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Primary Sclerosing Cholangitis

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General Surgery

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Pearls and Pitfalls

  • With sclerosing cholangitis, strictures involve the extrahepatic bile ducts exclusively in 10% of patients, solely the intrahepatic bile ducts in 15%, and both intrahepatic and extrahepatic bile ducts in 75%.

  • Cholangiocarcinoma (CCA) develops in 8–18% of patients with primary sclerosing cholangitis (PSC); the diagnosis of CCA is made concurrently with that of PSC in 40–60% of patients.

  • The diagnosis of CCA in the presence of PSC is notoriously difficult; biliary brushings and cytology have a sensitivity of less than 40%.

  • Repeated endoscopic therapy of dominant strictures may delay the diagnosis of CCA and affect survival adversely.

  • Operative resection of dominant extrahepatic strictures may prevent development of CCA, prolong survival, and delay need for liver transplantation.

  • Once cirrhosis develops, liver transplantation is the optimal therapy for patients with PSC; outcomes are equivalent to, or better than, those of transplantation for other indications.

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Selected Readings

  • Ahrendt SA, Pitt HA, Kalloo AN, et al. (1998) Primary sclerosing cholangitis: resect, dilate, or transplant? Ann Surg 227:412–423

    Article  PubMed  CAS  Google Scholar 

  • Ahrendt SA, Pitt HA, Nakeeb A, et al. (1999) Diagnosis and management of cholangiocarcinoma in primary sclerosing cholangitis. J Gastrointest Surg 3:357–368

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  • Baluyut AR, Sherman S, Lehman GA, et al. (2001) Impact of endoscopic therapy on the survival of patients with primary sclerosing cholangitis. Gastrointest Endosc 53:308–312

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  • Cameron J (1990) Atlas of surgery, vol. 1. BC Decker, Philadelphia

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  • Cameron JL, Pitt HA, Zinner MJ, et al. (1988) Resection of hepatic duct bifurcation and transhepatic stenting for sclerosing cholangitis. Ann Surg 207:614–622

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  • Graziadei IW, Weisner RH, Marotta PJ, et al. (1999) Long-term results of patients undergoing liver transplantation for primary sclerosing cholangitis. Hepatology 30:1121–1127

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Author information

Authors and Affiliations

  1. Indiana University School of Medicine, 1001 West 10th St., 46202, OPE 313 Indianapolis, IN, USA

    Nicholas J. Zyromski (Assistant Professor of Surgery)

  2. Indiana University School of Medicine, 1001 West 10th St., 46202, OPE 313 Indianapolis, IN, USA

    Henry A. Pitt (Professor of Surgery)

Authors
  1. Nicholas J. Zyromski
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  2. Henry A. Pitt
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Editor information

Editors and Affiliations

  1. Department of Surgery, Deputy Director, Comprehensive Cancer Center University of Alabama School of Medicine, Birmingham, AL, USA

    Kirby I. Bland MD (Fay Fletcher Kerner Professor and Chairman) (Fay Fletcher Kerner Professor and Chairman)

  2. Department of General and Visceral Surgery, University of Heidelberg, Heidelberg, Germany

    Markus W. Büchler MD (Professor of Surgery and Chairman) (Professor of Surgery and Chairman)

  3. Department of Surgery, University Hospital, Santiago, Chile

    Attila Csendes MD, FACS (Hon) (Professor of Surgery and Chairman) (Professor of Surgery and Chairman)

  4. James C. Mason Professor of Surgery Department of Surgery, Mayo Clinic College of Medicine, Rochester, MN, USA

    Michael G. Sarr MD

  5. Department of Clinical and Surgical Sciences, The University of Edinburgh Royal Infirmary of Edinburgh, Edinburgh, UK

    O. James Garden MBChB, MD, FRCS (Ed), FRCP (Ed), FRACS (Hon) (Regius Professor of Clinical Surgery) (Regius Professor of Clinical Surgery)

  6. Department of Surgery, The University of Hong Kong Queen Mary Hospital, Hong Kong, China

    John Wong BSc (Med (Syd)), MBBS (Syd), PhD (Syd), MD (Hon (Syd)), FRACS, FRCS (Edin), FRCS (Glasg), FACS (Hon) (Chair Professor) (Chair Professor)

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Zyromski, N.J., Pitt, H.A. (2009). Primary Sclerosing Cholangitis. In: Bland, K.I., Büchler, M.W., Csendes, A., Sarr, M.G., Garden, O.J., Wong, J. (eds) General Surgery. Springer, London. https://doi.org/10.1007/978-1-84628-833-3_109

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  • DOI: https://doi.org/10.1007/978-1-84628-833-3_109

  • Publisher Name: Springer, London

  • Print ISBN: 978-1-84628-832-6

  • Online ISBN: 978-1-84628-833-3

  • eBook Packages: MedicineReference Module Medicine

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