Urgent message: Vigilance for risk factors and diagnostic methods related to Paget-Schroetter syndrome in patients presenting to urgent care with relevant risk factors is essential to initiating therapy on site and timely referral to a hospital and/or vascular surgeon.
Darshan Shah, MD, Shikhar Saxena, MD and Shailendra Saxena, MD, PhD
Upper extremity deep venous thrombosis (UEDVT) is estimated to constitute 1% to 4% of all cases of deep venous thrombosis (DVT).1 The incidence may be higher, however, because many patients with UEDVT are asymptomatic. This is important to note, as the consequences of undiagnosed UEDVT resulting in pulmonary embolism are the same as DVT in lower exterminates. Urgent care and primary care physicians must maintain a high index of suspicion to evaluate unilateral upper extremity swelling as a UEDVT. Here, we present a case of UEDVT/Paget-Schroetter syndrome in a young athletic man who happens to be a weightlifter.
A previously healthy 31-year-old man presented with a two-week history of right-side upper extremity swelling. He also had mild tingling and numbness in the right hand, mostly along the inner border of the hand. There was no history of trauma. He is a non-smoker and currently not taking any medications. There was no family history of venous thromboembolism. He had mild pain, but was mainly concerned about swollen biceps. He goes to the gym and lifts weight regularly.
On examination, vitals were stable.
The examination of HEENT, lungs, heart, abdomen and lower extremities was normal.
A focus examination of right upper extremity showed swollen upper arm extending up to the axilla. There was superficial venous congestion overlying the biceps brachii and deltoid muscles. There was no evidence of arterial insufficiency or neurological deficit. There was no axillary or supraclavicular lymphadenopathy.
The patient had an extensive laboratory work-up; the CBC, CMP, thyroid panel, lipid profile, D-dimer, proteins C and S, antithrombin levels, and factor V were all within normal limits.
The chest radiograph did not show any evidence of cervical rib.
Subsequently, the patient had venous duplex imaging of the right upper extremity, which confirmed a thrombus in the right subclavian vein. A venogram also confirmed the diagnosis of thrombus in the distal right subclavian vein (Figure 1).
Figure 1. Venogram showing a blocked and compressed subclavian vein.
The patient also had spiral CT of chest, which demonstrated bilateral lower lobe acute sub segmental pulmonary emboli.
A vascular surgeon was consulted, and thrombolysis was commenced on the day of admission with infusion of tissue plasminogen activator (TPA) following cannulation of the right subclavian vein via the basilic vein.
The patient was anticogulated using low molecular weight heparin (LMWH).
Venography performed two days later demonstrated resolving thrombus (Figure 2). However, a repeat CT revealed extrinsic compression of the right subclavian vein as it crosses the first rib. Hence, an uncomplicated right-sided first-rib resection along with middle scalenectomy was performed to eliminate the compression of the subclavian vein.
Figure 2. Venogram showing a recanalized subclavian vein post TPA therapy.
The patient was discharged on warfarin for three months and the INR was monitored closely to keep the value between 2 to 3.
The patient was re-evaluated every month and he improved significantly. He resumed all of his activities within three to four months after surgery without any residual symptoms.
UEDVT and Paget-Schroetter Syndrome
Paget-Schroetter is a symptomatic compression of the subclavian vein as it passes through the narrow space between skeletal and muscular component of the shoulder girdle.2,3
The anatomy of thoracic outlet is complicated. Neurovascular structures pass through a narrow space to enter the upper extremity. The compression of subclavian is usually due to overdeveloped anterior scalene muscle in a healthy young man who is engaged in vigorous exercise to develop neck and upper extremity muscles.4,5 The venous compression results into stasis, intimal changes, and inflammation resulting into thrombosis.
Risk Factors for Paget-Schroetter Syndrome
The risk factors implicated in UEDVT are similar to those for venous thrombosis elsewhere in the body. However, specific anatomical changes at the thoracic outlet resulting into compression of subclavian vein are usually attributed as risk factors for Paget-Schroetter syndrome6 (Table 1).
Table 1. Risk factors for Paget-Schroetter Syndrome
- Physical activity involving hyperabduction of the shoulder, as seen in weight lifters
- Motions often associated with tennis players and baseball pitchers
- Vigorous exercise of the neck and upper extremity muscles
- Overdeveloped anterior scalene muscle
- Rudimentary first rib
- Presence of cervical rib
- Congenital band between first and second ribs
- Fracture of the clavicle with callus formation
- Apical tumors of the superior sulcus of the lung (Pancoast tumor)
- Thoracic outlet syndrome
The clinical presentation of patients with Paget-Schroetter syndrome is summarized in Table 2. However, only 50% of patients presenting these symptoms have objective findings of UEDVT.
The patients may also present with complications associated with Paget-Schroetter syndrome. Physicians should have high index of suspicion in diagnosis Paget-Schroetter syndrome because most of the patients lack classical signs and symptoms of DVT. Recognizing the relevant risk facilitates early.
Table 2: Clinical Presentation of Paget-Schroetter Syndrome
- Pain in the arm, neck and shoulder region
- Diffuse arm swelling
- Discoloration, tenderness and venous distension on the affected arm
- Visible collateral veins on the affected arm
- Arm discoloration and palpable axillary vein
The differential diagnosis for Paget-Schroetter includes:
- lymphedema of the upper extremity
- localized hematoma in biceps muscle
- muscle contusion and strain
- superficial phlebitis
Patients with Paget-Schroetter may initially present as symptoms and signs associated with UEDVT (Table 3).
Table 3. Complications Associated with Paget-Schroetter Syndrome
- Signs and symptoms of superior vena cava syndrome
- Signs and symptoms of pulmonary embolism
- Venous gangrene of upper extremity
- Post-thrombotic syndrome
- Signs and symptoms of systemic sepsis secondary to infection of thrombus
Diagnosis of Paget-Schroetter is confirmed by imaging of the axillary-subclavian venous system. Various non-invasive and invasive methods are listed in Table 4. Though not all will be accessible in all urgent care facilities, physicians should use them before initiating definitive therapy for Paget-Schroetter syndrome. Therefore, referral may be indicated.
Table 4. Non-invasive and Invasive Methods to Diagnose Paget-Schroetter Syndrome
- B-mode ultrasonography: most widely used procedure
- Duplex ultrasonography
- Color Doppler ultrasonography
- Venography: standard for the diagnosis of Paget-Schroetter syndrome and UEDVT
- Magnetic resonance imaging (MRI)
- Computed tomography (CT)
Therapy of Paget-Schroetter Syndrome/UEDVT
The primary aim of therapy for UEDVT and Paget-Schroetter syndrome is to halt the progression of thrombi, prevent embolism, and allow natural thrombolytic mechanism to work effectively.7 It is likely this will be accomplished in a hospital setting, but treatment options are described here to present a fuller picture of the entire patient experience.
It is important to find the anatomical cause of venous compression in Paget-Schroetter syndrome, which is often at the level of first rib. Therefore, transaxillary resection of first rib is advised for decompression of vein.
The most widely recommended treatment for Paget-Schroetter syndrome is a staged, multimodal approached developed by Machleder, et al in 1993.8 It involves trascatheter thrombolytic therapy followed by transaxillary resection of first rib, and then anticoagulation with warfarin sodium for three months.
Low-dose urokinase and recombinant tissue plasminogen activater (rt-PA) are commonly used as thrombolytic agents. They are safe and effective for restoring the patency of an occluded vein.
The surgical resection of the first rib to decompress thoracic outlet is usually performed three weeks after thrombolysis to allow time for endothelial repair and to decrease the risk of recurrent thrombosis.5
Balloon angioplasty may also be used in cases of residual stricture.5 However, in the acute phase, most patients of UEDVT and Paget-Schroetter syndrome receive full-dose anticoagulation with high or low molecular weight heparin followed by several months of therapy with oral warfarin sodium.
Placement of a superior vena cava (SVC) filter is indicated in patients with UEDVT or Paget-Schroetter syndrome in whom anticoagulation is contraindicated. This will prevent pulmonary embolism.
The thrombosis in subclavian vein due to compression at thoracic outlet in a health young man was identified more than 100 years ago as “effort thrombosis,” or Paget-Schroetter syndrome.3
The etiology of subclavian vein thrombosis is often secondary to bony and muscular compression at the thoracic outlet. The syndrome is usually seen in healthy young males who are often engaged in vigorous exercise to develop upper extremity muscles. The presenting symptoms are acute swelling and pain in the region of biceps brachii and deltoid muscles and usually in the dominant arm.
It is often confused with rotator cuff tendonitis at the shoulder joint; thus, delays in diagnosis of subclavian vein compression. The diagnosis can readily confirmed by duplex ultrasonography or contrast venography.
In the acute phase, a patient’s treatment should be started with anticoagulation using unfractionated heparin or low-molecular weight heparin. However, the urgent care and primary care physicians should involve a vascular surgeon as early as possible for effective management of Paget-Schroetter syndrome.
Early thrombolysis has been shown to improve recanalization and patency of subclavian vein when compared with antocogulation alone. Failure to restore patency of subclavian vein earlier may result into pulmonary embolism or residual defect.
Management of residual defect is hard, controversial, and may require balloon angioplasty. All evidence, however, advocates an early aggressive approach for both the investigation and management of patients with Paget-Schroetter syndrome.
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