Download - Vascular Case Study Portal Cavernoma
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