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Thoracic Outlet Syndrome

Article  in  Neurosurgery · November 2004


DOI: 10.1227/01.NEU.0000137333.04342.4D · Source: PubMed

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TOPIC REVIEW

THORACIC OUTLET SYNDROME


Jason H. Huang, M.D. OBJECTIVE: Thoracic outlet syndrome (TOS) is one of the most controversial clinical
Department of Neurosurgery, entities in medicine. We provide a review of this difficult-to-treat disorder, including
Hospital of the University of
Pennsylvania, University of
a brief overview, clinical presentations, surgical anatomy, treatment options, and
Pennsylvania Medical Center, outcomes.
Philadelphia, Pennsylvania METHODS: TOS represents a spectrum of disorders encompassing three related
syndromes: compression of the brachial plexus (neurogenic TOS), compression of the
Eric L. Zager, M.D.
subclavian artery or vein (vascular TOS), and the nonspecific or disputed type of TOS.
Department of Neurosurgery,
Hospital of the University of
Neurovascular compression may be observed most commonly in the interscalene
Pennsylvania, University of triangle, but it also has been described in the costoclavicular space and in the
Pennsylvania Medical Center, subcoracoid space. Patients present with symptoms and signs of arterial insufficiency,
Philadelphia, Pennsylvania
venous obstruction, painless wasting of intrinsic hand muscles, paresthesia, and pain.
Reprint requests: A careful and detailed medical history and physical examination are the most impor-
Jason H. Huang, M.D., tant diagnostic tools for proper identification of TOS. Electromyography, nerve con-
Department of Neurosurgery, duction studies, and imaging of the cervical spine and the chest also can provide
3 Silverstein Pavilion,
3400 Spruce Street, helpful information regarding diagnosis. Clinical management usually starts with
Philadelphia, PA 19104. conservative treatment including exercise programs and physical therapy; when these
Email: huangj@uphs.upenn.edu therapies fail, patients are considered for surgery. Two of the most commonly used
Received, May 2, 2003.
surgical approaches are the supraclavicular exposure and the transaxillary approach
Accepted, May 24, 2004.
with first rib resection. On occasion, these approaches may be combined or, alterna-
tively, posterior subscapular exposure may be used in selected patients.
CONCLUSION: TOS is perhaps the most difficult entrapment neuropathy encountered
by neurosurgeons. Surgical intervention is indicated for vascular and true neurogenic
TOS and for some patients with the common or nonspecific type of TOS in whom
nonoperative therapies fail. With careful patient selection, operative intervention
usually yields satisfactory results.
KEY WORDS: Brachial plexopathy, First rib resection, Supraclavicular approach, Thoracic outlet syndrome,
Transaxillary approach

Neurosurgery 55:897-903, 2004 DOI: 10.1227/01.NEU.0000137333.04342.4D www.neurosurgery-online.com

T
horacic outlet syndrome (TOS) is one of the most con- curs between 20 and 50 years of age. From a practical stand-
troversial clinical entities in medicine. Although it is point, we divide patients with TOS into three groups: 1) those
generally accepted that TOS is caused by compression of with compression of the brachial plexus, also called neuro-
brachial plexus elements or subclavian vessels in their passage genic TOS; 2) those with compression of the subclavian vessels
from the cervical area toward the axilla and proximal arm, (either artery or vein), also called vascular TOS; and 3) those
there is a great deal of disagreement among clinicians regard- with nonspecific-type TOS, sometimes referred to as the dis-
ing its diagnostic criteria and optimal treatment (7, 16, 18, 24, puted or common type of TOS, consisting poorly defined
36, 45). Some physicians even doubt the existence of TOS. chronic pain syndrome with features suggestive of brachial
Because there is no objective confirmatory test for TOS, there plexus involvement (46). Occasionally, the neurological and
is also much disagreement among clinicians with regard to its vascular components may coexist in the same patient. The
true incidence (31, 46), with reported incidences ranging from neurogenic type of TOS is more common clinically than vas-
3 to 80 cases per 1000 population. A variety of diseases can cular TOS. The disputed or nonspecific-type TOS refers to a
mimic the presentation of TOS (Table 1), and in most situa- large group of patients with unexplained pain in the arm,
tions, TOS is a diagnosis of exclusion. TOS is more commonly scapular region, and cervical region. Their symptoms fre-
observed in women, and the onset of symptoms usually oc- quently are triggered by a discrete traumatic event such as a

NEUROSURGERY VOLUME 55 | NUMBER 4 | OCTOBER 2004 | 897


HUANG AND ZAGER

(Fig. 1). This triangle contains the trunks of the brachial plexus
TABLE 1. Differential diagnosis of thoracic outlet syndrome and the subclavian artery. The subclavian vein crosses anterior
to the anterior scalene muscle. Just distal to the interscalene
Cervical disc or osteophyte triangle, the neurovascular bundle enters the costoclavicular
Pancoast tumor triangle, which is bordered anteriorly by the middle third of
the clavicle, posteromedially by the first rib, and posterolater-
Nerve sheath tumor ally by the upper border of the scapula. Finally, the neurovas-
Ulnar and/or median nerve entrapment cular bundle enters the subcoracoid space beneath the cora-
coid process just deep to the pectoralis minor tendon.
Brachial plexitis
Compression or irritation of the brachial plexus elements
Syrinx or spinal cord tumor has been described in all three of these anatomic spaces, but
for practical purposes, the vast majority of cases of TOS in-
Shoulder pathology (e.g., rotator cuff injuries)
volve neural and/or vascular compression within the inter-
Fibromyalgia scalene triangle. This area may be small at rest and may
become even smaller with certain provocative maneuvers,
Multiple sclerosis
e.g., abduction and external rotation of the arm. Anomalous
Raynaud disease structures such as cervical ribs, anomalous muscles, and fi-
brous bands may constrict this triangle further. Anomalous
Acute coronary syndrome
fibrous bands are a more common cause of TOS than rib
Vasculitis anomalies (23, 30). The anomalous bands may originate from
a cervical or rudimentary 1st thoracic rib, C7 vertebra, Sibson’s
Vasospastic disorder
fascia (the suprapleural membrane) or the scalene muscles.
Complex regional pain syndrome Pang et al. (23, 24) have presented a detailed description and
illustration of the cervical band syndrome associated with
TOS. Repetitive trauma to the plexus elements, particularly
motor vehicle accident, and results of objective neurological the lower trunk and C8–T1 spinal nerves, is thought to play an
evaluation often are normal. The diagnosis of nonspecific-type important role in the pathogenesis of TOS. This may occur in
TOS depends on the presence of provocative reproduction of a variety of occupations, sports, and hobbies. The disputed or
symptoms as well as the absence of any other specific diag- nonspecific-type TOS frequently is triggered by a discrete
nosis. Unfortunately these provocative tests (e.g., Adson test) traumatic event such as a motor vehicle accident.
are themselves nonspecific, and results of neurophysiological
tests that are useful in documenting true neurogenic TOS are
normal in the nonspecific disorder. Finally, there is consider-
able disagreement among clinicians regarding treatment of
TOS and debate regarding conservative treatment versus sur-
gical intervention (6, 13, 17, 18, 21, 22, 25). The choice of
surgical approach is also controversial; both of the two pop-
ular approaches, the transaxillary first rib resection and the
supraclavicular approach, have been used widely and provide
good outcomes, although major surgical complications also
have been reported (4, 6, 10, 16, 19, 20, 29, 32, 43, 45).

SURGICAL ANATOMY
The term thoracic outlet has remained an unfortunate mis-
nomer for this anatomic region, which more properly should
be designated the thoracic inlet. The neurovascular bundle,
which contains the brachial plexus trunks and the subclavian
vessels, courses through three narrow passageways from the
base of the neck toward the axilla and the proximal arm. The
most important of these passageways clinically is the most
proximal, the interscalene triangle, which is bordered by the FIGURE 1. Illustration of the thoracic outlet anatomy showing the relation-
anterior scalene muscle anteriorly, the middle scalene muscle ship of the brachial plexus and subclavian artery to the clavicle and anterior
posteriorly, and the medial surface of the first rib inferiorly and middle scalene muscles.

898 | VOLUME 55 | NUMBER 4 | OCTOBER 2004 www.neurosurgery-online.com


THORACIC OUTLET SYNDROME

CLINICAL PRESENTATION decompression of the subclavian artery or vein and recon-


AND DIAGNOSIS struction of the damaged vessel if needed.
The true neurogenic and disputed types of TOS encompass
In the three types of TOS, compression of the vascular the vast majority of such patients evaluated by a surgeon. The
structures is relatively uncommon. It is usually easy to iden- contrast between these two types of TOS syndrome may be
tify with objective vascular modalities such as venography characterized by the relatively painless form, in which the
and arteriography (Fig. 2). Thrombosis of the subclavian vein neurological and electrodiagnostic findings are quite dra-
often is referred to as Paget-von Schrötter syndrome. It tends to matic, versus the type associated with chronic pain syndrome
occur in younger men and often is associated with strenuous and few definitive neurological or electrodiagnostic findings.
work. Patients often present with edema and cyanosis of the The classic finding in the patient with true neurogenic TOS is
upper extremity or distended superficial veins of the shoulder the so-called Gilliatt-Sumner hand with the most dramatic
and chest. Color flow duplex ultrasonography, magnetic res- degree of atrophy observed in the abductor pollicis brevis,
onance venography, or venography is used to assist in the with lesser involvement of the interossei and hypothenar mus-
diagnosis of subclavian vein compression and thrombosis. cles (Fig. 3) (12). Sensory loss is restricted to the ulnar aspect of
Similarly, compression of the subclavian artery often occurs in the hand and forearm, as predicted by the known compression
young adults with a history of vigorous arm activity. Patients of the lower trunk primarily. Upper trunk forms of TOS have
often present with pallor, pulselessness, and coolness of the been described, but the reports are authored by non-
affected upper extremity. Decreased blood pressure greater neurologists who evaluated patients with chronic pain syn-
than 20 mm Hg in the affected arm compared with the con- dromes rather than true neurogenic TOS. The typical patient
tralateral arm is often a reliable indicator of arterial involve- with the true neurogenic form of TOS is a young, thin female
ment. Rarely, patients can present with multiple small infarcts with a long neck and drooping shoulders (5, 24, 39, 47).
of the hand and fingers owing to embolization. Duplex ultra- However, we have observed cases in men and even in body-
sonography, magnetic resonance arteriography, computed to- builders with overly developed scalene musculature. Dull,
mographic angiography, and arteriography is used to confirm aching pain in the lateral neck, shoulder, axilla, parascapular
the diagnosis. Definitive treatment involves prompt surgical region, and inner aspect of the arm and forearm is often
described, but pain is not the cardinal feature, unlike the
common or nonspecific neurogenic form of TOS. Discomfort
may be provoked by repetitive use of the extremity, particu-
larly with overhead activities. Pain is often diffuse and non-
dermatomal, and paresthesia is most often restricted to the
ulnar aspect of the hand and forearm. There are no reliable
provocative tests, but the 90-degree abduction and external
rotation test seems to have the best predictive value (11, 28).
Tinel’s sign over the supraclavicular fossa also seems to be
important.

FIGURE 2. Schematic drawing showing proposed treatment algorithm for FIGURE 3. Photographs showing Gilliatt-Sumner hand in a patient TOS.
TOS. APB, abductor pollicis brevis; MRI, magnetic resonance imaging; Severe thenar wasting in the right (symptomatic) hand, mainly involving
MRA, magnetic resonance angiography; MRV, magnetic resonance venog- the abductor pollicis brevis, is observed. A, side view of both hands. B,
raphy; CTA, computed tomographic angiography; BP, brachial plexus; frontal view of the right hand. The thenar eminence has typical “scal-
C-spine, cervical spine; CXR, chest x-ray; TP, transverse process. loped” appearance.

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HUANG AND ZAGER

By far the most common patient to present to a surgeon’s


office with TOS has the nonspecific or common type. These
cases more often involve a traumatic event, e.g., a motor
vehicle accident or a work-related injury. Litigation often is an
important variable in these cases, and unfortunately it must be
included in the clinical decision-making equation. Physical
examination is difficult in these patients because of the ten-
dency to guard the extremity and to exhibit giveaway-type
weakness, and the sensory examination often is unreliable.
Atrophy is usually not observed except as a result of disuse in
truly chronic cases. Provocative maneuvers usually are posi-
tive in a wide anatomic area, often extending outside the
region innervated by the brachial plexus (Fig. 2). FIGURE 4. Anteroposterior x-ray of the cervical spine. The elongated
transverse process of the 7th cervical vertebra (dotted arrow) and a small
cervical rib on the left (solid arrow) are visible.
ELECTRODIAGNOSTIC AND
symptoms (e.g., tumors or degenerative disease of the cervical
IMAGING STUDIES spine) rather than to establish the diagnosis of TOS.
Nerve conduction studies and electromyography are often
helpful as components of the diagnostic evaluation of patients
with suspected TOS (35, 46). In advanced cases of neurogenic TREATMENT
TOS, nerve conduction studies commonly reveal the following
For most patients with TOS, common practice is to offer a
characteristics: 1) decreased amplitude in ulnar sensory action
course of conservative treatment. Conservative management in-
potentials; 2) decreased amplitude in median compound motor
cludes modification of behaviors by avoiding provocative activ-
action potentials; 3) normal or slightly decreased ulnar motor
ities and arm positions, in addition to individually tailored phys-
potentials; and 4) normal median nerve sensory potentials. Fi-
ical therapy programs that strengthen the muscles of the pectoral
brillation potentials (suggestive of active axonal loss) are most
girdle and help to restore normal posture (17, 22). The reported
pronounced in the median innervated abductor pollicis brevis
improvement with conservative management of TOS ranges
and less pronounced in the ulnar innervated first dorsal in-
from 50 to 90% (3, 13, 21, 22, 46). Only in extremely rare situations
terosseous and abductor digiti quinti. Electromyography often
is emergency surgery indicated, e.g., in patients with infarction
reveals severe abnormalities in the hand intrinsic muscles.
caused by vascular occlusion or recurrent embolism.
The electrodiagnostic findings described above can be ex-
Surgical intervention is indicated in the vascular forms of TOS,
plained by the pathogenesis of TOS. Although all three trunks of
which are usually resistant to conservative management. Surgi-
the brachial plexus enter the interscalene triangle, only the lower
cal treatment is also indicated in patients with the true neuro-
trunk (C8–T1), along with the subclavian artery, is in direct
genic form of TOS, in which conservative management most
contact with the tendinous portion of the scalene muscle and the
often is unhelpful. Many of these patients present with advanced
hard surface of the rib. All of the C8–T1 sensory fibers course in
atrophy. On the other hand, we offer surgery to patients with the
the ulnar nerve, which accounts for the decreased ulnar sensory
nonspecific or disputed type of TOS only as a last resort, after
potentials and normal median sensory potentials. The C8–T1
prolonged conservative management and a clear discussion em-
motor fibers are carried by both the median and ulnar nerves, but
phasizing the potential risks and complications of surgical explo-
the median motor fibers are preferentially injured as a result of
ration in this complex anatomic region.
their location within the lower trunk. Thus, the median motor
compound action potentials tend to reveal a greater degree of
abnormality than the ulnar motor potentials. SURGICAL APPROACHES
In addition to electrophysiological studies, imaging studies
can provide useful information in the diagnosis of TOS. Cer- The two most commonly used surgical approaches for treat-
vical spine and chest x-rays are important in the identification ment of TOS are the anterior supraclavicular and the transax-
of bony abnormalities, including cervical ribs or prominent, illary approach for resection of the first rib. Major neurovas-
often “peaked” C7 transverse processes (Fig. 4). Anomalous cular complications have been reported with both approaches.
cervical ribs are reported in approximately 10% of patients Surgeons typically use the approach with which they are most
with TOS (26, 30, 38). The estimated incidence of anomalous familiar to achieve the best outcome. A third approach, the
ribs as revealed by routine chest x-rays in the general popu- posterior subscapular approach, is used much less frequently.
lation varies from less than 0.01% to 0.5% (1, 2, 9, 27, 37, 40).
Most cervical ribs are not symptomatic, however. Magnetic Transaxillary Approach
resonance imaging and computed tomography are more use- The transaxillary approach with resection of the first rib is
ful for the identification of other conditions that might cause favored by most thoracic surgeons and many vascular sur-

900 | VOLUME 55 | NUMBER 4 | OCTOBER 2004 www.neurosurgery-online.com


THORACIC OUTLET SYNDROME

geons (31, 32, 44, 45). It was first popularized by Roos in 1966 long thoracic nerve injury or unsatisfactory healing of the
(29). With the patient in a posterolateral position and the arm levator and rhomboid muscles (7, 8, 24).
elevated above the head, an incision is made over the palpable
rib (usually the third rib) in the axillary fossa. The axillary fat, CONCLUSION
lymph nodes, and vessels are dissected away, and the anterior
scalene and middle scalene muscles are divided. The first rib TOS is perhaps the most difficult-to-treat entrapment neu-
is identified and resected. The advantage of this approach is ropathy encountered by neurosurgeons. Surgical intervention
that it allows easy access through a hidden incision to almost is indicated for patients with vascular and true neurogenic
the entire first rib yet unhindered by the neurovascular struc- TOS and in those with the nonspecific or disputed type of
tures. The shortcoming of this approach lies in its limited TOS, in whom nonoperative treatment fails. With careful pa-
exposure of the neural elements. Most congenital bands and tient selection, operative intervention usually can yield satis-
cervical ribs lie medial to the first rib and hidden behind the factory results.
neurovascular structures.
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46. Wilbourn AJ: The thoracic outlet syndrome is overdiagnosed. Arch Neurol
47:328–330, 1990.
47. Zager EL: Illustrative case, in Loftus C, Batjer HH (eds): Techniques in
H uang and Zager have provided a review of TOS, includ-
ing its various forms and commonly accepted treatment
strategies. They present and discuss three syndromes: 1) com-
Neurosurgery. Philadelphia, Lippincott-Raven, 2000, pp 2–4.
pression of the brachial plexus (neurogenic thoracic syn-
drome), 2) compression of the subclavian artery, and 3) com-
Acknowledgments
pression of the subclavian vein. They discuss in particular the
This work was supported in part by the American Association of Neurological presentation and management strategies for neurogenic TOS,
Surgeons Codman Award and David Kline Award (to JHH).
commonly known to neurosurgeons as cervical band syn-
drome, which frequently is underdiagnosed. The authors have
COMMENTS raised our awareness of this entity and have provided a strat-
egy for diagnosis and management.
T he authors have presented a review article on a complex and
challenging subject. They reviewed a literature that, at its
best, can be confusing and frustrating. It has always been my
Edward C. Benzel
Cleveland, Ohio

902 | VOLUME 55 | NUMBER 4 | OCTOBER 2004 www.neurosurgery-online.com


THORACIC OUTLET SYNDROME

T he authors review the history and the current diagnostic and


surgical management options of TOS. This topical review
provides basic and standard information to the practicing neu-
comes are not provided because it was the main intent of the
authors to discuss the entity and its clinical presentation.
Several additional observations are in order. Dr. Jim Camp-
rosurgeon in the area of TOS and serves as a reference guide for bell and his group at Johns Hopkins favor transaxillary first
researching this topic further. TOS is a complex problem, as is rib resection, and in their hands, this approach seems to work
delineated by the authors, in that it has multiple presentations. well. Our group favors a supraclavicular approach or, in se-
The concern is with regard to the concept of neurogenic TOS lected cases, a posterior subscapular approach. This is in part
versus nonspecific arm and shoulder discomfort with extension based on intraoperative nerve action potential recordings
to the scapula. In these instances, the surgical indications become found not only in Gilliatt-Sumner hand patients but also in
unclear, as does whether decompression is indicated. Further those with disputed TOS; the operative electrophysiological
workup is lacking, and the results are often less satisfying in findings are at a spinal nerve-to-trunk level, not more laterally
these patients. The authors, however, provide a treatment algo- at a trunk-to-division level (1, 2; G Tender, A Thomas, N
rithm that would be very useful to a practicing neurosurgeon Thomas, D Kline, in preparation). Thus, the problem, where
with regard to diagnostic tests, differential diagnosis, and poten- one exists, is a proximal one close to the spine, and indeed,
tial surgical treatment. Overall, this article provides a good re- when we see anatomic correlates, such as a medial scalene
view, but is only a starting point for neurosurgeons who may be fibrous edge or band with or without cervical rib or elongated
further interested in this area. C7 process, scalene minimus, or Sibson’s fascia entrapment,
those variants also are close to the exit of the spinal nerve from
P. David Adelson
the spine and do not occur more laterally.
Pittsburgh, Pennsylvania
David G. Kline
T he authors have provided a succinct review of TOS. They
emphasize the difficulty in making this diagnosis, espe-
cially for disputed neurogenic TOS. This means that all other
New Orleans, Louisiana

possible differential diagnoses need to be excluded as much as


possible before this diagnosis is entertained. Also highlighted 1. Kim D, Cho Y, Tiel R, Kline D: Outcomes of surgery in 1019 brachial plexus
lesions treated at Louisiana State University Health Sciences Center.
by the authors is the need for a good trial of physical and J Neurosurg 98:1005–1016, 2003.
occupational therapy before resorting to surgery. Descriptions 2. Kline D, Hudson A: Nerve Injuries: Operative Results for Major Nerve Injuries,
of the operative approaches are brief, of necessity, and out- Entrapments, and Tumors. Philadelphia, W.B. Saunders Co., 1995.

Plaster cast of pregnant woman found on Via Stabiana in Pompeii. Her body, covered in a fine layer of ash, created a mold that was later filled with plas-
ter using the Giuseppe Fiorelli method, named after the archaeologist who led the official excavation of the site in 1860. (Photo courtesy of Electa, Milan.)
Please see de Divitiis et al., pp. 989–1006.

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