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Vascular Case Study Portal Cavernoma

Vascular Case Study Portal Cavernoma. Antoinette Reinders Clinical Imaging Sciences UFS September 2012. Patient Demographics. 36 year old female patient Referred from PVT C/O Loss of weight, fatigue and malaise Right upper quadrant fullness No significant medical or surgical history

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Vascular Case Study Portal Cavernoma

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  1. Vascular Case StudyPortal Cavernoma Antoinette Reinders Clinical Imaging Sciences UFS September 2012

  2. Patient Demographics • 36 year old female patient • Referred from PVT • C/O • Loss of weight, fatigue and malaise • Right upper quadrant fullness • No significant medical or surgical history • No history of alcohol or tobacco usage • Clinical examination • Hepatomegaly with slight jaundice- ?Portal hypertension • Slightly raised ductal enzymes and ALP

  3. Special investigations • Abdominal Ultrasound • Normal liver • Conglomerate, venous vascular mass at portahepatis • Differential • Arteriovenous malformation? • Cholangiocarcinoma • Portal hypertension with esophagealvarices • Vascular tumour?

  4. Ultrasound

  5. Arteriography

  6. Arteriography

  7. Arteriography

  8. Arteriography

  9. Arteriography

  10. Arteriography

  11. Arteriography

  12. Portal Hypertension • Relative or absolute obstruction of splanchnic blood flow4 • Less commonly increased portal blood flow • Normal pressure 5 – 10 mm Hg • >10 mmHg = PHT • PV flow reduction = 7-12 cm/sec • Classification • Pre-Hepatic • Hepatic • Post-Hepatic • Multiple collateral pathways from high-pressure portal system – low pressure systemic system

  13. Portal Hypertension • Pre-Hepatic • Portal vein thrombosis (PVT) or Compression • Hepatic • Pre-Sinusoidal • Congenital Hepatic fibrosis • Alfa 1 anti-trypsin • Wilson • Myelofibrosis • Cystic fibrosis • Sinusoidal • Hepatitis • Sickle cell disease • Post-sinusoidal • Cirrhosis • Veno-occlusive disease of liver • Post-sinusoidal • Budd-Chiari • Constrictive pericarditis • Congenital Hepatic fibrosis

  14. Cavernous Transformation of Portal Vein (CTPV) • Rare condition • Various etiologies and diverse clinical presentations1 • Occurs after longstanding extra hepatic portal vein thrombosis (PVT) • Causing portal hypertension • Development and dilatations of multiple small vessels in and around the re-canalizing main portal vein • Main clinical presentation: • Gastroesophagelavariceal bleeding/Anaemia • +/- Obstructive jaundice • Hepatosplenomegaly • Mostly asymptomatic

  15. Anatomy • Two venous plexuses of bile ducts and gallbladder3 • Epicholedochal venous plexus of Saint • Fine reticular web on outer surface of CBD and hepatic ducts • Paracholedochal veins of Petren • Parallel to CBD • Connected with • Gastric, pancreatico-duodenal, portal veins and liver directly • Extrinsic compression of CBD

  16. Pathology • Liver’s compensatory mechanisms after PVT • 2/3 Blood supply from Portal vein (PV) • 1/3 Blood supply from Hepatic artery (HA) • HA dilates to compensate – “arterial rescue” • Rapid development of collaterals – “venous rescue” • In and around intra and extrahepaticbiliary tracts • Venous plexuses of Saint and Petrin3 • As well as gallbladder • Continues to organise as a cavernous transformation in 3 – 5 weeks

  17. Cavernous Transformation of Portal Vein (CTPV) • 80% of patients has “biliopathy”2,3 • Strictures or displacements in biliary ducts and main bile ducts • Changes of collateral vessels compressing on near biliary tracts and gallbladder • Longstanding PVT = Vascular neogenesis and fibrosis • Appears similar to cholangiocarcinoma on ERCP • “Pseudo-cholangiocarcinoma sign”1,2 • Mildly elevated bilirubin and ALP levels • +/- presence of CBD stone and biliary stricture3 • Colour and Spectral Doppler key to differentiation2

  18. Transabdominal ultrasound images of a 53 year old male patient who developed a portal cavernoma , post liver transplant. Notice the multiple collateral vessels surrounding the portahepatis region with Colour Doppler ultrasound

  19. Dhiman RK, Behera A, Chawla YK, Dilawari JB, Suri S. Portal hypertensive biliopathy. Gut 2007;56(7):1001-1008

  20. Pseudo-cholangiocarcinoma sign Dhiman RK, Behera A, Chawla YK, Dilawari JB, Suri S. Portal hypertensive biliopathy. Gut 2007;56(7):1001-1008

  21. Kesler A et al. Vascular and biliary abnormalities mimicking cholangiocarcinoma in patients with cavernous transformation of the portal vein – Role of colour Doppler Sonography. J Ultrasound Med 2007; 26:1089-1095

  22. Management Dhiman RK, Behera A, Chawla YK, Dilawari JB, Suri S. Portal hypertensive biliopathy. Gut 2007;56(7):1001-1008

  23. Cholangiocarcinoma • Second most common hepatic malignancy after hepatoma • Adenocarcinoma • Intrahepaticvsextrahepatic • Avg age at onset • Intrahepatic = 50 – 60 years (M>F) • 10 – 13 % of all cholangiocarcinomas • Extrahepatic = 60 – 70 years (M>F) • >90% of all cholangiocarcinomas • Clinical symptoms • Weight loss, fatigue, anaemia • Obstructive jaundice (painless) • Elevated Billirubin and ALP • Palpable mass

  24. Cholangiocarcinoma • Encasement/obliteration/infiltration of portal vein and hepatic artery • Intrahepatic duct dilatation • +/- Extrahepatic duct dilatation • “Long stricture” with ERCP • Hypervascular tumour with neovascularisation • Arterio-arterial collaterals along course of bile ducts • Exophytic tumour mass on CT • 100% as low attenuating mass

  25. Images available from: http://www.gastrohep.com/images/image and radiopaedia.org

  26. Bibliography • 1. Ramos R, Park Y, Shazad G, Garcia C, Cohen R. Cavernous transformation of portal vein secondary to portal vein thrombosis: A case report. J Clin Med Res 2011;4(1):81-84 • 2. Kesler A et al. Vascular and biliary abnormalities mimicking cholangiocarcinoma in patients with cavernous transformation of the portal vein – Role of colour Doppler Sonography. J Ultrasound Med 2007; 26:1089-1095 • 3. Dhiman RK, Behera A, Chawla YK, Dilawari JB, Suri S. Portal hypertensive biliopathy. Gut 2007;56(7):1001-1008 • 4. Lupescu I, Masala N, Capsa R, Campeanu N, Georgescu SA. CT and mri of acquired portal venous system anomalies. J Gastrointestin Liver Dis 2006:15(4), 393-398 • 5. Sorrentino D, Labombarda A, DeBiase F, Trevisi A, Giagu P. Cavernous transformation of the portal vein associated to multiorgan developmental abnormalities. Liver international 2004; 24: 80-83 • 6. Bayraktar Y et al. The “Pseudo-cholangiocarcinoma sign” in patients with cavernous transformation of the portal vein and its effect on the serum alkaline phosphatase and bilirubin levels. Am J of Gastroenterol. 1995, 90(11): 2015-2019 • 7. Dahnert W. Radiology Review Manual 6th Ed. Lippincott, Williams & Wilkins 2007. p696-698 • 8. Images available from http://www.radiopaedia.org • 9. Brant WE, Helms CA. Fundamentals of Diagnostic Radiology 3rd edition. Lippincott Williams & Wilkins 2007

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