1. An Interesting Case of Thoracic Outlet Syndrome
Laurel Romer, M.D.
Primary Care Conference
May 14, 2008

2. Financial Disclosure
I have received no outside financial support for this presentation.

3. Learning Objectives Understand the pathophysiology of TOS
Learn the provocative maneuvers to diagnose TOS
Understand treatment options for TOS

4. Outline Case Presentation Definition Anatomy Epidemiology
Differential Diagnosis
Symptoms
Physical Exam
Diagnostic Testing
Treatment

5. Case Presentation
EB is a 30 year old white female who presents with left upper extremity swelling and pain, which began while she was watching a movie in a theater. She says that when she left the movie, a couple of hours after the symptoms initially began, she noted that the swelling of her left arm was worse and there was pain in her shoulder region. She also could not get her ring off of her finger and her left hand was dusky. She did not have any shortness of breath or pleuritic chest pain. A few days before that the patient had been shoveling snow during a heavy snow storm and the day after that developed upper respiratory symptoms including sore throat, sharp chest pain, a sensation of her ears being plugged and swollen glands in her neck.

6. Case Presentation
The patient had a Doppler evaluation which revealed clot in the subclavian and axillary veins. A CT scan of the chest revealed no evidence of pulmonary embolism. She had a hypercoagulable workup which was negative. She was treated with heparin and then started on Coumadin and consideration was given a couple of times to giving thrombolytics but this was not done. She was taken off her OCP.

7. Case Presentation
Ultrasound of the left upper extremity was performed about 15 days after the onset of symptoms and revealed interval partial recanalization of the left subclavian vein and slight increase in flow within the left axillary vein in a patient with previous occlusive thrombus in these vessels.
MRI of the left upper extremity was performed to look for a compressive component to her vasculature in the left upper extremity as a cause for the DVT.
The impression was chronically occluded segment of left brachial vein and chronic occlusion of left brachiocephalic vein, suggestion of early recannulization in the left brachiocephalic vein central to the confluence of left internal jugular and left subclavian veins. Venous drainage of the left upper extremity is most likely occurring through venous collaterals in the left lower neck, which cross midline and drain on the right.

8. TOS - Definition Adson first described his maneuver in 1927
Thoracic Outlet Syndrome first coined in 1956
Upper extremity symptoms due to compression of the neurovascular bundle by various structures in the area just above the first rib and behind the clavicle
Etiologies include congenital bony structures, fibromuscular abnormalities, posture, certain movements, trauma
Trauma – MVA followed by repetitive stress at work

9. TOS - Anatomy Trauma – MVA followed by repetitive stress at work
Google Images

10. TOS - Epidemiology 3 to 80 cases per 1000 Ages 20-40
Women > Men (4:1)
Neurogenic TOS (90%) > Venous TOS > Arterial TOS (<1%)
Cervical ribs occur in < 1% of population
70% women
Incidence difficult to determine due to lack of objective diagnostic tests for TOS
Most cervical ribs are asymptomatic; having cervical rib predisposes to NTOS after neck trauma, usually whiplash injuries
ATOS almost always associated with a cervical rib, so a normal neck xray is a good screening test to R/O ATOS

11. TOS – Differential Diagnosis
Cervical disc disease
Cervical facet disease
Malignancies (Pancoast/local tumors, spinal cord tumors)
Peripheral nerve entrapments (ulnar or median nerve)
Brachial plexitis
Rotator cuff injuries
Fibromyalgia, muscle spasm
Neurologic disorders (MS)
Chest pain, angina
Vasculitis
Vasospastic disorder (Raynaud’s)
Neuropathic syndromes of upper extremity
Incidence difficult to determine due to lack of objective diagnostic tests for TOS
Talu, GK: Agri 17 (2005), 5-9.

12. TOS - Symptoms Neurogenic TOS
Pain, paresthesia, and weakness in the hand, arm and shoulder, plus neck pain and occipital headaches
Raynaud’s phenomenon, hand coldness and color changes are also seen frequently in NTOS
RP symptoms caused by sympathetic nerve fibers run in the roots of C8. T1 and the lower trunk of the brachial plexus; when nerves are compressed this causes activation producing Raynaud’s
Sanders RJ, et al. J Vasc Surg, 46(3), 2007,

13. TOS - Symptoms Venous TOS
Swelling of the arm, plus cyanosis is strong evidence of subclavian vein obstruction
Pain often present, but may be absent
Arm swelling distinguishes VTOS from ATOS and NTOS
Paresthesia is common in VTOS, but is due to swelling in the hand, not nerve compression in TO
Sanders RJ, et al. J Vasc Surg, 46(3), 2007,

14. TOS - Symptoms Arterial TOS
Digital ischemia, claudication, pallor, coldness, paresthesia and pain in the hand (but rarely in the shoulder/neck)
Symptoms are a result of arterial emboli from a mural thrombus in a subclavian artery aneurysm or from thrombus forming distal to subclavian artery stenosis
Sanders RJ, et al. J Vasc Surg, 46(3), 2007,

15. TOS – Physical Exam VTOS NTOS arm swelling cyanosis
distended superficial veins over the shoulder and chest wall
NTOS
Tenderness over scalene muscles
Positive provocative tests
Sanders RJ, et al. J Vasc Surg, 46(3), 2007,

16. TOS – Physical Exam Provocative tests Adson test
Neck rotation and head tilting (ear to shoulder) eliciting pain and paresthesia down the contralateral side
90°AER - Abducting arms to 90 degrees in external rotation, brings on symptoms within 60 seconds
Upper Limb Tension Test
Sanders RJ, et al. J Vasc Surg, 46(3), 2007,

17. CLOSE
Pain down the arm, especially at the elbow or paresthesias of the hand are positive findings
Positive response indicates compression of the nerve roots or the branches of the brachial plexus
Fig. Upper Limb Tension Test (ULTT).
Position 1: Arms abducted to 90° with elbows extended.
Position 2: Dorsiflex wrists.
Position 3: Tilt head to side, ear to shoulder. Each maneuver progressively increases stretch on the brachial plexus.
                                                   

18. TOS – Diagnostic Testing
Neck or chest xray
Detects cervical rib or elongated C7 transverse process
EMG/NCS
Normal in large majority of clinically + NTOS
Most common finding in NTOS is ulnar neuropathy
Recent study suggests NCV abnormalities of the sensory medial antebrachial cutaneous nerve are seen in NTOS
MRI/CT
Venography/venous duplex
VTOS
Arteriography
Only indicated in ATOS
If no cervical rib, virtually elimates the dx of ATOS
MRI- helpful along with postural maneuvers especially for demonstrating brachial plexus compression
CT with contrast and postural maneuvers is effective in demonstrating vascular compression
Duplex u/s with postural maneuvers is a good supplementary tool when CT and MRI are negative or when patient presents with classic picture of VTOS
Seror, O. Clin Neurophysiol 115 (2004),

19. TOS – Treatment Conservative Management Massage, hydrotherapy and PT
Behavioral modification/avoidance of provocative activities
PT to strengthen muscles of the pectoral girdle and restore normal posture
Improvement: 50-90%
Focus is on relaxing the scalene muscles and strengthening the postural muscles
NSAIDS, muscle relaxants can be useful adjuncts
VTOS – thrombolytics, heparin, coumadin, surgical decompression
ATOS – revascularization via thromboembolectomy with surgical reconstruction if aneurysm present
Positive prognostic factors: compliance with home exercise program, modification of behavior at home and work
Negative prognostic factors: obesity, prior trauma, severity of symptoms, compensation claims pending, psychiatric illness, long symptom duration

20. TOS – Treatment Definitive management
Surgical decompression of the neurovascular bundle
First rib resection
Scalenectomy
Subclavian artery reconstruction
Cervical sympathectomy
NTOS – sympathetic overactivity may require sympathectomy along with decompression

21. Case Presentation
A few months later, venous duplex ultrasound appears normal. There is no evidence of impingement on either side. Both veins are patent by duplex but there is bilateral impingement in multiple stress positions on both the right and left side.

22. Case Presentation
EB met with a vascular surgeon who felt that she was at high risk for recurrence of BUE DVT (off anticoagulants) without definitive treatment
A left 1st rib resection was performed
A few months later a right 1st rib resection was performed
She was taken off anticoagulants
She was restarted on her OCP along with Aspirin 81mg qd
She was strongly advised to continue longterm with a shoulder girdle strengthening exercise program
High risk based on her vascular duplex study which showed venous impingement in various stress positions

23. References Barkhordarian, S. J Hand Surg 32 (4/2007), 565-570.
Demondion, X, et al. Radiographics 26 (2006),
Sanders RJ, et al. J Vasc Surg, 46 (2007),
Seror, O. Clin Neurophysiol 115 (2004),
Talu, GK: Agri 17 (2005), 5-9.
Vanti C, et al. Eura Medicophys 43 (2007),
High risk based on her vascular duplex study which showed venous impingement in various stress positions