Download Portal Vein Thrombosis

Portal Vein Thrombosis
Sophia A. Virani, HMS III
Gillian Lieberman, MD
Beth Israel Deaconess Medical Center
Department of Radiology
March 2009
Agenda
Introduce index patient
 Discuss portal vein thrombosis (PVT)

 Etiology
 Menu of radiologic tests
 Complications
Review hepatic anatomy and vasculature
 Companion Cases
 Review various treatment options

Let’s meet our patient …
Ms. S: Initial presentation

Ms. S is a 61 year old woman with ulcerative colitis and
primary biliary cirrhosis (PBC) who was transferred from an
outside hospital with worsening abdominal pain, bloody
diarrhea and ascites
 Her ulcerative colitis had become steroid-unresponsive during the
weeks prior to admission
 She has had PBC for ten years and her illness has been complicated
by ascites and hepatic encephalopathy
A review of her outside hospital CT showed a possible portal
vein thrombus
Portal Vein Thrombus: Overview

Rare event but can have serious clinical consequences

Types
 Acute or chronic
 Bland or malignant

Presentation
 Often subtle or asymptomatic
►
Symptoms are often those of primary illness

GI bleeding, abdominal pain, varices,
varices, ascites and splenomegaly
 In symptomatic patients, variceal bleeding is the most common presentation

Complications
 Portal hypertension from increased pressure to portal vein obstruction
 Thrombus can spread to splanchnic veins and potentially cause mesenteric ischemia,
which is a surgical emergency
High clinical suspicion and effective interpretation of
radiologic findings are key to making the diagnosis of portal vein thrombus
Etiology of Portal Vein Thrombus





Cirrhosis
 25% of patients with PVT have cirrhosis
► 1% of patients with cirrhosis develop PVT
► Likelihood of developing thrombus is associated with severity of disease
 Stasis of flow in portal system predisposes to clot formation
 Deficiency of protein C, protein S and antithrombin III lead to a hypercoagulable
state
Malignancy
 Hepatocellular carcinoma (HCC), pancreatic cancer
 Development of thrombus is a poor prognostic factor in HCC
Hypercoagulable states
 Factor V Leiden, Prothrombin gene mutation, Protein C deficiency, Protein S
deficiency, Antithrombin III deficiency
 Myeloproliferative disease
Infection
 Rare cause of PVT
 Intra-abdominal infection such as diverticulitis and appendicitis can cause a
pylephlebitis of portal vein
 In the pediatric population, omphalitis (infection of umbilical stump) can spread to
portal vein
Post surgical


Post splenectomy
Post transplant, secondary to surgical manipulation of portal system
Often multifactorial
Etiology unknown in up to 1/3 of cases
Portal vein thrombosis is a
radiologic diagnosis.
Let’s take a look at the various
options for imaging ….
PVT: Menu of Radiologic Tests
Test
Indications &
Findings
Advantages
Disadvantages
Ultrasound
Echogenic
thrombus
No blood flow on
Doppler
Non
invasive
Readily available
Operator
dependent
Acute thrombi could be
hypoechoic or anechoic
Difficult to assess splenic vein
and other branches due to
background liver echogenicity
Contrast CT
Non-enhancing
filling
defect within lumen
Rim enhancement
Variations with phase
Readily
available
Non operator
dependent
Contraindicated
in patients with
contrast allergy, renal
insufficiency
MRI
Can
Non
Expense
see increased signal
intensity on T2
invasive
No contrast
Claustrophobia
Ascites
Angiography
MRA / CTA
Often
pre-operative or
during TIPS
Filling defect
standard
Shows extent, location
and severity of
thrombus
Invasive
Filling
Good
Invasive
defect
Gold
may cause artifact
visualization of
collaterals
Contrast
Contrast
Anatomy Review
Portal venogram showing main, left and
right portal veins
BIDMC PACS
Ms. S: Patent Left Portal Vein and Left Portal
Vein thrombosis on CT
Abdominal CT 1 month prior
to admission
Abdominal CT on admission
*
*
*
*
C+ Abdominal CT, axial image, portal venous phase.
Patent left portal vein
BIDMC
BIDMC PACS
BIDMC PACS
Left portal vein occlusion
* ascites
Portal Vein Thrombus: CT Findings

Non enhancing filling defect within lumen

Rim enhancement
 Flow through dilated vasa vasorum
 Increased flow around periphery of clot

Can see phase dependent changes
 Enhancement during arterial phase as hepatic artery compensates
for decreased blood flow from portal system
 Decreased attenuation during portal venous phase

Occasionally can see calcifications in chronic thrombus

Can see arterial phase enhancement if malignant thrombus
Ultrasound of Portal System

Can detect presence, direction and characteristics of portal
venous blood flow
 hepatopetal = flow toward liver
► Normal
direction of portal venous flow
 hepatofugal = flow away from liver



Normal portal venous flow is continuous hepatopetal with
minimal respiratory variation
Hepatofugal flow in the portal system develops when
pressure in portal system is greater than pressure in
collateral vessels
Thrombus in portal vein would show absence of flow and
an echogenic thrombus
Ms. S: Patent Portal Vein on US

Transverse color doppler ultrasound of
main portal vein

Transverse color doppler ultrasound of
left portal vein

Flow depicts patent main portal vein

Flow depicts patent left portal vein

Possible small echogenic thrombus in
left portal vein
Ms. S: Clinical Course


Ulcerative Colitis
 Treated with an infusion of Remicade, a TNF- inhibitor
 Received 2 units packed red blood cells
Primary biliary cirrhosis
 Developed hepatic encephalopathy
►


Treated with lactulose and rifaximin
 Therapeutic paracentesis
Tested positive for HIT antibodies
 Lower extremity ultrasound showed no DVTs
Acute PVT
 Review of outside hospital CT showed left portal vein thrombus
 Doppler ultrasound revealed patent left portal vein, though non-occlusive
thrombus could not be ruled out
 Anticoagulation contraindicated given GI bleeding
 Scheduled for follow up CT in 2 weeks
Represented within 2 weeks for acute decompensation of end
stage liver disease
Ms. S underwent ultrasound and
CT scans which showed
progression of her portal vein
thrombus.
Ms. S: Portal Vein Thrombus on Ultrasound
BIDMC PACS
Previous Ultrasound
Color doppler ultrasound, transverse view of liver
No flow in main portal vein
Echogenic thrombus
BIDMC PACS
Next Step?
Ms. S: Main Portal Vein Thrombus on CT

*


*
*
*
Contrast enhanced abdominal CT, axial images, portal venous phase
phase
BIDMC PACS
Non enhancing
filling defect in
main PV
Multiple collateral
vessels
ascites
Ms. S: Left and Right Portal Vein Thrombi on CT
Contrast enhanced abdominal CT, axial images, portal venous phase
phase
Left portal vein occlusion
BIDMC PACS
Right portal vein occlusion
Ms. S: Superior Mesenteric Vein Thrombus on
CT
Filling defect in
superior
mesenteric vein
(SMV)
Contrast enhanced abdominal CT, axial images, portal venous phase
phase
BIDMC PACS
Ms. S: Main Portal Vein and SMV Thrombus on CT

Thrombus in main
portal vein

Thrombus in SMV
*

Ascites
*

Multiple collateral
vessels
*
Contrast enhanced abdominal CT, portal venous phase, maximum intensity
intensity
projection, coronal reconstruction
BIDMC PACS
Ms. S: Interim Clinical Course

Acute liver failure
 MELD 28, up from 13 during last admission
 Total bilirubin 16.4, up from 2.6 during last admission


She developed a leukocytosis which was
concerning for possible spontaneous bacterial
peritonitis
Hypotensive and tachycardic
 Placement of central line

She became hypoxic to 88% on RA
 Chest x-ray to evaluate hypoxia
Ms. S: Pneumothorax and Pleural Effusion
on Chest X-RAY
BIDMC PACS
*
*
*
Chest tube
Portable anterioranterior-posterior chest xx-ray




Right pleural effusion
Left pneumothorax
Collapsed left lung
Central line  possible cause of pneumothorax

Chest tube with re-expanded left lung
Ms. S: Clinical Progression

Significant clinical decompensation, most likely
due to occlusive portal vein thrombus
 Anticoagulation contraindicated given GI bleed
 The transplant team was consulted
► She
was not a candidate for transplant because of her
ulcerative colitis and possible sepsis


She developed disseminated intravascular
coagulation and methicillin-resistent staph aureus
bacteremia
She was ultimately made comfort measures only
and passed away soon after
Ms. S’s case demonstrated an
acute, bland thrombus in the
setting of cirrhosis.
Let’s move on to see features of
portal vein thrombus caused by a
tumor in two companion patients
Companion Patient #1:
Portal Vein Tumor Thrombus on CT

71 year old male with a history of hepatitis B who presents with two
days of epigastric pain, frequent bowel movements and fecal
incontinence
Large
hypoattenuating
mass in right lobe
of liver extending
into portal vein
C+ CT scan, axial images, portal venous phase
BIDMC PACS
Tumor Thrombus: Radiologic Features
Companion patient #1

Often see dilation of portal
vein
 Diameter > 23mm*

Intrathrombus neovascularity
 Arterial enhancement on CT
 Pulsatile flow on doppler US

“Thread and streak sign”
 multiple enhancing intraluminal
smaller vessels that can be seen
at arterial phase imaging
BIDMC PACS

Contiguity to tumor
 Often with direct invasion
*Tublin et al., “Benign and malignant portal vein thrombosis.”
thrombosis.”
Companion Patient #1:
Inferior Vena Cava and Right Atrial Thrombi on CT
BIDMC PACS
BIDMC PACS
IVC tumor thrombus
Right atrial tumor thrombus
Companion Patient #2:
PVT and Cavernous Transformation on CT

52 year old male with a three year history of renal cell carcinoma with
liver metastases and known left portal vein thrombus
BIDMC PACS
BIDMC PACS
Contrast enhanced abdominal CT, portal venous phase, maximum intensity
intensity projection, coronal reconstruction
Left portal vein thrombus
Multiple hypoattenuating lesions consistent with metastatic disease
Multiple serpiginous periportal collateral vessels = CAVERNOUS TRANSFORMATION
Cavernous Transformation

Formation of multiple venous collaterals when portal
system is obstructed
 Necessary to drain tributaries of portal vein and maintain adequate hepatic
perfusion
 Can be porto-porto (bypassing obstruction) or porto-systemic

Good imaging of collaterals is important for surgical
planning

Imaging techniques
 CT, US, MRI, CTA, MRA
 Appearance: spongelike, serpiginous, corkscrew, netlike
Collateral Circulation


Reversal of flow into low pressure collateral veins due to
portal hypertension
Major porto-systemic collaterals
 Left gastric vein, short gastric veins, esophageal veins
► Anastomose with azygous system
► Esophageal varices
 Para-umbilical and abdominal wall veins
► Caput
medusa
 Splenorenal shunts
 Rectal varices

Bleeding from varices causes significant morbidity
Companion Patient #3:
Porto-Porto Collaterals on CT and MRA
Lee et al: Portal vein thrombosis: CT features
 Cavernous transformation on
contrast enhanced CT scan,
portal venous phase
Wang et. al “Cavernous transformation of the portal vein:
3D dynamic contrast enhanced MR angiography”
angiography”
 Cavernous transformation on MRI
angiography
Companion Patient #4: Cirrhosis on CT scan
57 year old male with a history of alcoholic cirrhosis, portal
hypertension, gastroesophageal varices and chronic portal vein
thrombosis
 Presented for possible transjugalar intrahepatic porto-systemic shunt
(TIPS) procedure to alleviate portal hypertension

Shrunken, cirrhotic liver
BIDMC PACS
The patient had a percutaneous
venogram to visualize the portal
system during his TIPS attempt
Companion Patient #4: Portal Vein Thrombus
on Percutaneous Venogram

Blocked
main portal
vein

Large
splenic
collateral to
left and
right portal
veins
effectively
replacing
main portal
vein
BIDMC PACS
Treatment Modalities for PVT

Treatment dependent on nature of clot



Treat for varices with sclerotherapy or banding
Direct thrombolysis with tPA or streptokinase in acute clot
Long-term anticoagulation


Mechanical thrombectomy
TIPS
 Acute, recent or chronic
 At least three months, longer if underlying hypercoagulable state
 Controversial
►




Contraindicated if complete occlusion or significant cavernous transformation
Can reduce risk of variceal bleeding
Often in conjunction with mechanical thrombectomy or direct thrombolysis
Indications: failed sclerotherapy, ascites, pre-operative
Risk of embolism
Summary
Portal vein thrombus is rare in the general
population but not rare in the cirrhotic
population
 Often asymptomatic

 Consider workup in a patient with variceal
bleed, new ascites or sudden decompensation
in a patient with cirrhosis

Ultrasound and CT are first line for
diagnosis
Acknowledgments





Brian Midkiff, MD
Martin Smith, MD
Salamao Faintuch, MD
Erica Gupta, MD
Sadhna Nandwana, MD
Gillian Lieberman, MD
 Maria Levantakis

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