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.[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]
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
^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.
ISSN0039-6087.
PMID18108679.
^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.
PMID18108679.
^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.
PMID5901846.
^Peivandi, Mohammad Taghi; Nazemian, Zohreh (2011). "Clavicular Fracture and Upper-Extremity Deep Venous Thrombosis". Orthopedics. 34 (3): 227.
doi:
10.3928/01477447-20110124-28.
PMID21410116.
^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.
^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.
ISSN1432-086X.
PMID19641959.
S2CID8572126.
^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.
ISSN1432-086X.
PMID21448772.
S2CID13162412.
^Paget J (1866). "On gouty and some other forms of phlebitis". St. Bartholomew's Hospital Reports. 2: 82–92.
^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.