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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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