Vascular Case StudyPortal Cavernoma

Antoinette ReindersClinical Imaging Sciences

UFSSeptember 2012

Patient Demographics36 year old female patientReferred from PVTC/O

Loss of weight, fatigue and malaiseRight upper quadrant fullness

No significant medical or surgical historyNo history of alcohol or tobacco usageClinical examination

Hepatomegaly with slight jaundice- ?Portal hypertension

Slightly raised ductal enzymes and ALP

Special investigationsAbdominal Ultrasound

Normal liverConglomerate, venous vascular mass at porta

hepatisDifferential

Arteriovenous malformation? Cholangiocarcinoma Portal hypertension with esophageal varices Vascular tumour?

Ultrasound

Arteriography

Arteriography

Arteriography

Arteriography

Arteriography

Arteriography

Arteriography

Portal HypertensionRelative or absolute obstruction of splanchnic blood

flow4

Less commonly increased portal blood flowNormal pressure 5 – 10 mm Hg>10 mmHg = PHT PV flow reduction = 7-12 cm/sec

ClassificationPre-HepaticHepaticPost-HepaticMultiple collateral pathways from high-pressure portal

system – low pressure systemic system

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

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 hypertensionDevelopment and dilatations of multiple small vessels

in and around the re-canalizing main portal veinMain clinical presentation:

Gastroesophagela variceal bleeding/Anaemia +/- Obstructive jaundice Hepatosplenomegaly Mostly asymptomatic

AnatomyTwo venous plexuses of bile ducts and

gallbladder3

Epicholedochal venous plexus of Saint Fine reticular web on outer surface of CBD and

hepatic ductsParacholedochal veins of Petren

Parallel to CBD Connected with

Gastric, pancreatico-duodenal, portal veins and liver directly

Extrinsic compression of CBD

PathologyLiver’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 Petrin3

As well as gallbladder Continues to organise as a cavernous

transformation in 3 – 5 weeks

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

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

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

Pseudo-cholangiocarcinoma sign

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

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

Management

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

CholangiocarcinomaSecond most common hepatic malignancy after hepatoma

AdenocarcinomaIntrahepatic 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 cholangiocarcinomasClinical symptoms

Weight loss, fatigue, anaemiaObstructive jaundice (painless)Elevated Billirubin and ALPPalpable mass

CholangiocarcinomaEncasement/obliteration/infiltration of portal

vein and hepatic arteryIntrahepatic duct dilatation

+/- Extrahepatic duct dilatation“Long stricture” with ERCP

Hypervascular tumour with neovascularisationArterio-arterial collaterals along course of bile

ductsExophytic tumour mass on CT

100% as low attenuating mass

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

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