Paget-Schroetter Syndrome

Vascular Atlas

A 20 year old male presents to the emergency department with right upper extremity swelling, heaviness, and bluish discoloration. He is an aspiring professional baseball player and has recently been engaged in an intense training program. The swelling began 2 days prior to his presentation and progressed to the sensation of right arm heaviness and eventually to a bluish-gray discoloration. He denied nutritional supplements including steroids. Duplex ultrasound of the upper extremity revealed extensive subclavian-axillary thrombosis. Unfractionated heparin infusion was initiated and he was taken to the catheterization laboratory where he underwent placement of an ultrasound-assisted thrombolytic catheter. After 24 hours of low dose tissue plasminogen activator infusion (1 mg/hour), repeat angiography showed some vessel recanalization, however, with significant residual vein thrombosis. Subsequent balloon angioplasty revealed evidence of external venous compression. The patient was referred to orthopedic surgery and sports medicine for consideration of first rib resection.

Paget-Schroetter 2

Paget-Schroetter Syndrome, also described as effort thrombosis, is a clinical syndrome characterized by the venous complications of thoracic outlet syndrome. External venous compression causes intimal microtrauma, stasis, and ultimately thrombosis. The syndrome is classically described in athletes and those subject to repetitive upper extremity movements. Congenital anatomic anomalies including cervical ribs, costosternal ligament and scalene muscle hypertrophy are often implicated. Physical exam is typically significant for unilateral swollen extremity, and in more chronic cases, the classic venous collaterals known as Urschel’s sign may be present.

Diagnosis is often made with Duplex compression ultrasonography with angiography described as the diagnostic gold standard. Additional imaging to rule out anatomic variations should be done including lateral cervical spine X-rays to screen for cervical ribs. If no apparent anatomic anomalies are found, hypercoagubility and age-appropriate malignancy screening should be done.
Treatment strategies should be individually tailored however correction of any anatomical compression is paramount to reduce the risk of recurrence. Standard therapy includes, at a minimum, anticoagulation for at least 3 months. For patients presenting within 14 days of symptoms onset, catheter based therapies including thrombolytics, thrombectomy, and angioplasty without stenting, should be considered to reduce the risk of post-thrombotic syndrome.

References:

  1. Venkata M. Alla, Nagendra Natarajan,et al. Paget-Schroetter Syndrome: Review of Pathogenesis and Treatment of Effort Thrombosis. West J Emerg Med. 2010 September; 11(4): 358–362.
  2. Urschel HC Jr, Patel AN. Surgery remains the most effective treatment for Paget-Schroetter syndrome: 50 years’ experience. Ann Thorac Surg. 2008 Jul;86(1):254-60.
  3. Kearon C, Akl EA, Comerota AJ, Prandoni P, et al. American College of Chest Physicians. 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 Feb;141(2Suppl):e419S-94S.

About Author

Ari J. Mintz, DO

Ari J. Mintz, DO is an internal medicine resident at Lahey Hospital and Medical Center in Burlington, MA. His clinical interests include peripheral arterial disease, noninvasive imaging, and advanced therapeutic approaches to venous thromboembolism. 

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