We need you! See something you could improve? Make an edit and help improve WikSM for everyone.
Paget Schroetter Syndrome
From WikiSM
(Redirected from Paget-Schroetter Syndrome)
Contents
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
- The Subclavian Vein passes by the intersection of the Clavicle and 1st Rib
- Hypertrophic Anterior Scalene can compress the vein posteriorly
- Subclavius muscle can also compress the subclavian vein at the costoclavicular junction
- Anatomically small costoclavicular space can occur due to
- Hypertrophy of Anterior Scalene or Subclavius
- Abnormal bone morphology of the Clavicle or 1st Rib
- Subclavian vein can be compressed even in anatomically normal patients[7]
- Thoracic Outlet anatomic boundaries
- Superior: Clavicle
- Inferior: 1st Rib
- Medial: Costoclavicular Ligament
- Lateral: Anterior Scalene
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
- Fractures
- Proximal Humerus Fracture
- Humeral Shaft Fracture
- Clavicle Fracture
- Scapula Fracture
- First Rib Fracture (traumatic or atraumatic)
- Dislocations & Separations
- Arthropathies
- Muscle & Tendon Injuries
- Rotator Cuff
- Bursopathies
- Ligament Injuries
- Neuropathies
- Other
- Pediatrics
- Coracoid Avulsion Fracture
- Humeral Head Epiphysiolysis (Little League Shoulder)
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
- Standard Radiographs Shoulder as screening tools
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
- 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
- Internal
- External
- Sports Medicine Review Shoulder Pain: https://www.sportsmedreview.com/by-joint/shoulder/
References
- ↑ Paget J. Clinical lectures and essays. London: Longmans, Green & Co, 1875.
- ↑ von Schroetter L. Erkrankungen der Gefasse, in Nathnagel Handbuch der Pathologie und Therapie. Wein: Holder, 1884
- ↑ 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.
- ↑ Prandoni P, Bernardi E. Upper extremity deep vein thrombosis. Curr Opin Pulm Med 1999;5:222-6.
- ↑ DeFelice GS, Paletta GA Jr, Phillips BB, Wright RW. Effort thrombosis in the elite throwing athlete. Am J Sports Med 2002;30:708-12.
- ↑ Illig, Karl A., and Adam J. Doyle. "A comprehensive review of Paget-Schroetter syndrome." Journal of vascular surgery 51.6 (2010): 1538-1547.
- ↑ Adams JT, DeWeese JA, Mahoney EB, Rob CG. Intermittent subclavian vein obstruction without thrombosis. Surgery 1968;68:147-65.
- ↑ Urschel HC Jr, Razzuk MA. Paget-Schroetter syndrome: what is the best management? Ann Thorac Surg 2000;69:1663-8; discussion 1668-9
- ↑ . 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.
- ↑ 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
- ↑ 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.
- ↑ Chin EE, Zimmerman PT, Grant EG. Sonographic evaluation of upper extremity deep venous thrombosis. J Ultrasound Med 2005;24:829- 38; quiz 839-40.
- ↑ Adams JT, DeWeese JA. “Effort” thrombosis of the axillary and subclavian veins. J Trauma 1971;11:923-30.
- ↑ 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
- ↑ Becker DM, Philbrick JT, Walker FB 4th. Axillary and subclavian venous thrombosis. Prognosis and treatment. Arch Intern Med 1991; 151:1934-43.
- ↑ Urschel HC Jr, Razzuk MA. Paget-Schroetter syndrome: what is the best management? Ann Thorac Surg 2000;69:1663-8; discussion 1668-9.
- ↑ 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.
- ↑ Urschel HC Jr, Razzuk MA. Neurovascular compression in the thoracic outlet: changing management over 50 years. Ann Surg 1998;228: 609-17.
- ↑ 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
- ↑ 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.