Cholangioscopic Characterization of Dominant Bile Duct Stenoses in Patients with Primary Sclerosing Cholangitis

Background and Study Aims: Primary sclerosing cholangitis (PSC) is associated with the development of cholangiocarcinoma in up to 10 % of patients. Cholangiography or endoscopic tissue sampling does not reliably distinguish between cholangiocarcinoma and a benign dominant bile duct stenosis. The aim of the present study was to assess the value of cholangioscopy for distinguishing between benign and malignant dominant stenoses in PSC patients.
Patients and Methods: Fifty-three PSC patients with dominant bile duct stenoses were prospectively studied. Transpapillary cholangioscopy and endoscopic tissue sampling were carried out in addition to endoscopic retrograde cholangiography (ERC). The cholangiography and cholangioscopic findings were classified as malignant or benign by the investigators. A final diagnosis of malignant stenosis was based on positive histology and/or cytology, whereas a benign condition was assumed in cases of negative tissue sampling and uneventful extended clinical follow-up.
Results: Twelve PSC patients (23 %) had dominant bile duct stenoses caused by cholangiocarcinoma, whereas 41 of the 53 patients (77 %) had benign dominant bile duct stenoses. Cholangioscopy was significantly superior to ERC for detecting malignancy in terms of its sensitivity (92 % vs. 66 %; P = 0.25), specificity (93 % vs. 51 %; P < 0.001), accuracy (93 % vs. 55 %; P < 0.001), positive predictive value (79 % vs. 29 %; P < 0.001), and negative predictive value (97 % vs. 84 %; P < 0.001). Transpapillary cholangioscopy is more sensitive and specific for characterizing malignant bile duct stenosis in comparison with endoscopic brush cytology.
Conclusions: Transpapillary cholangioscopy significantly increases the ability to distinguish between malignant and benign dominant bile duct stenoses in patients with PSC.

References

• 1 Chapman R WG, Marborgh B A, Rhodes J M. et al . Primary sclerosing cholangitis: a review of clinical features, cholangiography and hepatic histology.  Gut. 1980;  21 870-877
• 2 La Russo N F, Wiesner R H, Ludwig J. et al . Primary sclerosing cholangitis.  N Engl J Med. 1984;  310 899-903
• 3 Farrant J M, Hayllar K M, Wilkinson M L. et al . Natural history and prognostic variables in primary sclerosing cholangitis.  Gastroenterology. 1991;  100 1710-1717
• 4 Broomé U, Olsson R, Lööf L. et al . Natural history and prognostic factor in 305 Swedish patients with primary sclerosing cholangitis.  Gut. 1996;  38 610-615
• 5 Ponsioen C Y, Vrouenraets S ME, Prawirodidjo W. et al . Natural history of primary sclerosing cholangitis and prognostic value of cholangiography in a Dutch population.  Gut. 2002;  51 562-566
• 6 Rosen C B, Nagorney D M, Wiesner R H. et al . Cholangiocarcinoma complicating primary sclerosing cholangitis.  Ann Surg. 1991;  213 21-25
• 7 Abu-Elmagd K M, Selby R, Iwatsuki S. et al . Cholangiocarcinoma and sclerosing cholangitis: clinical characteristics and effect on survival after liver transplantation.  Transplant Proc. 1993;  25 1124-1125
• 8 Knechtle S J, D’Alessandro A M, Harms B A. et al . Relationships between sclerosing cholangitis, inflammatory bowel disease, and cancer in patients undergoing liver transplantation.  Surgery. 1995;  118 615-620
• 9 Nashan B, Schlitt H J, Tusch G. et al . Biliary malignancies in primary sclerosing cholangitis: timing for liver transplantation.  Hepatology. 1996;  23 1105-1111
• 10 MacCarty R L, La Russo N F, May G R. et al . Cholangiocarcinoma complicating primary sclerosing cholangitis: cholangiographic appearances.  Radiology. 1985;  156 43-46
• 11 Stiehl A, Rudolph G, Klöters-Plachky P. 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;  36 151-156
• 12 Berquist A, Glaumann H, Persson B. et al . Risk factors and clinical presentation of hepatobiliary carcinoma in patients with primary sclerosing cholangitis: a case-control study.  Hepatology. 1998;  27 311-316
• 13 Ponsioen C Y, Vrouenraets S ME, van Milligen de Witt  A WM. et al . Value of brush cytology for dominant strictures in primary sclerosing cholangitis.  Endoscopy. 1999;  31 305-309
• 14 Lindberg B, Arnelo U, Bergquist A. et al . Diagnosis of biliary strictures in conjugation with endoscopic retrograde cholangiopancreatography, with special reference to patients with primary sclerosing cholangitis.  Endoscopy. 2002;  34 909-916
• 15 Nichols J C, Gores G J, LaRusso N F. et al . Diagnostic role of serum CA19 - 9 for cholangiocarcinoma in patients with primary sclerosing cholangitis.  Mayo Clinic Proc. 1993;  68 874-879
• 16 Ramage J K, Donaghy A, Farrant J M. et al . Serum tumor markers for the diagnosis of cholangiocarcinoma in primary sclerosing cholangitis.  Gastroenterology. 1995;  108 865-869
• 17 Björnsson E, Kilander A, Olsson R. Ca19-9 and CEA are unreliable markers for cholangiocarcinoma in patients with primary sclerosing cholangitis.  Liver. 1999;  19 510-508
• 18 Hultcrantz R, Olsson R, Danielsson A. et al . A 3-year prospective study on serum tumor markers used for detecting cholangiocarcinoma in patients with primary sclerosing cholangitis.  J Hepatol. 1999;  30 669-673
• 19 Nimura Y, Kamiya J, Hayakawa N. et al . Cholangioscopic differentiation of biliary strictures and polyps.  Endoscopy. 1989;  21 351-356
• 20 Nimura Y. Staging of biliary carcinoma: cholangiography and cholangioscopy.  Endoscopy. 1993;  25 76-80
• 21 Seo D W, Kim M H, Lee S K. et al . Usefulness of cholangioscopy in patients with focal stricture of the intrahepatic duct unrelated to intrahepatic stones.  Gastrointest Endosc. 1999;  49 204-209
• 22 Christensen M, Matzen P, Schulze S. et al . Complications of ERCP: a prospective study.  Gastrointest Endosc. 2004;  60 721-731