Vascular Thoracic Outlet Syndrome

Two distinct terms are used to describe vascular thoracic outlet syndrome (TOS) depending on which structure is predominantly affected: venous TOS (due to subclavian vein compression) and arterial TOS (due to subclavian artery compression). Although the venous and arterial subtypes of TOS affect only 3% and <1% of all TOS patients respectively, the diagnostic and management approaches to venous and arterial TOS have undergone considerable evolution due to the recent emergence of minimally invasive endovascular techniques such as catheter-directed arterial and venous thrombolysis, and balloon angioplasty. In this review, we discuss the anatomical factors, etiology, pathogenesis and clinical presentation of vascular TOS patients. In addition, we use the most up to date observational evidence available to provide a contemporary approach to the diagnosis and management of venous TOS and arterial TOS patients. Two distinct terms are used to describe vascular thoracic outlet syndrome (TOS) depending on which structure is predominantly affected: venous TOS (due to subclavian vein compression) and arterial TOS (due to subclavian artery compression). Although the venous and arterial subtypes of TOS affect only 3% and <1% of all TOS patients respectively, the diagnostic and management approaches to venous and arterial TOS have undergone considerable evolution due to the recent emergence of minimally invasive endovascular techniques such as catheter-directed arterial and venous thrombolysis, and balloon angioplasty. In this review, we discuss the anatomical factors, etiology, pathogenesis and clinical presentation of vascular TOS patients. In addition, we use the most up to date observational evidence available to provide a contemporary approach to the diagnosis and management of venous TOS and arterial TOS patients. Central MessageThe approach to vascular TOS has undergone significant evolution given the emergence of endovascular therapies over the last decade. The approach to vascular TOS has undergone significant evolution given the emergence of endovascular therapies over the last decade. PerspectiveVascular thoracic outlet syndrome (TOS) can be divided into 2 forms (arterial and venous) depending on which structure is compressed in the thoracic outlet. Clinical evidence for vascular TOS is limited to case series, with a paucity of randomized controlled trials. Our review focuses on the contemporary diagnostic and management approach to vascular TOS given the increased popularity of endovascular techniques. Vascular thoracic outlet syndrome (TOS) can be divided into 2 forms (arterial and venous) depending on which structure is compressed in the thoracic outlet. Clinical evidence for vascular TOS is limited to case series, with a paucity of randomized controlled trials. Our review focuses on the contemporary diagnostic and management approach to vascular TOS given the increased popularity of endovascular techniques. See Editorial Commentary page 158–159. See Editorial Commentary page 158–159. TOS refers to a constellation of signs and symptoms attributable to compression of the neurovascular bundle in the thoracic outlet region of the upper extremity. A total of 3 distinct terms are used to describe TOS depending on which structure is predominantly affected: neurogenic TOS (nTOS) from brachial plexus compression, venous TOS (vTOS) from subclavian vein compression, and arterial TOS (aTOS) from subclavian artery compression. The most common form by far is nTOS, which accounts for more than 90% of all TOS cases. This review focuses on the venous and arterial subtypes of TOS, which are seen in 3% and <1% of TOS patients, respectively. During the past decade, the approach to vTOS and aTOS has undergone considerable evolution due to the emergence of minimally invasive endovascular therapies. The thoracic outlet area comprises 3 anatomic spaces: scalene triangle, costoclavicular space, and pectoralis minor (PM) space (Fig. 1). The borders of the anterior scalene muscle, middle scalene muscle, and first rib define the scalene triangle; trunks of the brachial plexus and the subclavian artery pass through this space (Fig. 1A). Cervical ribs and anomalous first ribs may compress the scalene triangle, resulting in symptoms of nTOS or aTOS. The costoclavicular space, which is the area between the first rib and the clavicle housing all 3 major structures (subclavian artery, vein, and brachial plexus), represents the most common site of subclavian vein compression (Fig. 1B). The PM space, defined by the PM muscle anteriorly and the chest wall posteriorly, is an extension of the thoracic outlet and a common site of neurovascular compression. Most patients with TOS are adults between the age of 20 and 50 years, although adolescents aged 18 years or younger present with vascular TOS more frequently than adults.1Chang K. Graf E. Davis K. et al.Spectrum of thoracic outlet syndrome presentation in adolescents.Arch Surg. 2011; 146: 1383-1387Crossref PubMed Scopus (30) Google Scholar Factors that increase the risk of neurovascular compression and development of symptomatic TOS include congenital anomalies in the thoracic outlet, repetitive motion or stress activities, and traumatic injuries. Arterial TOS is associated with bone abnormalities or trauma in nearly all cases, whereas vTOS is most often the result of repetitive overhead arm and shoulder activities, such as swimming, throwing, or weight-lifting in combination with anomalies of the costoclavicular space. Arterial TOS is most commonly associated with bone abnormalities of the thoracic outlet, with cervical ribs being present in up to 85% of patients with aTOS in contemporary series.2Aljabri B. Al-Omran M. Surgical management of vascular thoracic outlet syndrome: A teaching hospital experience.Ann Vasc Dis. 2013; 6: 74-79Crossref PubMed Google Scholar, 3Criado E. Berguer R. Greenfield L. The spectrum of arterial compression at the thoracic outlet.J Vasc Surg. 2010; 52: 406-411Abstract Full Text Full Text PDF PubMed Scopus (69) Google Scholar, 4Davidović L.B. Koncar I.B. Pejkić S.D. et al.Arterial complications of thoracic outlet syndrome.Am Surg. 2009; 75: 235-239PubMed Google Scholar, 5Orlando M.S. Likes K.C. Mirza S. et al.A decade of excellent outcomes after surgical intervention in 538 patients with thoracic outlet syndrome.J Am Coll Surg. 2015; 220: 934-939Abstract Full Text Full Text PDF PubMed Scopus (76) Google Scholar Cervical ribs are present in <1% of the general population, and about 70% of individuals with cervical ribs are women. Cervical ribs that cause symptoms are often large, and can have bony fusion to the first rib that results in subclavian artery compression.6Chang K.Z. Likes K. Davis K. et al.The significance of cervical ribs in thoracic outlet syndrome.J Vasc Surg. 2013; 57: 771-775Abstract Full Text Full Text PDF PubMed Scopus (75) Google Scholar This causes injury to the third segment of the subclavian artery, leading to intimal damage, thrombosis, distal embolism or poststenotic dilation, and aneurysm formation. Rarely, complete occlusion of the subclavian artery may occur. Other less common anatomic abnormalities causing aTOS include anomalous first rib, prominent C7 transverse process, callus formation from an old clavicular or first rib fracture, and fibrocartilaginous band. In vTOS, the subclavian vein is often compressed between the first rib, costoclavicular ligament and subclavius tendon within the costoclavicular space (Fig. 1B). Repetitive arm movements traumatize the vein, causing posttraumatic inflammation, focal intimal fibrosis, stenosis, blood flow stasis, and eventual thrombosis leading to acute symptoms of upper extremity deep venous thrombosis. This clinical condition is referred to as effort thrombosis or Paget-Schroetter syndrome, and is often observed in young individuals and competitive athletes who engage in physical activities requiring repetitive arm and shoulder movements.7Chandra V. Little C. Lee J.T. Thoracic outlet syndrome in high-performance athletes.J Vasc Surg. 2014; 60: 1012-1018Abstract Full Text Full Text PDF PubMed Scopus (81) Google Scholar Chronic compression and repetitive trauma also cause inflammation external to the subclavian vein. This point is underscored by the fibrotic, hypertrophied and relatively fixed surrounding anatomic structures often observed during vTOS surgery. Early in the disease process, aTOS patients most commonly present with chronic arm, shoulder, or neck pain with increasing activity due to subclavian artery stenosis or thrombosis. Hand or arm ischemia due to arterial embolization is the most common acute presentation of aTOS. A subset of aTOS patients present with concomitant symptoms of nTOS, such as chronic arm or hand paresthesias, numbness, or weakness from coexisting brachial nerve compression.8Likes K. Rochlin D.H. Call D. et al.Coexistence of arterial compression in patients with neurogenic thoracic outlet syndrome.JAMA Surg. 2014; 149: 1240-1243Crossref PubMed Scopus (42) Google Scholar Physical examination for aTOS focuses on measuring bilateral blood pressures of the upper extremities to asses for a marked discrepancy between the symptomatic and asymptomatic side, palpating for cervical ribs or a pulsatile mass in the supraclavicular area, examining hands for signs of digital ischemia, and auscultating for bruits in the supraclavicular fossa. Moreover, narrowing the artery through shoulder abduction may provoke bruits if not present at rest. The classic clinical finding of vTOS is upper extremity edema with dilated subcutaneous collateral veins evident over the arm, shoulder, and chest wall. These collateral veins develop to accommodate the increased venous pressure from subclavian vein stenosis or thrombosis. In the largest series of vTOS to date (626 extremities), Urschel and Patel9Urschel H.C. Patel A.N. Surgery remains the most effective treatment for Paget-Schroetter syndrome: 50 years׳ experience.Ann Thorac Surg. 2008; 86: 254-260Abstract Full Text Full Text PDF PubMed Scopus (146) Google Scholar reported that vTOS patients most commonly present with visible collateral veins around the shoulder (99%), followed by arm swelling (96%), bluish discoloration (94%), aching pain with exercise (33%), and cervical ribs (10%). Only 4% of the patients presented with minimal symptoms. Severe complications of acute deep venous thrombosis, such as pulmonary embolism or venous gangrene, are rare. Plain radiography of the neck and chest with cervical spine view or thoracic inlet view will often demonstrate bone abnormalities that may aid in the diagnosis of TOS. Cervical ribs, prominent, or elongated C7 transverse processes, abnormal or elongated first ribs, and large fracture calluses are easily detected on plain radiographs. In a study of 31 patients with TOS, only 2 had normal plain radiographs, whereas the other 29 had abnormalities, such as cervical ribs and enlarged transverse processes detectable on plain radiographs.10Lascelles R.G. Mohr P.D. Neary D. et al.The thoracic outlet syndrome.Brain. 1977; 100: 601-612Crossref PubMed Scopus (29) Google Scholar However, the negative predictive value of plain radiography is debatable, and more definitive diagnostic imaging is required in nearly all cases to establish or exclude a diagnosis of TOS. Duplex ultrasound (DUS) examination is an effective initial approach in confirming clinical suspicion of vascular TOS. Aneurysmal change, arterial stenosis, and thrombosis can be detected by DUS of the subclavian and axillary arteries. Moreover, DUS has the advantage of assessing dynamic blood flow during compression maneuvers (hyperabduction), with a decrease in arterial diameter, changes in peak velocity or reproducible symptoms considered to be diagnostic of aTOS. Figure 2 shows an arterial DUS of the upper extremity with normal flow in the left brachial artery at rest, and complete cessation of flow with 45° of arm abduction above the shoulder—a classic finding in aTOS. DUS also has good accuracy in detecting subclavian-axillary vein thrombosis, although its use is limited in providing information about surrounding structures or preoperative planning. Conventional catheter-based arteriography or venography has traditionally been considered the gold standard diagnostic test for vascular TOS as it provides information about the exact location and nature of the vascular compression. However, because of its invasive nature, and lack of visualization of surrounding structures means catheter-based angiography and venography are largely reserved for intraprocedural interventional guidance. Contrast-enhanced computed tomography (CT) and magnetic resonance (MR) are now widely available, reliable, and reproducible modalities used to establish the diagnosis of vascular TOS. CT and MR are typically performed as 2-step procedures (neutral position and arm abduction) to reproduce the vascular compression seen on provocative maneuvers. CT angiography or venography provides superior analysis of the vasculature in relation to the bony structures, whereas MR angiography or venography is more efficient in the depiction of accessory muscles, muscle hypertrophy and fibrous bands.11Demondion X. Herbinet P. Van Sint Jan S. et al.Imaging assessment of thoracic outlet syndrome.RadioGraphics. 2006; 26: 1735-1750Crossref PubMed Scopus (158) Google Scholar Both of these imaging modalities are useful in delineating the exact location and nature of the vascular compression, and aid in surgical planning. The American College of Radiology Appropriateness Criteria recommendations suggest that CT and MR angiography or venography are both appropriate in establishing the diagnosis of TOS12Moriarty J.M. Bandyk D.F. Broderick D.F. et al.ACR appropriateness criteria imaging in the diagnosis of thoracic outlet syndrome.J Am Coll Radiol. 2015; 12: 438-443Abstract Full Text Full Text PDF PubMed Scopus (32) Google Scholar; therefore, which imaging modality is selected likely depends on clinician and institutional experience and expertise. Figure 3 presents a CT angiogram showing moderate impingement of the left subclavian artery by a cervical rib with poststenoic dilatation, and distal embolization to the left brachial artery in a patient with an acute ischemic arm secondary to aTOS. The management strategy for aTOS is guided by the nature and severity of arterial complications. Asymptomatic patients with subclavian artery compression without evidence of arterial degeneration may be managed nonsurgically due to a low risk of complications. It is reasonable to follow these patients with serial imaging of the arterial system, as the natural history of these patients is not clearly defined. We prefer to perform arterial DUS of the upper extremity every 6 months in these patients, although it is unclear if this practice leads to earlier diagnosis of arterial complications. Surgical treatment is required for patients with symptoms and evidence of arterial complications, such as intimal damage, mural thrombus, embolization, poststenotic dilatation, or aneurysm formation. The appropriate surgical strategy is guided by 3 main principles: decompression, arterial resection, and distal revascularization.1.Decompression: At minimum, relieving the arterial compression involves resection of cervical or first ribs, fibrous bands, scalenectomy, and removing any other associated anomalies. Some authors suggest that the first rib should be routinely removed to prevent recurrence of symptoms because it acts as a key insertion point for that cause vascular K. Rochlin D.H. et or first ribs are the cause of thoracic outlet Vasc Surg. 2014; Full Text Full Text PDF PubMed Scopus Google Scholar that anterior and middle is as effective as with first rib resection, and that a approach leads to less a risk of or plexus injury and hospital The treatment of thoracic outlet syndrome: A of Vasc Surg. Full Text Full Text PDF PubMed Scopus Google Scholar, et thoracic outlet for the first Surg. PubMed Scopus Google Scholar However, the evidence for this is and largely on patients with a past history of neurogenic TOS. for approach be over of any of arterial such as a subclavian artery aneurysm or stenosis with intimal is to prevent ischemic complications of the upper Vascular in the form of or may be required depending on the of the subclavian artery evidence of distal is or may be used in with arterial to of the are 2 main surgical approaches to thoracic outlet and The advantage of the approach is that it provides complete visualization of the first rib for and minimal risk to neurovascular structures that are from the first et approach for thoracic outlet syndrome: results of Surg. 2011; PubMed Scopus Google Scholar However, this approach is largely used for nTOS, as it is not for vascular The supraclavicular approach is for aTOS as it resection of the cervical and first ribs, structures, and vascular However, be to and the brachial plexus and other neurovascular structures, such as the and thoracic and the subclavian vein with this The supraclavicular of the thoracic outlet is in Figure series and a complete of aTOS symptoms in of B. Al-Omran M. Surgical management of vascular thoracic outlet syndrome: A teaching hospital experience.Ann Vasc Dis. 2013; 6: 74-79Crossref PubMed Google Scholar, 3Criado E. Berguer R. Greenfield L. The spectrum of arterial compression at the thoracic outlet.J Vasc Surg. 2010; 52: 406-411Abstract Full Text Full Text PDF PubMed Scopus (69) Google Scholar, 4Davidović L.B. Koncar I.B. Pejkić S.D. et al.Arterial complications of thoracic outlet syndrome.Am Surg. 2009; 75: 235-239PubMed Google Scholar, 5Orlando M.S. Likes K.C. Mirza S. et al.A decade of excellent outcomes after surgical intervention in 538 patients with thoracic outlet syndrome.J Am Coll Surg. 2015; 220: 934-939Abstract Full Text Full Text PDF PubMed Scopus (76) Google Scholar of the largest and most recent series, which cases of that in this is low and to nerve injury to the brachial plexus that with B. Al-Omran M. Surgical management of vascular thoracic outlet syndrome: A teaching hospital experience.Ann Vasc Dis. 2013; 6: 74-79Crossref PubMed Google Scholar Other complications include arterial or Although surgical is considered the gold standard approach to case have endovascular of the subclavian artery in combination with surgical M. et endovascular and surgical approach for the management of subclavian artery occlusion due to thoracic outlet syndrome.J Surg. 2011; 26: PubMed Scopus Google Scholar, C. K. et and first rib resection for thoracic outlet syndrome by an aneurysm of the subclavian Google Scholar The advantage of this is a less invasive approach to subclavian artery this be a risk of fracture at this location due to compression from external structures such as the clavicle and first D. et and cause for 6: PubMed Scopus Google Scholar is subclavian artery can be considered a to surgical for a distal or is treatment if the upper is from be followed by thoracic outlet and arterial to the of such as subclavian artery stenosis or of which arterial is surgical is to thoracic outlet The natural history of acute subclavian-axillary vein thrombosis is associated with and series have reported or symptoms in of vTOS patients with H.C. Patel A.N. Surgery remains the most effective treatment for Paget-Schroetter syndrome: 50 years׳ experience.Ann Thorac Surg. 2008; 86: 254-260Abstract Full Text Full Text PDF PubMed Scopus (146) Google Scholar treatment for vTOS involves of 3 therapies in to thrombolysis, decompression, and is selected depends on the clinical presentation of patients with contemporary series have treatment for vTOS on M.S. Likes K.C. Mirza S. et al.A decade of excellent outcomes after surgical intervention in 538 patients with thoracic outlet syndrome.J Am Coll Surg. 2015; 220: 934-939Abstract Full Text Full Text PDF PubMed Scopus (76) Google Scholar, H.C. Patel A.N. Surgery remains the most effective treatment for Paget-Schroetter syndrome: 50 years׳ experience.Ann Thorac Surg. 2008; 86: 254-260Abstract Full Text Full Text PDF PubMed Scopus (146) Google Scholar, J.M. et clinical and treatment for Paget-Schroetter Surg. 2013; 100: PubMed Scopus Google Scholar, L. S. et treatment with is effective in the management of venous thoracic outlet syndrome A ultrasound study in patients with due to venous thoracic outlet 2011; 26: PubMed Scopus Google Scholar, K. et and in first rib resection and for and chronic subclavian vein Vasc Surg. 2010; 52: Full Text Full Text PDF PubMed Scopus Google Scholar, Paget-Schroetter syndrome with and Vasc Surg. Full Text Full Text PDF PubMed Scopus Google Scholar, et treatment for outcomes in venous thoracic outlet 2009; Full Text Full Text PDF PubMed Scopus Google Scholar, et treatment of venous thoracic outlet syndrome: surgical and Vasc Surg. Full Text Full Text PDF PubMed Scopus Google Scholar, et of effort thrombosis of the subclavian Vasc Surg. Full Text Full Text PDF PubMed Scopus Google Scholar, S. et surgical management of the competitive with effort thrombosis of the subclavian vein Vasc Surg. 2008; Full Text Full Text PDF PubMed Scopus Google Scholar, et the treatment of venous thoracic outlet PubMed Scopus Google Scholar, J.T. et recurrence after management of Paget-Schroetter syndrome.J Vasc Surg. 2006; Full Text Full Text PDF PubMed Scopus (146) Google Scholar as a the approach to vTOS management can be on 3 patient (Fig. patients catheter-directed with surgical of the thoracic outlet has been reported to clinical A of may be required venous is by of a subclavian vein stenosis after may be considered to the risk of with chronic stenosis or occlusion of the subclavian vein with evidence of vTOS from surgical may be considered in patients with total occlusion in an to A of may be required venous is by of a subclavian vein stenosis may be considered to the risk of with chronic symptoms of venous without evidence of or significant stenosis only surgical or are not and approaches have all been for subclavian vein approach is selected often depends on experience and as is evidence to approach over are to rib resection with to the thoracic outlet, and the most common site of venous compression is in the costoclavicular space, first rib resection is routinely in vTOS patients in combination with first rib resection has been associated with of in patients with D. et intervention for thoracic outlet syndrome of Vasc Surg. 2009; Full Text Full Text PDF PubMed Scopus Google Scholar A have the of venous and the thoracic outlet, with in a costoclavicular is associated with of fracture and J.T. et recurrence after management of Paget-Schroetter syndrome.J Vasc Surg. 2006; Full Text Full Text PDF PubMed Scopus (146) Google Scholar, M. et experience with venous in vein Vasc Surg. 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