thoracic outlet syndrome - handfoundation.org

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THORACIC OUTLET SYNDROME A Lee Osterman, MD The Philadelphia Hand Center Compression neuropathies – Getting it right so you don't have t do it again or deal with complications The presentation of thoracic outlet syndrome may vary dramatically from patient to patient, including a variety of symptoms and signs. Objective tests such as electrodiagnostic studies are notoriously unreliable in characterizing this entity. Indeed, the very existence of TOS as a discrete entity has been controversial. 1 Those who accept its existence admit that the diagnosis must be a clinical one, based upon the gestalt rather than a punch list or algorithm. The variability and complexity of this syndrome lends itself to mistakes in diagnosis as well as in surgical treatment. Even experienced clinicians must be vigilant and humble when approaching the TOS patient. Anatomy An understanding of the anatomy of the thoracic outlet is crucial in avoiding errors in diagnosis as well as in surgical treatment. Variations in the bony, muscular, vascular and neural anatomy all contribute to the presentation of the TOS patient. Over 30% of patients may demonstrate congenital anomalies that present additional challenges to the surgeon. 2 A history of prior trauma 3 or surgery about the thorax, neck or shoulder, must raise suspicion for alterations in the anatomy. Although neural compression tends to generate the most obvious symptoms, arterial or venous compression may predominate in any particular patient. When the symptoms are localized to the subclavian artery 4 , subclavian vein 5 or a defined segment of the brachial 1 Wilbourn AJ: The Thoracic outlet syndrome is overdiagnosed. Arch Neurol 1990:47:328-330. 2 Roos DB: Congenital anomalies associated with thoracic outlet syndrome. Anatomy, symptoms, diagnosis and treatment. Am J Surg 1976:132:771-778. 3 Mulder DS, Greenwood FAH, Brooks CE: Posttraumatic thoracic outlet syndrome. J Trauma 1973:13:706-715. 4 Cormier JM, Amrane M, Ward A, et al: Arterial complications of the thoracic outlet syndrome. Fifty-five operative cases. J Vasc Surg 1989:9:778-787.

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Page 1: THORACIC OUTLET SYNDROME - handfoundation.org

THORACIC OUTLET SYNDROME A Lee Osterman, MD

The Philadelphia Hand Center Compression neuropathies – Getting it right so you don't have to

do it again or deal with complications

The presentation of thoracic outlet syndrome may vary dramatically from patient to patient, including a variety of symptoms and signs. Objective tests such as electrodiagnostic studies are notoriously unreliable in characterizing this entity. Indeed, the very existence of TOS as a discrete entity has been controversial.1 Those who accept its existence admit that the diagnosis must be a clinical one, based upon the gestalt rather than a punch list or algorithm. The variability and complexity of this syndrome lends itself to mistakes in diagnosis as well as in surgical treatment. Even experienced clinicians must be vigilant and humble when approaching the TOS patient. Anatomy An understanding of the anatomy of the thoracic outlet is crucial in avoiding errors in diagnosis as well as in surgical treatment. Variations in the bony, muscular, vascular and neural anatomy all contribute to the presentation of the TOS patient. Over 30% of patients may demonstrate congenital anomalies that present additional challenges to the surgeon.2 A history of prior trauma3 or surgery about the thorax, neck or shoulder, must raise suspicion for alterations in the anatomy. Although neural compression tends to generate the most obvious symptoms, arterial or venous compression may predominate in any particular patient. When the symptoms are localized to the subclavian artery4, subclavian vein5 or a defined segment of the brachial

1 Wilbourn AJ: The Thoracic outlet syndrome is overdiagnosed. Arch Neurol 1990:47:328-330. 2 Roos DB: Congenital anomalies associated with thoracic outlet syndrome. Anatomy, symptoms, diagnosis and treatment. Am J Surg 1976:132:771-778. 3 Mulder DS, Greenwood FAH, Brooks CE: Posttraumatic thoracic outlet syndrome. J Trauma 1973:13:706-715. 4 Cormier JM, Amrane M, Ward A, et al: Arterial complications of the thoracic outlet syndrome. Fifty-five operative cases. J Vasc Surg 1989:9:778-787.

Page 2: THORACIC OUTLET SYNDROME - handfoundation.org

plexus, the clinician should be suspicious for an anatomic variation.6 When the symptoms involve the entire contents of the thoracic outlet, postural and scalene abnormalities become the primary suspects. At surgery, all potential sites of compression must be examined systematically and thoroughly. Diagnosis Although the diagnosis of TOS must primarily be clinical, it must be distinguished from other causes of generalized upper extremity pain. It is essential, therefore, to perform a thorough physical exam, including the cervical spine, the shoulder and the entire upper extremity bilaterally, even when the symptoms are unilateral or relatively localized. The myriad complaints attributable to thoracic outlet syndrome, their variability and diffuseness tend to upset the clinician who wants a classic textbook description.7 Facial complaints, headaches, arm heaviness or swelling and diffuse weakness are more frequent than the classic numbness or vascular symptoms that occur with an elevated upper extremity. A common error performed in the examination includes the failure to identify subtle sensory changes which may be present on the medial aspect of the forearm or the ulnar hand. The medial antebrachial cutaneous nerve originates directly from the plexus and thus can be helpful in defining a site of brachial plexus irritation when such altered sensation is present. Weakness and atrophy may be difficult to distinguish from apprehension in some patients. Body habitus observations such as the presence of a gazelle-like neck, asymmetric shoulder height, large breasts8, or slouching posture should raise the physician’s antenna as to the possibility of thoracic outlet syndrome. Much like peripheral nerve testing, a supraclavicular or infraclavicular Tinel’s sign is specific but not particularly sensitive. Direct thumb pressure over the plexus that recreates the patient’s symptoms has been a more common and rewarding diagnostic finding. Grip strength testing measured in multiple positions is a standard of our upper extremity exam.9 The presence of an invalid grip pattern bodes a poor prognosis for treatment and may suggest that factors other than thoracic outlet syndrome may account for the patient’s disability.

5 Judy KI, Heymann RI: Vascular complications of thoracic outlet syndrome. Am J Surg 1972:123:521-531. 6 Adson AW: Surgical treatment for symptoms produced by cervical ribs and the scalenus anticus muscle. Surg Gynecol Obstet 1947:85:687-700. 7 Warrens AN, Heaton JM: Thoracic outlet compression syndrome: The lack of reliability of its clinical assessment. Ann R Coll Surg Engl 1987:69:203-204. 8 Kaye BL: Neurologic changes with excessively large breasts. South Med J 1972;65:177-180. 9 Harkonen R, Piirtomaa M, Alaranta H. Grip strength and hand position of the dynamometer in 204 Finnish adults. JHS 1993;18B:1:129-132.

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Provocative maneuvers may have relatively high sensitivity when performed correctly; however, the specificity is notably poor. Simple obliteration of the pulse with Adson’s and Wright’s maneuvers should not be considered positive unless accompanied by reproduction of the patient’s symptoms. Many normal individuals may demonstrate pulse obliteration, including, for example, 60% of the linemen of a college football team. The elevated arm test, felt to be pathognomonic by its describer, Dr. David Roos, has been shown by us to be equally positive in a patient with isolated peripheral neuropathies, including cubital tunnel and carpal tunnel syndromes.

• Cervical radiculopathy and/or degenerative disc disease10 • Brachial neuritis11 • Peripheral nerve compression12 (carpal tunnel syndrome, cubital tunnel

syndrome) • Nerve injury about the shoulder13 (suprascapular, long thoracic, axillary) • Syringomyelia14 • Multiple sclerosis15 • Vascular syndromes16 (Raynaud’s, quadrilateral space compression17) • Cardiac pathology18 • Mechanical shoulder problems (rotator cuff, impingement19, “Dead arm

syndrome”20) • Myofascial syndromes21 • Tumors of the supraclavicular fossa or lung22 • Complex regional pain syndromes23

10 Murphey F. Sources and patterns of pain in disc disease. Clin Neurosurg 1968;15:343-51. 11 McCarty EC, Tsairis P, Warren RF: Brachial neuritis. Clin Orthop 1999 Nov;(368):37-43. 12 Carroll RE, Hurst LC: The relationship of thoracic outlet syndrome and carpal tunnel syndrome. Clin Ortho Apr 1982;164:149-153. 13 Goslin KL, Krivickas LS: Proximal neuropathies of the upper extremity. Neurol Clin 1999 Aug;17(3):525-48. 14 Singer GL, Brust JC, Challenor YB: Syringomyelia presenting as shoulder dysfunction. Arch Phys Med Rehabil 1992 Mar;73(3):285-8. 15 Melville ID: The differential diagnosis of nerve compression syndromes in the arm and hand. The neurologist's approach. Hand 1972 Jun;4(2):111-4. 16 Kutz JE, Rowland EB Jr: Vascular compression about the shoulder. Hand Clin. 1993 Feb;9(1):131-8. 17 Cahill BR, Palmer RE: Quadrilateral space syndrome. J Hand Surg [Am] 1983 Jan;8(1):65-9. 18 Urschel HC Jr, Razzuk MA, Hyland JW, et al: Thoracic outlet syndrome masquerading as coronary artery disease (pseudoangina). Ann Thorac Surg 1973;16:239-248. 19 Boyle JJ: Is the pain and dysfunction of shoulder impingement lesion really second rib syndrome in disguise? Two case reports. Man Ther 1999 Feb;4(1):44-8. 20 Leffert RD, Gumley G: The relationship between dead arm syndrome and thoracic outlet syndrome. Clin Orthop 1987;223:20-31. 21 Lauder TD: Musculoskeletal disorders that frequently mimic radiculopathy. Phys Med Rehabil Clin N Am 2002 Aug;13(3):469-85. 22 Detterbeck FC: Pancoast (superior sulcus) tumors. Ann Thorac Surg 1997 Jun;63(6):1810-8. 23 Raja SN, Grabow TS: Complex regional pain syndrome I (reflex sympathetic dystrophy). Anesthesiology 2002 May;96(5):1254-60.

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In patients presenting with vascular complaints, bruit auscultation over the plexus area is important. In longstanding thoracic outlet syndrome, there may be post-stenotic dilatation of the subclavian artery producing an audible bruit. This auscultation can also be enhanced if it is performed with the arm in the symptomatic provocative position. Failure to identify one of the above causes of upper extremity pain may result in persistence or exacerbation of symptoms. Thoracic outlet syndrome often occurs concurrently with other diagnoses. We have noted the frequent association of mechanical shoulder pathology and brachial plexopathy at the thoracic outlet area. Finally, though debated by some, we have found a “double crush” phenomenon24,; i.e., an increased association of thoracic outlet syndrome with downstream peripheral compression neuropathy such as cubital, radial, and carpal tunnel syndromes. In such cases of multiple areas of compression neuropathy, the clinician should consider hereditary sensitivity to pressure palsy or intrinsic neuropathy (diabetes) as playing a potential role.25 Ancillary Studies Imaging: Plain x-rays of the cervical are essential in the evaluation of the thoracic outlet syndrome. The frequency of cervical ribs is 0.5 – 1%.26,27 There is a particularly high association of the “sucked candy” partial cervical rib and fibrous bands that predispose the thoracic outlet decompression. If there are cervical findings then an MRI of the cervical spine is helpful to assess root and/or disc involvement.

24 Wood VE, Biondi J: Double-crush nerve compression in thoracic-outlet syndrome. JBJS [Am] 1990; 72:85-87. 25 Jaeger SH, Singer DI, Whitenack SH, Mandel S: Nerve injury complications. Management of neurogenic pain syndromes. Hand Clin 1986 Feb; 2(1):217-234. 26 Pollack EW: Surgical anatomy of the thoracic outlet syndrome. Surg Gynecol Obstet 1980:150:97-103. 27 Sanders RJ, Hammond SL. Management of cervical ribs and anomalous first ribs causing neurogenic thoracic outlet syndrome. J Vasc Surg 2002 Jul; 36(1):51-56.

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MRI of the brachial plexus itself has not been helpful in our experience. We use it when we are concerned with a possible tumor or other unusual mass. In a prospective series of 40 patients undergoing thoracic outlet decompression, the brachial plexus MRI yielded only one helpful result: a Schwannoma was visualized. This tumor was already suspected, however, because of a palpable mass in the supraclavicular fossa. If there is a concern for a pulmonary problem, then routine chest x-ray and apical lordotics may be beneficial. A CT scan is useful if there is a clavicular abnormality such as a malunion or nonunion with abundant callous or sternoclavicular instability. Such a study details the preoperative bony deformity and its relationship to the outlet. With few exceptions we have abandoned the use of invasive vascular studies.28 Such exceptions include a patient with distal emboli or gangrene, a palpable bruit, clavicular mass or a patient with previous vascular surgery in the area. Recently, I have used an MRA, which has been helpful in such cases and may replace invasive vascular imaging. Other vascular studies include pulse volume recording. Such noninvasive recording, even when positive in provocative positions, has a yield comparable to a clinical Adson’s test. We still occasionally will use it to quantitate flow in the unilateral patient especially if cold intolerance and bluishness are dominant symptoms. Electrical studies: Electrical studies are notoriously unreliable in thoracic outlet syndrome.29 We use them routinely not to confirm brachial plexopathy at the thoracic outlet but to evaluate cervical radiculopathy and other distal peripheral compression problems. Provocative brachial plexus conduction studies are controversial and examiner dependent.30 We have also used somatic evoked potential studies which are more reliable in defining brachial plexus irritation.31,32 Patients without evidence of any electrical involvement in such studies with a normal neurologic examination are generally advised that despite their symptoms, no defined nerve damage exists and, hence, nonoperative treatment is recommended. Nonoperative Treatment 28 Sallstrom J, Thulesius O. Noninvasive-investigation of vascular compression in patients with thoracic outlet syndrome. Clin Physiol 1982;2:117-25. 29 Urschel HC Jr, Razzuk MA, Wood RE, et al: Objective diagnosis (ulnar nerve conduction velocity) and current therapy of the thoracic outlet syndrome. Ann Thorac Surg 1971:12:608-620. 30 Wilbourn AJ, Lederman RJ: Evidence for conduction delay in thoracic-outlet syndrome is challenged [letter]. N Engl J Med 1984;310:1052-1053. 31 Veilleux M, Stevens JC, Campbell JK: Somatosensory evoked potentials: Lack of value for diagnosis of thoracic outlet syndrome. Muscle Nerve 198:11:571-575. 32 Machleder HI, Moll F, Nuwer M, Jordan S: Somatosensory evoked potentials in the assessment of thoracic outlet compression syndrome. J Vasc Surg 1987;6:177-184.

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Most experienced clinicians utilize nonoperative regimens as their initial treatment for TOS. The most common errors in nonoperative treatment include failure to customize a treatment program for the particular patient and failure to continue the nonoperative plan for a sufficient duration to effectively alter the patient’s symptoms.33 Non-narcotic medication such as Elavil or Neurontin may lower these symptoms to a tolerable level. Surgical Treatment The first step in surgical treatment should be communicating with the patient the goals and limitations of surgery. This discussion must include a delineation of the potential complications, the risk of persistence of symptoms, exacerbation of symptoms, and recurrence. Our surgical outcome data at three years has shown 78% of patients are improved, 17% are unchanged, and 5% are worse. The worsening generally relates to increased pain, possibly due to perineural scarring. All forms have complications: catastrophic and minor have been reported. The patients have been directly informed of the major complications that include death secondary to uncontrolled subclavian hemorrhage, the possibility of multiple transfusions, damage to the plexus or phrenic nerve with transient or permanent paralysis, and pneumothorax possibly requiring a chest tube. While not meant to specifically frighten the patient, the frank discussion of these complications emphasizes the seriousness of the proposed surgery and often causes the patient to reconsider the severity of their symptoms against the backdrop of such potential complications. It is also important to explain that thoracic outlet decompression will relieve only compressive symptoms and those neural symptoms related to intraneural scarring will not be affected. Patients with concomitant pathology contributing to their symptoms must understand the specific symptoms being addressed in this operation. Electrodiagnostic evidence of cervical radiculopathy or peripheral neuropathy may require that additional treatment be rendered prior to their thoracic outlet decompression or even simultaneously. Females with excessively large breasts may benefit from mammoplasty prior to thoracic outlet decompression. The patient with mechanical shoulder pathology may benefit from a less risky shoulder procedure and get some secondary relief by taking the mechanical drag off the plexus. Failure to address these sites of pathology or, more importantly, failure to communicate with the patient the goals and expected outcome of their surgery may adversely affect their objective and subjective outcomes. Perhaps the most common error in the treatment of thoracic outlet syndrome lies in the selection criteria for surgical candidates. Operative intervention for thoracic outlet syndrome should be reserved for patients with very clear indications: 1) vascular insufficiency34 2) subclavian vein thrombosis 3) neurologic compromise 4) failure of

33 Peet RM, Henriksen JD, Anderson TP, et al: Thoracic-outlet syndrome: Evaluation of a therapeutic exercise program. Staff Meetings Mayo Clin 1956;31:281-287. 34 Thompson JF, Webster JHH: First rib resection for vascular complications of thoracic outlet syndrome. Br J Surg 1990:77:555-557.

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an appropriately designed and closely supervised therapy program with intractable pain. Generally most thoracic outlet syndrome patients will have some response to an appropriate nerve gliding program, and failure to respond should alert the physician to other possible non-surgical entities as well as to a potential poor surgical prognosis. Above all, a reproducible and valid clinical examination is essential in defining a surgical candidate. Lastly, narcotic pain medications should be weaned or at least under the management of a pain control center. Intraoperative errors often stem from the difficulty in recognizing landmarks in this area of frequent anatomic variability. The surgeon may plan on performing a 1st rib resection but fail to resect the complete rib. Occasionally, the 2nd rib is resected inadvertently, leaving the 1st rib in place. Or, perhaps, the 1st rib is resected, but a cervical rib is not identified. Sometimes a cervical rib is resected, but an abnormal 1st rib is left. The key to avoiding such errors lies in both preoperative x-rays and intraoperative x-rays when there is doubt as to the rib level. We routinely use intraoperative nerve stimulation and monitoring. This allows us to actively map out the plexus as well as identify the phrenic nerve as it crosses the anterior scalene. Exposure must be sufficient to identify the normal and abnormal anatomy, including the offending structures, the structures being decompressed and the structures at risk. It is impossible to assess whether sufficient rib resection has been performed without an exposure large enough to visually inspect the contents of the thoracic outlet adjacent to the rib. Furthermore, damage to the pleura, subclavian vessels or brachial plexus becomes far more likely when the surgeon is unable to visualize them. Multiple excellent approaches to the thoracic outlet have been described. However, as with any procedure, selection of the appropriate approach is of paramount importance in avoiding preventable complications.35 The foremost consideration should be the surgeon’s experience and comfort level with a particular approach. Once this has been taken into account, there are certain caveats with particular approaches that merit mention.36 Transaxillary 1st rib resection37 should be performed with the patient in the lateral decubitus position with an assistant to control the upper extremity. Utilization of a traction apparatus to hold the upper extremity may compromise the exposure as well as result in neurapraxic injury. This approach also requires complete skeletal muscle

35 Sanders RJ, Pearce WH: The treatment of thoracic outlet syndrome: A comparison of different operations. J Vasc Surg 1989;10:626-634. 36 Kostic S, Kulka F: Reasons behind surgical failures in thoracic outlet syndrome. Int Surg 1990;75:159-161. 37 Roos DB: Transaxillary approach for first rib resection to relieve thoracic outlet syndrome. Ann Surg 1966;163:354-358.

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relaxation be induced by the anesthesiologist to allow full access to all the structures. The surgeon should be aware of the intercostobrachial nerve, which should be spared to prevent posterior arm numbness. The long thoracic nerve may be at risk as it courses through or immediately posterior to the middle scalene. The dome of the pleura may be damaged during rib resection as it is adherent to the 1st rib and the suprapleural membrane. When performed carefully, this approach provides excellent access to the lower brachial plexus, but it does not allow treatment of the upper plexus. The posterior approach requires violation of the trapezius, levator scapulae and rhomboid muscles. Thus, it should not be performed in patients with trapezius weakness, ptotic scapulae, or patients who plan on returning to heavy labor upon recovery. Also, the surgeon must recognize that since this approach does not utilize intermuscular planes, it is often bloody and meticulous hemostasis is required throughout the case to maintain visualization as well as to avoid excessive blood loss. The transclavicular approach should not be performed in the presence of trapezial paralysis or weakness. This may result in an unstable and painful shoulder girdle despite adequate decompression of the thoracic outlet. Likewise, claviculectomy should not be performed in these patients. Prior to wound closure, the surgeon must carefully inspect the field and obtain meticulous hemostasis. A simple water seal approach should be done to assess the presence of a pneumothorax. If an air leak is detected, then an intraoperative chest x-ray is indicated; and, if the pneumothorax warrants it, a chest tube should be placed. Post-operative Care A routine postoperative chest x-ray is done in the recovery room to identify any pneumothorax that may have been missed during the closure. A small pneumothorax merits a repeat chest x-ray the following morning to identify whether there has been any increase in size. A large or symptomatic pneumothorax should be treated with a chest tube. A shoulder immobilizer is worn for comfort. Gentle range of motion exercises are started within the first week to avoid stiffness and atrophy about the shoulder girdle. Patients may gradually resume activities of daily living in approximately one month. A nerve gliding and stretching program is started at one week. The patient should not do any heavy lifting or extended arm work in the perioperative period for up to six weeks. At that time if all is going well, a strengthening program is begun. Some patients require permanent restriction relative to job activities. Conclusion Clinicians must be aware of the variability in anatomy, presentation, and treatment options for thoracic outlet syndrome. Careful design of nonoperative regimens and selection of surgical patients enhances the likelihood of successful treatment.

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s The presentation of thoracic outlet syndrome may vary dramatically from patient to patient, including a variety of symptoms and signs. Objective tests such as electrodiagnostic studies are notoriously unreliable in characterizing this entity. Indeed, the very existence of TOS as a discrete entity has been controversial.38 Those who accept its existence admit that the diagnosis must be a clinical one, based upon the gestalt rather than a punch list or algorithm. The variability and complexity of this syndrome lends itself to mistakes in diagnosis as well as in surgical treatment. Even experienced clinicians must be vigilant and humble when approaching the TOS patient. Anatomy An understanding of the anatomy of the thoracic outlet is crucial in avoiding errors in diagnosis as well as in surgical treatment. Variations in the bony, muscular, vascular and neural anatomy all contribute to the presentation of the TOS patient. Over 30% of patients may demonstrate congenital anomalies that present additional challenges to the surgeon.39 A history of prior trauma40 or surgery about the thorax, neck or shoulder, must raise suspicion for alterations in the anatomy. Although neural compression tends to generate the most obvious symptoms, arterial or venous compression may predominate in any particular patient. When the symptoms are localized to the subclavian artery41, subclavian vein42 or a defined segment of the brachial plexus, the clinician should be suspicious for an anatomic variation.43 When the symptoms involve the entire contents of the thoracic outlet, postural and scalene abnormalities become the primary suspects. At surgery, all potential sites of compression must be examined systematically and thoroughly. 38 Wilbourn AJ: The Thoracic outlet syndrome is overdiagnosed. Arch Neurol 1990:47:328-330. 39 Roos DB: Congenital anomalies associated with thoracic outlet syndrome. Anatomy, symptoms, diagnosis and treatment. Am J Surg 1976:132:771-778. 40 Mulder DS, Greenwood FAH, Brooks CE: Posttraumatic thoracic outlet syndrome. J Trauma 1973:13:706-715. 41 Cormier JM, Amrane M, Ward A, et al: Arterial complications of the thoracic outlet syndrome. Fifty-five operative cases. J Vasc Surg 1989:9:778-787. 42 Judy KI, Heymann RI: Vascular complications of thoracic outlet syndrome. Am J Surg 1972:123:521-531. 43 Adson AW: Surgical treatment for symptoms produced by cervical ribs and the scalenus anticus muscle. Surg Gynecol Obstet 1947:85:687-700.

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Diagnosis Although the diagnosis of TOS must primarily be clinical, it must be distinguished from other causes of generalized upper extremity pain. It is essential, therefore, to perform a thorough physical exam, including the cervical spine, the shoulder and the entire upper extremity bilaterally, even when the symptoms are unilateral or relatively localized. The myriad complaints attributable to thoracic outlet syndrome, their variability and diffuseness tend to upset the clinician who wants a classic textbook description.44 Facial complaints, headaches, arm heaviness or swelling and diffuse weakness are more frequent than the classic numbness or vascular symptoms that occur with an elevated upper extremity. A common error performed in the examination includes the failure to identify subtle sensory changes which may be present on the medial aspect of the forearm or the ulnar hand. The medial antebrachial cutaneous nerve originates directly from the plexus and thus can be helpful in defining a site of brachial plexus irritation when such altered sensation is present. Weakness and atrophy may be difficult to distinguish from apprehension in some patients. Body habitus observations such as the presence of a gazelle-like neck, asymmetric shoulder height, large breasts45, or slouching posture should raise the physician’s antenna as to the possibility of thoracic outlet syndrome. Much like peripheral nerve testing, a supraclavicular or infraclavicular Tinel’s sign is specific but not particularly sensitive. Direct thumb pressure over the plexus that recreates the patient’s symptoms has been a more common and rewarding diagnostic finding. Grip strength testing measured in multiple positions is a standard of our upper extremity exam.46 The presence of an invalid grip pattern bodes a poor prognosis for treatment and may suggest that factors other than thoracic outlet syndrome may account for the patient’s disability. Provocative maneuvers may have relatively high sensitivity when performed correctly; however, the specificity is notably poor. Simple obliteration of the pulse with Adson’s and Wright’s maneuvers should not be considered positive unless accompanied by reproduction of the patient’s symptoms. Many normal individuals may demonstrate pulse obliteration, including, for example, 60% of the linemen of a college football team. The elevated arm test, felt to be pathognomonic by its describer, Dr. David Roos, has been shown by us to be equally positive in a patient with isolated peripheral neuropathies, including cubital tunnel and carpal tunnel syndromes.

44 Warrens AN, Heaton JM: Thoracic outlet compression syndrome: The lack of reliability of its clinical assessment. Ann R Coll Surg Engl 1987:69:203-204. 45 Kaye BL: Neurologic changes with excessively large breasts. South Med J 1972;65:177-180. 46 Harkonen R, Piirtomaa M, Alaranta H. Grip strength and hand position of the dynamometer in 204 Finnish adults. JHS 1993;18B:1:129-132.

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• Cervical radiculopathy and/or degenerative disc disease47 • Brachial neuritis48 • Peripheral nerve compression49 (carpal tunnel syndrome, cubital tunnel

syndrome) • Nerve injury about the shoulder50 (suprascapular, long thoracic, axillary) • Syringomyelia51 • Multiple sclerosis52 • Vascular syndromes53 (Raynaud’s, quadrilateral space compression54) • Cardiac pathology55 • Mechanical shoulder problems (rotator cuff, impingement56, “Dead arm

syndrome”57) • Myofascial syndromes58 • Tumors of the supraclavicular fossa or lung59 • Complex regional pain syndromes60

In patients presenting with vascular complaints, bruit auscultation over the plexus area is important. In longstanding thoracic outlet syndrome, there may be post-stenotic dilatation of the subclavian artery producing an audible bruit. This auscultation can also be enhanced if it is performed with the arm in the symptomatic provocative position. Failure to identify one of the above causes of upper extremity pain may result in persistence or exacerbation of symptoms. Thoracic outlet syndrome often occurs concurrently with other diagnoses. We have noted the frequent association of mechanical shoulder pathology and brachial plexopathy at the thoracic outlet area.

47 Murphey F. Sources and patterns of pain in disc disease. Clin Neurosurg 1968;15:343-51. 48 McCarty EC, Tsairis P, Warren RF: Brachial neuritis. Clin Orthop 1999 Nov;(368):37-43. 49 Carroll RE, Hurst LC: The relationship of thoracic outlet syndrome and carpal tunnel syndrome. Clin Ortho Apr 1982;164:149-153. 50 Goslin KL, Krivickas LS: Proximal neuropathies of the upper extremity. Neurol Clin 1999 Aug;17(3):525-48. 51 Singer GL, Brust JC, Challenor YB: Syringomyelia presenting as shoulder dysfunction. Arch Phys Med Rehabil 1992 Mar;73(3):285-8. 52 Melville ID: The differential diagnosis of nerve compression syndromes in the arm and hand. The neurologist's approach. Hand 1972 Jun;4(2):111-4. 53 Kutz JE, Rowland EB Jr: Vascular compression about the shoulder. Hand Clin. 1993 Feb;9(1):131-8. 54 Cahill BR, Palmer RE: Quadrilateral space syndrome. J Hand Surg [Am] 1983 Jan;8(1):65-9. 55 Urschel HC Jr, Razzuk MA, Hyland JW, et al: Thoracic outlet syndrome masquerading as coronary artery disease (pseudoangina). Ann Thorac Surg 1973;16:239-248. 56 Boyle JJ: Is the pain and dysfunction of shoulder impingement lesion really second rib syndrome in disguise? Two case reports. Man Ther 1999 Feb;4(1):44-8. 57 Leffert RD, Gumley G: The relationship between dead arm syndrome and thoracic outlet syndrome. Clin Orthop 1987;223:20-31. 58 Lauder TD: Musculoskeletal disorders that frequently mimic radiculopathy. Phys Med Rehabil Clin N Am 2002 Aug;13(3):469-85. 59 Detterbeck FC: Pancoast (superior sulcus) tumors. Ann Thorac Surg 1997 Jun;63(6):1810-8. 60 Raja SN, Grabow TS: Complex regional pain syndrome I (reflex sympathetic dystrophy). Anesthesiology 2002 May;96(5):1254-60.

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Finally, though debated by some, we have found a “double crush” phenomenon61,; i.e., an increased association of thoracic outlet syndrome with downstream peripheral compression neuropathy such as cubital, radial, and carpal tunnel syndromes. In such cases of multiple areas of compression neuropathy, the clinician should consider hereditary sensitivity to pressure palsy or intrinsic neuropathy (diabetes) as playing a potential role.62 Ancillary Studies Imaging: Plain x-rays of the cervical are essential in the evaluation of the thoracic outlet syndrome. The frequency of cervical ribs is 0.5 – 1%.63,64 There is a particularly high association of the “sucked candy” partial cervical rib and fibrous bands that predispose the thoracic outlet decompression. If there are cervical findings then an MRI of the cervical spine is helpful to assess root and/or disc involvement. MRI of the brachial plexus itself has not been helpful in our experience. We use it when we are concerned with a possible tumor or other unusual mass. In a prospective series of 40 patients undergoing thoracic outlet decompression, the brachial plexus MRI yielded only one helpful result: a Schwannoma was visualized. This tumor was already suspected, however, because of a palpable mass in the supraclavicular fossa. If there is a concern for a pulmonary problem, then routine chest x-ray and apical lordotics may be beneficial. A CT scan is useful if there is a clavicular abnormality such as a malunion or nonunion with abundant callous or sternoclavicular instability. Such a study details the preoperative bony deformity and its relationship to the outlet. With few exceptions we have abandoned the use of invasive vascular studies.65 Such exceptions include a patient with distal emboli or gangrene, a palpable bruit, clavicular mass or a patient with previous vascular surgery in the area. Recently, I have used an MRA, which has been helpful in such cases and may replace invasive vascular imaging. Other vascular studies include pulse volume recording. Such noninvasive recording, even when positive in provocative positions, has a yield comparable to a clinical Adson’s test. We still occasionally will use it to quantitate flow in the unilateral patient especially if cold intolerance and bluishness are dominant symptoms.

61 Wood VE, Biondi J: Double-crush nerve compression in thoracic-outlet syndrome. JBJS [Am] 1990; 72:85-87. 62 Jaeger SH, Singer DI, Whitenack SH, Mandel S: Nerve injury complications. Management of neurogenic pain syndromes. Hand Clin 1986 Feb; 2(1):217-234. 63 Pollack EW: Surgical anatomy of the thoracic outlet syndrome. Surg Gynecol Obstet 1980:150:97-103. 64 Sanders RJ, Hammond SL. Management of cervical ribs and anomalous first ribs causing neurogenic thoracic outlet syndrome. J Vasc Surg 2002 Jul; 36(1):51-56. 65 Sallstrom J, Thulesius O. Noninvasive-investigation of vascular compression in patients with thoracic outlet syndrome. Clin Physiol 1982;2:117-25.

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Electrical studies: Electrical studies are notoriously unreliable in thoracic outlet syndrome.66 We use them routinely not to confirm brachial plexopathy at the thoracic outlet but to evaluate cervical radiculopathy and other distal peripheral compression problems. Provocative brachial plexus conduction studies are controversial and examiner dependent.67 We have also used somatic evoked potential studies which are more reliable in defining brachial plexus irritation.68,69 Patients without evidence of any electrical involvement in such studies with a normal neurologic examination are generally advised that despite their symptoms, no defined nerve damage exists and, hence, nonoperative treatment is recommended. Nonoperative Treatment Most experienced clinicians utilize nonoperative regimens as their initial treatment for TOS. The most common errors in nonoperative treatment include failure to customize a treatment program for the particular patient and failure to continue the nonoperative plan for a sufficient duration to effectively alter the patient’s symptoms.70 Non-narcotic medication such as Elavil or Neurontin may lower these symptoms to a tolerable level. Surgical Treatment The first step in surgical treatment should be communicating with the patient the goals and limitations of surgery. This discussion must include a delineation of the potential complications, the risk of persistence of symptoms, exacerbation of symptoms, and recurrence. Our surgical outcome data at three years has shown 78% of patients are improved, 17% are unchanged, and 5% are worse. The worsening generally relates to increased pain, possibly due to perineural scarring. All forms have complications: catastrophic and minor have been reported. The patients have been directly informed of the major complications that include death secondary to uncontrolled subclavian hemorrhage, the possibility of multiple transfusions, damage to the plexus or phrenic nerve with transient or permanent paralysis, and pneumothorax possibly requiring a chest tube. While not meant to specifically frighten the patient, the frank discussion of these complications emphasizes the seriousness of the proposed surgery and often causes the patient to reconsider the severity of their symptoms against 66 Urschel HC Jr, Razzuk MA, Wood RE, et al: Objective diagnosis (ulnar nerve conduction velocity) and current therapy of the thoracic outlet syndrome. Ann Thorac Surg 1971:12:608-620. 67 Wilbourn AJ, Lederman RJ: Evidence for conduction delay in thoracic-outlet syndrome is challenged [letter]. N Engl J Med 1984;310:1052-1053. 68 Veilleux M, Stevens JC, Campbell JK: Somatosensory evoked potentials: Lack of value for diagnosis of thoracic outlet syndrome. Muscle Nerve 198:11:571-575. 69 Machleder HI, Moll F, Nuwer M, Jordan S: Somatosensory evoked potentials in the assessment of thoracic outlet compression syndrome. J Vasc Surg 1987;6:177-184. 70 Peet RM, Henriksen JD, Anderson TP, et al: Thoracic-outlet syndrome: Evaluation of a therapeutic exercise program. Staff Meetings Mayo Clin 1956;31:281-287.

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the backdrop of such potential complications. It is also important to explain that thoracic outlet decompression will relieve only compressive symptoms and those neural symptoms related to intraneural scarring will not be affected. Patients with concomitant pathology contributing to their symptoms must understand the specific symptoms being addressed in this operation. Electrodiagnostic evidence of cervical radiculopathy or peripheral neuropathy may require that additional treatment be rendered prior to their thoracic outlet decompression or even simultaneously. Females with excessively large breasts may benefit from mammoplasty prior to thoracic outlet decompression. The patient with mechanical shoulder pathology may benefit from a less risky shoulder procedure and get some secondary relief by taking the mechanical drag off the plexus. Failure to address these sites of pathology or, more importantly, failure to communicate with the patient the goals and expected outcome of their surgery may adversely affect their objective and subjective outcomes. Perhaps the most common error in the treatment of thoracic outlet syndrome lies in the selection criteria for surgical candidates. Operative intervention for thoracic outlet syndrome should be reserved for patients with very clear indications: 1) vascular insufficiency71 2) subclavian vein thrombosis 3) neurologic compromise 4) failure of an appropriately designed and closely supervised therapy program with intractable pain. Generally most thoracic outlet syndrome patients will have some response to an appropriate nerve gliding program, and failure to respond should alert the physician to other possible non-surgical entities as well as to a potential poor surgical prognosis. Above all, a reproducible and valid clinical examination is essential in defining a surgical candidate. Lastly, narcotic pain medications should be weaned or at least under the management of a pain control center. Intraoperative errors often stem from the difficulty in recognizing landmarks in this area of frequent anatomic variability. The surgeon may plan on performing a 1st rib resection but fail to resect the complete rib. Occasionally, the 2nd rib is resected inadvertently, leaving the 1st rib in place. Or, perhaps, the 1st rib is resected, but a cervical rib is not identified. Sometimes a cervical rib is resected, but an abnormal 1st rib is left. The key to avoiding such errors lies in both preoperative x-rays and intraoperative x-rays when there is doubt as to the rib level. We routinely use intraoperative nerve stimulation and monitoring. This allows us to actively map out the plexus as well as identify the phrenic nerve as it crosses the anterior scalene. Exposure must be sufficient to identify the normal and abnormal anatomy, including the offending structures, the structures being decompressed and the structures at risk. It is impossible to assess whether sufficient rib resection has been performed without an exposure large enough to visually inspect the contents of the thoracic outlet adjacent to 71 Thompson JF, Webster JHH: First rib resection for vascular complications of thoracic outlet syndrome. Br J Surg 1990:77:555-557.

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the rib. Furthermore, damage to the pleura, subclavian vessels or brachial plexus becomes far more likely when the surgeon is unable to visualize them. Multiple excellent approaches to the thoracic outlet have been described. However, as with any procedure, selection of the appropriate approach is of paramount importance in avoiding preventable complications.72 The foremost consideration should be the surgeon’s experience and comfort level with a particular approach. Once this has been taken into account, there are certain caveats with particular approaches that merit mention.73 Transaxillary 1st rib resection74 should be performed with the patient in the lateral decubitus position with an assistant to control the upper extremity. Utilization of a traction apparatus to hold the upper extremity may compromise the exposure as well as result in neurapraxic injury. This approach also requires complete skeletal muscle relaxation be induced by the anesthesiologist to allow full access to all the structures. The surgeon should be aware of the intercostobrachial nerve, which should be spared to prevent posterior arm numbness. The long thoracic nerve may be at risk as it courses through or immediately posterior to the middle scalene. The dome of the pleura may be damaged during rib resection as it is adherent to the 1st rib and the suprapleural membrane. When performed carefully, this approach provides excellent access to the lower brachial plexus, but it does not allow treatment of the upper plexus. The posterior approach requires violation of the trapezius, levator scapulae and rhomboid muscles. Thus, it should not be performed in patients with trapezius weakness, ptotic scapulae, or patients who plan on returning to heavy labor upon recovery. Also, the surgeon must recognize that since this approach does not utilize intermuscular planes, it is often bloody and meticulous hemostasis is required throughout the case to maintain visualization as well as to avoid excessive blood loss. The transclavicular approach should not be performed in the presence of trapezial paralysis or weakness. This may result in an unstable and painful shoulder girdle despite adequate decompression of the thoracic outlet. Likewise, claviculectomy should not be performed in these patients. Prior to wound closure, the surgeon must carefully inspect the field and obtain meticulous hemostasis. A simple water seal approach should be done to assess the presence of a pneumothorax. If an air leak is detected, then an intraoperative chest x-ray is indicated; and, if the pneumothorax warrants it, a chest tube should be placed.

72 Sanders RJ, Pearce WH: The treatment of thoracic outlet syndrome: A comparison of different operations. J Vasc Surg 1989;10:626-634. 73 Kostic S, Kulka F: Reasons behind surgical failures in thoracic outlet syndrome. Int Surg 1990;75:159-161. 74 Roos DB: Transaxillary approach for first rib resection to relieve thoracic outlet syndrome. Ann Surg 1966;163:354-358.

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Post-operative Care A routine postoperative chest x-ray is done in the recovery room to identify any pneumothorax that may have been missed during the closure. A small pneumothorax merits a repeat chest x-ray the following morning to identify whether there has been any increase in size. A large or symptomatic pneumothorax should be treated with a chest tube. A shoulder immobilizer is worn for comfort. Gentle range of motion exercises are started within the first week to avoid stiffness and atrophy about the shoulder girdle. Patients may gradually resume activities of daily living in approximately one month. A nerve gliding and stretching program is started at one week. The patient should not do any heavy lifting or extended arm work in the perioperative period for up to six weeks. At that time if all is going well, a strengthening program is begun. Some patients require permanent restriction relative to job activities. Conclusion Clinicians must be aware of the variability in anatomy, presentation, and treatment options for thoracic outlet syndrome. Careful design of nonoperative regimens and selection of surgical patients enhances the likelihood of successful treatment.

The presentation of thoracic outlet syndrome may vary dramatically from patient to patient, including a variety of symptoms and signs. Objective tests such as electrodiagnostic studies are notoriously unreliable in characterizing this entity. Indeed, the very existence of TOS as a discrete entity has been controversial.75 Those who accept its existence admit that the diagnosis must be a clinical one, based upon the gestalt rather than a punch list or algorithm. The variability and complexity of this syndrome lends itself to mistakes in diagnosis as well as in surgical treatment. Even experienced clinicians must be vigilant and humble when approaching the TOS patient. Anatomy An understanding of the anatomy of the thoracic outlet is crucial in avoiding errors in diagnosis as well as in surgical treatment. Variations in the bony, muscular, vascular and neural anatomy all contribute to the presentation of the TOS patient. Over 30% of patients may demonstrate congenital anomalies that present additional challenges to the surgeon.76

75 Wilbourn AJ: The Thoracic outlet syndrome is overdiagnosed. Arch Neurol 1990:47:328-330. 76 Roos DB: Congenital anomalies associated with thoracic outlet syndrome. Anatomy, symptoms, diagnosis and treatment. Am J Surg 1976:132:771-778.

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A history of prior trauma77 or surgery about the thorax, neck or shoulder, must raise suspicion for alterations in the anatomy. Although neural compression tends to generate the most obvious symptoms, arterial or venous compression may predominate in any particular patient. When the symptoms are localized to the subclavian artery78, subclavian vein79 or a defined segment of the brachial plexus, the clinician should be suspicious for an anatomic variation.80 When the symptoms involve the entire contents of the thoracic outlet, postural and scalene abnormalities become the primary suspects. At surgery, all potential sites of compression must be examined systematically and thoroughly. Diagnosis Although the diagnosis of TOS must primarily be clinical, it must be distinguished from other causes of generalized upper extremity pain. It is essential, therefore, to perform a thorough physical exam, including the cervical spine, the shoulder and the entire upper extremity bilaterally, even when the symptoms are unilateral or relatively localized. The myriad complaints attributable to thoracic outlet syndrome, their variability and diffuseness tend to upset the clinician who wants a classic textbook description.81 Facial complaints, headaches, arm heaviness or swelling and diffuse weakness are more frequent than the classic numbness or vascular symptoms that occur with an elevated upper extremity. A common error performed in the examination includes the failure to identify subtle sensory changes which may be present on the medial aspect of the forearm or the ulnar hand. The medial antebrachial cutaneous nerve originates directly from the plexus and thus can be helpful in defining a site of brachial plexus irritation when such altered sensation is present. Weakness and atrophy may be difficult to distinguish from apprehension in some patients.

77 Mulder DS, Greenwood FAH, Brooks CE: Posttraumatic thoracic outlet syndrome. J Trauma 1973:13:706-715. 78 Cormier JM, Amrane M, Ward A, et al: Arterial complications of the thoracic outlet syndrome. Fifty-five operative cases. J Vasc Surg 1989:9:778-787. 79 Judy KI, Heymann RI: Vascular complications of thoracic outlet syndrome. Am J Surg 1972:123:521-531. 80 Adson AW: Surgical treatment for symptoms produced by cervical ribs and the scalenus anticus muscle. Surg Gynecol Obstet 1947:85:687-700. 81 Warrens AN, Heaton JM: Thoracic outlet compression syndrome: The lack of reliability of its clinical assessment. Ann R Coll Surg Engl 1987:69:203-204.

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Body habitus observations such as the presence of a gazelle-like neck, asymmetric shoulder height, large breasts82, or slouching posture should raise the physician’s antenna as to the possibility of thoracic outlet syndrome. Much like peripheral nerve testing, a supraclavicular or infraclavicular Tinel’s sign is specific but not particularly sensitive. Direct thumb pressure over the plexus that recreates the patient’s symptoms has been a more common and rewarding diagnostic finding. Grip strength testing measured in multiple positions is a standard of our upper extremity exam.83 The presence of an invalid grip pattern bodes a poor prognosis for treatment and may suggest that factors other than thoracic outlet syndrome may account for the patient’s disability. Provocative maneuvers may have relatively high sensitivity when performed correctly; however, the specificity is notably poor. Simple obliteration of the pulse with Adson’s and Wright’s maneuvers should not be considered positive unless accompanied by reproduction of the patient’s symptoms. Many normal individuals may demonstrate pulse obliteration, including, for example, 60% of the linemen of a college football team. The elevated arm test, felt to be pathognomonic by its describer, Dr. David Roos, has been shown by us to be equally positive in a patient with isolated peripheral neuropathies, including cubital tunnel and carpal tunnel syndromes.

• Cervical radiculopathy and/or degenerative disc disease84 • Brachial neuritis85 • Peripheral nerve compression86 (carpal tunnel syndrome, cubital tunnel

syndrome) • Nerve injury about the shoulder87 (suprascapular, long thoracic, axillary) • Syringomyelia88 • Multiple sclerosis89 • Vascular syndromes90 (Raynaud’s, quadrilateral space compression91) • Cardiac pathology92 • Mechanical shoulder problems (rotator cuff, impingement93, “Dead arm

syndrome”94)

82 Kaye BL: Neurologic changes with excessively large breasts. South Med J 1972;65:177-180. 83 Harkonen R, Piirtomaa M, Alaranta H. Grip strength and hand position of the dynamometer in 204 Finnish adults. JHS 1993;18B:1:129-132. 84 Murphey F. Sources and patterns of pain in disc disease. Clin Neurosurg 1968;15:343-51. 85 McCarty EC, Tsairis P, Warren RF: Brachial neuritis. Clin Orthop 1999 Nov;(368):37-43. 86 Carroll RE, Hurst LC: The relationship of thoracic outlet syndrome and carpal tunnel syndrome. Clin Ortho Apr 1982;164:149-153. 87 Goslin KL, Krivickas LS: Proximal neuropathies of the upper extremity. Neurol Clin 1999 Aug;17(3):525-48. 88 Singer GL, Brust JC, Challenor YB: Syringomyelia presenting as shoulder dysfunction. Arch Phys Med Rehabil 1992 Mar;73(3):285-8. 89 Melville ID: The differential diagnosis of nerve compression syndromes in the arm and hand. The neurologist's approach. Hand 1972 Jun;4(2):111-4. 90 Kutz JE, Rowland EB Jr: Vascular compression about the shoulder. Hand Clin. 1993 Feb;9(1):131-8. 91 Cahill BR, Palmer RE: Quadrilateral space syndrome. J Hand Surg [Am] 1983 Jan;8(1):65-9. 92 Urschel HC Jr, Razzuk MA, Hyland JW, et al: Thoracic outlet syndrome masquerading as coronary artery disease (pseudoangina). Ann Thorac Surg 1973;16:239-248.

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• Myofascial syndromes95 • Tumors of the supraclavicular fossa or lung96 • Complex regional pain syndromes97

In patients presenting with vascular complaints, bruit auscultation over the plexus area is important. In longstanding thoracic outlet syndrome, there may be post-stenotic dilatation of the subclavian artery producing an audible bruit. This auscultation can also be enhanced if it is performed with the arm in the symptomatic provocative position. Failure to identify one of the above causes of upper extremity pain may result in persistence or exacerbation of symptoms. Thoracic outlet syndrome often occurs concurrently with other diagnoses. We have noted the frequent association of mechanical shoulder pathology and brachial plexopathy at the thoracic outlet area. Finally, though debated by some, we have found a “double crush” phenomenon98,; i.e., an increased association of thoracic outlet syndrome with downstream peripheral compression neuropathy such as cubital, radial, and carpal tunnel syndromes. In such cases of multiple areas of compression neuropathy, the clinician should consider hereditary sensitivity to pressure palsy or intrinsic neuropathy (diabetes) as playing a potential role.99 Ancillary Studies Imaging: Plain x-rays of the cervical are essential in the evaluation of the thoracic outlet syndrome. The frequency of cervical ribs is 0.5 – 1%.100,101 There is a particularly high association of the “sucked candy” partial cervical rib and fibrous bands that predispose the thoracic outlet decompression. If there are cervical findings then an MRI of the cervical spine is helpful to assess root and/or disc involvement. MRI of the brachial plexus itself has not been helpful in our experience. We use it when we are concerned with a possible tumor or other unusual mass. In a prospective series of 40 patients undergoing thoracic outlet decompression, the brachial plexus MRI yielded

93 Boyle JJ: Is the pain and dysfunction of shoulder impingement lesion really second rib syndrome in disguise? Two case reports. Man Ther 1999 Feb;4(1):44-8. 94 Leffert RD, Gumley G: The relationship between dead arm syndrome and thoracic outlet syndrome. Clin Orthop 1987;223:20-31. 95 Lauder TD: Musculoskeletal disorders that frequently mimic radiculopathy. Phys Med Rehabil Clin N Am 2002 Aug;13(3):469-85. 96 Detterbeck FC: Pancoast (superior sulcus) tumors. Ann Thorac Surg 1997 Jun;63(6):1810-8. 97 Raja SN, Grabow TS: Complex regional pain syndrome I (reflex sympathetic dystrophy). Anesthesiology 2002 May;96(5):1254-60. 98 Wood VE, Biondi J: Double-crush nerve compression in thoracic-outlet syndrome. JBJS [Am] 1990; 72:85-87. 99 Jaeger SH, Singer DI, Whitenack SH, Mandel S: Nerve injury complications. Management of neurogenic pain syndromes. Hand Clin 1986 Feb; 2(1):217-234. 100 Pollack EW: Surgical anatomy of the thoracic outlet syndrome. Surg Gynecol Obstet 1980:150:97-103. 101 Sanders RJ, Hammond SL. Management of cervical ribs and anomalous first ribs causing neurogenic thoracic outlet syndrome. J Vasc Surg 2002 Jul; 36(1):51-56.

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only one helpful result: a Schwannoma was visualized. This tumor was already suspected, however, because of a palpable mass in the supraclavicular fossa. If there is a concern for a pulmonary problem, then routine chest x-ray and apical lordotics may be beneficial. A CT scan is useful if there is a clavicular abnormality such as a malunion or nonunion with abundant callous or sternoclavicular instability. Such a study details the preoperative bony deformity and its relationship to the outlet. With few exceptions we have abandoned the use of invasive vascular studies.102 Such exceptions include a patient with distal emboli or gangrene, a palpable bruit, clavicular mass or a patient with previous vascular surgery in the area. Recently, I have used an MRA, which has been helpful in such cases and may replace invasive vascular imaging. Other vascular studies include pulse volume recording. Such noninvasive recording, even when positive in provocative positions, has a yield comparable to a clinical Adson’s test. We still occasionally will use it to quantitate flow in the unilateral patient especially if cold intolerance and bluishness are dominant symptoms. Electrical studies: Electrical studies are notoriously unreliable in thoracic outlet syndrome.103 We use them routinely not to confirm brachial plexopathy at the thoracic outlet but to evaluate cervical radiculopathy and other distal peripheral compression problems. Provocative brachial plexus conduction studies are controversial and examiner dependent.104 We have also used somatic evoked potential studies which are more reliable in defining brachial plexus irritation.105,106 Patients without evidence of any electrical involvement in such studies with a normal neurologic examination are generally advised that despite their symptoms, no defined nerve damage exists and, hence, nonoperative treatment is recommended. Nonoperative Treatment Most experienced clinicians utilize nonoperative regimens as their initial treatment for TOS. The most common errors in nonoperative treatment include failure to customize a treatment program for the particular patient and failure to continue the nonoperative plan 102 Sallstrom J, Thulesius O. Noninvasive-investigation of vascular compression in patients with thoracic outlet syndrome. Clin Physiol 1982;2:117-25. 103 Urschel HC Jr, Razzuk MA, Wood RE, et al: Objective diagnosis (ulnar nerve conduction velocity) and current therapy of the thoracic outlet syndrome. Ann Thorac Surg 1971:12:608-620. 104 Wilbourn AJ, Lederman RJ: Evidence for conduction delay in thoracic-outlet syndrome is challenged [letter]. N Engl J Med 1984;310:1052-1053. 105 Veilleux M, Stevens JC, Campbell JK: Somatosensory evoked potentials: Lack of value for diagnosis of thoracic outlet syndrome. Muscle Nerve 198:11:571-575. 106 Machleder HI, Moll F, Nuwer M, Jordan S: Somatosensory evoked potentials in the assessment of thoracic outlet compression syndrome. J Vasc Surg 1987;6:177-184.

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for a sufficient duration to effectively alter the patient’s symptoms.107 Non-narcotic medication such as Elavil or Neurontin may lower these symptoms to a tolerable level. Surgical Treatment The first step in surgical treatment should be communicating with the patient the goals and limitations of surgery. This discussion must include a delineation of the potential complications, the risk of persistence of symptoms, exacerbation of symptoms, and recurrence. Our surgical outcome data at three years has shown 78% of patients are improved, 17% are unchanged, and 5% are worse. The worsening generally relates to increased pain, possibly due to perineural scarring. All forms have complications: catastrophic and minor have been reported. The patients have been directly informed of the major complications that include death secondary to uncontrolled subclavian hemorrhage, the possibility of multiple transfusions, damage to the plexus or phrenic nerve with transient or permanent paralysis, and pneumothorax possibly requiring a chest tube. While not meant to specifically frighten the patient, the frank discussion of these complications emphasizes the seriousness of the proposed surgery and often causes the patient to reconsider the severity of their symptoms against the backdrop of such potential complications. It is also important to explain that thoracic outlet decompression will relieve only compressive symptoms and those neural symptoms related to intraneural scarring will not be affected. Patients with concomitant pathology contributing to their symptoms must understand the specific symptoms being addressed in this operation. Electrodiagnostic evidence of cervical radiculopathy or peripheral neuropathy may require that additional treatment be rendered prior to their thoracic outlet decompression or even simultaneously. Females with excessively large breasts may benefit from mammoplasty prior to thoracic outlet decompression. The patient with mechanical shoulder pathology may benefit from a less risky shoulder procedure and get some secondary relief by taking the mechanical drag off the plexus. Failure to address these sites of pathology or, more importantly, failure to communicate with the patient the goals and expected outcome of their surgery may adversely affect their objective and subjective outcomes. Perhaps the most common error in the treatment of thoracic outlet syndrome lies in the selection criteria for surgical candidates. Operative intervention for thoracic outlet syndrome should be reserved for patients with very clear indications: 1) vascular insufficiency108 2) subclavian vein thrombosis 3) neurologic compromise 4) failure of an appropriately designed and closely supervised therapy program with intractable pain. Generally most thoracic outlet syndrome patients will have some response to an appropriate nerve gliding program, and failure to respond should alert the physician to other possible non-surgical entities as well as to a potential poor surgical prognosis.

107 Peet RM, Henriksen JD, Anderson TP, et al: Thoracic-outlet syndrome: Evaluation of a therapeutic exercise program. Staff Meetings Mayo Clin 1956;31:281-287. 108 Thompson JF, Webster JHH: First rib resection for vascular complications of thoracic outlet syndrome. Br J Surg 1990:77:555-557.

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Above all, a reproducible and valid clinical examination is essential in defining a surgical candidate. Lastly, narcotic pain medications should be weaned or at least under the management of a pain control center. Intraoperative errors often stem from the difficulty in recognizing landmarks in this area of frequent anatomic variability. The surgeon may plan on performing a 1st rib resection but fail to resect the complete rib. Occasionally, the 2nd rib is resected inadvertently, leaving the 1st rib in place. Or, perhaps, the 1st rib is resected, but a cervical rib is not identified. Sometimes a cervical rib is resected, but an abnormal 1st rib is left. The key to avoiding such errors lies in both preoperative x-rays and intraoperative x-rays when there is doubt as to the rib level. We routinely use intraoperative nerve stimulation and monitoring. This allows us to actively map out the plexus as well as identify the phrenic nerve as it crosses the anterior scalene. Exposure must be sufficient to identify the normal and abnormal anatomy, including the offending structures, the structures being decompressed and the structures at risk. It is impossible to assess whether sufficient rib resection has been performed without an exposure large enough to visually inspect the contents of the thoracic outlet adjacent to the rib. Furthermore, damage to the pleura, subclavian vessels or brachial plexus becomes far more likely when the surgeon is unable to visualize them. Multiple excellent approaches to the thoracic outlet have been described. However, as with any procedure, selection of the appropriate approach is of paramount importance in avoiding preventable complications.109 The foremost consideration should be the surgeon’s experience and comfort level with a particular approach. Once this has been taken into account, there are certain caveats with particular approaches that merit mention.110 Transaxillary 1st rib resection111 should be performed with the patient in the lateral decubitus position with an assistant to control the upper extremity. Utilization of a traction apparatus to hold the upper extremity may compromise the exposure as well as result in neurapraxic injury. This approach also requires complete skeletal muscle relaxation be induced by the anesthesiologist to allow full access to all the structures. The surgeon should be aware of the intercostobrachial nerve, which should be spared to prevent posterior arm numbness. The long thoracic nerve may be at risk as it courses through or immediately posterior to the middle scalene. The dome of the pleura may be

109 Sanders RJ, Pearce WH: The treatment of thoracic outlet syndrome: A comparison of different operations. J Vasc Surg 1989;10:626-634. 110 Kostic S, Kulka F: Reasons behind surgical failures in thoracic outlet syndrome. Int Surg 1990;75:159-161. 111 Roos DB: Transaxillary approach for first rib resection to relieve thoracic outlet syndrome. Ann Surg 1966;163:354-358.

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damaged during rib resection as it is adherent to the 1st rib and the suprapleural membrane. When performed carefully, this approach provides excellent access to the lower brachial plexus, but it does not allow treatment of the upper plexus. The posterior approach requires violation of the trapezius, levator scapulae and rhomboid muscles. Thus, it should not be performed in patients with trapezius weakness, ptotic scapulae, or patients who plan on returning to heavy labor upon recovery. Also, the surgeon must recognize that since this approach does not utilize intermuscular planes, it is often bloody and meticulous hemostasis is required throughout the case to maintain visualization as well as to avoid excessive blood loss. The transclavicular approach should not be performed in the presence of trapezial paralysis or weakness. This may result in an unstable and painful shoulder girdle despite adequate decompression of the thoracic outlet. Likewise, claviculectomy should not be performed in these patients. Prior to wound closure, the surgeon must carefully inspect the field and obtain meticulous hemostasis. A simple water seal approach should be done to assess the presence of a pneumothorax. If an air leak is detected, then an intraoperative chest x-ray is indicated; and, if the pneumothorax warrants it, a chest tube should be placed. Post-operative Care A routine postoperative chest x-ray is done in the recovery room to identify any pneumothorax that may have been missed during the closure. A small pneumothorax merits a repeat chest x-ray the following morning to identify whether there has been any increase in size. A large or symptomatic pneumothorax should be treated with a chest tube. A shoulder immobilizer is worn for comfort. Gentle range of motion exercises are started within the first week to avoid stiffness and atrophy about the shoulder girdle. Patients may gradually resume activities of daily living in approximately one month. A nerve gliding and stretching program is started at one week. The patient should not do any heavy lifting or extended arm work in the perioperative period for up to six weeks. At that time if all is going well, a strengthening program is begun. Some patients require permanent restriction relative to job activities. Conclusion Clinicians must be aware of the variability in anatomy, presentation, and treatment options for thoracic outlet syndrome. Careful design of nonoperative regimens and selection of surgical patients enhances the likelihood of successful treatment. References

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1. Wilbourn AJ: The Thoracic outlet syndrome is overdiagnosed. Arch Neurol 1990:47:328-330.

2. Roos DB: Congenital anomalies associated with thoracic outlet syndrome. Anatomy, symptoms, diagnosis and treatment. Am J Surg 1976:132:771-778.

3. Mulder DS, Greenwood FAH, Brooks CE: Posttraumatic thoracic outlet syndrome. J Trauma 1973:13:706-715.

4. Cormier JM, Amrane M, Ward A, et al: Arterial complications of the thoracic outlet syndrome. Fifty-five operative cases. J Vasc Surg 1989:9:778-787.

5. Judy KI, Heymann RI: Vascular complications of thoracic outlet syndrome. Am J Surg 1972:123:521-531.

6. Adson AW: Surgical treatment for symptoms produced by cervical ribs and the scalenus anticus muscle. Surg Gynecol Obstet 1947:85:687-700.

7. Warrens AN, Heaton JM: Thoracic outlet compression syndrome: The lack of reliability of its clinical assessment. Ann R Coll Surg Engl 1987:69:203-204.

8. Kaye BL: Neurologic changes with excessively large breasts. South Med J 1972;65:177-180.

9. Harkonen R, Piirtomaa M, Alaranta H. Grip strength and hand position of the dynamometer in 204 Finnish adults. JHS 1993;18B:1:129-132.

10. Murphey F. Sources and patterns of pain in disc disease. Clin Neurosurg 1968;15:343-51.

11. McCarty EC, Tsairis P, Warren RF: Brachial neuritis. Clin Orthop 1999 Nov;(368):37-43. 12. Carroll RE, Hurst LC: The relationship of thoracic outlet syndrome and carpal tunnel syndrome. Clin Ortho Apr 1982;164:149-153. 13. Goslin KL, Krivickas LS: Proximal neuropathies of the upper extremity. Neurol Clin 1999 Aug;17(3):525-48. 14. Singer GL, Brust JC, Challenor YB: Syringomyelia presenting as shoulder dysfunction. Arch Phys Med Rehabil 1992 Mar;73(3):285-8. 15. Melville ID: The differential diagnosis of nerve compression syndromes in the arm and hand. The neurologist's approach. Hand 1972 Jun;4(2):111-4. 16. Kutz JE, Rowland EB Jr: Vascular compression about the shoulder. Hand Clin. 1993 Feb;9(1):131-8. 17. Cahill BR, Palmer RE: Quadrilateral space syndrome. J Hand Surg [Am] 1983 Jan;8(1):65-9. 18. Urschel HC Jr, Razzuk MA, Hyland JW, et al: Thoracic outlet syndrome masquerading as coronary artery disease (pseudoangina). Ann Thorac Surg 1973;16:239-248. 19. Boyle JJ: Is the pain and dysfunction of shoulder impingement lesion really second rib syndrome in disguise? Two case reports. Man Ther 1999 Feb;4(1):44-8.

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