Skip to main content
PMC home page
Annals of Surgery logoLink to Annals of Surgery
. 1998 Mar;227(3):412–423. doi: 10.1097/00000658-199803000-00014

Primary sclerosing cholangitis: resect, dilate, or transplant?

S A Ahrendt 1, H A Pitt 1, A N Kalloo 1, A C Venbrux 1, A S Klein 1, H F Herlong 1, J Coleman 1, K D Lillemoe 1, J L Cameron 1
PMCID: PMC1191280  PMID: 9527065

Abstract

OBJECTIVE: The current study examines the results of extrahepatic biliary resection, nonoperative endoscopic biliary dilation with or without percutaneous stenting, and liver transplantation in the management of patients with primary sclerosing cholangitis (PSC). SUMMARY BACKGROUND DATA: Primary sclerosing cholangitis is a progressive inflammatory disease leading to secondary biliary cirrhosis. The most effective management of sclerosing cholangitis before the onset of cirrhosis remains unclear. METHODS: From 1980 to 1994, 146 patients with PSC were managed with either resection of the extrahepatic bile ducts and long-term transhepatic stenting (50 patients), nonoperative endoscopic biliary dilation with or without percutaneous stenting (54 patients), medical therapy (28 patients), and/or liver transplantation (21 patients). RESULTS: Procedure-related morbidity and mortality rates were similar between surgically resected and nonoperatively managed patients. In noncirrhotic patients, the serum bilirubin level was significantly (p < 0.05) reduced from preoperative levels (8.3+/-1.5 mg/dL) 1 (1.7+/-0.4 mg/dL) and 3 (2.7+/-0.9 mg/ dL) years after resection, but not after endoscopic or percutaneous management. For noncirrhotic PSC patients, overall 5-year survival (85% vs. 59%) and survival until death or transplantation (82% vs. 46%) were significantly longer (p < 0.05) after resection than after nonoperative dilation with or without stenting. For cirrhotic patients, survival after liver transplantation was longer than after resection or nonoperative dilation with or without stenting. Five patients developed cholangiocarcinoma, including three (6%) of the nonoperatively managed patients but none of the resected patients. CONCLUSIONS: In carefully selected noncirrhotic patients with PSC, resection and long-term stenting remains a good option. Patients with cirrhosis should undergo liver transplantation.

Full text

PDF
414

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Abu-Elmagd K. M., Malinchoc M., Dickson E. R., Fung J. J., Murtaugh P. A., Langworthy A. L., Demetris A. J., Krom R. A., Van Thiel D. H., Starzl T. E. Efficacy of hepatic transplantation in patients with primary sclerosing cholangitis. Surg Gynecol Obstet. 1993 Oct;177(4):335–344. [PMC free article] [PubMed] [Google Scholar]
  2. Abu-Elmagd K. M., Selby R., Iwatsuki S., Fung J., Tzakis A., Todo S., Demetris A. J., Baddour N., Irish W., Van Thiel D. H. Cholangiocarcinoma and sclerosing cholangitis: clinical characteristics and effect on survival after liver transplantation. Transplant Proc. 1993 Feb;25(1 Pt 2):1124–1125. [PMC free article] [PubMed] [Google Scholar]
  3. Beuers U., Spengler U., Kruis W., Aydemir U., Wiebecke B., Heldwein W., Weinzierl M., Pape G. R., Sauerbruch T., Paumgartner G. Ursodeoxycholic acid for treatment of primary sclerosing cholangitis: a placebo-controlled trial. Hepatology. 1992 Sep;16(3):707–714. doi: 10.1002/hep.1840160315. [DOI] [PubMed] [Google Scholar]
  4. Cameron J. L., Gayler B. W., Sanfey H., Milligan F., Kaufman S., Maddrey W. C., Herlong H. F. Sclerosing cholangitis. Anatomical distribution of obstructive lesions. Ann Surg. 1984 Jul;200(1):54–60. doi: 10.1097/00000658-198407000-00009. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. Cameron J. L., Pitt H. A., Zinner M. J., Herlong H. F., Kaufman S. L., Boitnott J. K., Coleman J. Resection of hepatic duct bifurcation and transhepatic stenting for sclerosing cholangitis. Ann Surg. 1988 May;207(5):614–622. doi: 10.1097/00000658-198805000-00016. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Chapman R. W., Arborgh B. A., Rhodes J. M., Summerfield J. A., Dick R., Scheuer P. J., Sherlock S. Primary sclerosing cholangitis: a review of its clinical features, cholangiography, and hepatic histology. Gut. 1980 Oct;21(10):870–877. doi: 10.1136/gut.21.10.870. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. Dickson E. R., Murtaugh P. A., Wiesner R. H., Grambsch P. M., Fleming T. R., Ludwig J., LaRusso N. F., Malinchoc M., Chapman R. W., Kaplan M. M. Primary sclerosing cholangitis: refinement and validation of survival models. Gastroenterology. 1992 Dec;103(6):1893–1901. doi: 10.1016/0016-5085(92)91449-e. [DOI] [PubMed] [Google Scholar]
  8. Farges O., Malassagne B., Sebagh M., Bismuth H. Primary sclerosing cholangitis: liver transplantation or biliary surgery. Surgery. 1995 Feb;117(2):146–155. doi: 10.1016/s0039-6060(05)80078-9. [DOI] [PubMed] [Google Scholar]
  9. Gaing A. A., Geders J. M., Cohen S. A., Siegel J. H. Endoscopic management of primary sclerosing cholangitis: review, and report of an open series. Am J Gastroenterol. 1993 Dec;88(12):2000–2008. [PubMed] [Google Scholar]
  10. Harrison R. F., Davies M. H., Neuberger J. M., Hubscher S. G. Fibrous and obliterative cholangitis in liver allografts: evidence of recurrent primary sclerosing cholangitis? Hepatology. 1994 Aug;20(2):356–361. [PubMed] [Google Scholar]
  11. Ismail T., Angrisani L., Powell J. E., Hübscher S., Buckels J., Neuberger J., Elias E., McMaster P. Primary sclerosing cholangitis: surgical options, prognostic variables and outcome. Br J Surg. 1991 May;78(5):564–567. doi: 10.1002/bjs.1800780515. [DOI] [PubMed] [Google Scholar]
  12. Johnson G. K., Geenen J. E., Venu R. P., Schmalz M. J., Hogan W. J. Endoscopic treatment of biliary tract strictures in sclerosing cholangitis: a larger series and recommendations for treatment. Gastrointest Endosc. 1991 Jan-Feb;37(1):38–43. doi: 10.1016/s0016-5107(91)70618-4. [DOI] [PubMed] [Google Scholar]
  13. Knechtle S. J., D'Alessandro A. M., Harms B. A., Pirsch J. D., Belzer F. O., Kalayoglu M. Relationships between sclerosing cholangitis, inflammatory bowel disease, and cancer in patients undergoing liver transplantation. Surgery. 1995 Oct;118(4):615–620. doi: 10.1016/s0039-6060(05)80026-1. [DOI] [PubMed] [Google Scholar]
  14. Knox T. A., Kaplan M. M. Treatment of primary sclerosing cholangitis with oral methotrexate. Am J Gastroenterol. 1991 May;86(5):546–552. [PubMed] [Google Scholar]
  15. Lee J. G., Schutz S. M., England R. E., Leung J. W., Cotton P. B. Endoscopic therapy of sclerosing cholangitis. Hepatology. 1995 Mar;21(3):661–667. [PubMed] [Google Scholar]
  16. Lee Y. M., Kaplan M. M. Primary sclerosing cholangitis. N Engl J Med. 1995 Apr 6;332(14):924–933. doi: 10.1056/NEJM199504063321406. [DOI] [PubMed] [Google Scholar]
  17. Lemmer E. R., Bornman P. C., Krige J. E., Wright J. P., Beningfield S., Jaskiewicz K., Kirsch R. E., Kahn D., Terblanche J. T., Robson S. C. Primary sclerosing cholangitis. Requiem for biliary drainage operations? Arch Surg. 1994 Jul;129(7):723–728. doi: 10.1001/archsurg.1994.01420310055009. [DOI] [PubMed] [Google Scholar]
  18. Ludwig J., Barham S. S., LaRusso N. F., Elveback L. R., Wiesner R. H., McCall J. T. Morphologic features of chronic hepatitis associated with primary sclerosing cholangitis and chronic ulcerative colitis. Hepatology. 1981 Nov-Dec;1(6):632–640. doi: 10.1002/hep.1840010612. [DOI] [PubMed] [Google Scholar]
  19. MacCarty R. L., LaRusso N. F., Wiesner R. H., Ludwig J. Primary sclerosing cholangitis: findings on cholangiography and pancreatography. Radiology. 1983 Oct;149(1):39–44. doi: 10.1148/radiology.149.1.6412283. [DOI] [PubMed] [Google Scholar]
  20. McEntee G., Wiesner R. H., Rosen C., Cooper J., Wahlstrom E. A comparative study of patients undergoing liver transplantation for primary sclerosing cholangitis and primary biliary cirrhosis. Transplant Proc. 1991 Feb;23(1 Pt 2):1563–1564. [PubMed] [Google Scholar]
  21. Mueller P. R., vanSonnenberg E., Ferrucci J. T., Jr, Weyman P. J., Butch R. J., Malt R. A., Burhenne H. J. Biliary stricture dilatation: multicenter review of clinical management in 73 patients. Radiology. 1986 Jul;160(1):17–22. doi: 10.1148/radiology.160.1.3715030. [DOI] [PubMed] [Google Scholar]
  22. Myburgh J. A. Surgical biliary drainage in primary sclerosing cholangitis. The role of the Hepp-Couinaud approach. Arch Surg. 1994 Oct;129(10):1057–1062. doi: 10.1001/archsurg.1994.01420340071012. [DOI] [PubMed] [Google Scholar]
  23. Nakeeb A., Lipsett P. A., Lillemoe K. D., Fox-Talbot M. K., Coleman J., Cameron J. L., Pitt H. A. Biliary carcinoembryonic antigen levels are a marker for cholangiocarcinoma. Am J Surg. 1996 Jan;171(1):147–153. doi: 10.1016/S0002-9610(99)80090-7. [DOI] [PubMed] [Google Scholar]
  24. Narumi S., Roberts J. P., Emond J. C., Lake J., Ascher N. L. Liver transplantation for sclerosing cholangitis. Hepatology. 1995 Aug;22(2):451–457. [PubMed] [Google Scholar]
  25. Nichols J. C., Gores G. J., LaRusso N. F., Wiesner R. H., Nagorney D. M., Ritts R. E., Jr Diagnostic role of serum CA 19-9 for cholangiocarcinoma in patients with primary sclerosing cholangitis. Mayo Clin Proc. 1993 Sep;68(9):874–879. doi: 10.1016/s0025-6196(12)60696-x. [DOI] [PubMed] [Google Scholar]
  26. Pitt H. A., Thompson H. H., Tompkins R. K., Longmire W. P., Jr Primary sclerosing cholangitis: results of an aggressive surgical approach. Ann Surg. 1982 Sep;196(3):259–268. doi: 10.1097/00000658-198209000-00004. [DOI] [PMC free article] [PubMed] [Google Scholar]
  27. Porayko M. K., Wiesner R. H., LaRusso N. F., Ludwig J., MacCarty R. L., Steiner B. L., Twomey C. K., Zinsmeister A. R. Patients with asymptomatic primary sclerosing cholangitis frequently have progressive disease. Gastroenterology. 1990 Jun;98(6):1594–1602. doi: 10.1016/0016-5085(90)91096-o. [DOI] [PubMed] [Google Scholar]
  28. Ramage J. K., Donaghy A., Farrant J. M., Iorns R., Williams R. Serum tumor markers for the diagnosis of cholangiocarcinoma in primary sclerosing cholangitis. Gastroenterology. 1995 Mar;108(3):865–869. doi: 10.1016/0016-5085(95)90462-x. [DOI] [PubMed] [Google Scholar]
  29. Rosen C. B., Nagorney D. M., Wiesner R. H., Coffey R. J., Jr, LaRusso N. F. Cholangiocarcinoma complicating primary sclerosing cholangitis. Ann Surg. 1991 Jan;213(1):21–25. doi: 10.1097/00000658-199101000-00004. [DOI] [PMC free article] [PubMed] [Google Scholar]
  30. Wiesner R. H. Current concepts in primary sclerosing cholangitis. Mayo Clin Proc. 1994 Oct;69(10):969–982. doi: 10.1016/s0025-6196(12)61822-9. [DOI] [PubMed] [Google Scholar]
  31. Wiesner R. H., Grambsch P. M., Dickson E. R., Ludwig J., MacCarty R. L., Hunter E. B., Fleming T. R., Fisher L. D., Beaver S. J., LaRusso N. F. Primary sclerosing cholangitis: natural history, prognostic factors and survival analysis. Hepatology. 1989 Oct;10(4):430–436. doi: 10.1002/hep.1840100406. [DOI] [PubMed] [Google Scholar]
  32. Wiesner R. H., LaRusso N. F. Clinicopathologic features of the syndrome of primary sclerosing cholangitis. Gastroenterology. 1980 Aug;79(2):200–206. [PubMed] [Google Scholar]

Articles from Annals of Surgery are provided here courtesy of Lippincott, Williams, and Wilkins

RESOURCES