1. 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 porta hepatis Differential Arteriovenous malformation? Cholangiocarcinoma Portal hypertension with esophageal varices Vascular tumour?

4. Ultrasound

5. Arteriography

12. Portal Hypertension Relative or absolute obstruction of splanchnic blood flow 4 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 presentations 1 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: Gastroesophagela variceal bleeding/Anaemia +/- Obstructive jaundice Hepatosplenomegaly Mostly asymptomatic

15. Anatomy Two venous plexuses of bile ducts and gallbladder 3 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 extrahepatic biliary tracts Venous plexuses of Saint and Petrin 3 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 stricture 3 Colour and Spectral Doppler key to differentiation 2

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 porta hepatis 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 Intrahepatic vs extrahepatic 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.orghttp://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 6 th Ed. Lippincott, Williams & Wilkins 2007. p696-698 8. Images available from http://www.radiopaedia.orghttp://www.radiopaedia.org 9. Brant WE, Helms CA. Fundamentals of Diagnostic Radiology 3 rd edition. Lippincott Williams & Wilkins 2007