MR imaging of primary sclerosing cholangitis (PSC) using the hepatobiliary specific contrast agent Gd-EOB-DTPA Poster No.: C-0019 Congress: ECR 2010 Type: Educational Exhibit Topic: Abdominal Viscera (Solid Organs) Authors: K. I. Ringe, N. Hellige, T. Weissmüller, P. Bolzen, M. P. Manns, M. Galanski, J. Lotz; Hannover/DE Keywords: Gd-EOB-DTPA, primary sclerosing cholangitis, hepatobiliary imaging DOI: 10.1594/ecr2010/C-0019 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. www.myesr.org Page 1 of 13
Learning objectives To learn about typical MRI findings in patients with Primary Sclerosing Cholangitis (PSC). To learn about the added value of a hepatobiliary specific contrast agent. Background Primary Sclerosing Cholangitis (PSC) PSC is a chronic cholestatic liver disease characterized by fibrosing inflammatory destruction of the intra- and / or extrahepatic bile ducts. The majority of cases is associated with inflammatory bowel disease, the exact etiology is unknown. PSC leads in the course of time to bile stasis, hepatic fibrosis, cirrhosis and end-stage liver disease. Patients with PSC have a significantly increased risk of developing cholangiocarcinoma. Gd-EOB-DTPA (Primovist ) Gd-EOB-DTPA is a hepatobiliary-specific contrast agent with imaging properties of conventional extracellular as well as of liver specific contrast agents. In patients with normal liver and kidney function approximately 50% of the administered dose is excreted through the hepatobiliary pathway, 50% via the kidneys. After i.v. injection, Gd-EOB-DTPA is transported from the extracellular space into the hepatocytes by the ATP-dependent organic anion transporting polypeptide (OATP1) and subsequently excreted into the biliary canaliculi by the canalicular multispecific organic anion transporter (cmoat). Since bilirubin is also excreted via the OATP1 receptor, biliary excretion of GD-EOB-DTPA depends on the overall liver function. Imaging findings OR Procedure details HEPATOBILIARY IMAGING WITH GD-EOB-DTPA The hepatic uptake of Gd-EOB-DTPA allows for data acquisition during the hepatocyte phase in addition to the usual dynamic examination. Page 2 of 13
In patients with normal liver function this hepatocyte phase is usually reached within 20 minutes and lasts for at least 60 minutes (Figure 1). To optimize workflow the individual sequences can be reordered as given below (Table 1). The time period of more than 15 minutes between the completion of the dynamic series and the acquisition of the hepatocyte phase images can be used for diffusion and T2w imaging. Fig.: Table 1: MR pulse sequence protocol for comprehensive liver examination. Fig.: Figure 1: 36 year old male with colitis ulcerosa, elevated liver function tests and suspicion of PSC. T2w 3D MRCP (a), late images 20 minutes after contrast injection in Page 3 of 13
the axial (b) and coronal (c) plane. Unsuspicious depiction of the hepatobiliary system with homogenous contrast uptake and timely biliary contrast excretion. IMAGING FINDINGS OF PSC A) Alterations of the intra- and extrahepatic bile ducts: Segmental fibrosis of intra- and / or extrahepatic bile ducts with saccular dilatation of normal intervening areas result in the characteristic beads-ona-string appearance dominant strictures: diameter of the common bile duct <1.5mm and common hepatic duct <1mm, respectively (in up 45% and 58% of patients, respectively) (Figure 2 and 3) bacterial cholangitis (usually after endoscopic intervention or surgery) Fig.: Figure 2: 60 year old female with known PSC. a: T2w MRCP shows ubiquitary dilatation and dicontinuation of intrahepatic bile ducts as well as central stenosis. b: Late imaging (40 min. p.i.) after Gd-EOB-DTPA injection depicts reduced uptake of contrast in the left lobe and proper biliary secretion in the right lobe (->). Page 4 of 13
Fig.: Figure 3: 31 year old male with known PSC. T2w MRCP (a) depicts central high degree stenosis (->). No associated mass is visible on pre contrast (b) and portal venous (c) VIBE images. Late imaging (20 min p.i.) demonstrates patent bile ducts and timely biliary contrast excretion. B) Parenchymal changes: findings of cirrhosis (e.g. irregularity of liver contour, nodular reticular pattern of liver parenchyma) and associated portal hypertension inhomogenous uptake of hepatobiliary-specific contrast agent (Figure 4) Fig.: Figure 4: 30 year old female with known PSC. a: T2w imaging depicts multiple enlarged lymph nodes (->) in the liver hilum and hepatoduodenal ligament. b: Late imaging (40 min. p.i.) shows significantly reduced contrast uptake in the liver with a heterogeneous nodular appearance, consistent with advanced cirrhosis. Contrast excretion via the kidneys. Page 5 of 13
C) Findings of malignancy: Cholangiocarcinoma develops in up to 23% of patients (Figure 5) Gallbladder neoplasia in terms of dysplasia, adenoma, carcinoma (Figure 6) HCC: patients with end-stage liver disease are at increased risk, but HCC occurs infrequently in patients with PSC Fig.: Figure 5: 62 year old male, T1w images (a,c) and T2w 3D MRCP. 40 min. p.i. the common bile duct (CBD) can be clearly visualized after biliary contrast secretion. A large tumor encasing the CBD is seen (o), consistent with a central cholangiocellular carcinoma (Klatskin Tumor). In addition, focal cholangitis in the left lobe is present (->). Fig.: Figure 6: 80 year old female. T2w imaging (a) depicts gallbladder filled with multiple stones. Arterial phase imaging (b) and late imaging 60 minutes post contrast injection (c) shows thickened gallbladder wall. In addition, signal alterations in the adjacent liver parenchyma is seen (d). Surgery confirmed gallbladder carcinoma with infiltration of the liver D) Other findings: Page 6 of 13
associated hilar lymphadenopathy post-operative changes and complications (bile duct anastomosis, bile duct leakage, bilioma) (Figure 7 and 8) Fig.: Figure 7: 37 year old male with known PSC. Status post left hemihepatectomy and hepaticojejunostomy. Late imaging (a,b,c) 20 minutes post contrast injection shows patent anastomosis with timely duodenal contrast excretion. Fig.: Figure 8: 20 year old female, status post cholecystectomy and iatrogenic bile duct injury. T1w images in the axial (a) and coronal (b) plane 40 minutes after contrast injection. Active contrast secretion into a subhepatic bilioma is seen (o) and exact localization of the leakage is possible (->). Images for this section: Page 7 of 13
Fig. 1: Figure 2: 60 year old female with known PSC. a: T2w MRCP shows ubiquitary dilatation and dicontinuation of intrahepatic bile ducts as well as central stenosis. b: Late imaging (40 min. p.i.) after Gd-EOB-DTPA injection depicts reduced uptake of contrast in the left lobe and proper biliary secretion in the right lobe (->). Fig. 2: Figure 1: 36 year old male with colitis ulcerosa, elevated liver function tests and suspicion of PSC. T2w 3D MRCP (a), late images 20 minutes after contrast injection in the axial (b) and coronal (c) plane. Unsuspicious depiction of the hepatobiliary system with homogenous contrast uptake and timely biliary contrast excretion. Page 8 of 13
Fig. 3: Figure 4: 30 year old female with known PSC. a: T2w imaging depicts multiple enlarged lymph nodes (->) in the liver hilum and hepatoduodenal ligament. b: Late imaging (40 min. p.i.) shows significantly reduced contrast uptake in the liver with a heterogeneous nodular appearance, consistent with advanced cirrhosis. Contrast excretion via the kidneys. Fig. 4: Figure 5: 62 year old male, T1w images (a,c) and T2w 3D MRCP. 40 min. p.i. the common bile duct (CBD) can be clearly visualized after biliary contrast secretion. A large tumor encasing the CBD is seen (o), consistent with a central cholangiocellular carcinoma (Klatskin Tumor). In addition, focal cholangitis in the left lobe is present (->). Page 9 of 13
Fig. 5: Figure 8: 20 year old female, status post cholecystectomy and iatrogenic bile duct injury. T1w images in the axial (a) and coronal (b) plane 40 minutes after contrast injection. Active contrast secretion into a subhepatic bilioma is seen (o) and exact localization of the leakage is possible (->). Fig. 6: Table 1: MR pulse sequence protocol for comprehensive liver examination. Page 10 of 13
Fig. 7: Figure 3: 31 year old male with known PSC. T2w MRCP (a) depicts central high degree stenosis (->). No associated mass is visible on pre contrast (b) and portal venous (c) VIBE images. Late imaging (20 min p.i.) demonstrates patent bile ducts and timely biliary contrast excretion. Fig. 8: Figure 6: 80 year old female. T2w imaging (a) depicts gallbladder filled with multiple stones. Arterial phase imaging (b) and late imaging 60 minutes post contrast injection (c) shows thickened gallbladder wall. In addition, signal alterations in the adjacent liver parenchyma is seen (d). Surgery confirmed gallbladder carcinoma with infiltration of the liver Fig. 9: Figure 7: 37 year old male with known PSC. Status post left hemihepatectomy and hepaticojejunostomy. Late imaging (a,b,c) 20 minutes post contrast injection shows patent anastomosis with timely duodenal contrast excretion. Page 11 of 13
Conclusion The spectrum of MRI appearances of PSC is diverse. MRCP enables the static assessment of the intra- and extrahepatic biliary tract in patients with PSC. By adding a hepatobiliary contrast agent to the imaging protocol assessment of the hepatic parenchyma including hepatic lesions and functional status of the bile ducts is possible. The value of Gd-EOB-DTPA in the assessment of small duct PSC needs to be evaluated in future studies. Personal Information Kristina Imeen Ringe, M.D. Hannover Medical School Department of Radiology Carl-Neuberg-Str. 1 30625 Hannover Germany Contact: ringe.kristina@mh-hannover.de References Björnsson E, Angulo P. Cholangicarcinoma in young individuals with and without primary sclerosing cholangitis. Am J Gastroenterol 2007; 102: 1677-1682 Ringe KI, Gupta RT, Brady CM, et al. Respiratory triggered 3D T2w MR Cholangiography after the injection of Gadoxetate Disodium: is it still reliable? Radiology in press Silveira MG, Lindor KD. Clinical features and management of primary sclerosing cholangitis. World J Gastroenterol 2008; 14(21): 3338-3349 Page 12 of 13
Stiehl A et al. Development of dominant bile duct stenoses in patients with primary sclerosing cholangitis treated with ursodesoxycholic acid: outcome after endoscopic treatment, J Hepatol 2002; 36: 151-156 Page 13 of 13