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

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

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

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

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.

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.

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.

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

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

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

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.

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, 601-604.

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, 601-604.

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, 601-604.

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, 601-604.

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, 601-604.

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.                                                    

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), 2316-2322.

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

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

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.

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

References Barkhordarian, S. J Hand Surg 32 (4/2007), 565-570. Demondion, X, et al. Radiographics 26 (2006), 1735-1750. Sanders RJ, et al. J Vasc Surg, 46 (2007), 601-604. Seror, O. Clin Neurophysiol 115 (2004), 2316-2322. Talu, GK: Agri 17 (2005), 5-9. Vanti C, et al. Eura Medicophys 43 (2007), 55-70. High risk based on her vascular duplex study which showed venous impingement in various stress positions