Paget–Schroetter disease

For other diseases named after Paget, see Paget's disease (disambiguation).
Paget-Schrotter disease
Other names: Paget–von Schrötter disease
Anterior view of right upper limb and thorax
SpecialtyVascular surgery

Paget–Schroetter disease (also known as 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.[4] 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

Paget-Schroetter syndrome -Prethrombolysis venogram

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. [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[13] first proposed the idea of venous thrombosis causing upper extremity pain and swelling,[14] and Leopold von Schrötter later linked the clinical syndrome to thrombosis of the axillary and subclavian veins.[15]

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. 88 (2): 89–127. ISSN 0039-6087. PMID 18108679.
  2. ↑ Hughes, ES (1949). "Venous obstruction in the upper extremity; Paget–Schroetter's syndrome; a review of 320 cases". Surg Gynecol Obstet. 88 (2): 89–127. PMID 18108679.
  3. ↑ Drapanas, T; Curran, WL (1966). "Thrombectomy in the treatment of "effort" thrombosis of the axillary and subclavian veins". Journal of Trauma. 6 (6): 107–19. doi:10.1097/00005373-196601000-00012. PMID 5901846.
  4. 1 2 Flinterman LE, Van Der Meer FJ, Rosendaal FR, Doggen CJ (Aug 2008). "Current perspective of venous thrombosis in the upper extremity" (PDF). Journal of Thrombosis and Haemostasis. 6 (8): 1262–6. doi:10.1111/j.1538-7836.2008.03017.x. PMID 18485082.
  5. ↑ Peivandi, Mohammad Taghi; Nazemian, Zohreh (2011). "Clavicular Fracture and Upper-Extremity Deep Venous Thrombosis". Orthopedics. 34 (3): 227. doi:10.3928/01477447-20110124-28. PMID 21410116.
  6. ↑ Reina, Nick J.; Honet, Joseph C.; Brown, William; Beitman, Max; Chodoroff, Gary (1988). "Paget-Schroetter syndrome in a viola player". Medical Problems of Performing Artists. 3 (1): 24.
  7. ↑ Di Nisio, M.; Van Sluis, G. L.; Bossuyt, P. M. M.; Büller, H. R.; Porreca, E.; Rutjes, A. W. S. (2010-04-01). "Accuracy of diagnostic tests for clinically suspected upper extremity deep vein thrombosis: a systematic review". Journal of Thrombosis and Haemostasis. 8 (4): 684–692. doi:10.1111/j.1538-7836.2010.03771.x. ISSN 1538-7836. PMID 20141579.
  8. ↑ Mai, Cuc; Hunt, Daniel (2011-05-01). "Upper-extremity deep venous thrombosis: a review". The American Journal of Medicine. 124 (5): 402–407. doi:10.1016/j.amjmed.2010.11.022. ISSN 1555-7162. PMID 21531227.
  9. ↑ Madden CC, Putukian M, Young CC, McCarty EC. Netter's Sports Medicine. Saunders. Philadelphia, 2010.
  10. ↑ Kearon, Clive; Akl, Elie A.; Comerota, Anthony J.; Prandoni, Paolo; Bounameaux, Henri; Goldhaber, Samuel Z.; Nelson, Michael E.; Wells, Philip S.; Gould, Michael K. (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. 141 (2 Suppl): e419S–94S. doi:10.1378/chest.11-2301. ISSN 1931-3543. PMC 3278049. PMID 22315268.
  11. ↑ Vik, Anders; Holme, Pål Andre; Singh, Kulbir; Dorenberg, Eric; Nordhus, Kåre Christian; Kumar, Satish; Hansen, John-Bjarne (2009-09-01). "Catheter-directed thrombolysis for treatment of deep venous thrombosis in the upper extremities". Cardiovascular and Interventional Radiology. 32 (5): 980–987. doi:10.1007/s00270-009-9655-y. ISSN 1432-086X. PMID 19641959. S2CID 8572126.
  12. ↑ Thompson, J. F.; Winterborn, R. J.; Bays, S.; White, H.; Kinsella, D. C.; Watkinson, A. F. (2011-10-01). "Venous thoracic outlet compression and the Paget-Schroetter syndrome: a review and recommendations for management". Cardiovascular and Interventional Radiology. 34 (5): 903–910. doi:10.1007/s00270-011-0148-4. ISSN 1432-086X. PMID 21448772. S2CID 13162412.
  13. ↑ Paget-von Schrötter disease at Who Named It?
  14. ↑ Paget J (1866). "On gouty and some other forms of phlebitis". St. Bartholomew's Hospital Reports. 2: 82–92.
  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.
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