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    Thoracic outlet syndromeaspects of

    diagnosis in the differential diagnosis of

    handarm vibration syndromeR. A. Cooke

    Background Thoracic outlet syndrome (TOS) is a cause of vascular and neurological co

    promise to the arm and hand, and may manifest as Raynauds phenomenon. It m

    be under-diagnosed.

    Aim This review was undertaken in order to clarify the diagnostic and investigat

    features of TOS that may differentiate it from handarm vibration syndrome.

    Methods A tiered review of the world literature was undertaken using Medline and Embase

    the primary search engines.Conclusion Thoracic outlet syndrome most commonly presents with neurological symptoms

    the arm. Vascular symptoms, including Raynauds phenomenon, may occur in ~10

    of cases. Careful clinical assessment by history and examination may reveal t

    elements of forearm and upper arm symptoms with postural exacerbation, wh

    distinguish this condition from handarm vibration syndrome. The usefulness

    investigation is unclear, but Doppler and neuroelectric studies may be valuab

    Magnetic resonance imaging scan is the investigation of choice.

    Key words Raynauds phenomenon; thoracic outlet syndrome.

    Received 6 June 2003

    Accepted 10 June 2003

    Introduction

    Thoracic outlet syndrome (TOS) refers to abnormal

    compression of nerve, arterial and, less frequently, venous

    structures at the base of the neck or thoracic outlet. It

    has also been referred to as scalenus anticus syndrome,

    cervical rib syndrome, costo-clavicular syndrome, hyper-

    abduction syndrome and TOS [1]. It is recognized as a

    cause of Raynauds phenomenon, as well as of neuro-

    logical symptoms affecting the upper limb. It follows that

    TOS must be considered as a possible cause of symptoms

    comparable with those of handarm vibration syndrome.

    Potential causes of TOS are a cervical rib, abnormalligamentous tissue and hypertrophy of the scalenus

    anterior muscle, as well as postural effects that interfere

    with the normal relationship between the first rib and

    those structures overlying it.

    It has been suggested that TOS may be the most und

    rated, overlooked and misdiagnosed peripheral ner

    compression in the upper extremity [2], as well as t

    most important and difficult to manage. A retrospect

    study of cases of TOS presenting to an emergency depa

    ment during a 29 year period concluded that a lack

    thorough evaluation resulted in under-diagnosis of TO

    [3]. Ouriel [4] suggested that the relative rarity of vascu

    disorders of the arm accounts for even experienc

    vascular clinicians being unfamiliar with upper extrem

    diagnostic testing.

    Anatomy

    The thoracic outlet is bounded by the first thorac

    vertebra, the superior border of the manubrium ster

    anteriorly, and the first rib and costal cartilage lateral

    The structures passing through this area and into t

    upper limb are the subclavian artery and vein, and t

    nervous structures of the brachial plexus (see Figure 1)

    Correspondence to: Dr R. A. Cooke,Summers Place,Whitbourne,Worcester

    WR6 5ST, UK. Tel: +44 1886 821644; fax: +44 1886 821944;

    e-mail: [email protected]

    Occupational Medicine2003;53:331

    DOI: 10.1093/occmed/kqg

    Occupational Medicine, Vol. 53 No. 5,

    Society of Occupational Medicine; all rights reserved 331

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    Incidence

    The normal frequency of cervical anomalies in the

    population is 0.121%.However,Boleset al.[5] reported

    15 members of a single family (the two parents and their

    13 children) affected with a TOS secondary to a cervical

    rib and or an apophysomegaly of the seventh cervical

    vertebra. Sallstrom and Thulesius [6] reported that the

    prevalence of TOS was almost twice as common in

    women as in men (ratio 1.76:1).

    Risk factors

    In many cases, an anatomical abnormality will be present

    and be the underlying reason for the development of

    symptoms of TOS.There may be occupational influences

    to provoke or exacerbate symptoms, such as working

    repeatedly with the arms at or above shoulder height.

    The throwing athlete is at risk for neurovascular

    injuries of the shoulder because of the demands placed

    upon the shoulder by repetitive throwing motions. The

    most commonly recognized neurovascular compression

    syndromes are axillary artery occlusion, effort throm-

    bosis, quadrilateral space syndrome and TOS [7]. After

    studying 167 patients exposed to handarm vibration,Kakosky [8] suggested that such exposure may play a part

    in the development of TOS,and conversely that TOS may

    contribute to Raynauds phenomenon of occupational

    origin.

    Clinical presentation

    Blanchardet al.[9] described five syndromes involving

    the thoracic outlet, namely:

    1. Arterial, due to a well formed cervical rib or to an

    abnormal first rib.

    2. Neurological, related to the fibrous band associated

    with a rudimentary cervical rib or a giant transverse

    process of C7. In 1980, Roos [10] reported the

    results of 1120 operations for TOS, indicating that

    most TOS patients have anomalous fibro-muscular

    bands near the brachial plexus and subclavian vessels.He identified nine different types of these soft tissue

    anomalies not visible radiographically, describing

    them as often multiple, and sometimes associated

    with bone abnormalities as well.

    3. Venouseffort thrombosis.

    4. Late post-traumatic, secondary to a fracture of the

    clavicle.

    5. The syndrome previously called scalenus anticus

    syndrome, being due to either trauma, typically a

    whiplash type of injury, or hypotonic shoulder

    muscles, occurring mostly in women, and responding

    well to specific and simple exercises.

    TOS can present with symptoms and signs of any of

    these, namely arterial insufficiency, venous obstruction,

    painless wasting of intrinsic hand muscles and pain [2].

    More than 90% of the patients present with neurolog-

    ical symptomspain, paraesthesiae, or arm and hand

    weaknessand 10% also have vascular problems [11]. In

    the 10% of patients who have vascular involvement,

    urgent investigation and early surgery may be necessary

    if ischaemic complications are to be avoided [12].

    Branchereau et al. [13] reported that among 10 cases

    of cervical ribs operated upon in seven patients, the

    predominantly neurological symptoms and signs were

    associated with vascular problems in three cases, withintermittent compression of the subclavian vessels.

    Dangerous vascular complications are the embolization

    of a subclavian aneurysm and the acute thrombosis of the

    subclavian vein [14]. Desai and Robbs [15] reported a

    series of 26 patients, among whom 17 presented with a

    fixed pulse deficit, 13 had a palpable aneurysm and 12

    had distal embolization. Durhamet al.[16] reviewed 34

    patients treated for upper extremity symptoms of TOS

    from 1983 to 1993. They reported that 22 patients (27

    arms) had subclavian artery injury, which was most com-

    monly caused by compression by a bony abnormality

    (cervical rib, 16; anomalous first rib, 2; cervical rib and

    anomalous first rib, 2). Fourteen of the 27 arms had

    distal embolization. Twelve additional patients (nine

    athletes) had axillary artery involvement, all from arterial

    compression by the head of the humerus during ab-

    duction manoeuvres; all had concomitant compression

    of the posterior circumflex humeral artery. Axillary

    arterial injury included thrombosis (one), aneurysm

    (two) and symptomatic extrinsic compression only

    (nine). They concluded that most patients with TOS who

    have arterial involvement have a bony anomaly causing

    Figure 1. Anatomy.

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    subclavian artery compression. They demonstrated that

    humeral head compression of the axillary artery and its

    circumflex branches is a surprisingly common pathologic

    mechanism.

    Neurological presentation

    Initial symptoms are pain and paraesthesiae in the

    affected area, followed by sensory and motor deficits in

    advanced cases. Complaints of paraesthesiae and numb-

    ness will relate to the nerve compression component of

    TOS, whereas the pain associated with this syndrome

    is largely caused by muscle imbalance in the neck,

    shoulders and upper back [17]. Rayan [18] reported two

    patients with TOS secondary to cervical rib, both of

    whom had vague shoulder pain as well as neurological

    manifestations due to compression neuropathy of the

    lower trunk of the brachial plexus. One of the patients

    had been suspected initially to have carpal tunnel

    syndrome. Typically, it is the lower trunk of the brachialplexus that is affected, so that TOS manifests as symp-

    toms of lower cervical nerve involvementsC8 and

    T1with hypoaesthesia and paraesthesia [19]. This is

    likely to result in pain, sometimes accompanied by

    paraesthesiae, along the lateral (ulnar) border of the

    forearm, and into the corresponding area of the hand and

    fingers. Although rare, upper plexus TOS may manifest

    with symptoms due to the involvement of the C5C7

    nerve roots [19], which may then result in pain or

    paraesthesiae along the medial aspect of the arm and

    forearm, and into the hands and fingers. In either case,

    the pain or paraesthesiae may be aggravated by elevation

    of the arm or by carrying heavy weights.Muscles supplied by the median or ulnar nerves

    derived from these trunks may be weak and wasted.

    Novaket al.[23] found that two-point discrimination was

    normal in 98% of cases.

    Vascular presentation

    The most frequent vascular presentation of TOS may be

    that of Raynauds phenomenon [20], or may be the less

    clearly defined symptoms of coldness, cyanosis and

    swelling of the hands, possibly resulting from irritation of

    sympathetic fibres in the subclavian bundle. Upper limbpulses may be diminished, with bruits heard over the

    subclavian artery.

    Primary Raynauds phenomenon in patients with signs

    of TOS has been shown to be indistinguishable from that

    in those without signs of TOS in respect of family history,

    gender, thumb involvement or asymmetry of Raynauds

    phenomenon [21].

    In a study of 167 workers exposed to handarm

    vibration, all of whom had Raynauds phenomenon, with

    or without numbness and muscle weakness, Kakosky

    found that a brachial plexus nerve lesion was the mo

    commonly electrophysiologically demonstrated lesion.

    16.2% of patients compression of the subclavian arte

    was also demonstrated. It was concluded that handar

    vibration exposure may play a part in the development

    TOS, and conversely that TOS may contribute

    Raynauds phenomenon of occupational origin [8].

    A number of provocative tests have been describebased on the concept that change in shoulder/ne

    position may produce symptoms, including Adsons a

    Roos tests. Pulse obliteration indicates some element

    anatomic tightness,but it has been suggested that witho

    symptom reproduction, pulse change has no diagnos

    relationship to TOS [22]. Novaket al.[23] reported th

    94% had positive provocative position and compressi

    test results, and suggested that clinical assessment

    TOS is best achieved by reproduction of symptoms w

    compression and positional provocative testing.

    In a study of TOS shoulder manoeuvres in 53 healt

    subjects, the outcomes of pulse alteration or paraestheswere unreliable in general. However, TOS shoulder ma

    oeuvres have reasonably low false-positive rates when

    positive outcome is defined as pain after Adsons te

    costo-clavicular manoeuvre or supraclavicular pressu

    discontinuation of the elevated arm stress test seconda

    to pain; pain in the same arm with two or more ma

    oeuvres; or any symptom in the same arm with three

    more manoeuvres [24]. Warrens and Heaton [2

    assessed manoeuvres advocated for the assessment

    thoracic outlet compression on 64 randomly chos

    volunteers. Although only 17% had any symptoms

    thoracic outlet compression, 58% had a positive result

    at least one of the manoeuvres. Only 2% were positive

    more than two manoeuvres. They concluded by sugge

    ing that the low specificity devalues these tests in clinic

    practice. Gillardet al.[26] found that provocative te

    had mean sensitivity and specificity values of 72 and 53

    respectively, with better values for the Adson test [posit

    predictive value (PPV) = 85%] and the hyperabducti

    test (PPV = 92%).

    Kitamura et al. [27] assessed the influence of de

    breathing on Adsons test by using laser Doppler flo

    metry to quantitatively assess changes in blood fl

    measured at the arm during Adsons test, the rever

    Adson test and deep breathing in the sitting position.

    10 patients who were found during surgery to ha

    anatomical abnormalities in the thoracic outlet are

    preoperative administration of Adsons test had n

    indicated any compression that caused cardiac wa

    disappearance and continued blood flow reduction. Th

    concluded that Adsons test, which involves compressi

    by the scalene muscles, is greatly affected by de

    breathing during the test, and that the results of this te

    do not faithfully reflect vascular compression.

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    Clinical investigation

    The diagnosis of thoracic outlet compression syndrome is

    usually made on the basis of an adequate history and

    physical examination. Diagnosis is largely one of exclu-

    sion,but it may be aided in some patients by angiography,

    assessment of the conduction velocity of the ulnar nerve

    and other objective tests [28]. It has been suggested that

    electromyograms, arteriograms and venograms are not

    helpful for the neurological type of TOS [11]. Stanton

    et al.[29] suggested that nerve conduction velocities and

    directional Doppler studies are the most useful adjuncts

    in diagnosis.

    Plain radiology

    Plain X-ray of the chest or thoracic outlet may demon-

    strate a supplementary rib, usually arising from the

    seventh cervical vertebra, but sometimes from the sixth

    and rarely from the fifth. Thirty-one patients with TOSwere studied by Lascelleset al.[30], who found that the

    majority of patients had radiological abnormalities and all

    had structural lesions in the superior thoracic aperture

    seen at operation.

    Computerized tomography (CT) scan

    Where a cervical rib is present, CT may demonstrate not

    only the bony abnormality but also the relationship of any

    bony deformity with arterial compression [31], Novak

    et al.[23] reported that 32% of the patients studied had acompressive anatomical abnormality identified by CT

    scan.Three-dimensional CT imaging is effective for iden-

    tifying the structures in the thoracic outlet [32]. A helical

    CT arterial and/or venous angiogram with postural

    manoeuvres has been shown to be useful in providing

    accurate information on the location and mechanism of

    vascular compression [26,33].

    Magnetic resonance imaging (MRI) scan

    MRI scan is the imaging method of choice for evaluatingthe anatomy and pathology of the brachial plexus [34].

    However, this may require elevation of the arm or

    hyperabduction manoeuvres to demonstrate compression

    of vascular or neurological structures [35,36]. Gado-

    linium enhancement angiography allows rapid evaluation

    and comparison of the vascular structures in both the

    neutral and abducted positions [37]. Since conventional

    MRI scanners may not allow investigation with the arm

    in abduction, an open magnet scanner may be seen as

    essential for this [38].

    Nerve conduction and electromyograph

    (EMG) studies

    Novaket al.[23] found that only one patient out of 50

    with TOS had a positive nerve conduction study/EMG at

    the brachial plexus level. However,others have concluded

    that neuroelectric studies are inconsistently helpful, but

    may be useful in ruling out other, more distal nerve en-

    trapments [22]. Gruss [14] suggested that a neurological

    examination with measuring of the proximal ulnar and

    median nerve conduction times is mandatory.

    Doppler studies

    Hachullaet al.[39] reviewed 95 healthy subjects examined

    regarding the presence of TOS by clinical examination

    and by Doppler flowmetry during performance of Adson,

    hyperabduction and abductionexternal rotation man-

    oeuvres. The response was considered positive when the

    radial pulse disappeared for the clinical test, and when theflow was totally arrested for the Doppler flowmetry. They

    concluded that Doppler flowmetry is useful for the TOS

    diagnosis only when the clinical evaluation is abnormal,

    that total arrest of flow is sometimes temporary, that

    arterial flow must be examined for at least 20 s, and that

    total arrest of flow is never seen during Adson manoeuvre

    or hyperabduction at 45 or 90 in healthy subjects.

    Novakovic et al. [40] examined 34 patients and 30

    asymptomatic volunteers, and studied the retroscalenal

    part of the subclavian artery using real-time (B-mode)

    duplex imaging. They found significant reduction of

    lumen diameter of the subclavian artery during Adsons

    manoeuvre in 51% of cases. During Adsons costo-

    clavicular and hyperabduction manoeuvre, they found

    significant changes in linear blood velocity, reduction of

    blood flow and turbulence in 91% of cases. Adsons

    manoeuvre was positive in 52%, costo-clavicular in 29%

    and hyperabduction (to 90) in 19% of cases.

    Wadhwaniet al.[41] concluded that colour Doppler

    sonography is a non-invasive, effective method compared

    with digital subtraction angiography in the diagnosis of

    TOS.

    Sallstrom and Thulesius [6] found a significant correla-

    tion between arterial compression measured by Doppler

    flowmetry and the presence of compression of the plexus,but the validity of this method was not satisfactory, giving

    both false-positive and -negative results. They also

    reported a very good correlation between arterial com-

    pression assessed clinically and by Doppler flowmetry.

    They concluded that the Doppler technique added very

    little with significant vascular compression being easily

    detected by clinical assessment. Gillard et al. [26] re-

    ported that Doppler ultrasonography visualized vascular

    parietal abnormalities and confirmed the diagnosis in

    some patients.

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    Angiography

    Angiography is an effective method of vascular inves-

    tigation, but is highly invasive. In TOS, it may be used to

    demonstrate compression of the subclavian artery.

    Ostheium and Schmitt [42] showed that half a series of

    18 patients so investigated had occlusion of the digital

    arteries on the side of the compressed subclavian artery.

    Conclusion

    Careful assessment is required of the patient who

    presents with neurological and/or vascular symptoms

    in the upper limb, including Raynauds phenomenon.

    Thoracic outlet syndrome should be considered with

    particular consideration to the following

    pain as a primary feature of the presentation; neurological symptoms or findings suggestive of a

    dermatomal distribution, particularly C8 or T1, but

    also less commonly at higher levels;

    exacerbation of symptoms with elevation of the armeither given as a part of the history or on examination.

    Careful clinical assessment is likely to indicate the

    diagnosis. Although there is variable evidence regarding

    the usefulness of postural/ provocative tests, the com-

    bination of pulse obliteration and reproduction of

    symptoms by such manoeuvres must be useful.

    Clinical investigations also appear to have mixed

    reviews. Nerve conduction studies are likely to be useful,

    if only to exclude other neurological lesions. Plain X-ray

    may demonstrate a cervical rib. CT scan is likely to

    demonstrate the presence of non-bony lesions. Dopplerstudies may be useful, although some studies suggest that

    their value is no greater than that of good clinical

    assessment. MRI is the investigation of choice.

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