Paget–Schroetter disease

Signs and symptoms

The condition is relatively rare. It usually presents in young and otherwise healthy patients, and also occurs more often in males than females. The syndrome also became known as "effort-induced thrombosis" in the 1960s, as it has been reported to occur after vigorous activity, though it can also occur due to anatomic abnormality such as clavicle impingement or spontaneously. It may develop as a sequela of thoracic outlet syndrome. It is differentiated from secondary causes of upper extremity thrombosis caused by intravascular catheters. Paget–Schroetter syndrome was described once for a viola player who suddenly increased practice time 10-fold, creating enough repetitive pressure against the brachiocephalic and external jugular veins to cause thrombosis.

Symptoms may include sudden onset of pain, warmth, redness, blueness and swelling in the arm. Diagnosis is usually confirmed with an ultrasound. These DVTs have the potential to cause a pulmonary embolism.

Diagnosis

Duplex ultrasonography MR Venography

Prevention

Prevention of Paget–Schroetter disease can be accomplished by gradual increases in activity and by avoiding strenuous upper extremity activity.

Treatment

The traditional treatment for thrombosis is the same as for a lower extremity DVT, and involves systemic anticoagulation to prevent a pulmonary embolus. Some have also recommended thrombolysis with catheter-directed alteplase or mechanical thrombectomy with a large-bore catheter and manual aspiration providing definitive intervention with an endovascular approach. If there is thoracic outlet syndrome or other anatomical cause then surgery can be considered to correct the underlying defect.

History

The condition is named after two men. James Paget first proposed the idea of venous thrombosis causing upper extremity pain and swelling, and Leopold von Schrötter later linked the clinical syndrome to thrombosis of the axillary and subclavian veins.

References

References

1. Hughes, E. S. R.. (1949-02-01). "Venous obstruction in the upper extremity; Paget-Schroetter's syndrome; a review of 320 cases". Surgery, Gynecology & Obstetrics.
2. Hughes, ES. (1949). "Venous obstruction in the upper extremity; Paget–Schroetter's syndrome; a review of 320 cases". Surg Gynecol Obstet.
3. (1966). "Thrombectomy in the treatment of "effort" thrombosis of the axillary and subclavian veins". Journal of Trauma.
4. (Aug 2008). "Current perspective of venous thrombosis in the upper extremity". Journal of Thrombosis and Haemostasis.
5. (2011). "Clavicular Fracture and Upper-Extremity Deep Venous Thrombosis". Orthopedics.
6. (1988). "Paget-Schroetter syndrome in a viola player". Medical Problems of Performing Artists.
7. (2010-04-01). "Accuracy of diagnostic tests for clinically suspected upper extremity deep vein thrombosis: a systematic review". Journal of Thrombosis and Haemostasis.
8. (2011-05-01). "Upper-extremity deep venous thrombosis: a review". The American Journal of Medicine.
9. Madden CC, Putukian M, Young CC, McCarty EC. Netter's Sports Medicine. Saunders. Philadelphia, 2010.
10. (2012-02-01). "Antithrombotic therapy for VTE disease: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines". Chest.
11. (2009-09-01). "Catheter-directed thrombolysis for treatment of deep venous thrombosis in the upper extremities". CardioVascular and Interventional Radiology.
12. (2011-10-01). "Venous thoracic outlet compression and the Paget-Schroetter syndrome: a review and recommendations for management". CardioVascular and Interventional Radiology.
13. {{WhoNamedIt. synd. 1924. Paget-von Schrötter disease
14. Paget J. (1866). "On gouty and some other forms of phlebitis". St. Bartholomew's Hospital Reports.
15. L. von Schrötter. Erkrankungen der Gefässe. Nothnagel’s Handbuch der speciellen Pathologie und Therapie, 1901. Volume XV, II. Theil, II. Hälfte: Erkrankungen der Venen. Wien, Hölder, 1899: 533–535.