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New Perspectives on Neurogenic Thoracic Outlet SyndromePublished
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New Perspectives on Neurogenic Thoracic OutletSyndrome
The quality of life for patients with neurogenic thoracic outlet
syndrome is profoundly diminished.Emerging evidence supports
minimally invasive chemodenervation of the cervicothoracic
musculaturewith onobotulinum toxin as a treatment option.By Paul J
Christo, MD, MBA [1] Volume 14, Issue #8
[2]The definition,incidence, diagnosis, and treatment of
thoracic outlet syndrome (TOS) are somewhat
controversial.Originally coined in 1956, the term TOS indicated a
compression of the neurovascular structures in theinterscalene
triangle corresponding to the possible etiology of the symptoms.1
The controversy iscentered on the fact that TOS refers to the
anatomy or location of the problem without identifying
thecauseeither vascular or neurogenic.
Therefore, TOS generally is defined as a group of disorders
caused by compression of the brachialplexus, subclavian artery, or
subclavian vein in the thoracic outlet, the area between the
clavicle(collarbone) at the base of the neck and the first rib,
including the front of the shoulders and chest. TOSis a progressive
condition marked by the impingement of the nerves and blood vessels
that feed thethoracic outlet.
Neurogenic TOS (NTOS), the most common form of TOC, can result
from inadequate space caused byscalene hypertrophy, fibrosis, or
congenital abnormalities, such as the occurrence of a cervical
rib.Other causes include repetitive motions that can enlarge or
change the tissue in or near the thoracicoutlet (similar to carpel
tunnel syndrome). These repetitive activities include assembly line
work, typing,and other movements; hyperextension-flexion injuries;
neck injuries from motor vehicle accidents(whiplash); and
sports-related injuries, particularly from swimming, baseball
(pitching), weightlifting,and volleyball.
Frequent symptoms of NTOS include numbness; tingling in the
fingers; pain in the neck, shoulder orarm; muscle spasms around the
scapula; headaches; and weakness in the upper extremities (Table
1).2
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New Perspectives on Neurogenic Thoracic Outlet SyndromePublished
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[3]
Pathophysiology
In many patients, the etiology of NTOS involves a combination of
a double hit of a congenitalpredisposition and an injury to the
area that compromises the outlet. The narrowed space affects
thescalene muscles, the brachial plexus, the long thoracic and
suprascapular nerves, and the stellateganglion (Figure 1).
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New Perspectives on Neurogenic Thoracic Outlet SyndromePublished
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[4]
Although the notion of NTOS as a complex spectrum disorder
provokes some controversy in the field, itsimpact on patients is
beyond dispute. Data indicate that the quality of life for a
patient with untreatedTOS is as impaired as that of someone with
chronic heart failure.3
TOS has been divided into 3 forms:
Neurogenic TOS (brachial plexus compression)
1. True neurogenic TOS2. Common neurogenic TOS
Arterial (subclavian artery compression)Venous (subclavian vein
compression)
As noted, nearly all cases of TOS (95%) are neurogenic in
origin. NTOS is an underappreciated and oftenoverlooked cause of
shoulder and neck pain and numbness. Like patients with other
chronic painconditions, patients with untreated neurogenic TOS
experience a diminished quality of life, reducedfinancial
well-being, functional limitations, and an increased risk for
depression and anxiety.4-6
True NTOS, which is confirmed with objective findings, accounts
for only 1% of cases, whereas commonNTOS, which has symptoms
suggestive of brachial plexus compromise but no objective findings,
makesup 99% of neurogenic cases of TOS.7,8 The remaining cases of
TOS are arterial (1%) and venous(3%-5%).1
Neurogenic TOS occurs in an estimated 3 to 80 per 1,000
individuals, the wide range reflecting the lackof confirmation in
many patients with signs and symptoms indicative of the condition.
Women withNTOS outnumber men by 3 to 4:1. The syndrome is
particularly common in people who perform
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New Perspectives on Neurogenic Thoracic Outlet SyndromePublished
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repetitive tasks with their upper extremities, such as
violinists, data entry personnel, and workers onassembly lines.
Athletes with repetitive overhead arm motion, including volleyball
players, swimmers,baseball pitchers, and weightlifters, also are at
increased risk, as are people who have experienced necktrauma.9
Histologic studies suggest that injury to either the anterior
scalene muscle (ASM) or the middle scalenemuscle are the main
causative factors of NTOS. Muscle fibrosis is a prime finding on
examination ofexcised scalene muscles, with NTOS patients having 3
times as much scar tissue as unaffectedsubjects.8-10
The ASM derives from the transverse processes of the C3-C6
vertebrae. The muscle, which attaches tothe first rib, serves as an
accessory muscle of respiration, and also rotates the neck
slightly. Spasm ofthe ASM puts traction on the brachial plexus and
causes edema of the muscle and nerves, which, inturn, limits the
space of the outlet. Development of scar tissue and fibrosis of the
ASM further worsenneural compromise and perpetuate pain.8,11
Targeting treatment to relieve tension and spasm of the ASM can
interrupt the chain of events thatleads to NTOS.
Diagnosis
There is no one standard for the diagnosis of TOS. The diagnosis
of NTOS can be difficult because itoften has a nonspecific clinical
presentation. In a classic case, the patient will complain of
painoriginating in the area of the shoulder and radiating along the
inner aspect of the arm. Other commonsymptoms involve pain in the
neck; the trapezius, mastoid, and anterior chest wall musclesall
fromupper plexus compression (C5-C7). Physical examination will
reveal tenderness in the scalene muscles,trapezius, and chest wall.
Patients may have a positive Tinel sign over the brachial plexus in
the neck,reduced sensation in the fingers to light touch, and
positive provocative maneuvers.9
Complicating the differential diagnosis, however, is that the
entire arm often is involved withoutdermatomal preference. The
clinician must distinguish cervical radiculopathy from disk
herniation orstenosis and rule out carpal tunnel syndrome.
A thorough history and physical examination are key to accurate
diagnosis of NTOS. Testing for NTOS isunreliable. Ancillary testing
lacks sensitivity and specificity. Similarly, provocative testing,
including theAdson maneuver,12 has unknown reliability and
specificity. The Adson maneuver, in particular, producesmany false
positive results and no longer is considered useful for identifying
patients with NTOS.13
Provocative maneuvers, nerve tension tests, and thumb pressure
over the brachial plexus can assist inthe determination of NTOS,
but the elevated arm stress test, or Roos stress test, is perhaps
the mostreliable indicator.13 Another potentially useful diagnostic
test includes the Spurling test to identifycervical disk
disease.8,14,15
Imaging Studies
Patients with NTOS often have normal results on
electromyelography (EMG) and nerve conduction tests.However, these
studies can be used to exclude other causes of neuropathic
symptoms, such asradiculopathy, carpal tunnel syndrome, cubital
tunnel syndrome, and polyneuropathy.
A chest x-ray may be warranted to identify cases of cervical
rib. Magnetic resonance imaging andcomputed tomography (CT) also
can help to rule out conditions that mimic NTOS.
Some evidence suggests that a medial antebrachial cutaneous
nerve conduction study can detectmilder cases of NTOS. This test
measures sensory function of the lower trunk of the brachial plexus
and
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New Perspectives on Neurogenic Thoracic Outlet SyndromePublished
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often yields positive results in patients with negative findings
on an EMG or nerve conduction tests.However, additional studies are
required to validate the utility of the test.9,16
Anterior Scalene Block
First described in 1939, the anterior scalene block (ASB) is an
intramuscular confirmatory test for NTOS.The block paralyzes the
muscle in spasm, allowing the first rib to descend and decompresses
thethoracic outlet.17 A positive response to an ASB test correlates
well with good surgical outcomes,whereas temporary muscle
relaxation helps predict benefit from decompression. In one study,
EMG-guided block provided relief in 94% of patients who underwent
surgery.18
A variety of imaging techniques can improve the success of ASB.
CT guidance for scalene injections, inparticular, has been shown to
minimize Horners sign, dysphonia, brachial plexus block,
anddysphagia.17
Treatment
Conservative
Conservative treatment for NTOS involves steps to minimize
pressure on the brachial plexus, restoringmuscle balance in the
neck, and improving neural mobility. Correcting ergonomic issues
and poorposture can help, as can nerve glides, stretching
exercises, and biofeedback. A 14-month course ofpostural correction
and strengthening of the shoulder girdle led to significant
reductions in pain andhigh patient satisfaction in one study.19
Physical Therapy
Some data support the use of heat packs, exercise programs, and
cervical traction for the treatment ofNTOS.20,21 A course of
inpatient rehabilitation, followed by a home exercise program,
appears to have ahigh rate of satisfaction among patients who have
undergone this regimen. However, data suggest that,in general, no
single approach to physical therapy is sufficient on its own.
Indeed, without otherinterventions, physical therapy may lead to
worse outcomes for some patients. In one study, 42 patients(37
women, 5 men) diagnosed with NTOS who had participated in physical
therapy at least 6 monthsprior to the study were selected.22 At the
end of the follow-up period, 25 patients reported
symptomaticimprovement, 10 reported that they were the same, and 7
patients had worse symptoms. Poor overalloutcome was related to
obesity (P
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New Perspectives on Neurogenic Thoracic Outlet SyndromePublished
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Chemodenervation
Injection of onobotulinum toxin Type A (Botox) is a relatively
new and promising approach to thetreatment of NTOS.23 Studies
indicate that onobotulinum toxin is safe and effective for an
increasingnumber of neuromuscular ailments. Approved indications
for onobotulinum toxin injections includehemifacial spasm,
blepharospasm, strabismus, and chronic migraine, among others.
Successful off-labeluse also has been described for lumbosacral
myofascial pain, piriformis syndrome, and lateralepicondylitis.
Administration of onobotulinum toxin for NTOS involves a single,
low-dose injection (20 units) into theASM under CT-guidance. In one
study, 27 patients with NTOS experienced substantial pain relief
for upto 3 months following low-dose injections of onobotulinum
toxin under CT guidance.23 The primaryoutcome was pain and
sensation on a visual analog scale (VAS) at 1, 2, and 3 months
after therapy.Short Form McGill Pain Questionnaire scores were
evaluated before treatment and at 1, 2, and 3 monthsafter therapy.
Patients reported substantial relief from treatment at both 1 and 2
months, andstatistically and clinically significant relief in both
sensory and VAS scores at the 3-month point (29%and 15%,
respectively).23
Onobotulinum toxin reduces muscle overactivity in the area of
the injection by blocking the release ofacetylcholine, weakening
the muscle for as long as 3 to 4 months. The toxin also may reduce
pain andinflammation in some patients, perhaps by inhibiting the
release of neuropeptidesparticularlysubstance P and glutamatethat
are implicated in nociceptive transmission and
centralsensitization.24,25 Some evidence suggests that onobotulinum
toxin can improve wound healing andreduce scarring in injured
muscles.5,26
Injections of onobotulinum toxin represent a minimally invasive
approach for patients hoping to avoidsurgery, or a bridge to
surgery for those seeking to delay the procedure. Successful
injections mayobviate the need for surgeryand the potential
complications from surgeryand limit the time patientsmust take off
from work, home duties, and other activities of daily living. This
benefit can be substantialbecause the common course for surgical
patients involves 8 weeks of physical therapy starting 2 weeksafter
the procedure, necessitating 2 to 3 months leave from work, as well
as no heavy lifting (>10pounds) for 6 months.5
Although chemodenervation can be performed using multiple
imaging modalities, the evidence for CTguidance is strong (Table
2). CT allows clinicians to visualize nearby anatomy (in real-time
in the case ofCT fluoroscopy), and, unlike ultrasound, it is not
vulnerable to obscuring by adiposity or osseousstructures. CT
imaging is fast, accurate, reliable, and safe, leading to a higher
percentage of successfulanesthetic blocks compared to other
modalities: 82% versus 38% for ultrasound, 18% for EMG
+fluoroscopy, and 72% for EMG alone.17,27,28 This advantage is
borne out by the high rate of improvementafter surgery associated
with CT-guided blocks (70%) to confirm true cases of neurogenic
TOS.5,23
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New Perspectives on Neurogenic Thoracic Outlet SyndromePublished
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[5]
Keeping exposure time to 60 seconds or less limits the amount of
ionizing radiation patients receive.
Surgical Decompression
Multiple approaches to surgical decompression for NTOS are
available, although comparative efficacydata for the techniques do
not exist. Studies suggest that initial rates of success are high,
approaching90%; however, complications occur in more than 30% of
patients and longitudinal data show a 60%recurrence of symptoms
within the first year after surgery and 80% within the second year.
In addition,60% of patients report persistent disability within the
first year after surgery.29
Conclusion
Neurologic TOS is the most common type of TOS, as well as the
most often overlooked andmisdiagnosed form of the condition. It
causes persistent pain, impaired function, and emotional
distress.If untreated, the quality of life for patients with NTOS
is profoundly diminished. Emerging evidencesupports minimally
invasive chemodenervation of the cervicothoracic musculature with
onobotulinumtoxin. Clinicians and patients should consider this
approach before attempting surgical decompression.References:
References
1. Hooper TL, Denton J, McGalliard MK, Brisme JM, Sizer PS Jr.
Thoracic outlet syndrome: acontroversial clinical condition. Part
1: anatomy, and clinical examination/diagnosis. J Man ManipTher.
2010;18(2):74-83.
2. Christo PJ, McGreevy K. Updated perspectives on neurogenic
thoracic outlet syndrome. Curr PainHeadache Rep. 2011;15
(1):14-21.
3. Chang, DC, Rotellina-Coltvet LA, Mukherjee D, De Leon R,
Freischlag JA. Surgical intervention forthoracic outlet syndrome
improves patients quality of life. J Vasc Surg.
2009;49(3):630-635.
4. Institute of Medicine (US) Committee on Advancing Pain
Research, Care, and Education. Relieving pain in America: a
blueprint for transforming prevention, care, education and
research.Washington, DC: National Academies Press (US); 2011.
5. Stewart WF, Ricci JA, Chee E, Morganstein D, Lipton R. Lost
productive time and cost due tocommon pain conditions in the US
workforce. JAMA. 2003;290(18):2443-2454.
6. Leong IY, Farrell MJ, Helme RD, Gibson SJ. The relationship
between medical comorbidity and self-rated pain, mood disturbance,
and function in older people with chronic pain. J Gerontol A
BiolSci Med Sci. 2007;62(5):550-555.
7. Atasoy, E. Thoracic outlet compressionsyndrome. Orthop Clin
North Am. 1996;27(2):265-303.
8. Brantigan CO, Roos DB. Etiology ofneurogenic thoracic outlet
syndrome. Hand Clin. 2004;20(1);17-22.
9. Sanders RJ, Hammond SL, Rao NM.Thoracic outlet syndrome: a
review. Neurologist. 2008;14(6):365-373.
10. Machleder HI, Moll F, Verity MA. The anterior scalene muscle
in thoracic outlet compressionsyndrome. Histochemical and
morphometric studies. Arch Surg. 1986;121(10):1141-1144.
11. Atasoy E. Thoracic outlet syndrome: anatomy. Hand Clin.
2004;20(1);7-14.12. Demirbag D, Unlu E, Ozdemir F, et al. The
relationship between magnetic resonance imaging
findings and postural maneuver and physical examination tests in
patients with thoracic outletsyndrome: results of a double-blind,
controlled study. Arch Phys Med Rehabil. 2007;88(7):844-851.
13. Physiopedia. Roos stress test.
http://www.physio-pedia.com/Roos_Stress_Test. Accessed August17,
2014.
14. Roos, DB. New concepts of TOS that explain etiology,
symptoms, diagnosis and treatment. Vasc
Page 7 of 10
-
New Perspectives on Neurogenic Thoracic Outlet SyndromePublished
on Practical Pain Management
(http://www.practicalpainmanagement.com)
Surg. 1979;13:313-2115. Rayan GM, Jensen C. Thoracic outlet
syndrome: provocative examination maneuvers in a typical
population. J Shoulder Elbow Surg. 1995;4(2):113-117.16. Foley
JM, Finlayson H, Travlos A. A review of thoracic outlet syndrome
and the possible role of
botulinum toxin in the treatment of this syndrome. Toxins.
2012;4(11):1223-1235.17. Mashayekh A, Christo PJ, Yousem DM, Pillai
JJ. CT guided injection of the anterior and middle
scalene muscles: technique and complications. Am J Neuroradiol.
2011;32(3):495-500.18. Jordan SE, Machleder HI. Diagnosis of
thoracic outlet syndrome using electrophysiologically
guided anterior scalene blocks. Ann Vasc Surg.
1998:12(3):260-264.19. Crosby CA, Wehbe MA. Conservative treatment
for thoracic outlet syndrome. Hand Clin.
2004;20(1): 43-49.20. Taskaynatan MA, Balaban B, Yasar E, et al.
Cerivcal traction in conservative management of
thoracic outlet syndrome. J Musculoskeletal Pain. 2007;15 (1):
89-94.21. Gulbahar S, Akalin E, Baydar M, et al. Regular exercise
improves outcome in droopy shoulder
syndrome: a subgroup of thoracic outletsyndrome. J
Musculoskeletal Pain. 2005;13(4):21-26.
22. Novak CB, Collins ED, Mackinnon SE. Outcome following
conservative management of thoracicoutlet syndrome. J Hand Surg Am.
1995;20(4): 542-548.
23. Christo PJ, Christo DK, Carinici AJ, Freischlag FA. Single
CT-guided chemodenervation of theanterior scalene muscle with
botulinum toxin for neurogenic thoracic outlet syndrome. Pain
Med.2010;11(4): 504-511.
24. Aoki K. Review of proposed mechanism for the antinociceptive
action of botulinum toxin type A. Neurotoxicology.
2005;26(5):785-793.
25. Sheean G. Botulinum toxin for the treatment of
musculoskeletal pain and spasm. Curr PainHeadache Rep.
2002;6(6):460-469.
26. Childers MK, Wilson DJ, et al. Treatment of painful muscle
syndromes with botulinum toxin: Areview. J Musculoskel Rehab.
1998;10:89-96.
27. Torriani M, Gupta R, Donahue DM. Botulinum toxin injection
in neurogenic thoracic outletsyndrome: results and experience using
an ultrasound-guided approach. Skeletal
Radiol.2010;39(10):973-980.
28. Jordan SE, Ahn SS, Gelabert HA. Combining ultrasonography
and electromyography forbotulinum chemodenervation treatment of
thoracic outlet syndrome: comparison withfluoroscopy and
electromyography guidance. Pain Physician. 2007;10(4):541-546.
29. Franklin GM, Fulton-Kehoe D, Bradley C, Smith-Weller T.
Outcome of surgery for thoracic outletsyndrome in Washington state
workers compensation. Neurology. 2000;54(6):1252-1257.
View Sources [6] References
1. Hooper TL, Denton J, McGalliard MK, Brisme JM, Sizer PS Jr.
Thoracic outlet syndrome: acontroversial clinical condition. Part
1: anatomy, and clinical examination/diagnosis. J Man ManipTher.
2010;18(2):74-83.
2. Christo PJ, McGreevy K. Updated perspectives on neurogenic
thoracic outlet syndrome. Curr PainHeadache Rep. 2011;15
(1):14-21.
3. Chang, DC, Rotellina-Coltvet LA, Mukherjee D, De Leon R,
Freischlag JA. Surgical intervention forthoracic outlet syndrome
improves patients quality of life. J Vasc Surg.
2009;49(3):630-635.
4. Institute of Medicine (US) Committee on Advancing Pain
Research, Care, and Education. Relieving pain in America: a
blueprint for transforming prevention, care, education and
research.Washington, DC: National Academies Press (US); 2011.
5. Stewart WF, Ricci JA, Chee E, Morganstein D, Lipton R. Lost
productive time and cost due tocommon pain conditions in the US
workforce. JAMA. 2003;290(18):2443-2454.
6. Leong IY, Farrell MJ, Helme RD, Gibson SJ. The relationship
between medical comorbidity and self-rated pain, mood disturbance,
and function in older people with chronic pain. J Gerontol A
Biol
Page 8 of 10
-
New Perspectives on Neurogenic Thoracic Outlet SyndromePublished
on Practical Pain Management
(http://www.practicalpainmanagement.com)
Sci Med Sci. 2007;62(5):550-555.7. Atasoy, E. Thoracic outlet
compressionsyndrome. Orthop Clin North Am. 1996;27(2):265-303.
8. Brantigan CO, Roos DB. Etiology ofneurogenic thoracic outlet
syndrome. Hand Clin. 2004;20(1);17-22.
9. Sanders RJ, Hammond SL, Rao NM.Thoracic outlet syndrome: a
review. Neurologist. 2008;14(6):365-373.
10. Machleder HI, Moll F, Verity MA. The anterior scalene muscle
in thoracic outlet compressionsyndrome. Histochemical and
morphometric studies. Arch Surg. 1986;121(10):1141-1144.
11. Atasoy E. Thoracic outlet syndrome: anatomy. Hand Clin.
2004;20(1);7-14.12. Demirbag D, Unlu E, Ozdemir F, et al. The
relationship between magnetic resonance imaging
findings and postural maneuver and physical examination tests in
patients with thoracic outletsyndrome: results of a double-blind,
controlled study. Arch Phys Med Rehabil. 2007;88(7):844-851.
13. Physiopedia. Roos stress test.
http://www.physio-pedia.com/Roos_Stress_Test. Accessed August17,
2014.
14. Roos, DB. New concepts of TOS that explain etiology,
symptoms, diagnosis and treatment. VascSurg. 1979;13:313-21
15. Rayan GM, Jensen C. Thoracic outlet syndrome: provocative
examination maneuvers in a typicalpopulation. J Shoulder Elbow
Surg. 1995;4(2):113-117.
16. Foley JM, Finlayson H, Travlos A. A review of thoracic
outlet syndrome and the possible role ofbotulinum toxin in the
treatment of this syndrome. Toxins. 2012;4(11):1223-1235.
17. Mashayekh A, Christo PJ, Yousem DM, Pillai JJ. CT guided
injection of the anterior and middlescalene muscles: technique and
complications. Am J Neuroradiol. 2011;32(3):495-500.
18. Jordan SE, Machleder HI. Diagnosis of thoracic outlet
syndrome using electrophysiologicallyguided anterior scalene
blocks. Ann Vasc Surg. 1998:12(3):260-264.
19. Crosby CA, Wehbe MA. Conservative treatment for thoracic
outlet syndrome. Hand Clin.2004;20(1): 43-49.
20. Taskaynatan MA, Balaban B, Yasar E, et al. Cerivcal traction
in conservative management ofthoracic outlet syndrome. J
Musculoskeletal Pain. 2007;15 (1): 89-94.
21. Gulbahar S, Akalin E, Baydar M, et al. Regular exercise
improves outcome in droopy shouldersyndrome: a subgroup of thoracic
outletsyndrome. J Musculoskeletal Pain. 2005;13(4):21-26.
22. Novak CB, Collins ED, Mackinnon SE. Outcome following
conservative management of thoracicoutlet syndrome. J Hand Surg Am.
1995;20(4): 542-548.
23. Christo PJ, Christo DK, Carinici AJ, Freischlag FA. Single
CT-guided chemodenervation of theanterior scalene muscle with
botulinum toxin for neurogenic thoracic outlet syndrome. Pain
Med.2010;11(4): 504-511.
24. Aoki K. Review of proposed mechanism for the antinociceptive
action of botulinum toxin type A. Neurotoxicology.
2005;26(5):785-793.
25. Sheean G. Botulinum toxin for the treatment of
musculoskeletal pain and spasm. Curr PainHeadache Rep.
2002;6(6):460-469.
26. Childers MK, Wilson DJ, et al. Treatment of painful muscle
syndromes with botulinum toxin: Areview. J Musculoskel Rehab.
1998;10:89-96.
27. Torriani M, Gupta R, Donahue DM. Botulinum toxin injection
in neurogenic thoracic outletsyndrome: results and experience using
an ultrasound-guided approach. Skeletal
Radiol.2010;39(10):973-980.
28. Jordan SE, Ahn SS, Gelabert HA. Combining ultrasonography
and electromyography forbotulinum chemodenervation treatment of
thoracic outlet syndrome: comparison withfluoroscopy and
electromyography guidance. Pain Physician. 2007;10(4):541-546.
29. Franklin GM, Fulton-Kehoe D, Bradley C, Smith-Weller T.
Outcome of surgery for thoracic outletsyndrome in Washington state
workers compensation. Neurology. 2000;54(6):1252-1257.
First published on: September 1, 2014
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