Once a poorly understood and heavily debated disease within cardiovascular medicine, thoracic outlet syndrome (TOS) has recently been recognized as a more common syndrome than previously thought.1 The condition has many presentations, including vascular and neurogenic, but it is hypothesized that the common pathophysiology of TOS is due to a narrowing of the thoracic outlet.
The thoracic outlet is defined by an area through which the subclavian vein, subclavian artery, and brachial plexus all emerge as a neurovascular bundle. Anatomically, the outlet is defined superiorly and inferiorly by the clavicle and first rib, respectively; this area is called the costoclavicular space. The subclavian artery and brachial plexus emerge between the anterior and posterior scalene muscles, and the subclavian vein runs anterior to the anterior scalene muscles into the outlet from which they all continue distally into the arm.
There are numerous conditions that can lead to a narrowing of this space, including congenital bone and muscle anomalies, repetitive trauma, stress, depression, and poor posture.1 One study showed that 29% of patients with TOS have bone anomalies.2 Another study found that 43% of patients who received surgical correction for their TOS had an anomaly of scalene muscle development and/or muscle insertion, whereas only 8.5% had an anomalous cervical rib.3
Subacute upper extremity presentations
TOS is further subdivided into neurogenic, venous, and arterial subtypes, dependent on which structures become impinged within the outlet. The neurogenic type (nTOS) is the most common, and presents in approximately 95% of patients diagnosed with TOS. The venous subtype (vTOS) is present in 3% to 5% of patients. The arterial subtype (aTOS) is the most rare, comprising only 1% to 2% of presentations of those with TOS.1 Although the different types share a common pathophysiology of a narrowed outlet space, their clinical presentations differ widely and should be considered in unilateral presentations of subacute upper extremity complaints in primary care offices.
The following discusses nTOS, vTOS, and aTOS, and their diagnosis and clinical management.
Some physicians further subdivide nTOS patients based on where the brachial plexus becomes impinged.4 Those who advocate this philosophy distinguish impingement between the scalene muscles as an upper thoracic syndrome—also called cervical outlet syndrome—and impingement between the first rib and clavicle as a true thoracic outlet syndrome.5 Patients with nTOS most commonly present with symptoms of intermittent pain, numbness, and tingling on the ipsilateral side that are distributed in the arms, hands, and fingers. Cervical nTOS has a distribution between C5 and C7, whereas true nTOS has a distribution between C8 and T1.4,5 With regard to what causes nTOS, one study showed that 50% of nTOS patients have a history of repetitive trauma to the shoulder area, which is hypothesized to result in inflammatory changes and narrowing of the thoracic outlet.1
Even though nTOS is the most common subtype of TOS, the clinical evaluation for patients presenting with nTOS is the most poorly correlated of the three subtypes. However, there are two tests that can raise suspicion for TOS: the elevated arm stress test (EAST) and a lidocaine scalene block test.
A patient who presents with symptoms of TOS and a history of repetitive trauma to the shoulder should have an EAST examination to evaluate for nTOS. The EAST examination is performed by having the patient abduct the shoulder to 90° with the elbow flexed to 90° and then place his or her hands facing forward while opening and closing the hands for 3 minutes. A positive test result occurs when the observer sees a decrease in the ability to open and close the hand or a lowering of the patient’s arm with increased time in opening and closing the hand.1 The test is not very specific; however, with a thorough patient history, the test result may support further evaluation for nTOS.
One test that has historically been helpful in diagnosis of nTOS is the lidocaine scalene block test. With imaging guidance, the anterior scalene is injected with lidocaine; in patients with nTOS, a decrease to near resolution of symptoms for up to 4 hours is observed. The lidocaine block test has had a positive predictive value of 90% for success in patients receiving further treatment.6
Initial management for nTOS patients is nonoperative and consists of physical therapy and lifestyle modifications. Successful modifications include avoidance of aggravating behaviors, ergonomic modifications, and selective use of some pharmacologic agents, including nonsteroidal anti-inflammatory drugs, antidepressants, and muscle relaxants, with 60% to 70% of patients reporting resolution of symptoms.1 If 8 to 12 weeks of nonoperative management fails, patients may subsequently need to seek referral for surgical management options.
vTOS is due to the impingement of the subclavian vein with resulting thrombosis. Hughes7 describes this “effort thrombosis,” or Paget-Schroetter syndrome, as axillary-subclavian vein thrombosis associated with strenuous and repetitive activity of the upper extremities.8 These patients have been shown to have a correlation with a multitude of underlying aggravating factors, including trauma, repetitive motion, and (although rare), a history of hypercoagulability.1 Trauma to the subclavian vein results in damage to the intima musculature that perpetuates thrombus formation; it is usually seen in younger and more active patients with an association of reported repetitive trauma to the shoulder region. Patients present with an acute or chronic onset of unilateral upper extremity swelling with red-purple discoloration and pain. Some patients report a chronic feeling of heaviness in the extremity.7
Diagnosis of vTOS is made by a combination of clinical presentation and noninvasive studies. A duplex ultrasound of the subclavian vein in both the resting position and with the arm abducted to 90° is used. Results suggestive of vTOS are Doppler waveforms of both the subclavian and axillary veins showing significant decrease in vein velocity.
Treatment of vTOS is subjective to the treating physician’s preference. Nonoperative management of vTOS involves thrombolytic therapy or heparinization, whereas surgical management with thrombectomy and simultaneous first rib resection is another possibility.9,10 Operative management—thoracic outlet decompression—should be performed in symptomatic patients.
aTOS is the most infrequently seen subtype of thoracic outlet syndrome and is hypothesized to be due to recurrent friction of the subclavian artery with resultant fibrosis and subsequent stenosis of the subclavian artery. Arterial stenosis, as well as poststenotic aneurysms, may result in arterial thrombosis that can present with symptoms of decreased blood flow to the extremity. Chronically, the patient may complain of claudication or pain of the extremity with activity that subsides with rest. If emboli form, break off, and travel distally, patients may present with subacute focal symptoms such as an individual digit with decreased pallor.11 The most dangerous presentation is in patients who acutely form a completely thrombosed subclavian artery, resulting in decreased blood flow to the upper extremity. Of those patients with congenital bony abnormalities, the highest correlation was with patients diagnosed with aTOS.2
Similar to evaluation of vTOS, patients are best evaluated with Doppler waveforms of the subclavian and axillary arteries. In contrast to the Doppler study results found in patients with vTOS, patients with aTOS show increased velocity with stenosis of the artery, and absent velocities in cases of complete occlusion. Patients with aTOS have increased correlation with bony abnormalities compared with those with vTOS; as a result, patients with suspected aTOS should also receive a chest radiograph. As in patients with vTOS, thoracic outlet decompression should be performed in symptomatic patients.
Surgical management of TOS
After conservative management for each subtype of TOS fails, surgical management should be considered. Surgical approaches include the transaxillary approach, the supraclavicular approach, and the combined approach.
The transaxillary approach decompresses TOS by resecting the first rib. The supraclavicular approach is performed with anterior and middle scalenectomies with or without first rib resection. Both have been reported to have success rates between 75% and 99%; to date, no randomized clinical trial have been done to compare the two.12 In the combined approach, anterior and middle scalenectomies are done via the supraclavicular approach, and a first rib resection is done via the transaxillary approach. It has been reported that the combined approach improves long-term results and decreases recurrence rates.13
Complications of surgical procedures in these cases include injury to major neurovascular structures, which can be avoided with careful manipulation of structures and proximal control; supraclavicular nerve palsy, resulting in sensory deficit; phrenic nerve palsy, resulting in respiratory compromise; arterial or venous injury, resulting in bleeding or hemothorax; and thoracic duct injury, resulting in a chylous effusion.
Virginia Bailey, BA, is a medical student at the McGovern Medical School of The University of Texas; Justin Cardenas, BS, is a medical student at Baylor College of Medicine; and Maura Holcomb, MD, is a practicing dermatologist in Houston.
- Freischlag J, Orion K. Understanding thoracic outlet syndrome. Scientifica (Cairo). 2014;2014:248163.
- Weber AE, Criado E. Relevance of bone anomalies in patients with thoracic outlet syndrome. Ann Vasc Surg. 2014;28:924-932.
- Makhoul RG, Machleder HI. Developmental anomalies at the thoracic outlet: an analysis of 200 consecutive cases. J Vasc Surg. 1992;16:534-542.
- Pauliukas P. Thoracic outlet syndrome: anatomy, symptoms, diagnostic evaluation, and surgical treatment. Slideshare website. http://www.slideshare.net/povilas1/thoracic-outlet-syndrome-anatomy-symptoms-diagnostic-evaluation-and-surgical-treatment. Published September 12, 2011. Accessed February 16, 2017.
- Ranney D. Thoracic outlet: an anatomical redefinition that makes clinical sense. Clin Anat. 1996;9:50-52.
- Torriani M, Gupta R, Donahue DM. Sonographically guided anesthetic injection of anterior scalene muscle for investigation of neurogenic thoracic outlet syndrome. Skeletal Radiol. 2009;38:1083-1087.
- Hughes ES. Venous obstruction in the upper extremity; Paget-Schroetter’s syndrome; a review of 320 cases. Surg Gynecol Obstet. 1949;88:89-127.
- Alla VM, Natarajan N, Kaushik M, Warrier R, Nair CK. Paget-Schroetter syndrome. Review of pathogenesis and treatment of effort thrombosis. West J Emerg Med. 2010;11:358-362.
- Joffe HV, Goldhaber SZ. Upper-extremity deep vein thrombosis. Circulation. 2002;106:1874-1880.
- Drapanas T, Curran WL. Thrombectomy in the treatment of “effort” thrombosis of the axillary and subclavian veins. J Trauma. 1966;6:107-119.
- Azizzadeh A, Thompson RW. Clinical presentation and patient evaluation in aTOS. In: Illig KA, Thompson RW, Freischlag JA, Donahue DM, Jordan SE, Edgelow PI, eds. Thoracic Outlet Syndrome. London, UK: Springer-Verlag; 2013:551-556.
- Bharat A, Mackinnon SE, Patterson GA. Supraclavicular approach for thoracic outlet syndrome. In: Sugarbaker D, Bueno R, Colson Y, Jaklitsch M, Krasna M, Mentzer S, eds. Adult Chest Surgery. 2nd ed. New York, NY: McGraw-Hill; 2015:chap 142.
- Cinà C, Whiteacre L, Edwards R, Maggisano R. Treatment of thoracic outlet syndrome with combined scalenectomy and transaxillary first rib resection. Cardiovasc Surg. 1994;2:514-518
This article originally appeared on Clinical Advisor