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eMedicine - Deep Venous Thrombosis, Upper Extremity : Article by

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Author: Craig Greben, MD, Assistant Professor of Radiology, New York University School of Medicine, Chief, Division of Vascular and Interventional Radiology, North Shore University Hospital

Craig Greben is a member of the following medical societies: Society of Cardiovascular and Interventional Radiology

Coauthor(s): James Naidich, MD, Residency Director, North Shore University Hospital; Professor, Department of Radiology, New York University School of Medicine; Hearns W Charles, MD, Assistant Professor of Radiology, New York University School of Medicine; Consulting Staff, Division of Vascular and Interventional Radiology, New York University Medical Center; Jason J Naidich, MD, Assistant Professor of Radiology, New York University School of Medicine; Attending Physician, Division of Vascular and Interventional Radiology, North Shore University Hospital

Editors: Anthony Watkinson, MD, Professor of Interventional Radiology, The Peninsula Medical School; Consultant and Senior Lecturer, Department of Radiology, The Royal Devon and Exeter Hospital, UK; Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand; Douglas M Coldwell, MD, PhD, Professor of Interventional Radiology, Department of Radiology, Professor of Interventional Radiology, University of Texas Southwestern Medical Center; Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute; Kyung J Cho, MD, FACR, William Martel Professor of Radiology, Fellowship Program Director, Department of Radiology, Division of Interventional Radiology, University of Michigan Medical School

Author and Editor Disclosure

Synonyms and related keywords: DVT, effort thrombosis, effort-induced thrombosis, secondary thrombosis, exertional thrombosis, axillary-subclavian vein thrombosis, Paget-von Schrötter syndrome

Background

The 2 forms of upper-extremity deep venous thrombosis (DVT) are (1) effort-induced thrombosis (Paget-von Schrötter syndrome) and (2) secondary thrombosis.

Effort induced thrombosis, or Paget-von Schrötter syndrome, accounts for 25% of cases. Paget in England and von Schrötter in Germany independently described effort thrombosis more than 100 years ago. In this primary form of the disease, an underlying chronic venous compressive abnormality caused by the musculoskeletal structures in the costoclavicular space is present at the thoracic inlet and/or outlet.

In 75% of patients with secondary thrombosis, hypercoagulability and/or indwelling central venous catheters are important contributing factors. In fact, with the advent of central venous catheters, upper-extremity and brachiocephalic venous thrombosis has become a more common problem.

Pathophysiology

Factors contributing to upper extremity DVT are controversial, but the disease may be explained with the Virchow triad: stasis, hypercoagulability, and intimal damage. Intimal injury is likely to be more important than the other factors in the etiology of upper-extremity DVT.

This mechanism is particularly true of thrombosis of the axillosubclavian vein, compared with that of thrombosis in other large veins. Because of its relatively fixed position in the thoracic inlet or outlet, the axillosubclavian vein is exposed to repeated trauma with arm movement. This repetitive trauma and compression in the fixed costoclavicular space leads to intimal hyperplasia and venous stenosis.

Etiologies of secondary venous thrombosis include the following:

  • Use of central venous catheters

  • Use of permanent cardiac pacers

  • Hypercoagulable states

  • Reduced S and C protein levels

  • Mediastinal tumors and nodes

  • Mediastinal radiation and surgery

  • Trauma (eg, fractured clavicle)

Frequency

United States

Upper-extremity DVT now accounts for about 8% of all cases of DVT; this rate is increased from only 2% of all cases prior to 1966. This increased frequency is at least partly due to the increased use of central venous catheters.

Mortality/Morbidity

The main complications of upper-extremity DVT are pulmonary embolus and superior vena cava syndrome. If a venous access device is in place and if venous thrombosis is diagnosed, the access device may need to be removed. This removal can seriously affect the care of critically ill patients in whom venous access is difficult to secure.

  • The prevalence of both symptomatic and asymptomatic pulmonary embolism in patients with upper-extremity DVT is high (10-30%) and similar to the frequency observed in lower-extremity DVT. Patients with catheter-associated upper-extremity DVT have a higher risk for pulmonary embolism than that of other patients. In patients who cannot receive thrombolytic or anticoagulation therapy, superior vena caval filtration should be considered.


  • Horratas et al found that 65% of catheter-induced thromboses were left sided.1 Further, in a study of 100 patients who underwent central venous catheterizations for dialysis, Schillinger et al found that about 42% of their subclavian veins and only 10% of the jugular veins developed stenoses.2 Because of this observation, the right internal jugular vein should be the primary site of access whenever possible because it has the lowest risk of stenosis and thrombosis.

Sex

Effort-induced thrombosis occurs most frequently in young men younger than 45 years; this disease particularly affects body builders and other individuals after exercise.

Anatomy

Anatomic causes that contribute to DVT include the following: presence of a cervical rib, posttraumatic deformity of surrounding bony structures, and anomalous musculofascial bands.

The most common points of venous entrapment are between the following: the anterior and medial heads of the scalenus muscle of the first rib (scalenus anticus syndrome); the subclavius muscle, the clavicle, and the first rib; an anomalous cervical rib and the scalenus muscle.

Clinical Details

DVT should be suspected in the presence of pain, swelling, and functional impairment of the upper extremity. The neck and shoulder may be painful and swollen. The skin may be mottled, and the superficial collateral veins may be dilated.

Thoracic outlet syndrome may occur with arterial (least common), venous, or brachial-plexus symptoms (most common).

Preferred Examination

Upper-extremity venous thrombosis can be diagnosed with color flow duplex imaging, with a sensitivity of 78-100% and a specificity of 90-100%.

The central veins cannot always be accurately imaged sonographically; therefore, contrast-enhanced venography remains an important diagnostic tool. In the assessment of effort-induced thrombosis, venographic views include abduction, external rotation, and extension views.



Findings

Plain radiographic findings of a clavicle or first rib fracture or presence of a cervical rib can increase the suspicion of thoracic outlet syndrome and venous thrombosis.



Findings

CT is readily available and widely used.

Upper-extremity and central venous thrombosis is often incidentally diagnosed on contrast-enhanced CT scans of the chest. The thrombus is hypoattenuating compared with the hyperattenuating vein. This cross-sectional imaging modality provides excellent information about soft tissue structures (eg, tumor, lymphadenopathy) surrounding the vein that may account for the thrombosis.

Degree of Confidence

With new 3-dimensional (3D) reconstruction software packages, the image quality is good, and the findings are diagnostic.



Findings

The strength of magnetic resonance venography is in the evaluation of the central veins of the chest; the subclavian vein; the brachiocephalic vein; and the superior vena cava, an area poorly visualized with ultrasonography. Thrombosis is diagnosed as a filling defect in the vessel.



Findings

Ultrasonography is the imaging modality of choice. Real-time ultrasonography and color flow Doppler imaging are rapid, noninvasive means for the diagnosis of DVT. The lack of full compressibility, the absence of color flow signal and augmentation, and visualization of thrombus are used to make the diagnosis.



Findings

Venography with iodinated contrast enhancement is more costly and invasive than ultrasonography, but it remains the criterion standard in the evaluation of DVT. Patients with effort-induced thrombosis should undergo bilateral upper-extremity venography with provocative maneuvers.

For patients with renal insufficiency or allergy to contrast material, carbon dioxide and gadolinium-based materials are alternative contrast agents for venography. These agents can be used for diagnostic venography as well as for guiding catheter-directed thrombolysis.

Gadolinium-based contrast agents (gadopentetate dimeglumine [Magnevist], gadobenate dimeglumine [MultiHance], gadodiamide [Omniscan], gadoversetamide [OptiMARK], gadoteridol [ProHance]) have recently been linked to the development of nephrogenic systemic fibrosis (NSF) or nephrogenic fibrosing dermopathy (NFD). For more information, see the eMedicine topic Nephrogenic Fibrosing Dermopathy. The disease has occurred in patients with moderate to end-stage renal disease after being given a gadolinium-based contrast agent to enhance MRI or MRA scans. As of late December 2006, the FDA had received reports of 90 such cases. Worldwide, over 200 cases have been reported, according to the FDA. NSF/NFD is a debilitating and sometimes fatal disease. Characteristics include red or dark patches on the skin; burning, itching, swelling, hardening, and tightening of the skin; yellow spots on the whites of the eyes; joint stiffness with trouble movingor straightening the arms, hands, legs, or feet; pain deep in the hip bones or ribs; and muscle weakness. For more information, see the FDA Public Health Advisory or Medscape.

Patients are positioned with their arm in the neutral, extended, and hyperabducted positions. The diagnosis is made when an intraluminal filling defect is seen or when a deep venous structure does not fill and collateral veins are visualized.

False Positives/Negatives

The compressive features seen with provocative maneuvers during contrast-enhanced venography can occur in asymptomatic patients; therefore, not all patients with venous compression have venous thrombosis.



If demonstrated, the thrombus should be treated in a staged, multidisciplinary fashion. The treatment of Paget-von Schrötter syndrome consists of thrombolytic therapy; anticoagulation; surgical decompression; and, occasionally, angioplasty and/or stent placement. Secondary axillosubclavian vein thrombosis can be treated with conservative, surgical, or catheter-directed thrombolytic therapy. Lytic therapy is highly effective when started early. Large strictures of a central vein can be treated with metallic stents.

Interventions for primary thrombosis of the axillosubclavian vein

In the Machleder algorithm, the basilic vein is the preferred access site.

Catheter-directed thrombolysis may be performed. Current mechanical thrombectomy devices can potentially facilitate clot removal and thus shorten the thrombolytic infusion time and reduce the dose.

Anticoagulation may be administered for 6-12 weeks.

Thoracic-outlet decompression by means of transaxillary or transthoracic resection of the first rib may be indicated if significant disability or vein abnormality persists.

Follow-up venography and angioplasty may be performed to correct residual venous stenosis after decompression surgery. The use of vascular stents in this location is relatively contraindicated because long-term patency rates are low.

Treatment options for secondary thrombosis of the axillosubclavian vein

Traditional conservative therapies include catheter removal, bed rest, heat application, limb elevation, and anticoagulation. Symptomatic improvement after conservative therapy is the result of venous collateral recruitment and the prevention of clot propagation.

Invasive surgical therapy includes thrombectomy.

Catheter-directed thrombolysis may be appropriate. The median basilic vein is easily accessed with sonographic or venographic fluoroscopic guidance. A vascular sheath is placed, and an upper-extremity venogram is obtained. The extent of the clot is documented. A guidewire is passed through the clotted venous segment (wire traversal test), and a multi–side-hole infusion catheter of appropriate length is embedded in the clotted segment. Thrombolytic therapy with the agent of choice is begun. Most thrombosed veins are recanalized in 24 hours (some as long as 48-72 h). If the underlying vein is normal after completion thrombolysis, short-term anticoagulation is warranted. If the underlying vein is abnormal (eg, with irregularities or stenoses), 10-12 weeks of anticoagulation is advocated.

Thrombolytic agents

Three thrombolytic agents are currently available for use in the United States: urokinase (Abbokinase, Abbott Laboratories), alteplase (Activase, Genentech), and reteplase (Retavase, Centocor).

Absolute contraindications for thrombolytic therapy are the following: (1) active internal hemorrhage; (2) recent gastrointestinal hemorrhage; (3) central nervous system tumor, aneurysm, or arteriovenous malformation; and (4) recent cerebrovascular accident. Relative contraindications include the following: (1) recent major surgery, biopsy, or trauma; (2) postpartum (<10 d) status; (3) uncontrolled hypertension; (4) hemorrhagic retinopathy; and (5) left-sided intracardiac thrombus.

Some patients with upper-extremity venous thrombosis should be considered for superior vena caval filtration. Indications for placement of a superior vena caval filter include the following: (1) contraindication to anticoagulation; (2) recurrent pulmonary embolism despite adequate anticoagulation; (3) complications of anticoagulation; (4) chronic, recurrent pulmonary embolism associated with pulmonary hypertension; and (5) massive pulmonary embolism.



Media file 1:  This contrast-enhanced study was obtained through a Mediport placed through the chest wall through the internal jugular vein to facilitate chemotherapy. A thrombus has propagated peripherally from the tip of the catheter in the superior vena cava into both subclavian veins.
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Media file 2:  Image in the same patient as in Image 1 demonstrates thrombus in the left subclavian and axillary veins.
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Media type:  Image

Media file 3:  Image in the same patient as in Images 1-2 after a 16-hour catheter-directed thrombolytic infusion of tissue-type plasminogen activator (tPA) into the left axillosubclavian vein. Interval clot dissolution has occurred, and central flow in the venous system has been restored.
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Media type:  Image

Media file 4:  Thoracic outlet compression syndrome secondary to hypertrophied musculofascial bands. Venogram of the right upper extremity demonstrates a hemodynamically significant, notchlike stenotic defect in the subclavian vein with the patient's arm in the neutral position. Note filling of venous collaterals, even with the arm in the neutral position.
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Media type:  Image

Media file 5:  Image in the same patient as in Images 4-9 obtained with the right arm abducted. Note the occlusion of the subclavian vein and marked filling of venous collaterals.
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Media type:  Image

Media file 6:  Image shows thoracic outlet compression syndrome after first-rib resection on the right side (same patient as in Images 4-9).
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Media type:  Image

Media file 7:  Thoracic outlet compression syndrome in the same patient as in Images 4-9. Percutaneous transluminal angioplasty (PTA) was performed with a 10-mm balloon catheter to treat the venous stenosis, which persisted after transaxillary resection of the first rib. Note the significant waist in the balloon before it is completely inflated.
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Media file 8:  Same patient as in Images 4-9. After full inflation, the waist in the balloon catheter is eliminated.
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Media file 9:  Same patient as in Images 4-9. Postangioplasty contrast-enhanced venogram demonstrates successful treatment of the residual venous stenosis after thoracic outlet compression syndrome (TOCS) surgery to decompress the vascular space.
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Media type:  Image

Media file 10:  Superior vena cava syndrome in a patient with lung cancer. CT scan demonstrates a hypoattenuating thrombus that fills the superior vena cava. The patient was treated with anticoagulation alone.
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Media type:  CT

Media file 11:  Contrast-enhanced venogram shows a critical stenosis of the left subclavian vein in a patient with end-stage renal disease and an arteriovenous fistula in the left arm. The access site in the fistula bleeds excessively after dialysis.
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Media type:  X-RAY

Media file 12:  Critical stenosis in the left subclavian vein is treated with a Wallstent and 12-mm balloon angioplasty (same patient as in Images 11 and 13). The stenosis had recurred 3 times after balloon angioplasty alone.
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Media file 13:  Contrast-enhanced venogram demonstrates successful treatment of the critical stenosis in the left subclavian vein Good flow has been reestablished.
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Media type:  X-RAY

Media file 14:  CT scan in a patient with non-Hodgkin lymphoma demonstrates a soft-tissue mass surrounding a hyperintense right brachiocephalic vein.
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Media file 15:  Contrast-enhanced venogram shows circumferential narrowing of the right brachiocephalic vein in a patient with non-Hodgkin lymphoma.
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Deep Venous Thrombosis, Upper Extremity excerpt

Article Last Updated: Apr 5, 2007