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Primary (spontaneous) upper extremity deep vein thrombosis

Primary (spontaneous) upper extremity deep vein thrombosis
Literature review current through: Jan 2024.
This topic last updated: May 31, 2022.

INTRODUCTION — Primary "spontaneous" upper extremity deep vein thrombosis is rare and is defined as thrombosis of the deep veins draining the upper extremity due to anatomic abnormalities of the thoracic outlet causing axillosubclavian compression and subsequent thrombosis. The syndrome is appropriately termed venous thoracic outlet syndrome but is also referred to as Paget-Schroetter syndrome, and alternatively as "effort" thrombosis [1]. It typically presents in young, otherwise healthy individuals as sudden, severe upper extremity pain and swelling following vigorous upper extremity activity. An aggressive treatment approach that includes anticoagulation, catheter-directed thrombolysis, and thoracic outlet decompression is aimed at relieving acute symptoms and minimizing complications, including recurrent thromboembolism and post-thrombotic syndrome. The presentation and diagnosis of primary upper extremity deep vein thrombosis in children is similar to that of adults [2].

The epidemiology, risk factors, pathophysiology, clinical features, diagnosis, and treatment of primary (spontaneous) upper extremity venous thrombosis will be reviewed here. Catheter-induced upper extremity venous thrombosis and lower extremity deep vein thrombosis are discussed elsewhere. (See "Catheter-related upper extremity venous thrombosis in adults" and "Clinical presentation and diagnosis of the nonpregnant adult with suspected deep vein thrombosis of the lower extremity".)

UPPER EXTREMITY ANATOMY — The upper extremity veins are divided into the superficial and deep venous systems (figure 1).

Superficial veins — The main superficial veins of the upper extremity include the cephalic, basilic, median cubital, and accessory cephalic veins (figure 1). The basilic vein is a common access site for performing digital subtraction venography.

Deep veins — The deep veins of the upper extremity include the paired ulnar, radial, and interosseous veins in the forearm; paired brachial veins of the upper arm; and axillary vein. The axillary vein becomes the subclavian vein at the lower border of the teres major muscle (figure 2).

Thoracic outlet anatomy — The thoracic outlet is bounded by the bony structures of the spinal column, first ribs, and sternum (figure 3A). Compression of the venous structures that traverse the thoracic outlet occurs in two distinct spaces: the scalene triangle and the costoclavicular space.

Scalene triangle – The anterior border of the scalene triangle is formed by the anterior scalene muscle, which originates from the transverse processes of the third through sixth cervical vertebrae (C3-C6) and inserts on the inner borders and superior surfaces of the first rib. The posterior wall of the scalene triangle is formed by the middle scalene muscle, which arises from the transverse processes of the second through seventh cervical vertebrae (C2-C7) and inserts broadly onto the posterior aspects of the first rib. The superior border of the first rib forms the base of the scalene triangle. The trunks of the brachial plexus and the subclavian artery pass between the anterior and middle scalene muscles, while the subclavian vein courses anteromedial to the scalene triangle (figure 3B).

Costoclavicular space – The costoclavicular space comprises the area between the first rib and the clavicle. The brachial plexus, subclavian artery, and subclavian vein pass through this space. The subclavian vein is most likely to be compressed at this site.

PATHOGENESIS AND TERMINOLOGIES — Primary upper extremity deep vein thrombosis is defined as thrombosis of the deep veins draining the upper extremity due to an underlying anatomic anomaly at the thoracic outlet causing compression or repetitive injury to the underlying axillosubclavian vein [3-6]. Primary upper extremity deep vein thrombosis is a manifestation of venous thoracic outlet syndrome. (See "Overview of thoracic outlet syndromes", section on 'Venous TOS'.)

Thrombosis of the veins draining the upper extremity was originally postulated to be the cause of acute arm pain and swelling by Paget [7], and later Von Schroetter related the clinical syndrome specifically to the axillary and subclavian veins [8]. This clinical entity was referred to as Paget-Schroetter syndrome [9]. In the mid-20th century, the term "effort" thrombosis was coined [10], due to the fact that the syndrome often occurred in physically active individuals after unusually strenuous use of the arm and shoulder [11-13]. The term "spontaneous" upper extremity venous thrombosis has also been used, highlighting the often dramatic presentation in an otherwise healthy, young individual. For the purposes of our discussion, we will refer to the syndrome as primary upper extremity deep vein thrombosis to distinguish it from secondary causes, which are associated with inciting factors such as indwelling catheters or prothrombotic states. (See "Catheter-related upper extremity venous thrombosis in adults".)

Anatomic abnormalities of the thoracic outlet that result in compression of the vein can be congenital or acquired. Congenital anomalies consist of cervical ribs, supernumerary muscles, abnormal tendon insertions, or abnormal muscular or tendinous bands [14,15]. Acquired abnormalities include bony overgrowth due to bony fracture (eg, clavicle, first rib) [16-19], or hypertrophy of anterior scalene muscle or subclavius muscles, often related to repetitive lifting. The abnormalities of the thoracic outlet are often bilateral, and bilateral primary upper extremity deep vein thrombosis has been reported [20,21]. Anatomic abnormalities often narrow the costoclavicular space, predisposing the vein to compression between the first rib and muscle or tendon (figure 3A-B), or between anomalous tendon insertions. Less commonly, compression of the vein between the clavicle and a cervical rib can occur. Partial occlusion of the vein by a congenital web has also been reported [14,15]. (See 'Thoracic outlet anatomy' above and "Overview of thoracic central venous obstruction", section on 'Central venous anatomy'.)

Under some circumstances, it appears that an anatomic abnormality is not necessary to produce injury to the vein. Extremes in range of motion of the upper extremity can lead to movement of the clavicle relative to the first rib sufficient to cause venous compression. Repetitive overhead arm movements or hyperabduction and external rotation of the shoulder are most often implicated [22-24]. Repetitive injury causes perivenous fibrosis, which eventually leads to thrombosis. It is important to recognize that the patient often presents with acute onset of symptoms related to the thrombosis, but the underlining problem may be a chronic repetitive injury that had narrowed the vein.

EPIDEMIOLOGY AND RISK FACTORS — Upper extremity deep vein thrombosis (all causes) represents 1 to 4 percent of all cases of deep vein thrombosis [25]. Primary upper extremity deep vein thrombosis is rare with an estimated annual incidence of 1 to 2 cases per 100,000 population [11,25]. The majority of cases of upper extremity deep vein thrombosis are secondary and related to central venous cannulation (eg, central line, pacemaker) or prothrombotic states (eg, thrombophilia, malignancy) [1,11,26,27]. Primary upper extremity deep vein thrombosis has also been reported in children, typically adolescents, with clinical presentations that are similar to those of adults [28]. (See "Catheter-related upper extremity venous thrombosis in adults".)

Between 60 and 80 percent of patients with primary upper extremity deep vein thrombosis report a history of exercise or strenuous activity often involving the dominant upper extremity prior to the onset of symptoms. Strenuous activities include weight-lifting, rowing, or activities involving repetitive overhead arm movements, particularly hyperabduction, such as pitching [1,29,30]. The average age at presentation is in the early thirties, and the male to female ratio is 2:1 [1]. A predominance of right-handed individuals may explain why the right axillosubclavian vein is more commonly affected. (See 'Pathogenesis and terminologies' above.)

Risk factors — Risk factors for primary upper extremity deep vein thrombosis include the following [1,2,11,26,27,29-33]:

Younger age, including children, typically adolescents

Athletic muscular male

Strenuous upper extremity activity

Repetitive overarm hyperabduction

Anatomic abnormalities of the thoracic outlet (congenital, acquired)

Thrombophilia

There is a high prevalence of an underlying thrombophilic state in primary upper extremity deep venous thrombosis, particularly in the pediatric population [32]. In two small case series of children with primary upper extremity deep venous thrombosis, the incidences of thrombophilia workup were 25 and 50 percent [2,34]. (See 'Screening for hypercoagulability' below.)

CLINICAL PRESENTATIONS — Primary upper extremity deep vein thrombosis can present acutely with symptoms and signs of upper extremity deep vein thrombosis or pulmonary embolism, or with chronic or intermittent symptoms. In the pediatric population, primary upper extremity deep vein thrombosis presents with similar symptoms and thrombotic complications as with adults [2,35,36].

Acute upper extremity deep vein thrombosis — Acute presentations are due to sudden thrombosis of the axillosubclavian vein. The classic presentation is that of a young, athletic male presenting with acute onset of upper extremity pain and swelling in the dominant arm following a particularly strenuous activity [1,29,30]. Strenuous use of the arm prior to the onset of extremity swelling or pain is recalled in 40 to 80 percent of patients, and symptoms are generally noticed within 24 hours of the strenuous activity [11,12,24,37]. The majority of patients (70 to 80 percent) manifest with variable degrees of neck, shoulder, or axillary discomfort; arm heaviness; and pain associated with complaints of upper extremity swelling [38,39]. Swelling and pain typically improve with rest and elevation of the arm to the level of the heart, whereas elevation of the extremity overhead may aggravate the symptoms [40].

Physical examination generally reveals edema of the affected extremity, often accompanied by cyanosis of the hand and fingers. The patient may also have a low-grade fever. A palpable venous cord (superficial thrombophlebitis) may be apparent in associated superficial veins (eg, proximal cephalic vein). Dilated subcutaneous collateral veins, also known as Urschel's sign, may be noticeable over the upper chest and proximal upper extremity, particularly in those with an underlying chronic venous stenosis [12,29,38].

Patients with primary venous thrombosis due to compression of structures of the thoracic outlet may also have symptoms attributable to the arterial or neurologic structures that pass through this space. Distinguishing between neurogenic, arterial, and venous thoracic outlet syndrome is discussed elsewhere. (See "Overview of thoracic outlet syndromes", section on 'Clinical evaluation'.)

Reduced arterial blood flow is uncommon and is rarely related to venous congestion (phlegmasia cerulea dolens), particularly in the upper extremity [41,42]. However, if present, it represents an emergency and indicates the need for emergency treatment. (See 'Thrombolytic therapy' below.)

Coexistent signs related to brachial plexus compression (ie, neurogenic thoracic outlet syndrome) may be present, manifesting as paraesthesias or pain in the ulnar nerve distribution, tenderness over the supraclavicular fossa, and wasting of the intrinsic hand muscles. (See "Overview of thoracic outlet syndromes", section on 'Clinical evaluation'.)

Acute pulmonary embolism — In addition to upper extremity swelling and pain, upper extremity deep vein thrombosis can initially present as symptomatic or asymptomatic pulmonary embolism [13,30,39,43-47]. The clinical features, diagnosis, and treatment of pulmonary embolism are discussed in detail elsewhere. (See "Epidemiology and pathogenesis of acute pulmonary embolism in adults".)

Chronic or intermittent symptoms — In patients with partial thrombosis or chronic venous stenosis due to repetitive injury that causes activity-related obstruction, symptoms may be intermittent and less severe. If venous occlusion develops over a protracted period of time, edema or pain may be minimal, and increased venous collateral flow over the chest (Urschel's sign) may be the only clinical sign that is apparent [12,29,38,48].

DIAGNOSIS — A diagnosis of upper extremity venous outflow obstruction (ie, deep vein thrombosis or venous stenosis) may be suspected based upon the clinical presentation but should be confirmed with imaging, typically initially using ultrasound. Advanced venous imaging may be needed to demonstrate abnormal venous compression when there is a diagnostic dilemma. D-dimer is useful for excluding thrombosis as an etiology, but will not exclude venous stenosis without thrombosis as a source of symptoms. (See 'Clinical presentations' above and 'D-dimer' below and 'Imaging' below.)

Primary upper extremity deep vein thrombosis can be distinguished from secondary causes of deep vein thrombosis by the absence of venous instrumentation, a young, otherwise healthy patient demographic, and a more typically sudden onset of symptoms. The clinical features of upper extremity deep vein thrombosis are otherwise similar and include upper extremity edema and pain, and cyanosis of the skin due to venous congestion. For the patient who presents with the classic presentation, once a diagnosis of venous outflow obstruction is established, no further workup is necessary except a plain chest radiograph to identify any obvious bony abnormalities prior to intervention. In the absence of an obvious bony anatomic abnormality, the specific anatomic abnormality may not be determined until the time of surgical exploration. (See 'Approach to treatment' below.)

D-dimer — Plasma D-dimer, which is a degradation product of cross-linked fibrin, may be elevated in patients with upper extremity deep vein thrombosis [49], as in those with other lower extremity deep vein thrombosis or pulmonary embolism. However, although a plasma D-dimer >500 mcg/L is sensitive for thrombosis and has a high negative predictive value, it is not specific for the anatomic location of the thrombosis and will not exclude vein compression/stenosis as a source for symptoms [50]. (See "Overview of thoracic central venous obstruction".)

Imaging — B-mode ultrasound, color Doppler ultrasound, and duplex ultrasound have been used extensively in the diagnosis of deep vein obstruction. Noncompressibility of the vein on B-mode ultrasound with or without visible intraluminal thrombus is the major criterion for the diagnosis of venous thrombosis. We use duplex ultrasound as the initial test for diagnosing upper extremity venous outflow obstruction because it is noninvasive, inexpensive, and, in observational studies, has an acceptable sensitivity and specificity for the diagnosis of upper extremity deep vein thrombosis [39,51-58]. A systematic review evaluated 17 studies, concluding that compression ultrasonography is an acceptable alternative to standard contrast venography [51]. The summary estimates of the sensitivity of compression, Doppler ultrasound, and Doppler ultrasound with compression were 97, 84, and 81 percent, respectively, and specificities were 96, 94, and 93 percent, respectively. Disadvantages of ultrasound are that it is technician dependent and that nonocclusive mural thrombus and thrombus in the proximal subclavian or innominate veins may not be adequately seen as a result of acoustic shadowing by the overlying clavicle and sternum [57,59,60].

Although standard catheter-based (digital subtraction) venography provides the best definition of abnormal venous anatomy and is the standard with which other modalities are compared [51,61], it is generally not needed to solely establish a diagnosis of upper extremity deep vein thrombosis or compression. Because of its invasive nature, catheter-based venography is generally reserved for situations where noninvasive studies are equivocal but clinical suspicion remains high for a primary cause of venous outlet obstruction [1]. For patients with intermittent or chronic symptoms, extrinsic compression of the vein can be demonstrated during catheter-based venography by performing dynamic studies that place the arm in various positions during the study. The venogram may be normal at rest but abnormal (varying degrees of extrinsic compression with "new" venous collaterals) with arm abduction; however, vein compression with arm abduction can be a normal variant [62].

Less invasive methods of venography include computed tomographic (CT) and magnetic resonance (MR) venography [60,63-65]. These modalities are not typically used to establish an initial diagnosis of upper extremity venous outflow obstruction. However, in the situation with atypical symptoms or equivocal ultrasound study, these study modalities offer noninvasive methods to further evaluate central veins and surrounding structures. These studies are also useful for identifying other secondary causes for deep vein thrombosis (eg, tumor).

CT venography can be used to confirm or exclude central vein thrombus (image 1); however, like catheter-based venography, substantial contrast loads are required. CT venography has not been studied sufficiently to determine its sensitivity and specificity. A small study of 18 patients compared CT venography and digital subtraction venography for their ability to discriminate the severity and extent of venous obstruction, the cause of upper extremity deep vein thrombosis, and implications for the planning of treatment [65]. CT venography was felt to provide more information than digital subtraction venography, and in one half of the patients, the findings of CT venography changed the treatment plan.

Contrast-enhanced MR imaging is very specific in its ability to image subclavian vein thrombosis, but its sensitivity for thrombosis is too low to be a useful screening modality [60]. However, with proper protocol and dynamic sequencing, contrast-enhanced three-dimensional (3D) MR angiography is an excellent noninvasive alternative to venogram (image 2) [66]. One must remember that anatomic compression of vascular structures in thoracic outlet is common, and positive findings and the presence of pathological findings must be clinically correlated.

Screening for hypercoagulability — Because of the high prevalence of an underlying thrombophilic state in primary upper extremity deep venous thrombosis, screening for thrombophilia should be included in the evaluation [32]. (See "Evaluating adult patients with established venous thromboembolism for acquired and inherited risk factors".)

DIFFERENTIAL DIAGNOSIS — The differential diagnosis for upper extremity edema not related to primary upper extremity deep vein thrombosis includes edema related to other etiologies, secondary causes of venous thrombosis, and lymphedema.

Upper extremity deep vein thrombosis that occurs in the absence of instrumentation and with no identifiable anatomic abnormalities or other risk factors for venous thrombosis (eg, oral contraceptives) raises concern for occult malignancy, which will be diagnosed in up to 25 percent of patients within one year of a venous thromboembolic event [39,67]. If a primary cause for upper extremity deep vein thrombosis is not immediately apparent on imaging studies, the patient has no history of instrumentation, and the patient has none of the risk factors listed above for primary upper extremity deep venous thrombosis, we suggest a more formal laboratory evaluation to rule out secondary causes for upper extremity deep vein thrombosis, including coagulation studies, which should be drawn prior to the initiation of anticoagulation. (See "Risk and prevention of venous thromboembolism in adults with cancer".)

There are many causes of extremity edema that are not related to venous obstruction. The medical history will usually give a clue as to the potential etiology for edema (eg, history of heart failure). Although systemic etiologies typically present with bilateral extremity edema, this feature is not helpful given that anatomic abnormalities of the thoracic outlet are common and patients with primary upper extremity deep vein thrombosis can present with bilateral symptoms. Routine laboratory studies typically important in the evaluation of patients with extremity edema include a complete blood count, electrolytes, and liver function tests. These studies may point to an alternative etiology for upper extremity edema. The general approach to the patient with edema is discussed in detail elsewhere. (See "Pathophysiology and etiology of edema in adults" and "Clinical manifestations and evaluation of edema in adults".)

Upper extremity arm swelling can be due to lymphedema; however, swelling from acute venous thrombosis has a more abrupt onset, and an antecedent risk factor such as prior axillary lymph node dissection is lacking. (See "Clinical features and diagnosis of peripheral lymphedema".)

APPROACH TO TREATMENT — The goals of treatment of primary upper extremity deep vein thrombosis are relieving symptoms related to venous obstruction, preventing complications of deep vein thrombosis, and preventing recurrent thrombosis [1,68-70]. Treatment options include anticoagulation, thrombolysis, and surgical decompression of the thoracic outlet.

There is no "one size fits all" approach to the treatment of primary upper extremity deep vein thrombosis. Individualized care requires a team of specialists with sufficient experience and readily available resources and ancillary personnel [71,72]. No treatment or combination of treatments has been rigorously evaluated for the treatment of upper extremity deep vein thrombosis. As a result, recommendations are based upon available retrospective studies and indirect evidence provided from the experience with deep vein thrombosis of the lower extremity [73-75].

Admission or outpatient referral — Many patients with acute, severe symptoms related to primary upper extremity deep vein thrombosis will require admission to manage symptoms and in anticipation of thrombolytic therapy and/or surgical decompression.

Initial outpatient anticoagulation and outpatient referral for possible further treatment may be appropriate for patients with:

Minimal symptoms

Delayed presentation more than two weeks after the onset of symptoms

Intermittent symptoms due to venous obstruction without thrombosis

Our approach — Our approach to treatment is as follows:

For all patients identified with upper extremity deep vein thrombosis, we agree with guidelines from the American College of Chest Physicians, which recommend anticoagulation for a minimum of three months [73-75]. (See 'Anticoagulation' below.)

For patients identified with anatomic abnormalities of the thoracic outlet causing symptomatic venous compression and who are good-risk surgical patients, we suggest thoracic outlet decompression (with or without preceding thrombolysis). The specific procedure is targeted to the type of abnormality identified. For those in whom a specific abnormality has not been identified, we perform first rib resection, provided that a secondary cause of upper extremity venous thrombosis is not present. (See 'Thoracic outlet decompression' below.)

For patients with sudden-onset primary upper extremity axillosubclavian deep vein thrombosis with moderate-to-severe upper extremity symptoms of less than two weeks' duration, we suggest thrombolysis to eliminate thrombus to the extent that is possible prior to thoracic outlet decompression. Lysis is less effective when symptoms have been present for more than two weeks. (See 'Thrombolytic therapy' below.)

For patients with mild symptoms, intermittent symptoms, and those who present in a delayed manner (>2 weeks), anticoagulation alone (no thrombolysis) with or without thoracic outlet decompression may be appropriate. The natural history of these patients is unclear. (See 'Symptomatic care' below and 'Anticoagulation' below.)

Our recommendations are in general agreement with the guidelines from the American College of Chest Physicians (ACCP); however, the ACCP suggests anticoagulant therapy alone over thrombolysis for patients with acute upper extremity deep vein thrombosis that involves the axillary or more proximal veins [73-75]. They further state that patients are likely to choose thrombolytic therapy over anticoagulation alone if they are more likely to benefit from thrombolysis, have access to catheter-based therapy, attach a high value to the prevention of post-thrombotic syndrome, and attach a lower value to the initial complexity, cost, and risk of bleeding with thrombolytic therapy. This recommendation does not distinguish between primary and secondary causes of upper extremity deep vein thrombosis directly; however, given that patients with primary upper extremity deep vein thrombosis are more likely to benefit from thrombolysis compared with patients with secondary causes of deep vein thrombosis, we support a more aggressive treatment strategy. (See 'Rationale for aggressive treatment' below.)

Rationale for aggressive treatment — An aggressive approach that includes a combination of thrombolysis and thoracic outlet decompression with or without venoplasty (percutaneous, open) appears to improve long-term outcomes in patients with primary upper extremity deep vein thrombosis, particularly those with acute, moderate-to-severe symptoms [71,76-95]. This approach is also supported for treatment of adolescent patients with venous thoracic outlet syndrome [28]. With an aggressive approach, success rates for reestablishing subclavian vein patency are nearly 100 percent provided that thrombolysis is performed within two weeks of the onset of symptoms [96-98]. Although early intervention is advocated, patients with primary upper extremity deep vein thrombosis who present later than two weeks may also benefit from thoracic outlet decompression (no thrombolysis) given the high rates of recurrent thrombosis and long-term morbidity associated with anticoagulation alone [4,23,99,100].

In a worldwide clinical series of 606 patients with primary upper extremity deep vein thrombosis, early thrombolysis and first rib resection provided the best outcome, with 95 percent of the surgical cohort experiencing an excellent clinical outcome compared with 29 percent treated conservatively, which consisted of anticoagulation, arm elevation, and upper extremity compression [29]. Residual venous obstruction was present in 78 percent of patients. The clinical outcomes associated with anticoagulation alone were evaluated in a later series of 54 patients, nearly all of whom were treated with warfarin [22]. After a mean follow-up of five years, 22 percent had persistent severe venous outflow obstruction on follow-up ultrasound. Approximately 50 percent of the patients were asymptomatic, but 13 percent had severe or disabling symptoms. Subsequent pulmonary embolism was documented in 26 percent and was symptomatic in one third of the patients. By comparison, among patients treated with thrombolysis (without thoracic outlet decompression), 76 percent were asymptomatic after a mean follow-up of 55 months [22,96]. In other retrospective reviews, persistent symptoms and disability occurred in 41 to 91 percent of patients treated conservatively [1,29].

First rib resection without preoperative thrombolysis has been proposed for the management of primary subacute venous thrombosis. In the retrospective review, 45 of 110 patients underwent preoperative thrombolysis alone or thrombolysis and balloon venoplasty prior to thoracic outlet decompression. The remaining 65 patients were treated with anticoagulation alone prior to thoracic outlet decompression. Up to 80 percent of occluded axillosubclavian veins recanalized during the follow-up period in the anticoagulation group, and the overall rates of venous patency were similar between the groups [99]. However, this study did not address the patients who presented acutely.

INITIAL MANAGEMENT — Patients who are diagnosed with primary upper extremity deep vein thrombosis are initially managed with measures to improve their comfort and are anticoagulated. Anticoagulation helps to maintain patency of collateral veins and reduces propagation of thrombus. Anticoagulant therapy is also effective in preventing pulmonary embolism with lower extremity venous thrombosis and, by extrapolation, may also prevent embolism from upper extremity deep vein thrombosis [30,73-75]. The decision to proceed with thrombolysis or thoracic outlet decompression is based upon symptom severity and the type of associated anatomic abnormality. (See 'Approach to treatment' above.)

Symptomatic care — Symptomatic care of phlebitic symptoms related to upper extremity deep vein thrombosis includes upper extremity elevation and nonsteroidal anti-inflammatory drugs (NSAIDs) for pain management.

Arm elevation should help reduce upper extremity swelling. Compression may also help to reduce the edema and alleviate acute symptoms. Compression is likely unnecessary in treated patients for whom the lesion is corrected and edema has resolved. No data are available regarding a benefit of compression for preventing post-thrombotic syndrome following upper extremity DVT, and using compression stockings for this purpose is not supported by American College of Chest Physicians guideline on treatment of venous thromboembolism [101].

Anticoagulation — The choice of initial parenteral agent for anticoagulation in patients with primary upper extremity deep vein thrombosis depends upon the need for further treatment in the form of thrombolysis or thoracic outlet decompression. Treatment options are similar to those available for lower extremity deep vein thrombosis. (See "Overview of the treatment of proximal and distal lower extremity deep vein thrombosis (DVT)".)

In our practice, we begin parenteral anticoagulation once a diagnosis of deep vein thrombosis is made but after any necessary laboratory tests to evaluate for hypercoagulable states have been obtained. For patients with mild, intermittent, or chronic symptoms who will be managed on an outpatient basis with anticoagulation alone, oral anticoagulants (eg, dabigatran, rivaroxaban, apixaban, or edoxaban) appear as effective as vitamin K antagonist and offer more convenience for the patient [73-75,102-104].

When thrombolysis is anticipated, we administer unfractionated heparin (or alternative agents if contraindicated) and maintain therapeutic levels (aPTT 1.5 to 2.5 times control) until thrombolysis is initiated. During thrombolysis, the dose of heparin should be lowered to minimize bleeding complications, but once thrombolysis is completed, full anticoagulation can be resumed [105,106]. Similarly, the dose of heparin should be lowered around the time of surgical intervention. Once any necessary interventions are completed, bridging anticoagulation can be used to transition to long-term therapy in anticipation of discharge. (See "Perioperative management of patients receiving anticoagulants", section on 'Bridging anticoagulation'.)

Duration of anticoagulation — In patients with primary upper extremity deep vein thrombosis, anticoagulation should be continued for a minimum of three months following the initial thrombotic event, with a longer duration of therapy indicated for those who have had a recurrent event [73-75,107]. We maintain anticoagulation regardless of whether intervention (thrombolysis, thoracic outlet decompression) was performed.

THROMBOLYTIC THERAPY — The aim of thrombolytic therapy is prompt dissolution of thrombus to minimize inflammation and endothelial injury and to restore vein patency, which reduces extremity edema and associated symptoms [30,91,95,108,109]. Thrombolytic therapy appears to have the most benefit for patients who present with acute, moderate-to-severe symptoms related to sudden axillosubclavian thrombosis. In our experience, thrombi that have been symptomatic for up to two weeks have a reasonable chance of lysis with catheter-directed infusion of alteplase directed into the thrombus. Thrombolysis restores vein patency in 64 to 84 percent of patients, with better patency rates associated with earlier initiation of lytic therapy [95]. However, even after successful lysis that restores vein patency, up to one third of patients will re-occlude without thoracic outlet decompression [1,95]. (See 'Rationale for aggressive treatment' above.)

The general contraindications to thrombolytic therapy are given in the table (table 1). Symptomatic patients who present more than two weeks after the onset of symptoms are less likely to benefit from thrombolysis due to the organized nature of the clot and inflammatory changes in the vein.

Observational studies have shown that catheter-directed pharmacologic thrombolysis, which involves embedding an infusion catheter into the axillosubclavian vein thrombus, achieves higher rates of clot dissolution compared with systemic infusion and requires an overall lower dose and shorter duration of lysis, which reduces bleeding complications [30,95,109].

Catheter-directed pharmacologic upper extremity thrombolysis is performed in the following manner:

The basilic vein of the affected limb is accessed using ultrasound guidance.

A guidewire and catheter are used to traverse the thrombosed axillosubclavian vein.

A multi-sidehole infusion catheter is embedded within the thrombus and infusion of alteplase initiated at 0.01 mg/kg/hour [95,110]. The catheter is secured into position, and the patient is transferred to a monitored setting to assess for any bleeding complications. The progress of thrombolysis is evaluated by performing venography within 24 hours. A total dose of 20 to 25 mg alteplase can be safely used in most cases.

For patients undergoing catheter-directed thrombolysis, aspirin may be administered along with unfractionated heparin during infusion of the thrombolytic agent to counteract platelet activation and the potentially increased thrombogenicity that may be induced with lytic therapy [39].

Mechanical thrombolysis (eg, AngioJet, EKOS catheter) is often used in combination with pharmacologic thrombolysis [111]. There are limited data involving the use of these devices to treat upper extremity thrombosis. However, based upon available results for lower extremity deep vein thrombosis, these treatments may be useful in upper extremity thrombosis to rapidly extract a large burden of thrombus and reduce the overall dose and duration of lytic therapy [112]. (See "Endovenous intervention for thoracic central venous obstruction".)

Once venous patency has been reestablished with catheter-directed thrombolysis, it is important to perform completion venography to evaluate the residual axillosubclavian vein for persistent compression or stenosis. Occasionally, adjunctive percutaneous transluminal angioplasty (PTA) following successful thrombolysis is needed to open the vein sufficiently so that anticoagulant treatment can maintain patency until thoracic outlet decompression can be performed [1]. (See 'Venoplasty' below and 'Thoracic outlet decompression' below.)

THORACIC OUTLET DECOMPRESSION — For patients with primary upper extremity axillosubclavian deep vein thrombosis, and selected patients with axillosubclavian venous compression/stenosis, observational studies support surgical decompression of the thoracic outlet, which provides the lower rates of recurrent (or future) thrombosis, and reduced long-term morbidity compared with more conservative management [1,29,113]. (See 'Rationale for aggressive treatment' above.)

Thoracic outlet decompression may include any one of a combination of the procedures discussed below and is indicated for good-risk surgical patients with any of the following presentations [1,29,76,114,115]:

Patients who present with acute, moderate-to-severe symptoms due to primary upper extremity axillosubclavian thrombosis, following thrombolysis.

Symptomatic patients (intermittent or recurrent) with a thoracic outlet anatomic abnormality causing venous compression/stenosis. Objective evidence of venous thrombosis may or may not be present.

Thoracic outlet decompression may be indicated in patients with symptomatic subacute (>2 weeks) or symptomatic chronic thrombosis [99].

Following successful thrombolysis, some clinicians favor anticoagulation for one to three months to allow endothelial healing and resolution of acute inflammation before performing thoracic outlet decompression [76]. However, given the risk of re-thrombosis, we agree with the majority of surgeons in advocating surgical decompression during the same hospitalization as thrombolytic therapy [1,4,72]. Regardless of the timing of surgical decompression, anticoagulation is maintained until the surgery can be performed. (See 'Anticoagulation' above.)

For patients with residual thrombus following thrombolysis or those with chronic axillosubclavian thrombosis, the axillosubclavian vein can be reconstructed concurrent with thoracic outlet decompression or later using open or endovascular techniques, if needed. In a series of patients undergoing first rib resection for chronic symptoms related to primary subclavian vein thrombosis, subclavian vein thrombus resolved in 14 of 16 patients treated with ongoing anticoagulation [116]. (See 'Venoplasty' below.)

The aim of thoracic outlet decompression is to provide more space through which the neurovascular structures of the upper extremity can pass. Thoracic outlet decompression may include any one or a combination of the following procedures, the choice of which will depend upon the specific anatomic abnormality identified [23,116]. (See "Overview of thoracic outlet syndromes", section on 'Pathogenesis'.)

First rib resection

Cervical rib resection (less common for venous thoracic outlet syndrome)

Division of anomalous bands

Division of anomalous musculotendinous insertions

Scalenectomy

For patients identified with a cervical rib, cervical rib resection may be all that is required. However, when a cervical rib is not present, first rib resection is combined with dissection and division of structures identified at the time of surgical exploration as potentially causing compression on the neurovascular structures of the thoracic outlet, whether these were identified as a specific source of compression on prior imaging studies.

Multiple surgical approaches are used for thoracic outlet decompression: the transaxillary, supraclavicular, and infraclavicular approaches, each with advantages and disadvantages. With surgical robotic technological advancement, robotic endoscopic transaxillary first rib resection and robotic transthoracic first rib resection have also been successfully performed [117,118]. There are no trials comparing surgical approaches for decompression, and there remains no expert consensus as to which surgical approach or method of treating the injured venous segment is better. As such, the surgical approach depends largely upon the type of anatomic abnormalities identified and surgeon preference. These approaches and the complications associated with them are discussed separately. (See "Overview of thoracic outlet syndromes", section on 'Thoracic outlet decompression'.)

Complications of thoracic outlet decompression include hemopneumothorax, long thoracic nerve injury, incomplete rib resection that can lead to recurrent symptoms, brachial plexus injury, arterial injury, lymphatic leak, and surgical site infection.

VENOPLASTY — Although there is uniform agreement that thoracic outlet decompression should be performed early in patients with acute axillosubclavian thrombosis [1], debate exists over the management of the vein. Concurrent with thoracic outlet decompression, some feel that venolysis or venoplasty (percutaneous transluminal or open) is sufficient while others advocate vein repair (patch venoplasty, interposition vein graft, vein bypass, jugular turndown) [29,91,119]. There are insufficient data to support one approach over another.

Occasionally, percutaneous transluminal angioplasty (PTA) is needed to keep the vein open following thrombolysis until thoracic outlet decompression can be accomplished [1]. However, prior to thoracic outlet decompression, stenting should be avoided [48,96,120-122]. Shoulder movements subject the stent to repetitive compression that can lead to stent fracture. In one small observational study, stent use was found to be an independent risk factor for upper extremity rethrombosis [96]. Once thoracic outlet decompression has been performed, stenting for residual or recurrent stenosis may be warranted with acceptable clinical outcomes [1,23].

POSTPROCEDURAL IMAGING — We obtain a duplex ultrasound in the postsurgical period to confirm patency of axillosubclavian vein. If the patient experiences recurrent symptoms, we agree with others in advocating further imaging to evaluate the patency of the repaired venous segment [116].

PERIOPERATIVE MORBIDITY AND MORTALITY — Mortality related to primary upper extremity deep vein thrombosis is overall low, due to the relatively young population of patients who are typically affected. By comparison, mortality related to secondary causes of upper extremity deep vein thrombosis is higher, ranging from 15 to 50 percent, reflecting underlying comorbidities such as malignancy, renal failure, and multiorgan failure [13].

Recurrent thromboembolism — Recurrent upper extremity deep vein thrombosis (all causes) occurs in 2 to 8 percent of patients following treatment [30,123,124]. These rates are significantly lower than recurrence rates for lower extremity deep vein thrombosis, which in one long-term study was 30 percent at eight years [125].

A hypercoagulable state needs to be considered in patients who develop recurrent thromboembolism [13]. Inadequate thoracic outlet decompression can also result in re-thrombosis [126]. Higher rates of thrombophilia have been reported in patients with recurrent upper extremity deep vein thrombosis [33,127-130]. In one study, 90 percent of postoperative complications were associated with some form of thrombophilia [128]. The evaluation of thrombophilia is discussed elsewhere. (See "Evaluating adult patients with established venous thromboembolism for acquired and inherited risk factors".)

Post-thrombotic syndrome — Post-thrombotic syndrome refers to the development of symptoms or signs of chronic venous insufficiency related to a prior deep vein thrombosis. Post-thrombotic syndrome is discussed in detail elsewhere. (See "Post-thrombotic (postphlebitic) syndrome".)

Quality of life is reduced in patients with post-thrombotic syndrome, particularly if the dominant arm is affected. Severe upper extremity symptoms with skin ulceration are rare, but post-thrombotic syndrome affecting the upper extremity can result in occupational disability in patients whose job requires manual labor. Even in patients whose occupation does not involve the vigorous use of the arms, symptoms of post-thrombotic syndrome can limit other activities and adversely impact quality of life.

The incidence of post-thrombotic syndrome following upper extremity deep vein thrombosis (all causes) ranges from 7 to 44 percent but appears to be more prevalent following primary compared with secondary etiologies [13,30]. Since primary upper extremity deep vein thrombosis generally affects young, otherwise healthy individuals with an active lifestyle and long life expectancy, one of the aims of early aggressive treatment is minimizing symptoms of post-thrombotic syndrome. (See 'Rationale for aggressive treatment' above.)

In patients with primary upper extremity deep vein thrombosis, up to 53 percent of patients treated with anticoagulation alone historically developed post-thrombotic syndrome at five years [29,114]. With aggressive therapy that includes anticoagulation, thrombolysis, and thoracic outlet decompression, the incidence of residual symptoms ranges from 12 to 25 percent [1,29]. The risk of developing post-thrombotic syndrome may be greater in patients who have residual vein obstruction. However, in one study, no association between ultrasound findings and the development of post-thrombotic syndrome was found [131].

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Superficial vein thrombosis, deep vein thrombosis, and pulmonary embolism" and "Society guideline links: Chronic venous disorders".)

SUMMARY AND RECOMMENDATIONS

Primary upper extremity deep vein thrombosis – Primary upper extremity deep vein thrombosis is defined as thrombosis of the deep veins draining the upper extremity (axillary, subclavian) due to an underlying anatomic anomaly at the thoracic outlet causing compression or repetitive venous injury. This condition is also referred to as venous thoracic outlet syndrome (vTOS), Paget-Schroetter syndrome, and "effort" thrombosis. It represents one of many causes of thoracic central venous obstruction (TCVO) and is estimated to represent between 1 and 4 percent of all cases of thrombotic TCVO, with secondary causes of thrombosis related to central vein cannulation (eg, central line, pacemaker) or prothrombotic states (eg, thrombophilia, malignancy). (See 'Pathogenesis and terminologies' above.)

Risk factors – Anatomic abnormalities of the thoracic outlet can be congenital or acquired. Congenital anomalies consist of cervical ribs, supernumerary muscles, abnormal tendon insertions, or abnormal muscular or tendinous bands. Acquired abnormalities include bony overgrowth due to bony fracture (usually of the clavicle), or hypertrophy of anterior scalene muscle or subclavius muscles, often related to repetitive lifting. (See 'Epidemiology and risk factors' above.)

Clinical presentations – Primary upper extremity deep vein thrombosis typically presents in young, otherwise healthy individuals as sudden, severe arm swelling. Although more commonly associated with lower extremity deep vein thrombosis, pulmonary embolism from upper extremity deep vein thrombosis (primary and secondary) occurs in 4 to 10 percent of patients. The clinician should have a high index of suspicion for this disorder when a young patient with no inciting factors presents with signs of upper extremity venous thrombosis, or pulmonary embolism in the absence of lower extremity symptoms. (See 'Clinical presentations' above.)

Diagnosis – A diagnosis of upper extremity venous outflow obstruction (ie, deep vein thrombosis or venous stenosis) may be suspected based upon the clinical presentation but should be confirmed with imaging, typically initially using ultrasound. D-dimer is useful for excluding thrombosis as an etiology but will not exclude vein compression/stenosis without thrombosis as a source of symptoms. Once a diagnosis of venous outflow obstruction is established, a primary etiology should be sought to identify the underlying anatomic abnormality that is the source of the obstruction. We obtain a plain chest radiograph on all patients to identify any obvious bony abnormalities. More advanced imaging may be necessary to demonstrate abnormal muscular attachments or dynamic venous compression for atypical presentations. (See 'Diagnosis' above.)

Treatment – Treatment of primary upper extremity deep vein thrombosis is aimed at preventing pulmonary embolism, recurrent venous thrombosis, and post-thrombotic syndrome. (See 'Approach to treatment' above.)

For patients with acutely symptomatic primary upper extremity deep vein thrombosis, we recommend anticoagulation over no such therapy (Grade 1B). Anticoagulation helps to maintain patency of collateral veins, reduces propagation of thrombus, and is effective for preventing pulmonary embolism. We prefer to use unfractionated heparin to facilitate rapid dose adjustment in patients who will undergo other interventions such as thrombolysis and thoracic outlet decompression.

For patients with moderate-to-severe acute symptoms who are diagnosed with primary upper extremity deep vein thrombosis, we suggest thrombolysis over anticoagulation alone (Grade 2C). Following thrombolysis, we suggest thoracic outlet decompression, rather than no decompression (Grade 2C). This approach decreases the risk for recurrent thrombosis and post-thrombotic syndrome. Anticoagulation alone may be adequate for minimally symptomatic or intermittently symptomatic patients. For those who present in a delayed manner (>2 weeks from the onset of symptoms), decompression of thoracic outlet in addition to anticoagulation may be beneficial. (See 'Approach to treatment' above and 'Rationale for aggressive treatment' above.)

We agree with the American College of Chest Physicians, who recommend a minimum of three months of anticoagulation following an initial thrombotic event. We maintain anticoagulation for a minimum of three months regardless of whether intervention (thrombolysis, thoracic outlet decompression) was performed.

  1. Illig KA, Doyle AJ. A comprehensive review of Paget-Schroetter syndrome. J Vasc Surg 2010; 51:1538.
  2. Trenor CC 3rd, Fisher JG, Khan FA, et al. Paget-Schroetter syndrome in 21 children: outcomes after multidisciplinary care. J Pediatr 2015; 166:1493.
  3. Alla VM, Natarajan N, Kaushik M, et al. Paget-schroetter syndrome: review of pathogenesis and treatment of effort thrombosis. West J Emerg Med 2010; 11:358.
  4. Aziz S, Straehley CJ, Whelan TJ Jr. Effort-related axillosubclavian vein thrombosis. A new theory of pathogenesis and a plea for direct surgical intervention. Am J Surg 1986; 152:57.
  5. Bahm J. Critical review of pathophysiologic mechanisms in thoracic outlet syndrome (TOS). Acta Neurochir Suppl 2007; 100:137.
  6. Mustafa S, Stein PD, Patel KC, et al. Upper extremity deep venous thrombosis. Chest 2003; 123:1953.
  7. Paget J. Clinical lectures and essays, Longmans, Green, and Co, London 1875.
  8. von Schroetter L. Erkrankungen der Gefasse. In: Nathnagel Handbuch der Pathologie und Therapie, Anonymous Wein, Holder 1884.
  9. HUGHES ES. Venous obstruction in the upper extremity; Paget-Schroetter's syndrome; a review of 320 cases. Surg Gynecol Obstet 1949; 88:89.
  10. Drapanas T, Curran WL. Thrombectomy in the treatment of "effort" thrombosis of the axillary and subclavian veins. J Trauma 1966; 6:107.
  11. Lindblad B, Tengborn L, Bergqvist D. Deep vein thrombosis of the axillary-subclavian veins: epidemiologic data, effects of different types of treatment and late sequelae. Eur J Vasc Surg 1988; 2:161.
  12. Horattas MC, Wright DJ, Fenton AH, et al. Changing concepts of deep venous thrombosis of the upper extremity--report of a series and review of the literature. Surgery 1988; 104:561.
  13. Flinterman LE, Van Der Meer FJ, Rosendaal FR, Doggen CJ. Current perspective of venous thrombosis in the upper extremity. J Thromb Haemost 2008; 6:1262.
  14. Swinton NW Jr, Edgett JW Jr, Hall RJ. Primary subclavian-axillary vein thrombosis. Circulation 1968; 38:737.
  15. Fisher JB, Granson MA. Congenital venous web causing subclavian vein obstruction: a case report. J Vasc Surg 1989; 10:460.
  16. Johansen KH, Thomas GI. Late thoracic outlet syndrome secondary to malunion of the fractured clavicle: case report and review of the literature. J Trauma 2002; 52:607.
  17. Jain S, Monbaliu D, Thompson JF. Thoracic outlet syndrome caused by chronic retrosternal dislocation of the clavicle. Successful treatment by transaxillary resection of the first rib. J Bone Joint Surg Br 2002; 84:116.
  18. Duane TM, O'Connor JV, Scalea TM. Thoracic outlet syndrome resulting from first rib fracture. J Trauma 2007; 62:231.
  19. Peivandi MT, Nazemian Z. Clavicular fracture and upper-extremity deep venous thrombosis. Orthopedics 2011; 34:227.
  20. Stevenson IM, Parry EW. Radiological study of the aetiological factors in venous obstruction of the upper limb. J Cardiovasc Surg (Torino) 1975; 16:580.
  21. Machleder HI. Thrombolytic therapy and surgery for primary axillosubclavian vein thrombosis: current approach. Semin Vasc Surg 1996; 9:46.
  22. Héron E, Lozinguez O, Emmerich J, et al. Long-term sequelae of spontaneous axillary-subclavian venous thrombosis. Ann Intern Med 1999; 131:510.
  23. Urschel HC Jr, Razzuk MA. Paget-Schroetter syndrome: what is the best management? Ann Thorac Surg 2000; 69:1663.
  24. DiFelice GS, Paletta GA Jr, Phillips BB, Wright RW. Effort thrombosis in the elite throwing athlete. Am J Sports Med 2002; 30:708.
  25. Isma N, Svensson PJ, Gottsäter A, Lindblad B. Upper extremity deep venous thrombosis in the population-based Malmö thrombophilia study (MATS). Epidemiology, risk factors, recurrence risk, and mortality. Thromb Res 2010; 125:e335.
  26. Engelberger RP, Kucher N. Management of deep vein thrombosis of the upper extremity. Circulation 2012; 126:768.
  27. Kucher N. Clinical practice. Deep-vein thrombosis of the upper extremities. N Engl J Med 2011; 364:861.
  28. Modi BP, Chewning R, Kumar R. Venous thoracic outlet syndrome and Paget-Schroetter syndrome. Semin Pediatr Surg 2021; 30:151125.
  29. Urschel HC Jr, Patel AN. Surgery remains the most effective treatment for Paget-Schroetter syndrome: 50 years' experience. Ann Thorac Surg 2008; 86:254.
  30. Joffe HV, Goldhaber SZ. Upper-extremity deep vein thrombosis. Circulation 2002; 106:1874.
  31. Chu AS, Harkness J, Witmer CM. Spontaneous Subclavian Vein Thrombosis in a Healthy Adolescent Cheerleader: A Case of Paget-Schroetter Syndrome. Pediatr Emerg Care 2017; 33:e92.
  32. Hendler MF, Meschengieser SS, Blanco AN, et al. Primary upper-extremity deep vein thrombosis: high prevalence of thrombophilic defects. Am J Hematol 2004; 76:330.
  33. Martinelli I, Battaglioli T, Bucciarelli P, et al. Risk factors and recurrence rate of primary deep vein thrombosis of the upper extremities. Circulation 2004; 110:566.
  34. Brandão LR, Williams S, Kahr WH, et al. Exercise-induced deep vein thrombosis of the upper extremity. 2. A case series in children. Acta Haematol 2006; 115:221.
  35. Vercellio G, Baraldini V, Gatti C, et al. Thoracic outlet syndrome in paediatrics: clinical presentation, surgical treatment, and outcome in a series of eight children. J Pediatr Surg 2003; 38:58.
  36. Rehemutula A, Zhang L, Chen L, et al. Managing pediatric thoracic outlet syndrome. Ital J Pediatr 2015; 41:22.
  37. Adams JT, DeWeese JA. "Effort" thrombosis of the axillary and subclavian veins. J Trauma 1971; 11:923.
  38. Prandoni P, Bernardi E. Upper extremity deep vein thrombosis. Curr Opin Pulm Med 1999; 5:222.
  39. Joffe HV, Kucher N, Tapson VF, et al. Upper-extremity deep vein thrombosis: a prospective registry of 592 patients. Circulation 2004; 110:1605.
  40. Ozçakar L, Dönmez G, Yörübulut M, et al. Paget-Schroetter syndrome forerunning the diagnoses of thoracic outlet syndrome and thrombophilia. Clin Appl Thromb Hemost 2010; 16:351.
  41. Bolitho DG, Elwood ET, Roberts F. Phlegmasia cerulea dolens of the upper extremity. Ann Plast Surg 2000; 45:644.
  42. Petritsch B, Wendel F, Hahn D, Goltz JP. Phlegmasia cerulea dolens of the arm. J Vasc Access 2012; 13:399.
  43. Kucher N, Tapson VF, Goldhaber SZ, DVT FREE Steering Committee. Risk factors associated with symptomatic pulmonary embolism in a large cohort of deep vein thrombosis patients. Thromb Haemost 2005; 93:494.
  44. Muñoz FJ, Mismetti P, Poggio R, et al. Clinical outcome of patients with upper-extremity deep vein thrombosis: results from the RIETE Registry. Chest 2008; 133:143.
  45. Stein PD, Matta F, Musani MH, Diaczok B. Silent pulmonary embolism in patients with deep venous thrombosis: a systematic review. Am J Med 2010; 123:426.
  46. Mai C, Hunt D. Upper-extremity deep venous thrombosis: a review. Am J Med 2011; 124:402.
  47. Inoue K, Saito J, Miyazaki M, et al. A Kendo player with haemoptysis. Lancet 2004; 364:814.
  48. Urschel HC Jr, Patel AN. Paget-Schroetter syndrome therapy: failure of intravenous stents. Ann Thorac Surg 2003; 75:1693.
  49. Kleinjan A, Di Nisio M, Beyer-Westendorf J, et al. Safety and feasibility of a diagnostic algorithm combining clinical probability, d-dimer testing, and ultrasonography for suspected upper extremity deep venous thrombosis: a prospective management study. Ann Intern Med 2014; 160:451.
  50. Merminod T, Pellicciotta S, Bounameaux H. Limited usefulness of D-dimer in suspected deep vein thrombosis of the upper extremities. Blood Coagul Fibrinolysis 2006; 17:225.
  51. Di Nisio M, Van Sluis GL, Bossuyt PM, et al. Accuracy of diagnostic tests for clinically suspected upper extremity deep vein thrombosis: a systematic review. J Thromb Haemost 2010; 8:684.
  52. Kerr TM, Lutter KS, Moeller DM, et al. Upper extremity venous thrombosis diagnosed by duplex scanning. Am J Surg 1990; 160:202.
  53. Knudson GJ, Wiedmeyer DA, Erickson SJ, et al. Color Doppler sonographic imaging in the assessment of upper-extremity deep venous thrombosis. AJR Am J Roentgenol 1990; 154:399.
  54. Sajid MS, Ahmed N, Desai M, et al. Upper limb deep vein thrombosis: a literature review to streamline the protocol for management. Acta Haematol 2007; 118:10.
  55. Prandoni P, Polistena P, Bernardi E, et al. Upper-extremity deep vein thrombosis. Risk factors, diagnosis, and complications. Arch Intern Med 1997; 157:57.
  56. Baarslag HJ, van Beek EJ, Koopman MM, Reekers JA. Prospective study of color duplex ultrasonography compared with contrast venography in patients suspected of having deep venous thrombosis of the upper extremities. Ann Intern Med 2002; 136:865.
  57. Mustafa BO, Rathbun SW, Whitsett TL, Raskob GE. Sensitivity and specificity of ultrasonography in the diagnosis of upper extremity deep vein thrombosis: a systematic review. Arch Intern Med 2002; 162:401.
  58. Chin EE, Zimmerman PT, Grant EG. Sonographic evaluation of upper extremity deep venous thrombosis. J Ultrasound Med 2005; 24:829.
  59. Hübsch PJ, Stiglbauer RL, Schwaighofer BW, et al. Internal jugular and subclavian vein thrombosis caused by central venous catheters. Evaluation using Doppler blood flow imaging. J Ultrasound Med 1988; 7:629.
  60. Haire WD, Lynch TG, Lund GB, et al. Limitations of magnetic resonance imaging and ultrasound-directed (duplex) scanning in the diagnosis of subclavian vein thrombosis. J Vasc Surg 1991; 13:391.
  61. Desjardins B, Rybicki FJ, Kim HS, et al. ACR Appropriateness Criteria® Suspected upper extremity deep vein thrombosis. J Am Coll Radiol 2012; 9:613.
  62. Matsumura JS, Rilling WS, Pearce WH, et al. Helical computed tomography of the normal thoracic outlet. J Vasc Surg 1997; 26:776.
  63. Kroencke TJ, Taupitz M, Arnold R, et al. Three-dimensional gadolinium-enhanced magnetic resonance venography in suspected thrombo-occlusive disease of the central chest veins. Chest 2001; 120:1570.
  64. Baarslag HJ, Van Beek EJ, Reekers JA. Magnetic resonance venography in consecutive patients with suspected deep vein thrombosis of the upper extremity: initial experience. Acta Radiol 2004; 45:38.
  65. Kim H, Chung JW, Park JH, et al. Role of CT venography in the diagnosis and treatment of benign thoracic central venous obstruction. Korean J Radiol 2003; 4:146.
  66. Ersoy H, Steigner ML, Coyner KB, et al. Vascular thoracic outlet syndrome: protocol design and diagnostic value of contrast-enhanced 3D MR angiography and equilibrium phase imaging on 1.5- and 3-T MRI scanners. AJR Am J Roentgenol 2012; 198:1180.
  67. Girolami A, Prandoni P, Zanon E, et al. Venous thromboses of upper limbs are more frequently associated with occult cancer as compared with those of lower limbs. Blood Coagul Fibrinolysis 1999; 10:455.
  68. Becker DM, Philbrick JT, Walker FB 4th. Axillary and subclavian venous thrombosis. Prognosis and treatment. Arch Intern Med 1991; 151:1934.
  69. Naeem M, Soares G, Ahn S, Murphy TP. Paget-Schroetter syndrome: A review and Algorithm (WASPS-IR). Phlebology 2015; 30:675.
  70. Keir G, Marshall MB. Management Strategy for Patients With Chronic Subclavian Vein Thrombosis. Ann Thorac Surg 2017; 103:672.
  71. Melby SJ, Vedantham S, Narra VR, et al. Comprehensive surgical management of the competitive athlete with effort thrombosis of the subclavian vein (Paget-Schroetter syndrome). J Vasc Surg 2008; 47:809.
  72. Caparrelli DJ, Freischlag J. A unified approach to axillosubclavian venous thrombosis in a single hospital admission. Semin Vasc Surg 2005; 18:153.
  73. Stevens SM, Woller SC, Kreuziger LB, et al. Antithrombotic Therapy for VTE Disease: Second Update of the CHEST Guideline and Expert Panel Report. Chest 2021; 160:e545.
  74. Kearon C, Akl EA, Ornelas J, et al. Antithrombotic Therapy for VTE Disease: CHEST Guideline and Expert Panel Report. Chest 2016; 149:315.
  75. Kearon C, Akl EA, Comerota AJ, et al. Antithrombotic therapy for VTE disease: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. Chest 2012; 141:e419S.
  76. Machleder HI. Evaluation of a new treatment strategy for Paget-Schroetter syndrome: spontaneous thrombosis of the axillary-subclavian vein. J Vasc Surg 1993; 17:305.
  77. Rathbun SW, Stoner JA, Whitsett TL. Treatment of upper-extremity deep vein thrombosis. J Thromb Haemost 2011; 9:1924.
  78. Thompson JF, Winterborn RJ, Bays S, et al. Venous thoracic outlet compression and the Paget-Schroetter syndrome: a review and recommendations for management. Cardiovasc Intervent Radiol 2011; 34:903.
  79. Stone DH, Scali ST, Bjerk AA, et al. Aggressive treatment of idiopathic axillo-subclavian vein thrombosis provides excellent long-term function. J Vasc Surg 2010; 52:127.
  80. Lee WA, Hill BB, Harris EJ Jr, et al. Surgical intervention is not required for all patients with subclavian vein thrombosis. J Vasc Surg 2000; 32:57.
  81. Strange-Vognsen HH, Hauch O, Andersen J, Struckmann J. Resection of the first rib, following deep arm vein thrombolysis in patients with thoracic outlet syndrome. J Cardiovasc Surg (Torino) 1989; 30:430.
  82. de León RA, Chang DC, Hassoun HT, et al. Multiple treatment algorithms for successful outcomes in venous thoracic outlet syndrome. Surgery 2009; 145:500.
  83. Urschel HC Jr, Razzuk MA. Improved management of the Paget-Schroetter syndrome secondary to thoracic outlet compression. Ann Thorac Surg 1991; 52:1217.
  84. Donayre CE, White GH, Mehringer SM, Wilson SE. Pathogenesis determines late morbidity of axillosubclavian vein thrombosis. Am J Surg 1986; 152:179.
  85. AbuRahma AF, Sadler D, Stuart P, et al. Conventional versus thrombolytic therapy in spontaneous (effort) axillary-subclavian vein thrombosis. Am J Surg 1991; 161:459.
  86. Skalicka L, Lubanda JC, Jirat S, et al. Endovascular treatment combined with stratified surgery is effective in the management of venous thoracic outlet syndrome complications: a long term ultrasound follow-up study in patients with thrombotic events due to venous thoracic outlet syndrome. Heart Vessels 2011; 26:616.
  87. Lee MC, Grassi CJ, Belkin M, et al. Early operative intervention after thrombolytic therapy for primary subclavian vein thrombosis: an effective treatment approach. J Vasc Surg 1998; 27:1101.
  88. Gloviczki P, Kazmier FJ, Hollier LH. Axillary-subclavian venous occlusion: the morbidity of a nonlethal disease. J Vasc Surg 1986; 4:333.
  89. Prandoni P, Bernardi E, Marchiori A, et al. The long term clinical course of acute deep vein thrombosis of the arm: prospective cohort study. BMJ 2004; 329:484.
  90. Povlsen B, Belzberg A, Hansson T, Dorsi M. Treatment for thoracic outlet syndrome. Cochrane Database Syst Rev 2010; :CD007218.
  91. Molina JE, Hunter DW, Dietz CA. Protocols for Paget-Schroetter syndrome and late treatment of chronic subclavian vein obstruction. Ann Thorac Surg 2009; 87:416.
  92. Urschel HC Jr, Razzuk MA. Neurovascular compression in the thoracic outlet: changing management over 50 years. Ann Surg 1998; 228:609.
  93. Hrubý J, Semrád M, Vidim T, et al. [Outcomes of combined surgical and endovascular treatment of the venous thoracic outlet syndrome during 2000-2007 in the IInd Surgical Clinic of the VFN (General Faculty Hospital) and 1. LF UK (First Medical Faculty, Charles University) in Prague]. Rozhl Chir 2010; 89:69.
  94. Angle N, Gelabert HA, Farooq MM, et al. Safety and efficacy of early surgical decompression of the thoracic outlet for Paget-Schroetter syndrome. Ann Vasc Surg 2001; 15:37.
  95. Vik A, Holme PA, Singh K, et al. Catheter-directed thrombolysis for treatment of deep venous thrombosis in the upper extremities. Cardiovasc Intervent Radiol 2009; 32:980.
  96. Lee JT, Karwowski JK, Harris EJ, et al. Long-term thrombotic recurrence after nonoperative management of Paget-Schroetter syndrome. J Vasc Surg 2006; 43:1236.
  97. Molina JE, Hunter DW, Dietz CA. Paget-Schroetter syndrome treated with thrombolytics and immediate surgery. J Vasc Surg 2007; 45:328.
  98. Schneider DB, Dimuzio PJ, Martin ND, et al. Combination treatment of venous thoracic outlet syndrome: open surgical decompression and intraoperative angioplasty. J Vasc Surg 2004; 40:599.
  99. Guzzo JL, Chang K, Demos J, et al. Preoperative thrombolysis and venoplasty affords no benefit in patency following first rib resection and scalenectomy for subacute and chronic subclavian vein thrombosis. J Vasc Surg 2010; 52:658.
  100. Tilney ML, Griffiths HJ, Edwards EA. Natural history of major venous thrombosis of the upper extremity. Arch Surg 1970; 101:792.
  101. Stevens SM, Woller SC, Baumann Kreuziger L, et al. Executive Summary: Antithrombotic Therapy for VTE Disease: Second Update of the CHEST Guideline and Expert Panel Report. Chest 2021; 160:2247.
  102. van Dongen CJ, van den Belt AG, Prins MH, Lensing AW. Fixed dose subcutaneous low molecular weight heparins versus adjusted dose unfractionated heparin for venous thromboembolism. Cochrane Database Syst Rev 2004; :CD001100.
  103. Karabay O, Yetkin U, Onol H. Upper extremity deep vein thrombosis: clinical and treatment characteristics. J Int Med Res 2004; 32:429.
  104. Savage KJ, Wells PS, Schulz V, et al. Outpatient use of low molecular weight heparin (Dalteparin) for the treatment of deep vein thrombosis of the upper extremity. Thromb Haemost 1999; 82:1008.
  105. Enden T, Sandvik L, Kløw NE, et al. Catheter-directed Venous Thrombolysis in acute iliofemoral vein thrombosis--the CaVenT study: rationale and design of a multicenter, randomized, controlled, clinical trial (NCT00251771). Am Heart J 2007; 154:808.
  106. Schulman S, Beyth RJ, Kearon C, Levine MN. Hemorrhagic complications of anticoagulant and thrombolytic treatment: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines (8th Edition). Chest 2008; 133:257S.
  107. Schulman S, Lindmarker P, Holmström M, et al. Post-thrombotic syndrome, recurrence, and death 10 years after the first episode of venous thromboembolism treated with warfarin for 6 weeks or 6 months. J Thromb Haemost 2006; 4:734.
  108. Alesh I, Kayali F, Stein PD. Catheter-directed thrombolysis (intrathrombus injection) in treatment of deep venous thrombosis: a systematic review. Catheter Cardiovasc Interv 2007; 70:143.
  109. Shah AD, Bajakian DR, Olin JW, Lookstein RA. Power-pulse spray thrombectomy for treatment of Paget-Schroetter syndrome. AJR Am J Roentgenol 2007; 188:1215.
  110. Oklu R, Wicky S. Catheter-directed thrombolysis of deep venous thrombosis. Semin Thromb Hemost 2013; 39:446.
  111. Kim HS, Patra A, Paxton BE, et al. Catheter-directed thrombolysis with percutaneous rheolytic thrombectomy versus thrombolysis alone in upper and lower extremity deep vein thrombosis. Cardiovasc Intervent Radiol 2006; 29:1003.
  112. Dasari TW, Pappy R, Hennebry TA. Pharmacomechanical thrombolysis of acute and chronic symptomatic deep vein thrombosis: a systematic review of literature. Angiology 2012; 63:138.
  113. Lugo J, Tanious A, Armstrong P, et al. Acute Paget-Schroetter syndrome: does the first rib routinely need to be removed after thrombolysis? Ann Vasc Surg 2015; 29:1073.
  114. Urschel HC Jr. Management of the thoracic-outlet syndrome. N Engl J Med 1972; 286:1140.
  115. Molina JE. Surgery for effort thrombosis of the subclavian vein. J Thorac Cardiovasc Surg 1992; 103:341.
  116. Chang KZ, Likes K, Demos J, et al. Routine venography following transaxillary first rib resection and scalenectomy (FRRS) for chronic subclavian vein thrombosis ensures excellent outcomes and vein patency. Vasc Endovascular Surg 2012; 46:15.
  117. Martinez BD, Albeshri H, Chulkov M, et al. Development and evolution of a robotic surgical technique for the treatment of thoracic outlet syndrome. J Vasc Surg 2021; 74:938.
  118. Gharagozloo F, Atiquzzaman N, Meyer M, et al. Robotic first rib resection for thoracic outlet syndrome. J Thorac Dis 2021; 13:6141.
  119. Feugier P, Aleksic I, Salari R, et al. Long-term results of venous revascularization for Paget-Schroetter syndrome in athletes. Ann Vasc Surg 2001; 15:212.
  120. Meier GH, Pollak JS, Rosenblatt M, et al. Initial experience with venous stents in exertional axillary-subclavian vein thrombosis. J Vasc Surg 1996; 24:974.
  121. Oderich GS, Treiman GS, Schneider P, Bhirangi K. Stent placement for treatment of central and peripheral venous obstruction: a long-term multi-institutional experience. J Vasc Surg 2000; 32:760.
  122. Maintz D, Landwehr P, Gawenda M, Lackner K. Failure of Wallstents in the subclavian vein due to stent damage. Clin Imaging 2001; 25:133.
  123. Heit JA, Mohr DN, Silverstein MD, et al. Predictors of recurrence after deep vein thrombosis and pulmonary embolism: a population-based cohort study. Arch Intern Med 2000; 160:761.
  124. Molina JE. Reoperations after failed transaxillary first rib resection to treat Paget-Schroetter syndrome patients. Ann Thorac Surg 2011; 91:1717.
  125. Flinterman LE, van Hylckama Vlieg A, Rosendaal FR, Doggen CJ. Recurrent thrombosis and survival after a first venous thrombosis of the upper extremity. Circulation 2008; 118:1366.
  126. Samoila G, Twine CP, Williams IM. The infraclavicular approach for Paget-Schroetter syndrome. Ann R Coll Surg Engl 2018; 100:83.
  127. Gabriel F, Portolés O, Labiós M, et al. Usefulness of thrombophilia testing in venous thromboembolic disease: findings from the RIETE registry. Clin Appl Thromb Hemost 2013; 19:42.
  128. Cassada DC, Lipscomb AL, Stevens SL, et al. The importance of thrombophilia in the treatment of Paget-Schroetter syndrome. Ann Vasc Surg 2006; 20:596.
  129. Leebeek FW, Stadhouders NA, van Stein D, et al. Hypercoagulability states in upper-extremity deep venous thrombosis. Am J Hematol 2001; 67:15.
  130. Héron E, Lozinguez O, Alhenc-Gelas M, et al. Hypercoagulable states in primary upper-extremity deep vein thrombosis. Arch Intern Med 2000; 160:382.
  131. Persson LM, Arnhjort T, Lärfars G, Rosfors S. Hemodynamic and morphologic evaluation of sequelae of primary upper extremity deep venous thromboses treated with anticoagulation. J Vasc Surg 2006; 43:1230.
Topic 8211 Version 25.0

References

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