Thoracic outlet syndrome

Thoracic outlet syndrome

1. Thoracic Outlet Syndrome DR. SUNIL KUMAR SHARMA SENIOR RESIDENT,DEPT. OF NEUROLOGY G.M.C., KOTA

2. The term ‘thoracic outlet syndrome’ (TOS) was originally coined in 1956 by RM Peet . The simple definition of thoracic outlet syndrome is neurovascular symptoms in the upper extremities due to pressure on the nerves and vessels in the thoracic outlet area. The specific structures compressed are usually the nerves of the branchial plexus and occasionally the subclavian artery or subclavian vein.( RICHARD J. SANDERS, M.D) Thoracic Outlet Syndrome

3. Depending on the exact site of injury and the injury component of the neurovascular bundle, three distinct syndromes or a combination of these may be encountered. Neurological syndrome (95%) Venous syndrome.(4%) Arterial syndrome (1%) Pathogenesis

4. The symptoms and signs are mixed among these three types. They should be called Predominant Neurogenic , Arterial, or Venous. Before the complications of TOS occurs, there is a period where uncomplicated TOS is misdiagnosed. Delay from symptoms to diagnosis -3 months to 15 years

5. Uncomplicated TOS (Disputed TOS, Nonspecific TOS, Common TOS) The Uncomplicated Form should also be divided in -Predominant Neurogenic , -Predominant Arterial, and -Predominant Venous types.  Complicated Form(true TOS) New classification

6. The Uncomplicated Form is the most common and the most undiagnosed, or misdiagnosed. The Uncomplicated Form can present with - mild-to-severe pain, - positional paresthesias as the only symptom. - no atrophy of the hand muscles The symptoms are frequently intermittent and oscillating. New classification

7. The Complicated Form is easy to diagnose, but too late, Symptoms and signs - Slowly progressive unilateral atrophic weakness of the intrinsic hand muscles & Sensory abnormalities in the C8- T1 distribution in the Neurogenic type . -Non-positional ischemia of the fingers and hands, Thrombosis and or embolism of the arteries of the upper extremities, subclavian aneurysm, and cerebral embolism ,are symptoms of Arterial TOS . -Venous thrombosis of the subclavian / axillary veins, Paget-von Schrötter syndrome, these are the signs and symptoms of the Venous type.

8. Pain and paresthesias of the upper extremities are common in all the three types. Shoulder, neck, and chest pains, facial pain, and occipital headaches are usually ignored symptoms in the Predominant Neurogenic type, both in the Uncomplicated or Complicated Forms.

9. Incidence 1-2% Age – usually seen 20-50 yrs of age Sex- Female: Male – 3:1 No Racial predilection Neurogenic TOS >95 % Venous TOS – 4 % Arterial TOS – 1% Epidemiology

10. Interscalene triangle ( most commonly involved) -Inferiorly : 1 st rib -Ant : scaleneus anterior -Post : scaleneus medius . Costoclavicular space -Ant : clavicle, subclavius muscle -Post medial: 1 st rib -Post lateral: superior border of scapula Pectoralis minor space - Anteriorly by Pectoralis minor and posteriorly by Chest wall ANATOMY

11. ANATOMY

12. Brachial plexus Subclavian artery Subclavian vein Contents Interscalene triangle Coracoclavicular space Pectoralis minor space

13. Anatomical defects- Bony abnormalities- Cervical rib Long C7 transverse process Abnormal bands, ligaments Fracture clavicle/ 1 st rib Exostosis Etiologies

14. Muscle anomalies Anomalous insertion of scalene muscles Scalene muscle hypertrophy Scaleneus minimus Passage of the brachial plexus through the substance of the anterior scalene muscle, A broad, excessively anterior middle scalene muscle insertion on the first rib

15. Tumours Trauma Brachial plexus trauma/Whiplash injury Poor posture.   Drooping the shoulders or holding the head in a forward position.

16. Repetitive activity.   Typing on a computer, Athletes and swimmers Baseball pitcher Obesity Pregnancy.  

17. A cervical rib is a supernumerary (or extra) rib which arises from the seventh cervical vertebra. Sometimes known as "neck ribs" Congenital abnormality located above the normal first rib. A cervical rib is present in less than 1% of the normal population, have been reported in 5%–9% of patients with TOS B/L in 50%, common in right side. Usually asymptomatic Cervical rib

18. Neurogenic TOS (95 %) f/b Venous variant of TOS(4%) & arterial TOS (1%)variant. Such big difference in the frequency of clinical manifestations of neurogenic and vascular (venous and arterial) TOS is due to the high sensitivity of nerves for compression and irritation. The subclavian vessels: artery and vein are compressed almost as often as nerves Clinical features

19. Paraesthesia Pain in shoulder, arm, forearm and fingers Occipital headache – referred from tight scalene muscles Weakness of forearm, hand. Neurogenic TOS

20. Cervical outlet syndrome(Upper TOS) – when brachial plexus nerve roots are compressed in the scalene triangle , Upper nerve roots (C5 C6 C7) are most forcefully compressed. True thoracic outlet Syndrome(Lower TOS)- When the compression of brachial plexus is in the costoclavicular space ,usually lower roots (C8-T1) of the brachial nerve plexus are compressed . Neurogenic TOS

21. Fatigue Weakness Coldness Upper limb claudication Thrombosis Paraesthesia Gangrene Raynaud's phenomenon due to thrombosis with distal embolisation Arterial TOS

22. Edema Venous distension Collateral formation Cyanosis Paget- Schroetter syndrome – effort thrombosis "Effort" axillary-subclavian vein thrombosis (Paget- Schroetter syndrome) is an uncommon deep venous thrombosis due to repetitive activity of the upper limbs. Venous TOS

23. Diagnosis

24. Clinical tests

25. Patient seated with arms above 90 degrees of abduction and full external rotation with head in neutral position. Patient opens and closes hands into fists while holding the elevated position for 3 minutes. Positive test: pain and/or paresthesia and discontinuation with dropping of the arms for relief of pain. Sensitivity- 52–84% Specificity- 30–100 Roos test/Elevated arms stress test(EAST)

26. Roos test/Elevated arms stress test False + ve in Carpal tunnel syndrome, Ulnar neuropathy, fi bromyalgia

27. Adson maneuver may be performed seated or standing. The patient is requested to take a deep breath and rotate and extend their head as far as possible towards the unaffected side. The affected side arm is abducted with the elbow fl exed, and the examiner’s fi ngers should be placed on the radial pulse. The test will reproduce symptoms or obliterate the ipsilateral radial pulse One can also listen for a bruit underneath the clavicle during the Adson’s test to document compression. Adson maneuver

28. Adson maneuver

29. Wright's hyperabduction test Arm hyperabducted to 180 °-diminishing radial pulse. Neurovascular structures compressed in subcoracoid region by pectoralis minor tendon, head of humerus or coracoid process. Sens.-70–90 Spec.-29–53

30. Wright's hyperabduction test

31. Patient sits straight with arms at the side. Radial pulse is assessed. Patient retracts and depresses shoulders while protruding the chest. Position is held for up to 1 minute. Positive test: change in radial pulse and/or pain and paresthesia . Sens.-NT Spes.-53–100 Costoclavicular maneuver

32. Costoclavicular maneuver

33. Patient seated, Examiner passively rotates the head away from the affected side and gently flexes the neck forward to end range moving the ear toward the ventral chest. Positive test: forward flexion part of the movement is notably decreased with a hard end feel. Sens.-100 Spec.-NT Cervical rotation lateral flexion test

34. Cervical rotation lateral flexion test

35. Upper limb tension testing is sensitive for irritation of the neural tissue including cervical roots, brachial plexus and peripheral nerves . It has been advocated for the diagnosis of neurogenic TOS with reported high sensitivity. The test appears to be excellent for screening for sensitization of the neural tissue in the cervical spine, brachial plexus and upper limb but is not specific for one area. Upper limb tension test

36. Head is turned contralaterally , the arm is abducted with the elbow extended Sens-90%, Spec.-38%. Upper limb tension test

37. A more objective examination is the lidocaine scalene block test. Under image guidance, either computed tomography, ultrasound, or fl uoroscopy, the anterior scalene muscle is injected with lidocaine . Patients with nTOS should have some decrease or complete relief of symptoms for four hours. An initial lidocaine block, if positive, predicts 90% success for subsequent treatments including physical therapy and surgical intervention lidocaine scalene block test

38. Carpel tunnel syndrome Spinal canal tumors Shoulder myositis Angina pectoris Reynaud's disease Ulnar nerve compression - epicondylitis Differential diagnoses

39. Investigations

40. Chest x ray, cervical spine x ray USG/ Colour Doppler MRI, cervical myelography r/o narrowing of intrevertebral foramen, disc compression,intraspinal SOL. Vascular imaging(angiogram/ venogram ) r/o aneurysm, thrombosis Nerve conduction study, electromyography confirm neurogenic TOS, localise the area of compression- r/o CTS Ix.

41. Cervical ribs, Elongated C7 transverse process, Degenerative spine disease, Bone destruction related to a primary or secondary neoplasm X ray cervical spine

42. Chest and cervical X ray Cervical plain radiograph of a 27-year-old woman shows both a cervical rib (arrow) and an elongated C7 transverse process (arrowhead).

43.   Anteroposterior plain radiograph of the clavicle   Showing Excessive callus of the clavicle in a 36-year-old patient with neurologic TOS.

44. Duplex ultrasound Highly sensitive and specific test for venous stenosis or occlusion May demonstrate an increased flow velocity in the subclavian artery at the site of a stenosis in aTOS . USG/ Colour Doppler

45. Conventional arteriography and venography may demonstrate the presence of extrinsic compression. Do not allow a clear depiction of the impinging anatomic structure, Replaced by less invasive procedures (CT, MR imaging,ultrasonography ). Arteriography and venography

46. Angiography CTA / MRA or traditional angiography can be utilized to identify more clearly the occlusion, aneurysm, thrombolyisis / distal embolization . To plan surgical reconstruction

47. A 38-year-old female presented with intermittent pain and numbness in her fingers, exacerbated by certain movements. The images show a subtracted three-dimensional contrast-enhanced MRA sequence with ( A ) arms down and ( B ) arms raised. Severe compression of the subclavian artery can be seen on both sides (arrows)

48. Arterial compression in a 24-year-old woman. MR angiogram shows the subclavian artery stenosis (arrow).

49. three-dimensional reformatted CT Image shows the arterial compression and the relationship of the artery (arrow) to the surrounding anatomic structures.

50. Sagittal T1-weighted MR image  show a scalenus minimus muscle (straight arrow), which passes between the C8 nerve root (arrowhead) and subclavian artery (curved arrow)

51.   Electrodiagnostic Studies Nerve conduction studies can be normal in uncomplicated nTOS Ulnar sensory potential amplitude is reduced or absent Ulnar motor potential is reduced out of proportion to the median

52. Treatment

53. Non operative treatment Posture improving exercises. Breathing exercises. Avoid aggravating activities. Avoid repetitive upper extremity mechanical work and muscular trauma. Analgesics,muscle relaxants, antidepressants. Physiotherapy .

54. Botulinum toxin A ( botox ) can be used for temporary symptom relief. Botox takes two weeks to work but can last three months and can help patients progress with physical therapy. Botulinum toxin injection with ultrasound/EMG guidance is safe and well tolerated in subjects with suspected nTOS . Botulinum toxin A (Botox)

55. Surgical treatment Indications: Symptoms persists with non operative treatment. Associated vascular compression. Progression of neurological symptoms. Nerve conduction velocity < 60m/s

56. First rib resection-complete resection of the first rib resulted in superior outcomes Anterior scalenectomy – ant and middle scaleni resected Cervical Rib resection if present Transaxillary approach or supraclavicular approach If an aneurysm is present, the patient may require an arterial reconstruction in addition to FRRS. (FRRS) Fi rst rib resection and anterior scalenectomy

57. aTOS Surgical intervention, speci fi cally is indicated for both venous and arterial TOS Uniformly, all patients with arterial thoracic outlet syndrome will need full anticoagulation and varying degrees of surgical intervention Milder – Catheter directed thrombolysis before repair Severe ischemia usually requires surgical embolectomy (with or without intraoperative thrombolysis ) in conjunction with thoracic outlet decompression Vascular TOS-

58. Anticoagulation is resumed three days after FRRS in vTOS Invasive venography is performed two weeks postoperatively -Lesion-free patent subclavian veins -stop anticoagulation -Those undergoing additional endovascular treatment are continued on anticoagulation for 1- 2 months until followup Followup -(N)-stop Anticoagulant - Thrombosis-cont. for 6 months vTOS

59. Approximately 60–70% of patients with nTOS can be successfully treated with -Avoidance of activities that precipitate symptoms, -Ergonomic modi fi cations to the workplace, -selective use of pharmacologic agents such as nonsteroidal anti- in fl ammatories , antidepressants, and muscle relaxants. nTOS

60. Physical therapy is also a very important component for these patients. Conservative management should be attempted for 8–12 weeks before considering surgery. Those that fail, should undergo a lidocaine scalene muscle injection. If they respond to this block, they may be evaluated to see if they are physically fi t for FRRS.

61. As more and more patients are treated for TOS, the referral pattern has begun to change. Now, patients are being referred earlier with a shorter duration of symptoms, which improves their chance of a successful surgical treatment. A rise of presentation in adolescents has also been observed. Modern experience indicates that a multidisciplinary comprehensive approach to TOS improves outcomes.   Recent Developments in TOS

62. Although TOS has come a long way in the last half century, there are many avenues left to explore. Diagnosis still remains the most debated aspect of neurogenic TOS. Despite multiple maneuvers and even the lidocaine scalene block, the test results rely on patient symptomatology alone. Continued research would be beneficial to find a more objective analysis. Some have suggested using MRI or CTAs preoperatively to compare TOS patients with control patients. Future Research

63. Thank You

64. Bradley‘s Neurology in Clinical Practice – 6 th edition Understanding Thoracic Outlet Syndrome Julie freishchlag and kristine orion Imaging Assessment of Thoracic Outlet Syndrome -Xavier Demondion el al.  Neurogenic thoracic outlet sndrome : A case report and review of the literature. Boezaart , AP, et al. International Journal of Shoulder Surgery. 2010;4:27-35. Epidemiology and pathogenesis of thoracic outlet syndrome- Gustaw Wojcik1,2*, Barbara Sokolowska3 , Jolanta Piskorz Thoracic outlet syndrome : anatomy, symptoms, diagnostic evaluation and surgical treatment Prof., Dr. Scs. Povilas Pauliukas