Unusual Deep Vein Thromboses - Wild Apricot · 2014-02-03 · Unusual Deep Vein Thromboses Dr. Karl von Kemp Centrum voor Hart- en Vaatziekten ... Congestion of venous collaterals

Unusual Deep Vein Thromboses

Dr. Karl von Kemp

Centrum voor Hart- en Vaatziekten

UZ Brussel

Unusual DVT2 BSIM 3.12.2010

Unusual Deep Vein Thromboses

Upper extremity deep vein thrombosis

• Spontaneous

• Catheter-associated

Cerebral venous thrombosis

Retinal vein thrombosis

Part 1 :

Upper extremity venous thrombosis

Spontaneous upper extremity venous thrombosis

Catheter-induced upper extremity venous thrombosis



“Paget-Schroetter syndrome”

“Effort thrombosis”

Etiology :

• Extrinsic compression at the thoracic outlet

• Hypercoagulability : minor role


Anatomical Factors Predisposing toPaget–Schroetter Syndrome.



“Paget-Schroetter syndrome”

“Effort thrombosis”

Etiology :

• Extrinsic compression at the thoracic outlet

• Hypercoagulability : minor role

Clinical presentation:

• Dull aching pain axilla/shoulder

• Swelling of arm or hand, cyanosis, dilated collaterals

• Symptoms increase with exercise, improve with rest and

elevation of the arm

• Preceding strenuous exercise


Paget–Schroetter Syndrome



DIAGNOSIS

Only 50 % of clinically suspected UEVT have a

positive phlebogram.

Digital substraction phlebography

Duplex doppler ultrasound

• Sensitivity 70 – 100 %, specificity 93 %

• Proximal subclavian vein shadowed by clavicle and sternum

• Useful for screening purposes

MRI : sensitivity for non-occlusive thrombi ??

CT : insufficient data


Right Subclavian Angiogram Revealing Chronic Nonocclusive Thrombus (Thick Arrow) and Irregularities and Aneurysmal Dilatation (Thin Arrow) in

the Subclavian Vein.



EVOLUTION

Pulmonary embolism in > 30 %

Conservative management (anticoagulation only) :

< 50 % are asymptomatic after 5 years

Thrombolysis : > 75 % are asymptomatic after 5

years

An invasive approach is favored

• Younger and physically active patients

• Potential for severe physical limitation by chronic

venous insufficiency



MANAGEMENT

No firm data

Patients with recanalisation of the

subclavian vein fare better.

Many patients do well even with persistent

venous occlusion.

A combined approach probably gives the

best long term outcome but is not necessary

for all patients.


Suggested management for Paget-Schroetter syndrome (1/3)

Arm venogram

Catheter-directed

thrombolysis

Positive Negative

Investigate other

causes for symptoms

Succesful lysis No lysis

Anticoagulation

6 to 8 weeks

Anticoagulation

3 months

Repeat venogram No abnormality

Venous compression Discontinue anticoagulation

Surgical correction Evaluate for thrombophilia



Surgical correction

Anticoagulation

6 to 8 weeks

Repeat venogram No abnormality

Discontinue anticoagulation

Evaluate for thrombophilia

Venous stenosis

Balloon angioplasty

Anticoagulation

6 to 8 weeks

Repeat venogram



Repeat venogram

after balloon angioplasty

Persistent stenosis Collaterals absent

Discontinue anticoagulation

Evaluate for thrombophilia

Collaterals present

Consider indefinite anticoagulation

or repeat angioplasty


Evaluation for thrombophilia

Recommendation of the Thrombosis Guidelines Group :

A standard thrombophilia screening is recommended

for a first unexplained DVT at age < 45, in case of

family history of DVT, or in DVT at an unusual location.

Determine antithrombin, protein C, protein S, APCR,

prothrombin G20210A mutation, anticardiolipin

antibodies, lupus anticoagulant, factor VIII and

homocystein.

See www.bsth.be for details (TGG recommendations)

http://www.bsth.be/


When to screen for thrombophilia ?

At diagnosis and before initiation of therapy.

Activation of the coagulation cascade can cause false-

positive and false-negative results.

The most reliable time is 1 month after stopping the

anticoagulant treatment.


Catheter-induced upper extremity venous

thrombosis (UEVT).

Superficial thrombosis due to peripheral

catheters.

Endothelial trauma and vessel wall

inflammation.

Risks :

• Embolism

• Post-thrombotic symptoms


Risk factors for catheter-induced UEVT.

PICC = central catheter

Tip in v. brachiocefalica.

Infection

Hormonal therapy (if + thrombophilia; or IVF)

Chemical irritation (chemotherapy)

Highest incidence in cancer patients

• Up to 60 %

• 75 % are asymptomatic


Clinical presentation of catheter-induced UEVT.

Very often asymptomatic

Inability to draw blood from catheter

Congestion of venous collaterals

Pain/tenderness at insertion site, induration,

erythema : ΔΔ local tumor invasion.

Oedema, increases with exercise.

Pulmonary embolism may be the first

symptom

High index of suspicion requested !


Diagnosis of catheter-induced UEVT.

Duplex ultrasound

• Limitations cfr. Paget-Schroetter syndrome

• Prior to repeat catheterisation

Venography

• Through the catheter

• Conventional venography : on strict indication.


Management of catheter-induced UEVT (1).

More conservative than P.S. syndrome• Older pts, more sedentary, live shorter

• Have more severe problems than venous insufficiency

Prevention of embolisation : treatment = treatment for lower extremity DVT.

Maintain catheter function !

Removal of the catheter ? (does not eliminate the need for anticoagulation).

What in asymptomatic UEVT ?


Management of catheter-induced UEVT (2).

Thrombolysis : not recommended

Instillation of a fibrinolytic agent in an

occluded catheter can be considered (rTPA

or urokinase)

Prophylactic anticoagulation (LMWH) may

reduce thrombosis, does not reduce

occlusive thrombi : not recommended


Pacemaker leads and UEVT

Frequent : 5 – 25 % by venography

Only 1 – 3 % have symptomatic UEVT

ICD = pacemaker

Main problem : replacing electrodes or

upgrading the device (CRT)

Duplex ultrasound should always precede

such a procedure.

Anticoagulation (for cardiac indication)

seems to protect from UEVT.


Pacemaker leads and UEVT

Predictors of UEVT :

Multiple leads vs single lead

Hormone therapy

History of DVT

Insertion of a temporary PM preceding the

definitive PM

Presence of a PM preceding insertion of an ICD

Dual coil leads


Management of PM-lead-associated UEVT

Asymptomatic pts are usually not treated.

Anticoagulation is the cornerstone of therapy

in symptomatic patients.

Thrombolysis improves early patency but

does not reduce late post-thrombotic

syndrome.

Removal of a non-functional lead before

inserting a new lead.

Part 2 :

Cerebral venous thrombosis


Cerebral Venous Thrombosis

Less common type of stroke

Increased awareness and increased

availability of MRI leads to increased

diagnosis.

International Study on Cerebral Vein and

Dural Sinus Thrombosis (ISCVT) (Stroke 2009)

Younger patients (mean : 39 y).

Female predominance (pregnancy –

puerperium – contraception)


Cerebral Venous Thrombosis : Pathogenesis

Obstruction of dural sinus

Increased venous pressure

↑ Venular and

Capillary pressure

Impairment of CSF

absorption

↓ Capillary

perfusion

Venous and

capillary rupture

Blood-brain

barrier disruptionIncreased

Intracranial pressure

↓ Cerebral

perfusion

Parenchymal

haemorrhage

Vasogenic

edema

↓ Cerebral

blood flow

Failure of energetic

metabolism

Cytotoxic

edema


Cerebral Venous Thrombosis : Clinical Aspects

Highly variable clinical presentation :

Intracranial hypertension syndrome : headache ± vomiting (89 %), papilledema, visual problems

Focal syndrome : focal deficits, seizures.

Encephalopathy : multifocal signs, mental status changes, stupor, coma


Frequency of Thrombosis of the Major Cerebral Veins and Sinuses.


Postmortem Views of Sinus Thrombosis.


MRI of Sinus Thrombosis.

Stam J. N Engl J Med 2005;352:1791-1798.


Angiographic Image (Venous Phase) of Sinus Thrombosis.


CT Imaging of Sinus Thrombosis.

Stam J. N Engl J Med 2005;352:1791-1798.


Causes of and Risk Factors Associated with

Cerebral Venous Sinus Thrombosis

Genetic prothrombotic conditions

Antithrombin deficiency

Protein C and protein S deficiency

Factor V Leiden mutation

Prothrombin G20210A mutation

Hyperhomocysteinemia caused by gene mutation in MTHF reductase

Acquired prothrombotic states

Nephrotic syndrome

Antiphospholipid antibodies

Homocysteinemia

Pregnancy

Puerperium

Infections

Otitis, mastoiditis, sinusitis

Meningitis

Systemic infectious disease

Inflammatory disease

SLE

Wegener’s granulomatosis

Sarcoidosis

Inflammatory bowel disease

Behçet’s syndrome

Hematologic conditions

Polycythemia, primary and secondary

Thrombocythemia

Leukemia

Anemia, including paroxysmal nocturnal hemoglobinuria

Drugs

Oral contraceptives

Asparaginase

Mechanical causes, trauma

Head injury

Injury to sinuses or jugular vein, jugular catheterisation

Neurosurgical procedures

Lumbar puncture

Miscellaneous

Dehydration, especially in children

Cancer

Stam, J. N Engl J Med 2005; 352 ; 1791


Detection of thrombophilia in CVT.

• A cause of CVT will be found in 65 to 85 % of patients.

• There is generally an association of a genetic

thrombophilia with a precipitating factor : oral

contraception, pregnancy of puerperium, cranial

trauma, lumbar puncture.

• In patients over 40 without identified etiology, search for

malignancy.


Prognosis of CVT

5 % die in the acute phase

15 % overall death or dependency

Low risk of recurrence

Predictors of poor long-term prognosis :• Central nervous system infection

• Malignancy

• Thrombosis of the deep cerebral veins

• Hemorrhage on CT or MRI

• Glasgow coma scale < 9 on admission

• Mental state abnormality

• Age > 37 years

• Male gender.


Treatment of CVT (1)

Anticoagulation (LMWH followed by vit K

antagonists) is recommended.

Anticoagulation appears safe even in the

presence of intracerebral or subarachnoid

hemorrhage.

Endovascular thrombolysis could be performed

at experienced centers in patients with poor

prognosis who worsen despite adequate

anticoagulation.


Treatment for CVT (2)

Anticoagulant treatment will be administered

for 6 to 12 months.

Chronic anticoagulation is recommended for

patients with prothrombotic conditions,

including the antiphospholipid syndrome.

Oral contraception should be stopped.

CVT is not a contra-indication for

subsequent pregnancy.

Part 3 :

Retinal vein thrombosis


Retinal vein occlusion

is a frequent cause of loss of vision in the elderly;

is the second most frequent vascular disease of the

retina (after diabetes retinopathy).

Stasis and thrombosis in the retinal vein are

caused by atherosclerotic or inflammatory damage

in the adjacent artery.

Loss of vision is mainly due to macular edema (and

neovascularisation, vitreous hemorrhage, retinal detachment or

neovascular glaucoma).


Retinal vein occlusion

Is weakly associated with all thrombophilic

states.

Arterial hypertension is the strongest risk

factor.

There is a weaker association with diabetes,

hyperlipidemia, smoking and renal disease.


Types of retinal vein occlusion

Branch retinal vein occlusion (at an AV

intersection)

Central retinal vein occlusion (at lamina

cribrosa sclerae)

Branch RVO is 4 x more common than

central RVO and has a better prognosis.

Perfused or non perfused RVO


Branch Retinal-Vein Occlusion in the Superotemporal Quadrantof the Right Eye


Nonperfused Central Retinal-Vein Occlusion in the Left Eye


Diagnostic workup of RVO

Ophtalmologic assessment : fundoscopy,

fluorescein angiography, OCT…

Systemic workup :

1 : Check for cardiovascular risk factors

• No evidence that treatment of AHT or other risk

factor influences visual prognosis

• RVO should be considered end-organ damage by

AHT, implying more aggressive management.


Systemic workup of RVO

2. Check for cardiovascular disease (stroke,

PAD, coronary artery disease).

3. Routine laboratory testing : glycemia,

HbA1c, renal function, lipid levels, CBC

(hyperviscosity syndrome ?).

4. Thrombophilia testing

In younger patients (< 50)

Notion of preceding thrombotic disorders

Bilateral RVO


Treatment of renal vein occlusion.

No indication for anticoagulation.

Local treatment

Laser therapy

Intravitreal steroids

Intravitreal anti VEGF drugs


Conclusion.

The same basic process (venous

thrombosis) can cause damage by a variety

of mechanisms, depending on the site

involved.

Treatment for the same basic process can

vary from very aggresive to strict abstinence

of interfering with the thrombotic process.


Assessment of Cardiovascular Risk in Patients withRetinal-Vein Occlusion




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Unusual Deep Vein Thromboses - Wild Apricot · 2014-02-03 · Unusual Deep Vein Thromboses Dr. Karl von Kemp Centrum voor Hart- en Vaatziekten ... Congestion of venous collaterals