handarm vibration syndrome R. A. Cooke Background Thoracic outlet syndrome (TOS) is a cause of vascular and neurological com- promise to the arm and hand, and may manifest as Raynauds phenomenon. It may be under-diagnosed. Aim This review was undertaken in order to clarify the diagnostic and investigative 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 as the primary search engines. Conclusion Thoracic outlet syndrome most commonly presents with neurological symptoms in the arm. Vascular symptoms, including Raynauds phenomenon, may occur in ~10% of cases. Careful clinical assessment by history and examination may reveal the elements of forearm and upper arm symptoms with postural exacerbation, which distinguish this condition from handarm vibration syndrome. The usefulness of investigation is unclear, but Doppler and neuroelectric studies may be valuable. 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, abnormal ligamentous 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 under- rated, overlooked and misdiagnosed peripheral nerve compression in the upper extremity [2], as well as the most important and difficult to manage. A retrospective study of cases of TOS presenting to an emergency depart- ment during a 29 year period concluded that a lack of thorough evaluation resulted in under-diagnosis of TOS [3]. Ouriel [4] suggested that the relative rarity of vascular disorders of the arm accounts for even experienced vascular clinicians being unfamiliar with upper extremity diagnostic testing. Anatomy The thoracic outlet is bounded by the first thoracic vertebra, the superior border of the manubrium sterni anteriorly, and the first rib and costal cartilage laterally. The structures passing through this area and into the upper limb are the subclavian artery and vein, and the 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: Roger@vwfmed.fsnet.co.uk Occupational Medicine 2003;53:331336 DOI: 10.1093/occmed/kqg097 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, Boles et 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 Blanchard et 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, with intermittent 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. Durham et 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. 332 OCCUPATIONAL MEDICINE
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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 brachial plexus 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. Novak et 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 limb pulses 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 most commonly electrophysiologically demonstrated lesion. In 16.2% of patients compression of the subclavian artery was also demonstrated. It was concluded that handarm vibration exposure may play a part in the development of TOS, and conversely that TOS may contribute to Raynauds phenomenon of occupational origin [8]. A number of provocative tests have been described, based on the concept that change in shoulder/neck position may produce symptoms, including Adsons and Roos tests. Pulse obliteration indicates some element of anatomic tightness, but it has been suggested that without symptom reproduction, pulse change has no diagnostic relationship to TOS [22]. Novak et al. [23] reported that 94% had positive provocative position and compression test results, and suggested that clinical assessment of TOS is best achieved by reproduction of symptoms with compression and positional provocative testing. In a study of TOS shoulder manoeuvres in 53 healthy subjects, the outcomes of pulse alteration or paraesthesiae were unreliable in general. However, TOS shoulder man- oeuvres have reasonably low false-positive rates when a positive outcome is defined as pain after Adsons test, costo-clavicular manoeuvre or supraclavicular pressure; discontinuation of the elevated arm stress test secondary to pain; pain in the same arm with two or more man- oeuvres; or any symptom in the same arm with three of more manoeuvres [24]. Warrens and Heaton [25] assessed manoeuvres advocated for the assessment of thoracic outlet compression on 64 randomly chosen volunteers. Although only 17% had any symptoms of thoracic outlet compression, 58% had a positive result in at least one of the manoeuvres. Only 2% were positive for more than two manoeuvres. They concluded by suggest- ing that the low specificity devalues these tests in clinical practice. Gillard et al. [26] found that provocative tests had mean sensitivity and specificity values of 72 and 53%, respectively, with better values for the Adson test [positive predictive value (PPV) = 85%] and the hyperabduction test (PPV = 92%). Kitamura et al. [27] assessed the influence of deep breathing on Adsons test by using laser Doppler flow- metry to quantitatively assess changes in blood flow measured at the arm during Adsons test, the reverse Adson test and deep breathing in the sitting position. In 10 patients who were found during surgery to have anatomical abnormalities in the thoracic outlet area, preoperative administration of Adsons test had not indicated any compression that caused cardiac wave disappearance and continued blood flow reduction. They concluded that Adsons test, which involves compression by the scalene muscles, is greatly affected by deep breathing during the test, and that the results of this test do not faithfully reflect vascular compression. R. A. COOKE: THORACIC OUTLET SYNDROME 333
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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 TOS were studied by Lascelles et 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 a compressive anatomical abnormality identified by CT scan. Three-dimensional CTimaging 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 evaluating the 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 Novak et 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 Hachulla et 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 the flow 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. Wadhwani et 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. 334 OCCUPATIONAL MEDICINE
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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 arm
either 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. Doppler studies 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. References 1. Chirkov A, Zakhariev T, Stankev M, Markov D. The costoclavicular neurovascular compression syndrome. Khirurgiia 1995;48:510. 2. Sheth RN, Belzberg AJ. Diagnosis and treatment of thoracic outlet syndrome. Neurosurg Clin N Am 2001;12:295309. 3. Shukla PC, Carlton FB Jr. Diagnosis of thoracic outlet syndrome in the emergency department. South Med J 1996;89:212217. 4. Ouriel K. Noninvasive diagnosis of upper extremity vascular disease. SeminVasc Surg 1998;11:5459 [Review]. 5. Boles JM, MissoumA, Mocquard Y, et al. A familial case of thoracic outlet syndrome. Clinical, radiological study with treatment. Semaine Hop 1981;57:11721176. 6. Sallstrom J, Thulesius O. Non-invasive investigation of vascular compression in patients with thoracic outlet syndrome. Clin Physiol 1982;2:117125. 7. Baker CL Jr, Liu SH. Neurovascular injuries to the shoulder. J Orthop Sports Phys Ther 1993;18:360364. 8. Kakosky T. Tunnel syndromes of the upper extremities in workers using hand operated vibrating tools. Med Lav 1994;85:474480. 9. Blanchard B, Blanchard G, Forcier P, Cloutier LG. The thoracic outlet: true syndromes, disputed syndrome. Rev Med Suisse Romande 1992;112:253266. 10. Roos DB. Pathophysiology of congenital anomalies in thoracic outlet syndrome. Acta Chir Belg 1980;79:353361. 11. Morbidelli A, Miani S, Bortolani E. Complete cervical rib. Possible neurovascular implications of the upper limb. Min Chir 1989;44:11671172. 12. Young HA, Hardy DG. Thoracic outlet syndrome. Br J Hosp Med 1983;29:457461. 13. Branchereau A. Castro M. Devin R. Cervical ribs. Therapeutic deliberations apropos of 10 cases. Nouv Presse Med 1976;5:24512454. 14. Gruss D. Thoracic outlet syndrome. Acta Chir Aust 2000;32:1519. 15. Desai Y, Robbs JV. Arterial complications of the thoracic outlet syndrome. Eur J Vasc Endovasc Surg 1995;10:362365. 16. DurhamJR, Yao JS, Pearce WH, Nuber GM, McCarthy WJ 3rd. Arterial injuries in the thoracic outlet syndrome. JVasc Surg 1995;21:5769. 17. Mackinnon SE, Novak CB. Evaluation of the patient with thoracic outlet syndrome. Semin Thorac Cardiovasc Surg 1996;8:190200. 18. Rayan GM. Lower trunk brachial plexus compression neuropathy due to cervical rib in young athletes. Am J Sports Med 1988;16:7779. 19. Matsuyama T, Okuchi K, Goda K. Upper plexus thoracic outlet syndrome: case report. Neurol Med Chir (Tokyo) 2002;42:237241. 20. Maissonneuve H, Planchon B, de Faucal P, Mussini JM, Patra P. Vascular manifestations of thoracic outlet syndrome. Prospective study of 104 patients. J Mal Vasc 1991;16:220225. 21. Pistorius MA, Planchon B. Incidence of thoracic outlet syndrome on the epidemiology and clinical presentation of apparently primary Raynauds phenomenon. Int Angiol 1995;14:6064. 22. Youmans CR Jr, Smiley RH. Thoracic outlet syndrome with negative Adsons and hyperabduction maneuvers. Vasc Surg 1980;14:318329. 23. Novak CB, Mackinnon SE, Patterson GA. Evaluation of patients with thoracic outlet syndrome. J Hand Surg [Am] 1993;18:292299. 24. Plewa MC, Delinger M.The false-positive rate of thoracic outlet syndrome shoulder maneuvers in healthy subjects. Acad Emerg Med 1998;5:337342. 25. Warrens AN, Heaton JM. Thoracic outlet compression syndrome: the lack of reliability of its clinical assessment. Ann R Coll Surg Eng 1987;69:203204. 26. Gillard J, Perez-Cousin M, Hachulla E, et al. Diagnosing thoracic outlet syndrome: contribution of provocative tests, ultrasonography, electrophysiology, and helical computed tomography in 48 patients. Joint Bone Spine 2001;68:416424. R. A. COOKE: THORACIC OUTLET SYNDROME 335
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