Paget–Schroetter disease

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Paget-Schrotter disease
Other names: Paget–von Schrötter disease
Gray576.png
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[citation needed]

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. 4.0 4.1 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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