1. Effective Treatment Algorithm for the Management of Acute Upper Extremity Venous Thrombosis Secondary to Thoracic Outlet Syndrome (Paget-Schroetter Syndrome) Toufic Safa, MD, FACS Vascular & Endovascular Surgery St. Francis Hospital, Roslyn NY

2. ANATOMIC BOUNDARIES: Thoracic Outlet

3. Thoracic Outlet Syndrome Three Types: 1- 95% have neurological symptoms (C8 and T1) 2- 4% have venous symptoms 3- 1% have arterial symptoms Paget-Schroetter (Effort Thrombosis) describes the venous syndrome

4. Dr. James Paget and Dr. Leopold Von Schroetter

5. Risk factors for Paget-Schroetter Syndrome: 1.Hypertrophied Costo-Clavicular Ligament 2.Physical activity involving hyperabduction of the shoulder, as seen in weight lifters 3.Arm Motion often associated with tennis playing and baseball pitching 4.Vigorous exercise of the neck and upper extremity muscles 5.Overdeveloped anterior scalene, pectoralis minor, or subclavius muscles 6.Rudimentary first rib 7.Presence of cervical rib 8.Congenital band between first and second ribs 9.Fracture of the clavicle with callus formation 10.Apical tumors of the superior sulcus of the lung (Pancoast tumor)

6. Three Zones Where the Vein can be compressed: Scalene Anticus, Sbclavius, and Pec Minor

7. THORACIC OUTLET: Vein Compression

8. 2 / 100.000 per year 1-4% of all DVT INCIDENCE Lindblad B et al. DVT of the axillary-subclavian veins: epidemiologic data, treatment and late sequelae. Eur J Vasc Surg 1988; 2:161-5

9. Mean age:early 30s Male to Female ratio:2:1 Right Arm (dominant):80% Vigorous exercise:60-80% EPIDEMIOLOGY Illig KA and Doyle AJ. A comprehensive review of Paget-Schroetter syndrome. J Vasc Surg 2010;51:1538-47

10. YOUR TYPICAL PATIENT

11. SIGNS and SYMPTOMS of T.O.S. Pain in the upper limb Swelling Darkened Skin with increased turgor Feeling of heaviness Easily fatigued arm and hand Superficial vein distension Thrombophlebitis of the upper limb

14. Presentation – Young, healthy individuals – Often within 24 hours of a strenuous activity – Aching in shoulder or axilla; worse with activity – Swelling of the arm and hand Physical Exam – Non-pitting edema – Cyanosis of hand and fingers – Pulses are typically palpable

15. WORK-UP: 1- Venous Duplex Ultrasound 2- MR Venography: Arm in neutral and abducted positions 3- Conventional Venography

16. MR Venography : Arm down and Arm elevated Notice vein occlusion when arm is elevated

17. CONVENTIONAL VENOGRAPHY: Neutral and Abducted Positions

18. CONVENTIONAL VENOGRAPHY

19. Medical Therapy : Anticoagulation alone Interventional Therapy : Thrombolytic therapy, Angioplasty, followed by Anticoagulation Surgical Therapy : Decompression of thoracic outlet: First rib resection, excision of accessory cervical rib….etc TREATMENT of TOS: CONTROVERSIES

20. Interventional Therapy

21. Thrombolytic therapy with tPA: Trans-catheter Dosing: 10mg bolus followed by 0.01mg/kg/hour for 2-3 days or till clot is dissolved It is an off-label use for tPA Re-imaging in 24-hour intervals to re-assess Mechanical thrombectomy devices like Angiojet or TRELLIS can shorten the lysis times VENOUS CLOT LYSIS

22. Try NOT to STENT the Subclavian Venous Stenosis

23. SURGICAL TREATMENT OF T.O.S. FRIST RIB RESECTION Possible approaches

24. SURGICAL TREATMENT OF T.O.S. FRIST RIB RESECTION : Transaxillary approach - Patient Positioning

25. TRANSAXILLARY APPROACH: Need a Strong First Assistant

26. TRANSAXILLARY FIRST RIB RESECTION: NECESSARY TOOLS

27. TRANSAXILLARY FIRST RIB RESECTION: Initial View

28. TRANSAXILLARY FIRST RIB RESECTION: Mobilize Rib with Periosteal Elevator: Divide Anterior Scalene attachment to first rib

29. TRANSAXILLARY FIRST RIB RESECTION: Divide First Rib in the middle using Bone Cutter

30. TRANSAXILLARY FIRST RIB RESECTION: Divide Rib at costo-clavicular angle using angled Bone Cutter

31. TRANSAXILLARY FIRST RIB RESECTION: Divide remaining segment of Rib using same instrument

32. TRANSAXILLARY FIRST RIB RESECTION: FINAL VIEW – First Rib is out

33. INCISION IS COSMETICALLY CLOSED

34. SURGICAL TREATMENT OF T.O.S. COMPLICATIONS: o Minor  transient dysesthesia  pneumothorax  hemo- or chylo-thorax  Horner’s syndrome o Major :  venous or arterial injuries  brachial plexus injury

35. PERSONAL EXPERIENCE: 5 Patients in three years (3 males, 2 females, ages 16-44) All underwent duplex U/S and Venography All required lysis with tPA and mechanical thrombectomy with Angiojet Placed on heparin/coumadin for 4-6 weeks Four returned for transaxillary first rib resection One patient reclotted vein while on anti-coagulation and was managed conservatively Other four patients had full resolution of symptoms No major post-op complications

36. SURGICAL TREATMENT OF T.O.S. Case Example 1

37. SURGICAL TREATMENT OF T.O.S. Case Example 2

38. CASE EXAMPLE 3

39. CASE EXAMPLE 4

40. CASE EXAMPLE 5

41. Arm Swelling Venous Thrombosis Lysis with tPA followed by anticoagulation for 4-6 weeks Trans-axillary First Rib resection Re-thrombosis Full anti- coagulation Venous Stenosis Trans-axillary First Rib Resection SUMMARY: Algorithm for Arm Swelling (Venous TOS)

42. THANK YOU