Paget-Schroetter syndrome

Background

• axillary or subclavian vein thrombosis associated with strenuous and repetitive activity of the upper extremities affecting usually young athletic people

Clinical Features

• upper extremity swelling and discomfort, usually unilateral
• redness of the upper extremity
• dilated visible veins around the shoulder (Urschel’s sign)
• cyanosis
• may have a discrete precipitating event
  • usually sports-related arm exertion
• may be acute, subacute or chronic

Differential Diagnosis

• Thoracic outlet obstruction/Pancoast tumor
• lymphatic obstruction
• SVC syndrome
• cellulitis
• necrotizing fasciitis

Workup

• CBC, CMP, coags
• consider D-dimer
• Chest X-ray to rule out anatomic abnormalities or lung masses that might cause thoracic outlet obstruction
• Ultrasound with color Doppler- preferred initial test (sensitivity 78-100%, specificity 82-100%)
• MRI venography (noninvasive, but expensive and limited availability)
• Gold standard- contrast venography (use when ultrasound findings are equivocal but still have high clinical suspicion)

Management

1. Anticoagulation
   1. LMWH, Fondaparinux, Unfractionated Heparin
      1. Choice depends on further plans for intervention and disposition
      2. Bridge to Coumadin
2. Thrombolysis
   1. Catheter directed infusion of alteplase or urokinase
   2. For moderate to severe cases
3. Surgical decompression
   1. For moderate to severe cases

Disposition

• Depends on the severity of symptoms and the acuity of presentation
  • Mild/intermittent/chronic (>2weeks) symptoms
    • Outpatient management with LMWH bridging to Coumadin
  • Severe/acute presentation
    • Admit, consult vascular surgery for thrombectomy or thrombolysis

See Also

• Ultrasound: DVT
• DVT (Deep Venous Thrombosis)

Sources

1. Alla V, Ntarajan N, Kaushik M, Warrier R, Nair C. Paget-Schroetter Syndrome: Review of Pathogenesis and Treatment of Effort Thrombosis. Western Journal of Emergency Medicine, Vol XI, No 4, 2010, p 358
2. Chin E, Zimmerman P, Grant E. Sonographic Evaluation of Upper Extremity DVT. J Ultrasound Med, 2005; 24:829-838
3. Goshima K, White M. Overview of Thoracic Outlet Syndromes. UpToDate, 2012
4. Goshima K. Primary (spontaneous) Upper Extremity Deep Venous Thrombosis. UpToDate, 2013