unusual deep vein thromboses - wild apricot · 2014-02-03 · unusual deep vein thromboses dr. karl...
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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
Unusual DVT4 BSIM 3.12.2010
Spontaneous upper extremity venous thrombosis
“Paget-Schroetter syndrome”
“Effort thrombosis”
Etiology :
• Extrinsic compression at the thoracic outlet
• Hypercoagulability : minor role
Unusual DVT5 BSIM 3.12.2010
Anatomical Factors Predisposing toPaget–Schroetter Syndrome.
Unusual DVT6 BSIM 3.12.2010
Spontaneous upper extremity venous thrombosis
“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
Unusual DVT7 BSIM 3.12.2010
Paget–Schroetter Syndrome
Unusual DVT8 BSIM 3.12.2010
Spontaneous upper extremity venous thrombosis
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
Unusual DVT9 BSIM 3.12.2010
Right Subclavian Angiogram Revealing Chronic Nonocclusive Thrombus (Thick Arrow) and Irregularities and Aneurysmal Dilatation (Thin Arrow) in
the Subclavian Vein.
Unusual DVT10 BSIM 3.12.2010
Spontaneous upper extremity venous thrombosis
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
Unusual DVT11 BSIM 3.12.2010
Spontaneous upper extremity venous thrombosis
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.
Unusual DVT12 BSIM 3.12.2010
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
Unusual DVT13 BSIM 3.12.2010
Suggested management for Paget-Schroetter syndrome (2/3)
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
Unusual DVT14 BSIM 3.12.2010
Suggested management for Paget-Schroetter syndrome (3/3)
Repeat venogram
after balloon angioplasty
Persistent stenosis Collaterals absent
Discontinue anticoagulation
Evaluate for thrombophilia
Collaterals present
Consider indefinite anticoagulation
or repeat angioplasty
Unusual DVT15 BSIM 3.12.2010
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)
Unusual DVT16 BSIM 3.12.2010
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.
Unusual DVT17 BSIM 3.12.2010
Catheter-induced upper extremity venous
thrombosis (UEVT).
Superficial thrombosis due to peripheral
catheters.
Endothelial trauma and vessel wall
inflammation.
Risks :
• Embolism
• Post-thrombotic symptoms
Unusual DVT18 BSIM 3.12.2010
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
Unusual DVT19 BSIM 3.12.2010
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 !
Unusual DVT20 BSIM 3.12.2010
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.
Unusual DVT21 BSIM 3.12.2010
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 ?
Unusual DVT22 BSIM 3.12.2010
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
Unusual DVT23 BSIM 3.12.2010
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.
Unusual DVT24 BSIM 3.12.2010
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
Unusual DVT25 BSIM 3.12.2010
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
Unusual DVT27 BSIM 3.12.2010
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)
Unusual DVT28 BSIM 3.12.2010
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
Unusual DVT29 BSIM 3.12.2010
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
Unusual DVT30 BSIM 3.12.2010
Frequency of Thrombosis of the Major Cerebral Veins and Sinuses.
Unusual DVT31 BSIM 3.12.2010
Postmortem Views of Sinus Thrombosis.
Unusual DVT32 BSIM 3.12.2010
MRI of Sinus Thrombosis.
Stam J. N Engl J Med 2005;352:1791-1798.
Unusual DVT33 BSIM 3.12.2010
Angiographic Image (Venous Phase) of Sinus Thrombosis.
Unusual DVT34 BSIM 3.12.2010
CT Imaging of Sinus Thrombosis.
Stam J. N Engl J Med 2005;352:1791-1798.
Unusual DVT35 BSIM 3.12.2010
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
Unusual DVT36 BSIM 3.12.2010
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.
Unusual DVT37 BSIM 3.12.2010
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.
Unusual DVT38 BSIM 3.12.2010
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.
Unusual DVT39 BSIM 3.12.2010
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
Unusual DVT41 BSIM 3.12.2010
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).
Unusual DVT42 BSIM 3.12.2010
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.
Unusual DVT43 BSIM 3.12.2010
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
Unusual DVT44 BSIM 3.12.2010
Branch Retinal-Vein Occlusion in the Superotemporal Quadrantof the Right Eye
Unusual DVT45 BSIM 3.12.2010
Nonperfused Central Retinal-Vein Occlusion in the Left Eye
Unusual DVT46 BSIM 3.12.2010
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.
Unusual DVT47 BSIM 3.12.2010
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
Unusual DVT48 BSIM 3.12.2010
Treatment of renal vein occlusion.
No indication for anticoagulation.
Local treatment
Laser therapy
Intravitreal steroids
Intravitreal anti VEGF drugs
Unusual DVT49 BSIM 3.12.2010
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.
Unusual DVT50 BSIM 3.12.2010
Assessment of Cardiovascular Risk in Patients withRetinal-Vein Occlusion
Unusual DVT51 BSIM 3.12.2010
Unusual DVT52 BSIM 3.12.2010
Unusual DVT53 BSIM 3.12.2010