Unusual Deep Vein Thromboses. Dr. Karl von Kemp Centrum voor Hart- en Vaatziekten UZ Brussel

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1 Unusual Deep Vein Thromboses Dr. Karl von Kemp Centrum voor Hart- en Vaatziekten UZ Brussel

2 Unusual Deep Vein Thromboses Upper extremity deep vein thrombosis Spontaneous Catheter-associated Cerebral venous thrombosis Retinal vein thrombosis 2 Unusual DVT

3 Part 1 : Upper extremity venous thrombosis Spontaneous upper extremity venous thrombosis Catheter-induced upper extremity venous thrombosis

4 Spontaneous upper extremity venous thrombosis Paget-Schroetter syndrome Effort thrombosis Etiology : Extrinsic compression at the thoracic outlet Hypercoagulability : minor role 4 Unusual DVT

5 Anatomical Factors Predisposing to Paget Schroetter Syndrome. 5 Unusual DVT

6 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 6 Unusual DVT

7 Paget Schroetter Syndrome 7 Unusual DVT

8 Spontaneous upper extremity venous thrombosis DIAGNOSIS Only 50 % of clinically suspected UEVT have a positive phlebogram. Digital substraction phlebography Duplex doppler ultrasound Sensitivity %, specificity 93 % Proximal subclavian vein shadowed by clavicle and sternum Useful for screening purposes MRI : sensitivity for non-occlusive thrombi?? CT : insufficient data 8 Unusual DVT

9 Right Subclavian Angiogram Revealing Chronic Nonocclusive Thrombus (Thick Arrow) and Irregularities and Aneurysmal Dilatation (Thin Arrow) in the Subclavian Vein. 9 Unusual DVT

10 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 10 Unusual DVT

11 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. 11 Unusual DVT

12 Suggested management for Paget-Schroetter syndrome (1/3) Positive Catheter-directed thrombolysis Arm venogram Negative Investigate other causes for symptoms Succesful lysis Anticoagulation 6 to 8 weeks Repeat venogram Venous compression Surgical correction No lysis Anticoagulation 3 months No abnormality Discontinue anticoagulation Evaluate for thrombophilia 12 Unusual DVT

13 Suggested management for Paget-Schroetter syndrome (2/3) Surgical correction Anticoagulation 6 to 8 weeks Repeat venogram Venous stenosis Balloon angioplasty No abnormality Discontinue anticoagulation Evaluate for thrombophilia Anticoagulation 6 to 8 weeks Repeat venogram 13 Unusual DVT

14 Suggested management for Paget-Schroetter syndrome (3/3) Repeat venogram after balloon angioplasty Persistent stenosis Collaterals present Collaterals absent Discontinue anticoagulation Evaluate for thrombophilia Consider indefinite anticoagulation or repeat angioplasty 14 Unusual DVT

15 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 for details (TGG recommendations) 15 Unusual DVT

16 When to screen for thrombophilia? At diagnosis and before initiation of therapy. Activation of the coagulation cascade can cause falsepositive and false-negative results. The most reliable time is 1 month after stopping the anticoagulant treatment. 16 Unusual DVT

17 Catheter-induced upper extremity venous thrombosis (UEVT). Superficial thrombosis due to peripheral catheters. Endothelial trauma and vessel wall inflammation. Risks : Embolism Post-thrombotic symptoms 17 Unusual DVT

18 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 18 Unusual DVT

19 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! 19 Unusual DVT

20 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. 20 Unusual DVT

21 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? 21 Unusual DVT

22 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 22 Unusual DVT

23 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. 23 Unusual DVT

24 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 24 Unusual DVT

25 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. 25 Unusual DVT

26 Part 2 : Cerebral venous thrombosis

27 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) 27 Unusual DVT

28 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 disruption Increased Intracranial pressure Cerebral perfusion Parenchymal haemorrhage Vasogenic edema Cerebral blood flow Failure of energetic metabolism Cytotoxic edema 28 Unusual DVT

29 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 29 Unusual DVT

30 Frequency of Thrombosis of the Major Cerebral Veins and Sinuses. 30 Unusual DVT

31 Postmortem Views of Sinus Thrombosis. 31 Unusual DVT

32 MRI of Sinus Thrombosis. Stam J. N Engl J Med 2005;352: Unusual DVT

33 Angiographic Image (Venous Phase) of Sinus Thrombosis. 33 Unusual DVT

34 CT Imaging of Sinus Thrombosis. Stam J. N Engl J Med 2005;352: Unusual DVT

35 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 ; Unusual DVT

36 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. 36 Unusual DVT

37 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. 37 Unusual DVT

38 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. 38 Unusual DVT

39 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. 39 Unusual DVT

40 Part 3 : Retinal vein thrombosis

41 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). 41 Unusual DVT

42 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. 42 Unusual DVT

43 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 43 Unusual DVT

44 Branch Retinal-Vein Occlusion in the Superotemporal Quadrant of the Right Eye 44 Unusual DVT

45 Nonperfused Central Retinal-Vein Occlusion in the Left Eye 45 Unusual DVT

46 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. 46 Unusual DVT

47 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 47 Unusual DVT

48 Treatment of renal vein occlusion. No indication for anticoagulation. Local treatment Laser therapy Intravitreal steroids Intravitreal anti VEGF drugs 48 Unusual DVT

49 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. 49 Unusual DVT

50 Assessment of Cardiovascular Risk in Patients with Retinal-Vein Occlusion 50 Unusual DVT

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