When Is Bile Duct Resection Indicated for Biliary Strictures in Primary Sclerosing Cholangitis?

  • J. Camilo Barreto
  • J. Michael MillisEmail author
Part of the Difficult Decisions in Surgery: An Evidence-Based Approach book series (DDSURGERY)


Primary sclerosing cholangitis (PSC) has a variable clinical course, but often becomes a progressive disease that leads to chronic cholestasis, cirrhosis and liver failure. In addition, PSC is the most common risk factor for cholangiocarcinoma in western countries. The etiology is unclear, and as a result there are no specific medical therapies that change long-term outcomes. Liver transplantation offers the only potentially curative therapy but it is usually reserved for patients with advanced stage or cirrhosis. Earlier stages require alternative invasive treatment modalities to manage symptoms and address dominant strictures, which can be benign or malignant. The distinction between these may be extremely challenging, and has an influence on the treatment options, which include endoscopic dilatation, stenting, or surgery, either biliary bypass or extrahepatic bile duct resection. Endoscopic therapy has less morbidity, but surgical treatment has the advantage of not leaving potentially malignant or dysplastic strictures in place and may be associated with longer survival. When cholangiocarcinoma develops, it tends to appear at an advanced stage and prognosis is poor.


Primary sclerosing cholangitis Stricture Cancer Endoscopy Resection Morbidity 


  1. 1.
    Koro NS, Alkaade S. Role of endoscopy in primary sclerosing cholangitis. Curr Gastroenterol Rep. 2003;15:361.CrossRefGoogle Scholar
  2. 2.
    Aljiffry M, Renfrew PD, Walsh MJ, Laryea M, Molinari M. Analytical review of diagnosis and treatment strategies for dominant bile duct strictures in patients with primary sclerosing cholangitis. HPB (Oxf). 2011;13:79–90.CrossRefGoogle Scholar
  3. 3.
    Van Milligen de Wit AW, Rauws EA, van Bracht J, et al. Endoscopic stent therapy for dominant extrahepatic bile duct strictures in primary sclerosing cholangitis. Gastrointest Endosc. 1996;44:293–9.CrossRefGoogle Scholar
  4. 4.
    Olsson RG, Asztely MS. Prognostic value of cholangiography in primary sclerosing cholangitis. Eur J Gastroenterol Hepatol. 1995;7:251–4.PubMedGoogle Scholar
  5. 5.
    Stiehl A, Rudolph G, Kloters-Plachky P, Sauer P, Walker S, et al. Development of dominant bile duct stenoses in patients with primary sclerosing cholangitis treated with ursodeoxycholic acid: outcome after endoscopic treatment. J Hepatol. 2002;36:151–6.CrossRefPubMedGoogle Scholar
  6. 6.
    Tischendorf JJ, Kruger M, Trautwein C, et al. Cholangioscopic characterization of dominant bile duct stenoses in patients with primary sclerosing cholangitis. Endoscopy. 2006;38:665–9.CrossRefPubMedGoogle Scholar
  7. 7.
    Ponsloen CY, Lam K, van Milligen de Witt AW, Hulbregtse K, Tytgat GN. Four years experience with short-term stenting in primary sclerosing cholangitis. Am J Gastroenterol. 1999;94:2403–7.CrossRefGoogle Scholar
  8. 8.
    Baluyut AR, Sherman S, Lehrman GA, Hoen H, Chalasani N. Impact of endoscopic therapy on the survival of patients with primary sclerosing cholangitis. Gastrointest Endosc. 2001;53:308–12.CrossRefPubMedGoogle Scholar
  9. 9.
    Gotthardt DN, Rudolph G, Kloters-Plachky P, Kulaksiz H, Stiehl A. Endoscopic dilation of dominant stenoses in primary sclerosing cholangitis: outcome after long-treatment. Gastrointest Endosc. 2010;71:527–34.CrossRefPubMedGoogle Scholar
  10. 10.
    LaRusso NF, Shneider BL, Black D, et al. Primary sclerosing cholangitis: summary of a workshop. Hepatology. 2006;44:746–64.CrossRefPubMedGoogle Scholar
  11. 11.
    Kaya M, Petersen BT, Angulo P, et al. Balloon dilation compared to stenting of dominant strictures in primary sclerosing cholangitis. Am J Gastroenterol. 2001;96:1059–66.CrossRefPubMedGoogle Scholar
  12. 12.
    Domanjko B, Ahrendt SA. Indications for non-transplant surgery in primary sclerosing cholangitis. HPB. 2005;7:292–7.CrossRefGoogle Scholar
  13. 13.
    Pitt HA, Thompson HH, Tompkins RK, Longmire WP. Primary sclerosing cholangitis: results of an aggressive surgical approach. Ann Surg. 1982;196:259–68.CrossRefPubMedPubMedCentralGoogle Scholar
  14. 14.
    Ahrendt SA, Pitt HA, Kalloo AN, et al. Primary sclerosing cholangitis. Resect, dilate or transplant? Ann Surg. 1998;227:412–23.CrossRefPubMedPubMedCentralGoogle Scholar
  15. 15.
    Martin FM, Rossi RL, Nugent FW, et al. Surgical aspects of sclerosing cholangitis. Results in 178 patients. Ann Surg. 1990;212:551–6.CrossRefPubMedPubMedCentralGoogle Scholar
  16. 16.
    Ismail T, Angrisani L, Powell JE, et al. Primary sclerosing cholangitis: surgical options, prognostic variables and outcome. Br J Surg. 1991;78:564–7.CrossRefPubMedGoogle Scholar
  17. 17.
    Farges O, Malassagne B, Sebaugh M, Bismuth H. Primary sclerosing cholangitis: liver transplantation or biliary surgery. Surgery. 1995;117:146–55.CrossRefPubMedGoogle Scholar
  18. 18.
    Brandsaeter B, Friman S, Broome U, et al. Outcome following liver transplantation for primary sclerosing cholangitis in the Nordic countries. Scand J Gastroenterol. 2003;38:1776–83.CrossRefGoogle Scholar
  19. 19.
    Pawlik TM, Olbrecht VA, Pitt HA, et al. Primary sclerosing cholangitis: role of extrahepatic biliary resection. J Am Coll Surg. 2008;206:822–32.CrossRefPubMedGoogle Scholar
  20. 20.
    Myburgh JA. Surgical biliary drainage in primary sclerosing cholangitis. The role of the Hepp-Couinaud approach. Arch Surg. 1994;129:1057–62.CrossRefPubMedGoogle Scholar
  21. 21.
    Hutson DG, Russell E, Levi JU, et al. Dilatation of biliary strictures through the afferent limb of a Roux-en-Y choledochojejunostomy in patients with sclerosing cholangitis. World J Surg. 2001;25:1251–3.CrossRefPubMedGoogle Scholar
  22. 22.
    Bergquist A, Ekbom A, Olsson R, et al. Hepatic and extrahepatic malignancies in primary sclerosing cholangitis. J Hepatol. 2002;36:321–7.CrossRefPubMedGoogle Scholar
  23. 23.
    Abu-Elmagd KM, Selby R, Iwatsuki S, et al. Cholangiocarcinoma and sclerosing cholangitis: clinical characteristics and effect on survival after transplantation. Transplant Proc. 1993;25:1124–9.PubMedPubMedCentralGoogle Scholar
  24. 24.
    Lee JG, Schutz SM, England RE, Leung JW, Cotton PB. Endoscopic therapy of sclerosing cholangitis. Hepatology. 1995;21:661–7.PubMedGoogle Scholar
  25. 25.
    Rosen CB, Nagorney DM, Wiesner RH, Coffey RJ, LaRusso NF. Cholangiocarcinoma complicating primary sclerosing cholangitis. Ann Surg. 1991;213:21–5.CrossRefPubMedPubMedCentralGoogle Scholar
  26. 26.
    Eloubeidi MA, Chen VK, Jhala NC, et al. Endoscopic ultrasound-guided fine needle aspiration biopsy of suspected cholangiocarcinoma. Clin Gastroenterol Hepatol. 2004;2:209–13.CrossRefPubMedGoogle Scholar
  27. 27.
    Kipp BR, Stadheim LM, Halling SA, et al. A comparison of routine cytology and fluorescence in situ hybridization for the detection of malignant bile duct strictures. Am J Gastroenterol. 2004;99:1675–81.CrossRefPubMedGoogle Scholar
  28. 28.
    Linder S, Soderlund C. Endoscopic therapy in primary sclerosing cholangitis: outcome of treatment and risk of cancer. Hepatogastroenterology. 2001;48:387–92.PubMedGoogle Scholar
  29. 29.
    Chalasani N, Baluyut A, Ismail A, et al. Cholangiocarcinoma in patients with primary sclerosing cholangitis: a multicenter case-control study. Hepatology. 2000;31:7–11.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing Switzerland 2016

Authors and Affiliations

  1. 1.Section of General SurgeryUniversity of Chicago MedicineChicagoUSA
  2. 2.Department of SurgeryUniversity of Chicago HospitalsChicagoUSA

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