Thoracic outlet syndrome

Author(s): Philip A. Nelson, MD and Carley Sauter, MD

Originally published:12/28/2012

Last updated:04/05/2017

1. DISEASE/DISORDER:

Definition

Thoracic outlet syndrome (TOS) refers to a constellation of upper extremity symptoms from compression of neurovascular structures coursing through the thoracic inlet. Based on the principal site of compression or irritation, the disorder is classified as either true (classic) neurogenic TOS, vascular TOS, or nonspecific TOS.1

Etiology

TOS may result from compression or irritation of the brachial plexus, subclavian vein, subclavian artery, or a combination of the three.1 Compression may occur from soft tissue mass or abnormal musculoligamentous and bony structures; these changes may be related to trauma, overuse, congenital, or neoplasm.

Epidemiology including risk factors and primary prevention

Accurate diagnosis and incidence of TOS is difficult due to the absence of consistently reproducible objective measures. Some authors propose an incidence of 3-80 cases per 1000.2 Proportionately, neurogenic TOS (nTOS) is estimated to comprise over 90% of TOS. Venous TOS (vTOS) accounts for 3% and arterial TOS (aTOS) is estimated to have the lowest incidence, at less than 1%.4 The female/male ratio for nTOS is 3.5:1 and there is no sex predilection for the arterial type.5 Studies indicate that vTOS affects a similar proportion of men and women5. Both vTOS and aTOS usually develop in young patients without significant comorbidities.5

Patho-anatomy/physiology

TOS occurs secondary to compression of the subclavian artery, subclavian vein, or brachial plexus at the level of the thoracic inlet.1 The thoracic inlet can be divided into three sections: the interscalene triangle, the costoclavicular triangle, and the subcoracoid space.2 The neurovascular bundle can be compressed or irritated at any of these structures.  Compression most often occurs at the interscalene triangle.2 Interscalene triangle surface area can be decreased by cervical ribs, fibrous bands, and kyphotic musculoskeletal anatomy.2 Fibrous bands are more likely than rib anomalies to cause constrictions.2,7,8 Cervical ribs are a rarity; most are asymptomatic.4 Anomalous bands may arise from cervical or rudimentary first thoracic rib, C7 vertebra, subpleural membrane, or scalene muscles.2  Repetitive trauma to the neurovascular bundle at the thoracic inlet may lead to TOS, with the lower trunk or medial cord of the brachial plexus most affected.1 Functional thoracic outlet syndrome has been associated in case reports with postural deviation, including increased kyphosis exacerbating compression at the thoracic inlet.  Repetitive shoulder use and above-shoulder athletic endeavors (swimming, throwing) may increase injury risk6. Nonspecific thoracic outlet syndrome may be seen secondary to kyphotic posture, tight pectoralis muscles and latissimus dorsi.

Classic syndromes have been defined as follows:

Scalenus Anticus Syndrome: compression of subclavian artery or brachial plexus at interscalene triangle.

Costoclavicular Syndrome: compression of subclavian artery, subclavian vein, or brachial plexus between first thoracic rib and clavicle.

Hyperabduction Syndrome: compression of the subclavian or axillary artery, or brachial plexus between the pectoralis minor tendon.

Disease progression including natural history, disease phases or stages, disease trajectory (clinical features and presentation over time)

Natural History

nTOS can cause lasting nerve damage if left untreated, resulting in atrophy, pain and paresthesias.

vTOS can lead to complications including arm swelling, pain, ischemic ulceration, gangrene, and thromboembolic events.4

Clinical Presentation

It is important to evaluate for mimics of TOS in diagnostic phase.  Differential diagnosis includes C8-T1 radiculopathy, brachial plexopathy, carpal tunnel syndrome, ulnar neuropathy, intrinsic shoulder pathology, or myofascial pain.

Essentials of Assessment

History

Arterial TOS: Symptoms include limb coldness, pallor, arm fatigue and claudication with exercise and arm elevation.7

Venous TOS: Symptoms include swelling, edema, cyanosis, and exercise-induced limb pain. Patients may present with subclavian vein thrombosis; sudden onset of limb edema, dusky cyanosis, and pain.7

Neurogenic TOS: Symptoms include arm pain and paresthesias; cervical and shoulder symptoms. With nTOS, symptoms are initially mild and intermittent, increasing in frequency and severity with time.7   nTOS may present with painless hand muscle atrophy, weakness of grip strength due to hand intrinsic muscle wasting, and paresthesias.8

Physical examination

  • The objectivity of physical exam is questionable as a measure for diagnosis of TOS.9
  • Perform a comprehensive exam for all patients, evaluating for motor weakness and vascular compromise.
  • Examine upper extremities differences in color, temperature, moisture, atrophy, edema, finger nails, and hirsutism.
  • Test distal pulses with hands resting upon patient’s lap. Alteration of the radial pulse was thought to be pathognomonic for scalenus anticus syndrome, however, specificity is low (18-87%).7,10,11
  • Wright’s test: monitor the radial pulse with the arm in 90 degrees of abduction/external rotation, head neutral or rotated to opposite direction. Damping or loss of pulse may corroborate diagnosis of vascular TOS (positive in 7% of normals).7
  • Adson’s maneuver: performed with patient seated, forearms on knees, take long breath, elevating the chin and turning the head ipsilaterally; a positive test reproduces symptoms. This test is nonspecific.9
  • Roos test:  performed with patient’s arm at 90-degree abduction/external rotation, shoulder and elbow in frontal plane. The patient opens and closes hands in slow repetitive motion for 3 minutes. The test is positive if symptoms are reproduced including gradual pain in the neck and shoulder, progressing down the arm, with paresthesias in the forearm and digits. If arterial compression, arm pallor with extremity elevated and reactive hyperemia as limb is lowered.7 Specificity of Roos test is very poor.9

Functional assessment

Evaluation of grip strength, finger abduction strength, and repetitive above shoulder activity is important in assessing level of impairment in ADLs.

Laboratory studies

EDX studies to distinguish between cervical radiculopathy, peripheral mono- or poly- neuropathies, from nTOS.12

Typical EDX findings in neurologic TOS:

  • Nerve conduction studies (NCS)
    • Median motor NCS: reduced CMAP amplitude, normal latency
    • Median sensory NCS: normal
    • Ulnar motor NCS: +/- reduced CMAP amplitude, normal latency
    • Ulnar sensory NCS: reduced SNAP amplitude, normal latency
    • MABC sensory NCS: reduced/absent CMAP amplitude
  • Needle EMG
    • Fibrillations/positive waves, reduced recruitment, neurogenic MUAPs: especially in abductor pollicis brevis, also ulnar innervated hand muscles, other C8-T1 innervated muscles in severe cases

Imaging

The goal of imaging is to localize the site of compression and evaluate the compressing structure and the compressed structure. Comparing arms down with hyperabduction is an essential part of any testing modality. Cervical spine radiographs or MRI may be performed to rule out extra rib, axial disease, cervical radiculopathy, bony abnormalities, or neoplasm. Ultrasound is often used in the initial evaluation of patients with suspected vascular TOS and can be helpful as is noninvasive and can be performed with provocative maneuvers, however cannot adequately assess the plexus or evaluate deeper pathology.13 The optimal exam for evaluation of vascular TOS is contrast-enhanced MRI/MRA with provocative arm positioning. Noncontrast MRI with arm in abduction can be sufficient to diagnose nTOS.13 The advantage of MRI over CTA or CT alone is that it provides information on both vascular flow and characterizes surrounding soft tissue and bony structures without radiation exposure. However, asymptomatic patients may have abnormal testing, therefore imaging should be used as a confirmatory test based on clinical suspicion.5,14

Supplemental assessment tools

The Visual Analog Scale is useful for pain assessment. SF-36 and other functional assessment forms characterize functioning and track treatment success/failure.

Early predictions of outcomes

Persistent pain, weakness that does not improve despite 6 months of conservative management, or lack of improvement on EMG findings correlate with poorer prognosis with nonsurgical management and are indications for surgical intervention in cases of underlying anatomic compression.6

Environmental

Repetitive shoulder use and above-shoulder athletic (swimming, throwing) and work endeavors may increase injury risk. Workplace ergonomic modifications may be useful in cases where prolonged poor posture may lead to functional impairments.

Social role and social support system

Rehabilitation of TOS may require significant changes to ADLs and work life. Evaluation by a pain psychologist may be beneficial.  If hand intrinsic muscle strength and abduction strength is lost, upper extremity work capacity may be significantly impaired.

Professional Issues

Professional/work life may be impaired in due to weakness and pain. Modification of work duties may be indicated in certain cases.

3. Rehabilitation Management and Treatments

Available or current treatment guidelines

Goals of treatment are to prevent disability, manage ischemia, initiate appropriate anticoagulation or thrombolysis, and adequately manage pain.

REHABILITATION

The most common course of treatment for acute and subacute TOS is conservative management including behavioral modification, limitation of provocative activities (i.e. arm positions), and a customized course of physical therapy. Targeted physical therapy entails strengthening exercises of the pectoral girdle and postural training, improvement of scapular kinematics to counteract thoracic kyphosis.2,15   Other physical treatments include nerve gliding, mobilization of the first rib, and muscle energy treatments are used but have limited supporting evidence.16 Workplace ergonomic modifications may be useful in cases where prolonged poor posture may lead to functional impairments.

CHEMODENERVATION

Chemodenervation of the scalenes, pectoralis minor, and subclavius muscles with botulinum toxin has been used as a treatment for symptoms predominantly in nTOS, using various methods of guidance (ultrasound, EMG, CT).17-19 Case reports and case series suggest improvement in pain control and blood flow, however this has not been confirmed in a double-blinded, placebo controlled study.20

SURGERY

When acute or subacute progressive neurologic weakness occurs secondary to nTOS, surgical decompression should be considered.3   Guidelines are not established in cases in which pain is the only manifesting symptom. The surgical approach is determined by the major site of compression. Emergent surgery indicated in cases of severe vascular occlusion or acute embolism to ensure revascularization.2 Surgical decompression for vTOS and nTOS has shown mixed outcomes in quality of life measures, with some positive outcomes for short term but mixed results for long term outcomes and are dependent on patient selection.21-26

At different disease stages

As noted above, the most common course of treatment for acute and subacute nTOS is conservative management including behavioral modification, limitation of provocative activities, and physical therapy. If there is progressive or severe neurologic deficit (ie weakness), then surgery may be considered.

If symptoms are refractory to conservative care or in chronic TOS, then chemodenervation can be considered. Surgery is considered in chronic nTOS with progressive weakness.

Coordination of care

Good communication between physicians, therapists, radiologists, and surgeons is essential to the appropriate care of patients.

Patient & family education

Effective communication of disease etiology and prognosis along with work/life implications and modifications is always indicated.

Translation into practice: practice “pearls”/performance improvement in practice (PIPs)/changes in clinical practice behaviors and skills

The key to appropriate management is distinguishing nTOS from vTOS, as they have different treatment approaches. Intrinsic shoulder injury, cervical radiculopathy, poly- or mono-neuropathy, and other neuropathic syndromes must be ruled out as mimickers of TOS.

Electrodiagnostic testing may be helpful to establish diagnosis of neurogenic TOS.

MRI/MRA with provocative arm positions is the optimal imaging test for evaluation of TOS.

Nonspecific TOS, without objective imaging or electrodiagnostic findings, is controversial.

Cutting edge/emerging and unique concepts and practice

Cutting edge concepts and practice

For the evaluation of vascular TOS, contrast-enhanced MRI/MRA with provocative arm positioning best assess vascular flow. Noncontrast MRI with arm in abduction can be sufficient to diagnose nTOS.13 The advantage of MRI over CTA or CT alone is that it provides information on both vascular flow and characterizes surrounding soft tissue and bony structures without radiation exposure.

Gaps in the evidence-based knowledge

A major gap in evidence-based knowledge is the absence of an objective grading system for TOS upon which consensus for diagnosis and indications for treatment can be made. Some clinicians believe that surgical intervention is overused and that the diagnosis of TOS itself is controversial and not fully accepted.

References

  1. Kitirji B. Electromyography in Clinical Practice. Philadelphia, PA: Mosby; 2007:199-208.
  2. Huang JH, Zager EL. Thoracic outlet syndrome. Neurosurgery. 2004; 55(4):897-903.
  3. Hood DB, Kuehne J, Yellin AE, Weaver FA. Vascular complications of thoracic outlet syndrome. Am Surg 1997; 63:913.
  4. Sanders RJ. Diagnosis of thoracic outlet syndrome. Journal of Vascular Surgery. 2007; 46(3):606-607.
  5. Aghayev A, Rybicki FJ. State-of-the-art magnetic resonance imaging in vascular thoracic outlet syndrome. Magnetic Resonance Imaging Clinics of North America, 2015; 23(2):309-320.
  6. Safran MR. Nerve injury about the shoulder in athletes. Part 2: long thoracic nerve, spinal accessory nerve, burners/stingers, thoracic outlet syndrome. Am J Sports Med. 2004; 32(4):1063-1076.
  7. Brantigan C, Roos D. Diagnosing thoracic outlet syndrome. Hand Clin. 2004; 20:27-36.
  8. Ferrante MA. The thoracic outlet syndromes. Muscle & Nerve. 2012; 45(6):780-795.
  9. Nord KM, Kapoor P, Fisher J, Thomas G, Sundaram A, Scott K, Kothari MJ. False positive rate of thoracic outlet syndrome diagnostic maneuvers. Electromyogr Clin Neurophysiol. 2008; 48(2):67-74.
  10. Sanders RJ, Hammond SH. Management of cervical ribs and anomalous first ribs causing neurogenic thoracic outlet syndrome. Journal of Vascular Surgery, 2002;36:51-56.
  11. Marx RG, Bombardier C, Wright JG. What we know about the reliability and validity of physical examination tests used to examine the upper extremity. J Hand Surg. 1999;24A(1):185-192.
  12. Dumitru D. Electrodiagnostic Medicine, 2nd Edition. Philadelphia, PA: Hanley and Belfus, Inc; 2002:817-20.
  13. Moriarty JM, Bandyk DF, Broderick DF, Cornelius RS, Dill KE, Francois CJ, et al. ACR appropriateness criteria imaging in the diagnosis of thoracic outlet syndrome. Journal of the American College of Radiology, 2015; 12(5), 438-443.
  14. LaBan MM, Zierenberg AT, Yadavalli S, Zaidan S. Clavicle-induced narrowing of the thoracic outlet during shoulder abduction as imaged by computed tomographic angiography and enhanced by three-dimensional reformation. American Journal of Physical Medicine & Rehabilitation, 2011; 90(7), 572-578.
  15. Lindgren KA. Conservative treatment of thoracic outlet syndrome: A 2-year follow-up. Arch Phys Med Rehabil. 1997; 78:373-378.
  16. Orlando MS, Likes KC, Freischlag JA. Physical therapy in the management of patients with neurogenic thoracic outlet syndrome: In reply to Gambhir and colleagues. Journal of the American College of Surgeons, 205; 221(3):778-779.
  17. Danielson K, Odderson IR. Botulinum toxin type A improves blood flow in vascular thoracic outlet syndrome. American Journal of Physical Medicine & Rehabilitation, 2008; 87(11), 956-959.
  18. Christo PJ, Christo DK, Carinci AJ, Freischlag JA. Single CT-guided chemodenervation of the anterior scalene muscle with botulinum toxin for neurogenic thoracic outlet syndrome. Pain Medicine, 2010; 11(4):504-511.
  19. Jordan SE, Ahn SS, Gelabert HA. Combining ultrasonography and electromyography for botulinum chemodenervation treatment of thoracic outlet syndrome: comparison with fluoroscopy and electromyographic guidance. Pain Physician. 2007;10:541-546.
  20. Finlayson HC, O’Connor RJ, Brasher PM, Travlos A. Botulinum toxin injection for management of thoracic outlet syndrome: A double-blind, randomized, controlled trial. Pain, 2011; 152(9):2023-2028.
  21. Chang DC, Rotellini-Coltvet LA, Mukherjee D, De Leon R, Freischlag JA. Surgical intervention for thoracic outlet syndrome improves patient’s quality of life. Journal of Vascular Surgery, 2009; 49(3):630-5, discussion 635-7.
  22. Colli BO, Carlotti CJ, Assirati JJ, Marques WJ. Neurogenic thoracic outlet syndromes: A comparison of true and nonspecific syndromes after surgical treatment. Surgical Neurology, 2006; 65(3):262-271.
  23. Likes KC, Orlando MS, Salditch Q, Mirza S, Cohen A, Reifsnyder T. Lessons learned in the surgical treatment of neurogenic thoracic outlet syndrome over 10 years. Vascular & Endovascular Surgery, 2015; 49(1-2):8-11.
  24. Desai SS, Toliyat M, Dua A, Charlton-Ouw KM, Hossain M, Estrera AL. Outcomes of surgical paraclavicular thoracic outlet decompression. Annals of Vascular Surgery, 2014; 8(2):457-464.
  25. Rochlin DH, Likes KC, Gilson MM, Christo PJ, Freischlag J. Management of unresolved, recurrent, and/or contralateral neurogenic symptoms in patients following first rib resection and scalenectomy. Journal of Vascular Surgery, 2012; 56(4):1061-1068
  26. Scali S, Stone D, Bjerke A, Chang C, Rzucidlo E, Goodney P. Long-term functional results for the surgical management of neurogenic thoracic outlet syndrome. Vascular and Endovascular Surgery, 2010; 44(7):550-555.

Original Version of the Topic

Clark C. Smith, MD, Raj Telhan, MD. Thoracic Outlet Syndrome. 02/05/2013.

Author Disclosure

Philip A. Nelson, MD
Nothing to Disclose

Carley Sauter, MD
Nothing to Disclose

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