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Paget Schroetter Syndrome

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Other Names

  • Paget-Schroetter Syndrome
  • Venous Thoracic Outlet Syndrome
  • Effort Thrombosis
  • Subclavian Vein Thrombosis
  • Paget-von Schroetter Syndrome
  • Primary Effort Thrombosis

Background

  • This page refers to thrombosis of the Subclavian Vein at the costoclavicular junction, a disease termed Paget Schroetter Syndrome (PSS)
    • This should not be confused with Thoracic Outlet Syndrome (TOS), although PSS may be considered a sub-category of venous TOS

History

  • First described by Sir James Paget in 1875[1]
  • von Schroetter postulated in 1884 that this entity was the direct result of a muscle strain[2]

Incidence

  • Incidence estimated to be between 1.0 - 2.03 per 100,000 person years[3]
  • Upper extremity venous thrombosis amounts for 1-4% of all episodes of venous thrombosis[4]
  • One study estimated a case rate of 1 per 5 years among a high level baseball club[5]
  • Average patient is 30s, male, right upper extremity affected, with a history of vigorous exercise or activity

Pathophysiology

  • Unclear whether thrombosis occurs from single insult or cumulative effects of chronic injury[6]
  • Generally held belief is that it is a chronic compression and repetitive microtrauma
    • Subsequently inflammation, fibrosis, adhesions to surrounding anatomic structures
    • On arthroscopy, surgeons report dense collagenous scarring around the entrapped vein
  • In some cases, this is thought to be intermittent compression rather than chronic
    • No objective evidence of damage to the vein, typically normal venogram at rest
  • In cases of complete occlusion, collateral veins will be recruited including
    • cephalic vein, profunda branches with the transverse cervical, scapular, and external and internal jugular veins

Pathoanatomy


Risk Factors

  • History of Clavicle Fracture
  • Cervical Rib (controversial)
  • Repetitive or prolonged hyperabduction, external rotation of shoulder[8]
  • Hypercoagulable Condition[9]
  • Sports[10]
    • Baseball players
    • Swimmers
    • Weight lifters[11]
  • Occupational
    • Repetitive overhead arm motion (i.e. mechanics, electricians)

Differential Diagnosis


Clinical Features

  • General: Physical Exam Shoulder
  • History
    • Patient reports blue, swollen, heavy, painful arm
    • Majority of patients have a history of vigorous exercise, 85% within 24 hours of inciting event
    • Intermittent variant: episodic arm discoloration, swelling
  • Physical Exam
    • Sudden onset of aching, heaviness, swelling, red-blue discoloration of affected extremity
    • May observe dilated superficial collateral veins in more chronic cases
  • Special Tests

Evaluation

Radiographs

Ultrasound

  • Duplex ultrasound is now diagnostic gold standard
  • Highly accurate in experienced hands[12]
    • Sensitivity 78-100%
    • Specificity 82-100%
  • Findings
    • Non-commpressible vein
    • Lack of flow
    • Chronic clots will appear fibrotic and echogenic

MRI

  • Useful to exclude other etiologies
  • Venogram is a consideration

CT

  • Useful to exclude other etiologies
  • Venogram is a consideration

Venography

  • Indicated if high clinical concern, negative ultrasound
  • Diagnostic and can be therapeutic
  • Can be performed with distal peripheral vein
  • Findings
    • Occlusion of the subclavian vein at costoclavicular junction
    • Presence of collateral in more chronic cases
  • In the setting of normal venogram but high index of suspicion
    • Consider abducting arm to 90°
    • Consider tourniquet to occlude superficial venous system

Labs

  • Plasma D-dimer may be elevated
  • Specificity: 14% to 60% (need citation)
  • No diagnostic guidelines for PSS

Classification

  • N/A

Management

Prognosis

  • Untreated[13]
    • Residual upper extremity in 78% of cases
    • Persistent symptoms in 41-91%
    • Permanent disability in 39-68%
  • Treated
    • Good results in 50-66% of patients treated with anticoagulation alone
    • Good results in 80-90% of patients with thrombolysis, delayed thoracic outlet decompression
    • Good results in 90-95% of patients with thrombolysis, immediate thoracic outlet decompression
  • Chang et al: Only 77% of patients treated returned to work[14]

Nonoperative

  • Anticoagulation
    • No clear guidelines on duration of anticoagulation
    • CHEST guidelines recommend 3 months for UE DVT
    • 6-15% of patients develop pulmonary emboli[15]
    • Urschel et al: excellent or good” long-term results in only 29% treated with anticoagulation alone[16]
  • Catheter directed thrombolysis
    • Successful in 62-84% of cases[17]
    • Patients with acute thrombosis appear to due better than chronic thrombosis

Operative

  • Transaxillary first rib resection
    • Timing of decompression is controversial
    • Good surgical exposure, cosmesis
    • Good to excellent long term results in 85-95% of patients treated[18]
  • Decompression of costoclavicular junction
  • Angioplasting or stenting
    • High failure rate if performed before decompression
  • Venous reconstruction

Rehab and Return to Play

Rehabilitation

  • Needs to be updated

Return to Play

  • Needs to be updated

Complications

  • Post Thrombotic Syndrome (PTS)
    • Characterized by pain, heaviness and swelling of the affected extremity
    • Can be a chronic, debilitating condition
    • Seen in 7–46% of patients with upper extremity DVT[19]
  • Intrinsic venous defects
    • Caused by scarring due to persistent injury
  • Rethrombosis in up to 1/3 of patients[20]
  • Surgical

See Also


References


  1. Paget J. Clinical lectures and essays. London: Longmans, Green & Co, 1875.
  2. von Schroetter L. Erkrankungen der Gefasse, in Nathnagel Handbuch der Pathologie und Therapie. Wein: Holder, 1884
  3. Lindblad B, Tengborn L, Bergqvist D. Deep vein thrombosis of the axillary-subclavian veins: epidemiologic data, effects of different types of treatment and late sequelae. Eur J Vasc Surg 1988;2:161-5.
  4. Prandoni P, Bernardi E. Upper extremity deep vein thrombosis. Curr Opin Pulm Med 1999;5:222-6.
  5. DeFelice GS, Paletta GA Jr, Phillips BB, Wright RW. Effort thrombosis in the elite throwing athlete. Am J Sports Med 2002;30:708-12.
  6. Illig, Karl A., and Adam J. Doyle. "A comprehensive review of Paget-Schroetter syndrome." Journal of vascular surgery 51.6 (2010): 1538-1547.
  7. Adams JT, DeWeese JA, Mahoney EB, Rob CG. Intermittent subclavian vein obstruction without thrombosis. Surgery 1968;68:147-65.
  8. Urschel HC Jr, Razzuk MA. Paget-Schroetter syndrome: what is the best management? Ann Thorac Surg 2000;69:1663-8; discussion 1668-9
  9. . Cassada DC, Lipscomb AL, Stevens SL, Freeman MB, Grandas OH, Goldman MH. The importance of thrombophilia in the treatment of Paget-Schroetter syndrome. Ann Vasc Surg 2006;20:596-601.
  10. Chandra V, Little C, Lee JT. Thoracic outlet syndrome in high-performance athletes. J Vasc Surg 2014;60:1012-7; discussion 1017-8. 10.1016/j.jvs.2014.04.013
  11. DeLisa LC, Hensley CP, Jackson S. Diagnosis of Paget-Schroetter Syndrome/Primary Effort Thrombosis in a Recreational Weight Lifter. Phys Ther 2017;97:13-9.
  12. Chin EE, Zimmerman PT, Grant EG. Sonographic evaluation of upper extremity deep venous thrombosis. J Ultrasound Med 2005;24:829- 38; quiz 839-40.
  13. Adams JT, DeWeese JA. “Effort” thrombosis of the axillary and subclavian veins. J Trauma 1971;11:923-30.
  14. Chang DC, Rotellini-Coltvet LA, Mukherjee D, DeLeon R, Freischlag JA. Surgical intervention for thoracic outlet syndrome improves patients’ quality of life. J Vasc Surg 2009;49:630-7
  15. Becker DM, Philbrick JT, Walker FB 4th. Axillary and subclavian venous thrombosis. Prognosis and treatment. Arch Intern Med 1991; 151:1934-43.
  16. Urschel HC Jr, Razzuk MA. Paget-Schroetter syndrome: what is the best management? Ann Thorac Surg 2000;69:1663-8; discussion 1668-9.
  17. Doyle A, Wolford HY, Davies MG, Adams JT, Singh MJ, Saad WE, et al. Management of effort thrombosis of the subclavian vein: today’s treatment. Ann Vasc Surg 2007;21:723-9.
  18. Urschel HC Jr, Razzuk MA. Neurovascular compression in the thoracic outlet: changing management over 50 years. Ann Surg 1998;228: 609-17.
  19. Elman EE, Kahn SR. The post-thrombotic syndrome after upper extremity deep venous thrombosis in adults: a systematic review. Thromb Res 2006;117:609-14. 10.1016/j.thromres.2005.05.029
  20. Machleder HI. Upper extremity venous occlusion. In: Ernst CB, Stanely JC, editors. Current therapy in vascular surgery. 3rd ed. St Louis: Mosby-Year Book Inc 1995. p. 958-63.
Created by:
John Kiel on 4 July 2019 19:23:38
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Last edited:
13 November 2020 14:18:01
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