Paget–Schroetter disease explained

Paget–Schrotter disease
Synonyms:Paget–von Schrötter disease
Field:vascular surgery

Paget–Schroetter disease (which evolved from a venous thoracic outlet syndrome) is a form of upper extremity deep vein thrombosis (DVT), a medical condition in which blood clots form in the deep veins of the arms. These DVTs typically occur in the axillary and/or subclavian veins.[1]

Signs and symptoms

The condition is relatively rare.[2] 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,[3] as it has been reported to occur after vigorous activity,[4] though it can also occur due to anatomic abnormality such as clavicle impingement[5] 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.[6]

Symptoms may include sudden onset of pain, warmth, redness, blueness and swelling in the arm. Diagnosis is usually confirmed with an ultrasound.[7] These DVTs have the potential to cause a pulmonary embolism.[8]

Diagnosis

Duplex ultrasonographyMR Venography

Prevention

Prevention of Paget–Schroetter disease can be accomplished by gradual increases in activity and by avoiding strenuous upper extremity activity.[9]

Treatment

The traditional treatment for thrombosis is the same as for a lower extremity DVT, and involves systemic anticoagulation to prevent a pulmonary embolus.[10] 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.[11] If there is thoracic outlet syndrome or other anatomical cause then surgery can be considered to correct the underlying defect.[12]

History

The condition is named after two men. James Paget first proposed the idea of venous thrombosis causing upper extremity pain and swelling,[13] and Leopold von Schrötter later linked the clinical syndrome to thrombosis of the axillary and subclavian veins.[14]

Notes and References

  1. Venous obstruction in the upper extremity; Paget-Schroetter's syndrome; a review of 320 cases. Surgery, Gynecology & Obstetrics. 1949-02-01. 0039-6087. 18108679. 89–127. 88. 2. E. S. R.. Hughes.
  2. Hughes . ES . Venous obstruction in the upper extremity; Paget–Schroetter's syndrome; a review of 320 cases . Surg Gynecol Obstet . 1949 . 2 . 18108679 . 88 . 89–127.
  3. Drapanas, T . Curran, WL . Thrombectomy in the treatment of "effort" thrombosis of the axillary and subclavian veins . Journal of Trauma . 6 . 1966 . 6. 107–19 . 10.1097/00005373-196601000-00012 . 5901846 .
  4. Current perspective of venous thrombosis in the upper extremity . Flinterman LE, Van Der Meer FJ, Rosendaal FR, Doggen CJ . Journal of Thrombosis and Haemostasis . Aug 2008 . 6 . 1262–6 . 18485082 . 8 . 10.1111/j.1538-7836.2008.03017.x. free .
  5. Clavicular Fracture and Upper-Extremity Deep Venous Thrombosis. Orthopedics. 227. 34. 3. 10.3928/01477447-20110124-28. 21410116. Mohammad Taghi. Peivandi. Zohreh. Nazemian. 2011.
  6. Reina . Nick J. . Honet . Joseph C. . Brown . William . Beitman . Max . Chodoroff . Gary . Paget-Schroetter syndrome in a viola player . Medical Problems of Performing Artists . 3 . 1 . 24 . 1988.
  7. Accuracy of diagnostic tests for clinically suspected upper extremity deep vein thrombosis: a systematic review. Journal of Thrombosis and Haemostasis. 2010-04-01. 1538-7836. 20141579. 684–692. 8. 4. 10.1111/j.1538-7836.2010.03771.x. M.. Di Nisio. G. L.. Van Sluis. P. M. M.. Bossuyt. H. R.. Büller. E.. Porreca. A. W. S.. Rutjes. free.
  8. Upper-extremity deep venous thrombosis: a review. The American Journal of Medicine. 2011-05-01. 1555-7162. 21531227. 402–407. 124. 5. 10.1016/j.amjmed.2010.11.022. Cuc. Mai. Daniel. Hunt.
  9. Madden CC, Putukian M, Young CC, McCarty EC. Netter's Sports Medicine. Saunders. Philadelphia, 2010.
  10. Antithrombotic therapy for VTE disease: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. Chest. 2012-02-01. 1931-3543. 3278049. 22315268. e419S–94S. 141. 2 Suppl. 10.1378/chest.11-2301. Clive. Kearon. Elie A.. Akl. Anthony J.. Comerota. Paolo. Prandoni. Henri. Bounameaux. Samuel Z.. Goldhaber. Michael E.. Nelson. Philip S.. Wells. Michael K.. Gould.
  11. Catheter-directed thrombolysis for treatment of deep venous thrombosis in the upper extremities. CardioVascular and Interventional Radiology. 2009-09-01. 1432-086X. 19641959. 980–987. 32. 5. 10.1007/s00270-009-9655-y. Anders. Vik. Pål Andre. Holme. Kulbir. Singh. Eric. Dorenberg. Kåre Christian. Nordhus. Satish. Kumar. John-Bjarne. Hansen. 8572126.
  12. Venous thoracic outlet compression and the Paget-Schroetter syndrome: a review and recommendations for management. CardioVascular and Interventional Radiology. 2011-10-01. 1432-086X. 21448772. 903–910. 34. 5. 10.1007/s00270-011-0148-4. J. F.. Thompson. R. J.. Winterborn. S.. Bays. H.. White. D. C.. Kinsella. A. F.. Watkinson. 13162412.
  13. Paget J . On gouty and some other forms of phlebitis . St. Bartholomew's Hospital Reports . 2 . 82–92 . 1866.
  14. 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.

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