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Case Seventy Six - Thoracic Outlet Syndrome -
Variant of Paget - von Schroetter Syndrome

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Clinical History: 46 year old male with remote history of multiple injuries from Vietnam war, presenting with acute pain and numbness in right arm after hearing a "pop" while acting as a pall bearer the previous day.

Findings: Right upper extremity venogram demonstrates filling defects in the right axillary and subclavian veins, with external compression of the most medial aspect of the subclavian vein, at the bifurcation of the jugular vein and right brachiocephalic vein. Numerous collateral vessels are demonstrated.

After 24 hours of urokinase treatment, the thrombosed vessels are significantly improved. However, the external compression persists.

Diagnosis: Thoracic outlet syndrome. Variant of Paget - von Schroetter syndrome.

Discussion: Three potential compression sites responsible for thoracic outlet syndrome include:
1. Interscalene triangle - borders include anterior and medial scalene muscles and the first rib.
2. Costoclavicular space - between clavicle and first rib.
3. Pectoralis minor tunnel - space between pectoralis minor tendon anteriorly and coracoid process of scapula.

The majority of patients have one of the following congenital or acquired conditions in one or more of the potential compression sites:

Congenital lesion include cervical ribs, scalenus minimus muscle, wide insertion or hypertrophy of anterior scalene muscle, anomalous first rib, slender body habitus with a long thin neck. Acquired lesions include extreme muscular build, fractures to clavicle or first thoracic rib with nonanatomic alignment or exuberant callus formation, supraclavicular tumors or lymphadenopathy.

Effort thrombosis is a form of post-traumatic thrombosis of the axillary vein, usually observed in younger individuals following exercise or occupational activity. The etiology is compression of the axillary vein by either subclavian muscle or costocoracoid ligament during forced abduction. This is known as Paget-von Schroetter syndrome.

References:
Kadir, Saadon, M.D. Diagnostic Angiography. W. B. Saunders Co., Philadelphia, 1986.

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Submitted by:
Anne H. Nicklas, M.D.
Joseph P. Lipuma, M.D.