Mass-Like Extreme Wall Thickening of the Entire Common Bile Duct in a Case of Portal Cavernoma Cholangiopathy

Portal cavernoma cholangiopathy (PCC) refers to the cholangiographic abnormalities that occur in patients with portal cavernoma. These abnormalities may be either due to chronic portal vein thrombosis or extrahepatic portal vein occlusion. These abnormalities occur due to enlargement of the bridging...

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Main Authors: Veeraraghavan Gunasekaran, Supraja Laguduva Mohan, Sunitha Vellathussery Chakkalakkoombil, Kuppusamy Senthamizhselvan
Format: Article
Language:English
Published: Thieme Medical and Scientific Publishers Pvt. Ltd. 2022-01-01
Series:Journal of Gastrointestinal and Abdominal Radiology
Subjects:
Online Access:http://www.thieme-connect.de/DOI/DOI?10.1055/s-0041-1735216
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author Veeraraghavan Gunasekaran
Supraja Laguduva Mohan
Sunitha Vellathussery Chakkalakkoombil
Kuppusamy Senthamizhselvan
author_facet Veeraraghavan Gunasekaran
Supraja Laguduva Mohan
Sunitha Vellathussery Chakkalakkoombil
Kuppusamy Senthamizhselvan
author_sort Veeraraghavan Gunasekaran
collection DOAJ
description Portal cavernoma cholangiopathy (PCC) refers to the cholangiographic abnormalities that occur in patients with portal cavernoma. These abnormalities may be either due to chronic portal vein thrombosis or extrahepatic portal vein occlusion. These abnormalities occur due to enlargement of the bridging tortuous paracholedochal, epicholedochal, and cholecystic veins exerting pressure on the bile ducts. Ischemic changes can also occur in the bile duct due to portal vein thrombosis, which affects the microvascular circulation or associated hepatic artery thrombosis. These may be either reversible with shunt procedures such as transjugular intrahepatic portosystemic shunt (TIPS) or irreversible in the advanced stage, leading to recurrent episodes of biliary pain, cholangitis, and cholestasis. Occasionally it may present as mass-like diffuse common bile duct (CBD) wall thickening, which may be confused with mimickers like primary CBD lymphoma, immunoglobulin G4-related sclerosing cholangitis, and even cholangiocarcinoma on imaging. Thus, we need to be aware of the mass-forming PCC imaging findings to avoid unnecessary invasive procedures like biopsy or surgical intervention. Here, we present a case of PCC, which presented as mass-like diffuse CBD wall thickening with patent lumen on ultrasound that led to further workup with contrast-enhanced computed tomography and magnetic resonance imaging. The wall thickening showed persistent delayed enhancement, no significant diffusion restriction, and there was also associated superior mesenteric vein thrombosis with multiple mesenteric collaterals. A positron emission tomography-CT scan also ruled out malignant disease as there was no uptake. Finally, a diagnosis of mass-forming PCC was made by combining imaging features and excluding other causes.
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spelling doaj.art-e593b84a6f0849e098250c265ea64ae52022-12-22T02:06:52ZengThieme Medical and Scientific Publishers Pvt. Ltd.Journal of Gastrointestinal and Abdominal Radiology2581-91782022-01-01050106306910.1055/s-0041-1735216Mass-Like Extreme Wall Thickening of the Entire Common Bile Duct in a Case of Portal Cavernoma CholangiopathyVeeraraghavan Gunasekaran0Supraja Laguduva Mohan1Sunitha Vellathussery Chakkalakkoombil2Kuppusamy Senthamizhselvan3Department of Radiodiagnosis, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, IndiaDepartment of Radiodiagnosis, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, IndiaDepartment of Radiodiagnosis, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, IndiaDepartment of Medical Gastroenterology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, IndiaPortal cavernoma cholangiopathy (PCC) refers to the cholangiographic abnormalities that occur in patients with portal cavernoma. These abnormalities may be either due to chronic portal vein thrombosis or extrahepatic portal vein occlusion. These abnormalities occur due to enlargement of the bridging tortuous paracholedochal, epicholedochal, and cholecystic veins exerting pressure on the bile ducts. Ischemic changes can also occur in the bile duct due to portal vein thrombosis, which affects the microvascular circulation or associated hepatic artery thrombosis. These may be either reversible with shunt procedures such as transjugular intrahepatic portosystemic shunt (TIPS) or irreversible in the advanced stage, leading to recurrent episodes of biliary pain, cholangitis, and cholestasis. Occasionally it may present as mass-like diffuse common bile duct (CBD) wall thickening, which may be confused with mimickers like primary CBD lymphoma, immunoglobulin G4-related sclerosing cholangitis, and even cholangiocarcinoma on imaging. Thus, we need to be aware of the mass-forming PCC imaging findings to avoid unnecessary invasive procedures like biopsy or surgical intervention. Here, we present a case of PCC, which presented as mass-like diffuse CBD wall thickening with patent lumen on ultrasound that led to further workup with contrast-enhanced computed tomography and magnetic resonance imaging. The wall thickening showed persistent delayed enhancement, no significant diffusion restriction, and there was also associated superior mesenteric vein thrombosis with multiple mesenteric collaterals. A positron emission tomography-CT scan also ruled out malignant disease as there was no uptake. Finally, a diagnosis of mass-forming PCC was made by combining imaging features and excluding other causes.http://www.thieme-connect.de/DOI/DOI?10.1055/s-0041-1735216common bile ductmagnetic resonance imagingportal vein
spellingShingle Veeraraghavan Gunasekaran
Supraja Laguduva Mohan
Sunitha Vellathussery Chakkalakkoombil
Kuppusamy Senthamizhselvan
Mass-Like Extreme Wall Thickening of the Entire Common Bile Duct in a Case of Portal Cavernoma Cholangiopathy
Journal of Gastrointestinal and Abdominal Radiology
common bile duct
magnetic resonance imaging
portal vein
title Mass-Like Extreme Wall Thickening of the Entire Common Bile Duct in a Case of Portal Cavernoma Cholangiopathy
title_full Mass-Like Extreme Wall Thickening of the Entire Common Bile Duct in a Case of Portal Cavernoma Cholangiopathy
title_fullStr Mass-Like Extreme Wall Thickening of the Entire Common Bile Duct in a Case of Portal Cavernoma Cholangiopathy
title_full_unstemmed Mass-Like Extreme Wall Thickening of the Entire Common Bile Duct in a Case of Portal Cavernoma Cholangiopathy
title_short Mass-Like Extreme Wall Thickening of the Entire Common Bile Duct in a Case of Portal Cavernoma Cholangiopathy
title_sort mass like extreme wall thickening of the entire common bile duct in a case of portal cavernoma cholangiopathy
topic common bile duct
magnetic resonance imaging
portal vein
url http://www.thieme-connect.de/DOI/DOI?10.1055/s-0041-1735216
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AT sunithavellathusserychakkalakkoombil masslikeextremewallthickeningoftheentirecommonbileductinacaseofportalcavernomacholangiopathy
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