Paget-Schroetter disease pathophysiology
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Anahita Deylamsalehi, M.D.[2]
Overview
It is thought that Paget-Schroetter disease is a form of axillary-subclavian vein thrombosis resulted from compression of aforementioned veins. This condition is more common in athletes and/or manual labors. Some studies link Paget-Schroetter disease with anatomical defects involving the thoracic outlet.
Pathophysiology
Physiology
The subclavian vein passes through a space surrounded by the first rib, the scalenus anterior muscle and the subclavius muscle and tendon.[1]
Pathogenesis
- It is though that Paget-Schroetter disease (also known as Effort Thrombosis) is the result of axillary-subclavian vein compression and subsequent thrombosis, and is more common in athletes and/or manual labors.[2]
- Around 60-80% of cases with Paget-Schroetter disease have a history of repetitious activity of upper extremity.[3]
- Sports related to higher chance of developing this condition are baseball, softball, wrestling, playing ball, gymnastic, swimming, hockey, rowing, martial arts, backpacking, recreational badminton, weight training and billiards which require repetitive and constant upper extremity activities. [4][5]
- Repetitive overhead arm activities (e.g., auto repair and painting) have been reported in a number of cases.[6]
- Even with normal anatomical structure the Subclavian vein can be compressed through hyper-abduction (more prominent in arm abducted over 90 degrees), extension and/or external rotation.[7][8]
- In addition to compression, these activities can put tension on the Subclavian vein which lead to micro-trauma of the endothelium and initiate the coagulation cascade.[9]
- More compression can be seen with hypertrophied Scalenus Anterior or Subclavius muscle.
- Increased bulk of muscles like Pectoralis minor and Subscapularis muscles and presence of osseous exostosis or fibrous bands, as well as congenital or post-trauma bone deformities can cause further compression.[10]
- Restricted space around the Subclavian vein lead to blood flow stasis and immobilize the Subclavian vein.
- The Subclavian vein becomes more prone to endothelial trauma from arm movements when it's mobility has been limited.[11]
- Endothelial trauma followed by thrombosis and recanalization will result in intimal hyperplasia and inflammation, which can be more complicated by venous webs, extensive collateral formation and perivenular fibrosis.[12]
- There is no clear connection between Effort Thrombosis and hereditary or acquired thrombophilias, however coexisting Thrombophilia can rise the rate of postoperative complications after corrective surgery. [13]
Genetics
- No racial or genetic predisposition has been demonstrated.[14]
Associated Conditions
Conditions associated with the Paget-Schroetter disease include:
- Anatomical abnormalities involving the thoracic outlet:
- Paget-Schroetter disease or Effort Thrombosis is a venous variant of Thoracic Outlet Syndrome.
- Therefore abnormalities like cervical rib, congenital bands, hypertrophy of scalenus tendons and abnormal insertion of the costoclavicular ligament are associated with this variant of Thoracic Outlet Obstruction.[15]
Gross Pathology
- On gross pathology, the following findings can be noted:
- Presence of cervical rib
- Hypertrophy of scalenus tendons
- Congenital bands and abnormal insertion of the costoclavicular ligament could be expected. [16]
- Replacement of loose connective tissue that encircles the vein by dense collagen scar is seen at operation, which is resulted from trauma-induced inflammation and subsequent fibrosis. [17]
Microscopic Pathology
- No microscopic change is detected on microscopic histopathological analysis.
References
- ↑ Mall NA, Van Thiel GS, Heard WM, Paletta GA, Bush-Joseph C, Bach BR (2013). "Paget-schroetter syndrome: a review of effort thrombosis of the upper extremity from a sports medicine perspective". Sports Health. 5 (4): 353–6. doi:10.1177/1941738112470911. PMC 3899898. PMID 24459553.
- ↑ Alla VM, Natarajan N, Kaushik M, Warrier R, Nair CK (2010). "Paget-schroetter syndrome: review of pathogenesis and treatment of effort thrombosis". West J Emerg Med. 11 (4): 358–62. PMC 2967689. PMID 21079709.
- ↑ Rowan TL, Kazemi M (2012). "Paget Schroetter Syndrome: A case study of the chiropractor's role in recognizing and comanaging an important condition". J Can Chiropr Assoc. 56 (4): 256–61. PMC 3501911. PMID 23204568.
- ↑ Mall NA, Van Thiel GS, Heard WM, Paletta GA, Bush-Joseph C, Bach BR (2013). "Paget-schroetter syndrome: a review of effort thrombosis of the upper extremity from a sports medicine perspective". Sports Health. 5 (4): 353–6. doi:10.1177/1941738112470911. PMC 3899898. PMID 24459553.
- ↑ Illig, Karl A.; Doyle, Adam J. (2010). "A comprehensive review of Paget-Schroetter syndrome". Journal of Vascular Surgery. 51 (6): 1538–1547. doi:10.1016/j.jvs.2009.12.022. ISSN 0741-5214.
- ↑ Illig, Karl A.; Doyle, Adam J. (2010). "A comprehensive review of Paget-Schroetter syndrome". Journal of Vascular Surgery. 51 (6): 1538–1547. doi:10.1016/j.jvs.2009.12.022. ISSN 0741-5214.
- ↑ Alla VM, Natarajan N, Kaushik M, Warrier R, Nair CK (2010). "Paget-schroetter syndrome: review of pathogenesis and treatment of effort thrombosis". West J Emerg Med. 11 (4): 358–62. PMC 2967689. PMID 21079709.
- ↑ Illig, Karl A.; Doyle, Adam J. (2010). "A comprehensive review of Paget-Schroetter syndrome". Journal of Vascular Surgery. 51 (6): 1538–1547. doi:10.1016/j.jvs.2009.12.022. ISSN 0741-5214.
- ↑ Alla VM, Natarajan N, Kaushik M, Warrier R, Nair CK (2010). "Paget-schroetter syndrome: review of pathogenesis and treatment of effort thrombosis". West J Emerg Med. 11 (4): 358–62. PMC 2967689. PMID 21079709.
- ↑ Mall NA, Van Thiel GS, Heard WM, Paletta GA, Bush-Joseph C, Bach BR (2013). "Paget-schroetter syndrome: a review of effort thrombosis of the upper extremity from a sports medicine perspective". Sports Health. 5 (4): 353–6. doi:10.1177/1941738112470911. PMC 3899898. PMID 24459553.
- ↑ Alla VM, Natarajan N, Kaushik M, Warrier R, Nair CK (2010). "Paget-schroetter syndrome: review of pathogenesis and treatment of effort thrombosis". West J Emerg Med. 11 (4): 358–62. PMC 2967689. PMID 21079709.
- ↑ Alla VM, Natarajan N, Kaushik M, Warrier R, Nair CK (2010). "Paget-schroetter syndrome: review of pathogenesis and treatment of effort thrombosis". West J Emerg Med. 11 (4): 358–62. PMC 2967689. PMID 21079709.
- ↑ Alla VM, Natarajan N, Kaushik M, Warrier R, Nair CK (2010). "Paget-schroetter syndrome: review of pathogenesis and treatment of effort thrombosis". West J Emerg Med. 11 (4): 358–62. PMC 2967689. PMID 21079709.
- ↑ Lazea, C; Asavoaie, C (2019). "Paget–Schroetter syndrome in a teenager after throwing firecrackers – A case report". Nigerian Journal of Clinical Practice. 22 (7): 1022. doi:10.4103/njcp.njcp_230_18. ISSN 1119-3077.
- ↑ Alla VM, Natarajan N, Kaushik M, Warrier R, Nair CK (2010). "Paget-schroetter syndrome: review of pathogenesis and treatment of effort thrombosis". West J Emerg Med. 11 (4): 358–62. PMC 2967689. PMID 21079709.
- ↑ Urschel, Harold C.; Patel, Amit N. (2008). "Surgery Remains the Most Effective Treatment for Paget-Schroetter Syndrome: 50 Years' Experience". The Annals of Thoracic Surgery. 86 (1): 254–260. doi:10.1016/j.athoracsur.2008.03.021. ISSN 0003-4975.
- ↑ Illig, Karl A.; Doyle, Adam J. (2010). "A comprehensive review of Paget-Schroetter syndrome". Journal of Vascular Surgery. 51 (6): 1538–1547. doi:10.1016/j.jvs.2009.12.022. ISSN 0741-5214.