Portal biliopathy from the image to the diagnostic: a systematic illustrated overview

Portal biliopathy from the image to the diagnostic: a systematic illustrated overview Poster No.: C-1690 Congress: ECR 2018 Type: Educational Exhibit Authors: F. Stefan, M. D. M. Boros, E. M. Preda, I. G. Lupescu ; 1 1 2 2 2 2 Bucuresti/RO, Bucharest/RO Keywords: Obstruction / Occlusion, Contrast agent-other, MR, Image manipulation / Reconstruction, Vascular, Biliary Tract / Gallbladder, Abdomen, MR-Cholangiography DOI: 10.1594/ecr2018/C-1690 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 27

Learning objectives 1. 2. To familiarize the radiologists with the most important imaging features of portal cavernoma cholangiopathy. To discuss and illustrate the main differential diagnosis problems. Page 2 of 27

Background Portal biliopathy represents morphological changes in the biliary tree and gallbladder secondary to portal cavernoma [1]. Portal cavernoma Cavernous transformation of the portal vein appears when the native extrahepatic portal vein is thrombosed Table 1 on page 7 [2]. Extrahepatic obstruction of the portal vein (EHPVO) generate increased pressure in the splachnic territory. To bypass the obstruction, blood thends to flow through porto-portal collateral veins of the biliary tree, that form varices in and around the common bile duct (CBD). Porto-portal collateral veins of the biliary tree include paracholedochal and epicholedochal venous plexuses and cholecystic veins. The paracholedochal plexus consists of 3 o'clock and 9 o'clock marginal veins lying parallel to the CBD, which are interconnected with transvers channels. Near the hilum these marginal veins enter into branches of portal vein Fig. 1 on page 7 [3]. Table 1 References: Radiology, I.C. Fundeni, I. C. Fundeni - Bucuresti/RO Page 3 of 27

Fig. 1 References: Adapted from: Sharma Malay & Babu Ramesh. Portal Cavernoma Cholangiopathy: An Endoscopic Ultrasound Based Imaging Approach. Journal of Clinical and Experimental Hepatology. 4.[ DOI:10.1016/j.jceh.2013.08.015]. PATHOGENESIS There are few mechanisms incriminate to produce portal cavernoma cholangiopathy [4, 5]: 1. Reversible component of portal cavernoma cholangiopathy: Mechanical extrinsec compression causing biliary irregularities. Partial or complete resolution of these changes has been shown after shunt surgery and after transjugular intrahepatic portosystemic shunt. 2. Fixed component of portal cavernoma cholangiopathy : Page 4 of 27

Chronic ischemia of the bile duct wall leads to inflammation and fibrosis with high grade stenosis. The contribution of the portal vein to the microvascular blood flow through the bile duct is 62 %, so the extension of the thrombotic process to small venules of the bile ducts cause ischemia of the bile ducts. Also the decrease in the portal venous flow, causes dilatation of the hepatic arterial system, this may result in reduce supply toward the bile ducts. Encasement by a fibrous "tumor-like-cavernoma". Cavernous transformation also involves neogenesis and formation of connective tissue with increasing duration of thrombosis, resulting in fibrous hilar mass containing multiple tiny collateral veins. DIAGNOSIS Imaging evaluation of the portal venous system and biliary tree is usually performed with ultrasonography, computer tomography (CT) and magnetic resonance imaging (MRI). Ultrasonography The initial screening modality for the EHPVO and portal biliopathy is ultrasound with color Doppler because it is non-invasive, free of radiation, easily available and cheap. Findings on sonography vary from non-visualization of portal vein to a completely thrombosed vein with cavernoma formation seen as multiple tubular anechoic structures in the porta. Color Doppler sonography demonstrates flow in portal collaterals in the absence of flow in portal vein. Ultrasound can detect narrowing or stenosis of the CBD with associated proximal dilatation. Exact details of biliary are not possible on ultrasound. [5] Computer Tomography CT clearly depicts the cavernous transformation of the portal vein, presence of the intra and extrahepatic portions of the parabiliary and peribiliary plexuses, and gallbladder varices. The exact location of periportal collaterals with exact localization of portosystemic collaterals can be made out. CT can depict secondary biliary ductal dilatation caused by the portal collaterals. The main purpose of CT remains in ruling out other causes of biliary dilatation. However all the information about the vascular and biliary findings of portal biliopathy on CT comes at the expense of radiation. As the disease may require repetitive imaging, CT is not the preferred technique because of the risks of radiation exposure. [6] Magnetic Resonance Imaging MRI and magnetic resonance cholangiography( MRCP), plays an important role in the positive diagnostic. Diham et al. [5] made the statement that for the diagnosis to be established all of the following criteria would have to be fulfilled: 1. 2. presence of a portal cavernoma, typical cholangiographic changes on endoscopic retrograde cholangiography (ERC) or MRCP, Page 5 of 27

3. absence of other causes of these biliary changes like bile duct injury, primary sclerosing cholangitis, cholangiocarcinoma etc. Combination of contrast enhanced MRI with MRCP have replaced ERC as the diagnostic imaging of choice for portal biliopathy. The MRCP findings of the biliary abnormalities associated with portal cavernoma are: strictures, dilatation, sinuous appearance and common biliary duct dislocation. [2, 5, 7] Awareness of "tumor-like cavernoma" and review of the dynamic contrast (especially portal venous phase) images can help to put the correct diagnosis and save the patient from catastrophic effects of a potential biopsy. [1] Page 6 of 27

Images for this section: Table 1 Radiology, I.C. Fundeni, I. C. Fundeni - Bucuresti/RO Page 7 of 27

Fig. 1 Adapted from: Sharma Malay & Babu Ramesh. Portal Cavernoma Cholangiopathy: An Endoscopic Ultrasound Based Imaging Approach. Journal of Clinical and Experimental Hepatology. 4.[ DOI:10.1016/j.jceh.2013.08.015]. Page 8 of 27

Findings and procedure details MRI EXAMINATION PROTOCOL INCLUDED: Coronal/axial T2 ssfse short/long TE MRCP Oblique coronal +/- T2 ssfse long/short TE centered on the biliary tree Dynamic 3D contrast enhanced (Gd-BOPTA) Axial/cor 3D T1 sequence at 90 minutes after Gd- BOPTA iv injection. IMAGING FINDINGS We reanalyzed the MRI images of patients who were diagnosed with acquired portal cavernoma, spanning from 2007 to 2017. From the total of 114 patients with acquired portal cavernoma, only 16 had abnormal findings in the biliary tract. All the patients with portal biliopathy were asymptomatic. The MRCP findings of the biliary abnormalities associated with portal cavernoma found in our study are: 1. "tumor-like cavernoma"- uniform solid mass structure with encasement of the common bile duct and the hepatic ducts, mimicking a tumor, in which the venous collaterals could not be individualized clearly Fig. 2 on page 18 Page 9 of 27

Fig. 2: Tumor like cavernoma. 44 year old female patient with chronic myeloproliferative disorder and portal cavernoma. Axial T2-weighted FS (a, b), coronal ssfse short TE (c) and long TE (d), 3DT1 axial (e, f), 3DT1+K axial (g, h), 3DT1+K coronal (i) and 3DT1+K hepatobiliary phase coronal (j) images reveal: "tumor-like" portal cavernoma ( arrow) with encasement of the common bile duct and the hepatic ducts. Minimal dilatation of the left hepatic duct. References: Radiology, I.C. Fundeni, I. C. Fundeni - Bucuresti/RO 2. sinuous appearance Fig. 3 on page 18 Page 10 of 27

Fig. 3: Sinuos appearance. 37 year old male patient with cirrhosis due to chronic viral hepatitis. Axial T2-weighted FS (a), 3DT1+K axial (b) and coronal (e), 3DT1+K hepatobiliary phase axial (c) and coronal (f), ssfse long TE coronal (d) images showing: cavernous transformation of the extrahepatic portal vein and the posterior right branch of the portal vein (white arrow). Sinuous appearance of the common bile duct (yellow arrow). Mild dilatation of the intrahepatic bile duct in segments VI and VII. References: Radiology, I.C. Fundeni, I. C. Fundeni - Bucuresti/RO 3. common biliary duct dislocation Fig. 4 on page 19 Fig. 5 on page 20 Page 11 of 27

Fig. 4: Common biliary duct dislocation. 15 year old female patient with factor V Leiden mutation. Axial T2-weighted FS (a), ssfse long TE coronal (b), 3DT1+K axial (c) and coronal (d) images showing: cavernous transformation of the extrahepatic portal vein (asterisks) and the posterior right branch of the portal vein (white arrow). Mild dislocation of the common bile duct (black arrowhead). Multiple strictures with upstream dilatation of the left intrahepatic bile ducts. References: Radiology, I.C. Fundeni, I. C. Fundeni - Bucuresti/RO Page 12 of 27

Fig. 5: Common biliary duct dislocation. 31 year old male patient with idiopathic portal vein thrombosis. Coronal ssfse short (a) and long TE (b), 3DT1+K hepatobiliary phase coronal (c) and axial (e) images showing: Portal cavernoma (asterisks). Dislocation and compression of the mid and distal common bile duct (arrow) with proximal mild dilatation of the central intrahepatic bile ducts. References: Radiology, I.C. Fundeni, I. C. Fundeni - Bucuresti/RO 4. stricture Fig. 6 on page 21 Page 13 of 27

Fig. 6: Stricture. 26 year old female patient with hemathologic disorder and portal cavernoma. 3DT1+K axial (a,b) and coronal (e), axial T2-weighted FS (c,d), coronal ssfse long TE (f, g) images showing: mutiple biliary stenoses (arrowheads) with upstream dilatation (arrow), multiple irregularities of intrahepatic bile ducts and cystic duct (yellow arrow). References: Radiology, I.C. Fundeni, I. C. Fundeni - Bucuresti/RO 5. upstream dilatation Fig. 4 on page 19, Fig. 6 on page 21 DIFFERENTIAL DIAGNOSIS The main differential diagnosis is done with: I.Neoplastic: Cholangiocarcinoma- Klatskin tumor Fig. 7 on page 22 Hilar cholangiocarcinoma (HCCA) is a rare malignant tumor arising from the epithelium of the bile ducts. Diagnostic features include intrahepatic segmental biliary dilatation, periductal thickening, endoluminal lesions and direct tumor spread to the liver or adjacent vessels. Biliary dilatation is usually intrahepatic and often segmental and located proximally to an illdefined biliary mass near the hepatic hilus. The transition between dilated Page 14 of 27

and non dilated bile ducts is usually abrupt and this is a key feature for diagnosis. [8] Fig. 7: Klatskin tumor. Coronal ssfse short TE (a), MRCP (b), axial 3DT1+K (c), ADC (d) and DWI (e) images. Tumour mass (arrow) appearing to originate from the common hepatic duct with extension into the right and left hepatic ducts, proximal common bile duct. The mass is causing obstruction with bilateral intrahepatic biliary duct dilatation. References: Radiology, I.C. Fundeni, I. C. Fundeni - Bucuresti/RO II.Non-neoplastic pathologies: Primary sclerosing cholangitis (PSC) Fig. 8 on page 23 Approximately 60 to 80% of patients with PSC present with inflammatory bowel disease. The clinical presentation can vary, including cholestatic Page 15 of 27

laboratory findings and nonspecific symptoms. Diagnosis of PSC can be made by typical cholangiographic findings and the exclusion of secondary causes. The typical MRCP features include diffuse, multifocal short segmental strictures and mild dilatation in the intrahepatic and extrahepatic bile ducts alternating with normal ducts, which sometimes produce "beaded" appearance. As the fibrosis progresses and strictures worsen, the peripheral bile ducts are obliterated and become poorly visualized on MRCP showing a "pruned tree" appearance. [9] Fig. 8: 40 year old female patient with primary sclerosing cholangitis. Coronal ssfse short TE (a), MRCP (b) and coronal hepatobiliary phase (c), images showing: Multifocal intra- and extrahepatic biliary strictures alternating with segmental dilatation. Intrahepatic biliary stone (arrow) appearing as low signal filling defect. References: Radiology, I.C. Fundeni, I. C. Fundeni - Bucuresti/RO Biliary tree lithiasis Fig. 9 on page 24 MRCP is the best modality to identify choledocholithiasis, appearing as filling defects with low signal. The key finding for excluding portal biliopathy is absence of portal cavernoma. Fig. 9: Coronal ssfse short TE (a), MRCP (b) and coronal hepatobiliary phase (c) images showing:choledocolithiasis (arrow) in the terminal portion of the common bile duct with upstream dilatation. References: Radiology, I.C. Fundeni, I. C. Fundeni - Bucuresti/RO WHAT THE CLINICIAN SHOULD KNOW? Page 16 of 27

Presence or absence of: 1. biliary abnormalities: stricture, dilatation, sinuos appearance, biliary lithiasis; 2. vascular abnormalities: extrahepatic chronic portal vein thrombosis, portal cavernoma, gallbladder varices; 3. indirect signs of portal hypertension as ascites or splenomegaly or collateral splanchnic circulation. Page 17 of 27

Images for this section: Fig. 2: Tumor like cavernoma. 44 year old female patient with chronic myeloproliferative disorder and portal cavernoma. Axial T2-weighted FS (a, b), coronal ssfse short TE (c) and long TE (d), 3DT1 axial (e, f), 3DT1+K axial (g, h), 3DT1+K coronal (i) and 3DT1+K hepatobiliary phase coronal (j) images reveal: "tumor-like" portal cavernoma ( arrow) with encasement of the common bile duct and the hepatic ducts. Minimal dilatation of the left hepatic duct. Radiology, I.C. Fundeni, I. C. Fundeni - Bucuresti/RO Page 18 of 27

Fig. 3: Sinuos appearance. 37 year old male patient with cirrhosis due to chronic viral hepatitis. Axial T2-weighted FS (a), 3DT1+K axial (b) and coronal (e), 3DT1+K hepatobiliary phase axial (c) and coronal (f), ssfse long TE coronal (d) images showing: cavernous transformation of the extrahepatic portal vein and the posterior right branch of the portal vein (white arrow). Sinuous appearance of the common bile duct (yellow arrow). Mild dilatation of the intrahepatic bile duct in segments VI and VII. Radiology, I.C. Fundeni, I. C. Fundeni - Bucuresti/RO Page 19 of 27

Fig. 4: Common biliary duct dislocation. 15 year old female patient with factor V Leiden mutation. Axial T2-weighted FS (a), ssfse long TE coronal (b), 3DT1+K axial (c) and coronal (d) images showing: cavernous transformation of the extrahepatic portal vein (asterisks) and the posterior right branch of the portal vein (white arrow). Mild dislocation of the common bile duct (black arrowhead). Multiple strictures with upstream dilatation of the left intrahepatic bile ducts. Radiology, I.C. Fundeni, I. C. Fundeni - Bucuresti/RO Page 20 of 27

Fig. 5: Common biliary duct dislocation. 31 year old male patient with idiopathic portal vein thrombosis. Coronal ssfse short (a) and long TE (b), 3DT1+K hepatobiliary phase coronal (c) and axial (e) images showing: Portal cavernoma (asterisks). Dislocation and compression of the mid and distal common bile duct (arrow) with proximal mild dilatation of the central intrahepatic bile ducts. Radiology, I.C. Fundeni, I. C. Fundeni - Bucuresti/RO Page 21 of 27

Fig. 6: Stricture. 26 year old female patient with hemathologic disorder and portal cavernoma. 3DT1+K axial (a,b) and coronal (e), axial T2-weighted FS (c,d), coronal ssfse long TE (f, g) images showing: mutiple biliary stenoses (arrowheads) with upstream dilatation (arrow), multiple irregularities of intrahepatic bile ducts and cystic duct (yellow arrow). Radiology, I.C. Fundeni, I. C. Fundeni - Bucuresti/RO Page 22 of 27

Fig. 7: Klatskin tumor. Coronal ssfse short TE (a), MRCP (b), axial 3DT1+K (c), ADC (d) and DWI (e) images. Tumour mass (arrow) appearing to originate from the common hepatic duct with extension into the right and left hepatic ducts, proximal common bile duct. The mass is causing obstruction with bilateral intrahepatic biliary duct dilatation. Radiology, I.C. Fundeni, I. C. Fundeni - Bucuresti/RO Page 23 of 27

Fig. 8: 40 year old female patient with primary sclerosing cholangitis. Coronal ssfse short TE (a), MRCP (b) and coronal hepatobiliary phase (c), images showing: Multifocal intra- and extrahepatic biliary strictures alternating with segmental dilatation. Intrahepatic biliary stone (arrow) appearing as low signal filling defect. Radiology, I.C. Fundeni, I. C. Fundeni - Bucuresti/RO Fig. 9: Coronal ssfse short TE (a), MRCP (b) and coronal hepatobiliary phase (c) images showing:choledocolithiasis (arrow) in the terminal portion of the common bile duct with upstream dilatation. Radiology, I.C. Fundeni, I. C. Fundeni - Bucuresti/RO Page 24 of 27

Conclusion MRI, especially MRCP, is the imaging modality of choice for portal biliopathy diagnosis and follow-up, associated with axial 3D T1+Gd for mapping of the portal cavernoma. Page 25 of 27

Personal information Dr. Florentina Stefan Prof.Dr.Ioana G. Lupescu Radiology Medical Imaging and Interventional Radiology Depatment, Fundeni Clinical Institute, University of Medicine and Pharmacy "Carol Davila"- Bucharest mail: florentinastefan08@yahoo.com ilupescu@gmail.com Page 26 of 27

References 1. Arora A.: Imaging in portal cavernoma cholangiopathy: current understanding and future perspectives. AME Med J 2017; 2:102. 2. Khuroo MS et al.: Portal biliopathy. World J Gastroenterol 2016; 22(35): 7973-7982. 3. Sharma M et al.: Portal Cavernoma Cholangiopathy: An Endoscopic Ultrasound Based Imaging Approach. Journal of Clinical and Experimental Hepatology 2013; 4 [DOI:10.1016/j.jceh.2013.08.015]. 4. Puri P et al.: Journal of Clinical and Experimental Hepatology: S27-S33. PMC. Web. 4 Sept. 2017. 5. Dhiman RK et al: Portal Cavernoma Cholangiopathy: Consensus Statement of a Working Party of the Indian National Association for Study of the Liver. Journal of Clinical and Experimental Hepatology 2014; 4: S2-S14 [DOI:10.1016/j.jceh.2014.02.003]. 6. Kalra N, Shankar S, Khandelwal N.: Imaging of Portal Cavernoma Cholangiopathy. Journal of Clinical and Experimental Hepatology. 2014;4(Suppl 1):S44-S52. doi:10.1016/ j.jceh.2013.07.004. 7. Khan et al.: Portal biliopathy: A review of imaging features of nine patients. International Journal of Clinical Medicine 2017; 8, 604-617. 8. Valls, Carlos et al.: "Radiological Diagnosis and Staging of Hilar Cholangiocarcinoma." World Journal of Gastrointestinal Oncology 5.7 (2013): 115-126. PMC. Web. 30 Dec. 2017. 9. Seo, Nieun et al.: "Sclerosing Cholangitis: Clinicopathologic Features, Imaging Spectrum, and Systemic Approach to Differential Diagnosis." Korean Journal of Radiology 17.1 (2016): 25-38. Page 27 of 27