3):474–7.CrossRefPubMedGoogle Scholar
  7. 7.
    La Pergola E, Zen Y, Davenport M. Congenital choledochal malformation: search for a marker of epithelial instability. J Pediatr Surg. 2016;51(9):1445.CrossRefPubMedGoogle Scholar
  8. 8.
    Soares KC, Kim Y, Spolverato G, et al. Presentation and clinical outcomes of choledochal cysts in children and adults: a multi-institutional analysis. JAMA Surg. 2015;150(6):577–84.CrossRefPubMedGoogle Scholar
  9. 9.
    Soares KC, Goldstein SD, Ghaseb MA, Kamel I, et al. Pediatric choledochal cysts: diagnosis and current management. Pediatr Surg Int. 2017;33(6):637–50.  https://doi.org/10.1007/s00383-017-4083-6.CrossRefPubMedGoogle Scholar
  10. 10.
    Wiseman K, Buczkowski AK, Chung SW, et al. Epidemiology, presentation, diagnosis, and outcomes of choledochal cysts in adults in an urban environment. Am J Surg. 2005;189:527–31.CrossRefPubMedGoogle Scholar
  11. 11.
    Dabbas N, Davenport M. Congenital choledochal malformation: not just a problem for children. Ann R Coll Surg Engl. 2009;91:100–5.CrossRefPubMedPubMedCentralGoogle Scholar
  12. 12.
    Moslim MA, Takahashi H, Seifarth FG, et al. Choledochal cyst disease in a western center: a 30-year experience. J Gastrointest Surg. 2016;20:1453–63.CrossRefPubMedGoogle Scholar
  13. 13.
    Nicholl M, Pitt HA, Wolf P, Cooney J, et al. Choledochal cysts in western adults: complexities compared to children. J Gastrointest Surg. 2004;8:245–52.CrossRefPubMedGoogle Scholar
  14. 14.
    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(10):1255–60.CrossRefPubMedGoogle Scholar
  15. 15.
    Todani T, Watanabe Y, Fujii T, et al. Cylindrical dilatation of the choledochus: a special type of congenital bile duct dilatation. Surgery. 1985;98:964–9.PubMedPubMedCentralGoogle Scholar
  16. 16.
    Diao M, Li L, Cheng W. Congenital biliary dilatation may consist of 2 disease entities. J Pediatr Surg. 2011;46(8):1503–9.CrossRefPubMedPubMedCentralGoogle Scholar
  17. 17.
    Makin E, Davenport M. Understanding choledochal malformation. Arch Dis Child. 2012;97(1):69–72.CrossRefPubMedPubMedCentralGoogle Scholar
  18. 18.
    Nagi B, Kochhar R, Bhasin D, et al. Endoscopic retrograde cholangiopancreatography in the evaluation of anomalous junction of the pancreaticobiliary duct and related disorders. Abdom Imaging. 2003;28:847–52.CrossRefPubMedPubMedCentralGoogle Scholar
  19. 19.
    Ragot E, Mabrut JY, Ouaïssi M, Working Group of the French Surgical Association, 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(2):538–45.CrossRefPubMedGoogle Scholar
  20. 20.
    Wong KC, Lister J. Human fetal development of the hepato-pancreatic duct junction--a possible explanation of congenital dilatation of the biliary tract. J Pediatr Surg. 1981;16:139–45.CrossRefPubMedGoogle Scholar
  21. 21.
    Ito T, Ando H, Nagaya M, et al. Congenital dilatation of the common bile duct in children. The etiologic significance of the narrow segment distal to the dilated common bile duct. Z Kinderchir. 1984;39:40–5.PubMedGoogle Scholar
  22. 22.
    Babbit DP. Congenital choldeocal cyst: new etiological concept based on anomalous relationships of common bile duct and pancreatic bulb. Ann Radiol. 1969;12:231–41.Google Scholar
  23. 23.
    Weng R, Hu W, Cai S, et al. Prenatal diagnosis and prognosis assessment of congenital choledochal cyst in 21 cases. J Obstet Gynaecol. 2016;36(3):324–7.CrossRefPubMedGoogle Scholar
  24. 24.
    Farello GA, Cerofolini A, Rebonato M, et al. Congenital choledochal cyst: video-guided laparoscopic treatment. Surg Laparosc Endosc. 1995;5:354–8.PubMedGoogle Scholar
  25. 25.
    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. 2011;25:1567–73.CrossRefPubMedGoogle Scholar
  26. 26.
    Liem NT, Pham HD, Dung le A, et al. Early and intermediate outcomes of laparoscopic surgery for choledochal cysts with 400 patients. J Laparoendosc Adv Surg Tech A. 2012;22:599–603.CrossRefPubMedGoogle Scholar
  27. 27.
    Kasai M, Asakura Y, Taira Y. Surgical treatment of choledochal cyst. Ann Surg. 1970;172:844–51.CrossRefPubMedPubMedCentralGoogle Scholar
  28. 28.
    Watanabe Y, Toki A, Todani T. Bile duct cancer developed after cyst excision for choledochal cyst. J Hepato-Biliary-Pancreat Surg. 1999;6:207–12.CrossRefGoogle Scholar

Copyright information

© Springer Nature Singapore Pte Ltd. 2018

Authors and Affiliations

  • Filippo Parolini
    • 1
  • Mark Davenport
    • 2
  1. 1.Kings College HospitalLondonUK
  2. 2.Department of Paediatric SurgeryKings College HospitalLondonUK

Personalised recommendations