Paget-Schrötter Syndrome and the Thoracic Outlet. Carl Aschkenasi September 6, 2001
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1 Paget-Schrötter Syndrome and the Thoracic Outlet Carl Aschkenasi September 6,
2 Our Patient: Acute onset left arm pain J.B. is a 44 yo male lawyer Has had a hx of occasional mild L arm pain on exertion PMH: Hereditary angioedema, HTN Presented to ED last week w/ 1 wk h/o L arm swelling, cyanosis, pain, worse when carrying his briefcase Hand was cold, radial pulses present but R>L Tenderness to deep palpation over L anterior shoulder 2
3 Differential for this presentation Myocardial ischemia/infarction Musculoskeletal strain/trauma Angioedema Pancoast s tumor Cervical disk dz Cervical cord tumor/syringomyelia Brachial plexopathy Carpal tunnel syndrome Reflex sympathetic dystrophy Vasculitidies, NOS Thromboembolic dz Thoracic outlet syndrome 3
4 Anatomy Neurovasculature of the arm 4
5 The Thoracic Outlet Three tight spaces: 1. Between scalenes 2. Costoclav. space: clavicle first rib sup margin scapula 3. Under pec minor & coracoid (from Vesalius.com) 5
6 Mr. J.B. s images on admission 6
7 From the ED to the fluoroscopy suite: Venography of J.B. s L brachial vein. Humerus Vein with mottled filling defects and evidence of collateralization Lines and stuff (disregard) 7
8 Digital subtraction MR venography done the morning following J.B s initial presentation L internal jugular vein Low signal region within lumen representing thrombus in L subclavian vein Continuation of brachial vein, with portions out of plane, showing thrombus resolved due to overnight tpa thrombolysis (Coronal view, see next slide for horizontal view ) 8
9 Horizontal reformatting of J.B. s L subclavian vasculature showing thrombus in situ L subclavian vein w/ thrombus L subclavian artery (also affected on hyperabduction in J.B., but not shown here.) 9
10 Thoracic outlet syndrome TOS coined by Peet et. al in at Mayo 1956 Triad of sx: numbness, weakness, swelling Also: Color change/raynaud s Diffuse pain/parathesias (90%) in ulnar distribution) Weakness/atrophy (10%) Decreased pulses Gangrene Subclavian bruit, Pseudoangina 10
11 Pathophysiology of TOS/PSS From Makhoul & Machleder, J Vasc. Surg. (1992) 11
12 Paget-Schroetter syndrome: a close cousin to TOS Independently described by von Schroetter in Vienna in 1884 and Paget in London in 1875 Described as effort thrombosis of axillary/subclavian vein Secondary to anatomic anomalies and/or recurrent use/injury Also secondary to subcl. catheters, transvenous pacers, iv drug use 12
13 TOS/PSS Etiologies Cervical ribs (0.5% population!, 5% of folks w/ cervical rib have sx) Fibrous bands Laxity of costoclavicular joint Osteophytes Shoulder girdle trauma Postural disturbances: pendulant breasts, obesity, backpackers 13
14 Look out for cervical ribs! (from VirtualHospital.com) 14
15 TOS/PSS Factoids Three types: Neurologic, vascular, both Brachial plexus involved in 98% cases (dx d by CT) Arterial only in 1-5% of TOS pts Only 200 known pts in 140 yrs of English-language medical literature! Diagnostic typology after Scher et. al. (1984): Stage 0: no signs, no sx, no visible lesion Stage 1: Minimal stenosis and mild poststenotic dilation Stage 2: Aneurysm, intimal damage, mural thrombosis Dreaded complication: PE (in older series has been 5-10%) 15
16 (plus Valsalva) Examination for suspected thoracic outlet syndrome (after DeGowin and DeGowin, Diagnostic Examination) 16
17 Radiologic workup CXR Angiography Doppler (series: 10 of 13 diagnosed) MR veno/angiography IVUS 17
18 Compression at the costoclavicular triangle Neutral position, note mural thrombus Abduction 18 (from Univ. of Wisconsin Med. Sch. Radiology web site)
19 At angiography (humeral compression) collaterals Abduction From Urschel & Razzuk, Ann. Surg. (1998) Adduction 19
20 Management of TOS/PSS Conservative: Exercises/postural advice/support bra relief in 50-90% in 6wks Injections of steroids plus analgesic Thrombolysis PTCA/stenting Surgical: (<5% need surgery) Anterior rib resection Anterior scalene release Clavicular resection. Often the only successful treatment is to wait for collateralization!! 20
21 Surgery, anticoagulation, and stenting From Hall et. al., J Vasc. & Int. Radiol. (1995) 21
22 Presentation type and anatomic etiology in a 200-patient series From Makhoul & Machleder, J Vasc. Surg. (1992) 22
23 Case summary & follow-up: J.B. 1. Thrombolysis w/ t-pa was performed w/ good effect 2. Conventional angio and MR complemented each other and guided therapy 3. Heparin was started as a bridge to coumadin 4. Post-lysis angio showed some residual mural thrombus and persistent arterial stenosis 1. Pt was d/c d in stable condition w/ medical follow-up The end. 23
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