Acute Upper Extremity Deep Venous Thrombosis

Vineel Kurli, David S. Pryluck, Charan K. Singh and Timothy W.I. Clark

Upper extremity deep venous thrombosis (UEDVT) most commonly refers to thrombosis involving the axillary, subclavian, or brachiocephalic veins.¹ With an annual incidence of approximately 0.4 to 1 case per 10,000 people, UEDVT has become increasingly more common with higher utilization of long-term central venous access catheters and implanted cardiac devices.^2–5 Current understanding of UEDVT has evolved with recognition of significant potential morbidity and mortality, including thrombophlebitis, postthrombotic syndrome, pulmonary embolism, and rare but devastating phlegmasia cerulea dolens and venous gangrene.^6,7 Pulmonary embolism complicates UEDVT in approximately 5% of cases.⁸ Prompt diagnosis and management of UEDVT is therefore important to reduce the short- and long-term consequences of this condition.

To date, there are no prospective randomized controlled trials evaluating treatment of UEDVT. Despite the emergence of different therapeutic strategies, optimal management of acute UEDVT remains controversial and challenging. Catheter-directed thrombolysis after clot debulking with mechanical thrombectomy is becoming more widely adopted as the initial form of therapy, followed by surgery in primary UEDVT and balloon angioplasty in selected secondary causes of UEDVT.

Indications

Indications for endovascular therapy of UEDVT are guided by the etiology of the thrombosis. UEDVT can be broadly classified into two groups: primary and secondary.

Primary Uedvt: Paget-Schroetter Syndrome

Also referred to as effort-related thrombosis or thoracic outlet syndrome, this condition more commonly affects otherwise healthy, young, and highly functional individuals, with a male-to-female ratio of approximately 2 : 1.² The inciting event can be any form of excessive or repetitive arm activity, such as weight-lifting, rowing, and swimming,² in the presence of one or more compressive elements at the costoclavicular junction. These include hypertrophied or broad insertion of the subclavius and anterior scalene muscles or tendons, cervical ribs, long cervical transverse processes, musculofascial bands, and clavicular or first rib anomalies.^1,9,10 The underlying pathophysiology is thought to be chronic venous stasis secondary to anatomic compression, followed by an acute stress that produces venous intimal damage and inflammation with associated thrombosis.¹¹ Repeated trauma to the vessel can also result in dense perivascular fibrous scar tissue formation, causing persistent compression.¹ Untreated or inadequately treated patients may suffer from chronic disability due to symptoms of venous obstruction, leading to significant loss of occupational productivity and quality of life.¹²

Conservative treatment involving arm rest, elevation, and anticoagulation is associated with high failure rates and significant long-term disability. Development of intermittent venous distention was reported in 18% of patients in one series, with late symptoms of swelling, pain, and superficial thrombophlebitis in 68% of patients.¹³ Treatment is aimed at (1) restoring venous patency, (2) relieving intrinsic venous stenosis, and (3) surgical decompression of the thoracic outlet.¹⁴ Two multidisciplinary strategies are supported in the literature to treat effort-related UEDVT. The first is early thrombolysis followed by surgery and further endovascular interventions, including angioplasty or stenting if residual stenosis develops after surgery. The second strategy is early thrombolysis followed by a short period of anticoagulation and surgical treatment only of symptomatic patients.¹⁵

Secondary UEDVT

Secondary UEDVT is far more common and accounts for approximately 80% of all UEDVT.² It is associated with multiple underlying risk factors, the most common being central venous catheterization and malignancy. Approximately 60% to 70% of cases have a history of central venous catheter placement.^16,17 Endothelial injury at the insertion site or site of chronic vessel wall contact, turbulent or slow flow induced by the catheter, larger catheter sizes, longer duration of use, and a catheter tip position that is too proximal all predispose to thrombosis.¹¹

Other risk factors for secondary UEDVT include sepsis, immobility, previous history of venous thrombosis, liver and renal failure, external trauma, coagulation disorders, vasculitis, and treatment with oral contraceptives.6,18 Screening for hypercoagulable states (e.g., factor V Leiden, prothrombin gene mutation, homocystinemia, antiphospholipid antibody syndrome) should be considered in patients presenting with idiopathic UEDVT, recurrent DVT, or a family history of DVT without evidence of thoracic outlet compression.

Clinical Presentation

Patients with true effort-induced thrombosis will almost always be persistently symptomatic.¹⁹ Clinical presentation includes arm swelling, pain and tenderness, vague shoulder or neck discomfort, numbness, or functional impairment of the affected arm.^6,7 Edema is the most common physical sign. Other signs include erythema, tenderness, warmth of the affected arm, distended collateral veins over the shoulder girdle, and limited range of movement.^7,18 A history of repeated exercise or strenuous effort can be elicited from the patients with effort-related thrombosis, with the dominant arm being more commonly affected. If thoracic outlet syndrome is suspected, the supraclavicular fossa should be palpated for brachial plexus tenderness, the hand and arm inspected for atrophy, and provocative tests such as the Adson and Wright maneuvers performed. To perform the Adson test, the examiner extends the patient’s arm on the affected side while the patient extends the neck and rotates the head toward the same side. Weakening of the radial pulse with deep inspiration suggests compression of the subclavian artery. The Wright maneuver tests for reproduction of symptoms and weakening of the radial pulse when the patient’s shoulder is abducted and the humerus externally rotated.

Imaging Techniques

Confirming the clinical diagnosis of UEDVT requires imaging of the axillary/subclavian venous system. Doppler ultrasonography is a rapid, accurate, and noninvasive method of evaluating venous disease in the upper extremities and is currently the screening technique of choice for UEDVT.²⁰ Sensitivity ranges from 78% to 100%, and specificity ranges from 82% to 100%.²⁰ Limitations of Doppler ultrasonography include difficulty visualizing the medial segment of the subclavian vein, the brachiocephalic vein, and the confluence with the superior vena cava (SVC) posterior to the clavicle and sternum, potentially resulting in a false-negative study.^20,21

Venography remains the gold standard for diagnosis of UEDVT and enables diagnosis and initial treatment in a single setting. Although power injectors can be used, hand injections are commonly used and may reduce the risk of contrast agent extravasation.²¹ In patients with suspected effort thrombosis, evaluation should also include positional venography, with the arm held in an abducted and externally rotated position to assess the presence and severity of subclavian venous compression.¹¹ It is important to recognize that venous compression in this region can be a seen in normal asymptomatic individuals, and aggressive therapies such as surgical decompression should only be considered in the presence of other symptoms and findings.¹¹

Magnetic resonance venography (MRV) is accurate in evaluating the central thoracic veins, including the brachiocephalic veins and SVC. It has been shown to correlate well with conventional contrast venography and to provide better evaluation of central collateral veins.1,22 More recently, in a prospective study that assessed time-of-flight and gadolinium-enhanced magnetic resonance imaging (MRI) in patients with suspected UEDVT, the calculated sensitivity and specificity were 71% and 89% for time-of-flight and 50% and 80% for gadolinium-enhanced MRI.²³ The utility of MRV in the workup and management of patients with UEDVT is yet to be defined. Similarly, computed tomographic venography continues to have an evolving role in the assessment of UEDVT.²⁴