Thoracic outlet syndrome (TOS) is complex clinical entity characterized by various neurovascular signs and symptoms of the upper

limb. It includes several different types of disorders, as follows:

Neurologic TOS o Classic (true) neurologic TOS o Common (disputed) neurologic TOS Vascular TOS o Arterial TOS o Venous TOS Combined neurovascular TOS

TOS can involve various components of the brachial plexus, the blood vessels, or both at different sites between the base of the neck and the axilla. The arterial form is caused by compression of the subclavian artery, the venous form is caused by compression of the subclavian vein, and the neurologic form is caused by brachial plexus compression. Combined neurovascular TOS is usually traumatic. Most authors suggest that nonspecific neurologic TOS results from injury to the brachial plexus, by either traction or compression, at some point within the cervicoaxillary canal. True (classic) neurologic TOS, which is rare, is caused by congenital anomalies. Usually these anomalies include a taut fibrous band or rudimentary cervical rib Because of complex etiology and absence of good diagnostic tests, patient history is important in TOS.

In common neurogenic TOS, pain is the most common and earliest complaint. Detailed history characterizing the patient's pain may lead to appropriate diagnostic and therapeutic plans. o The location of pain is an important part of the history. Ask the patient to describe the pain location and type on a pain diagram (anterior/posterior and lateral view of human picture). Pain, numbness, and/or tingling of the upper extremity are common presenting features of neurogenic TOS. Sometimes the patient may report pain in the chest, neck, and/or face and even headache.[1] o Precipitating factors include repetitive or stressful activity, such as prolonged computer keyboard use or overhead work, which can provoke or intensify pain. Most patients report a history of an automobile accident or work-related injury. Alleviating factors may exist and may provide additional clues for possible etiologies. o Various terms can be used to describe quality of pain, but it is usually a dull aching type in neurogenic TOS. o Spreading or radiation of pain is also important in evaluation of neuropathic pain. If retrosternal pain (radiation from intercostobrachial nerve, a branch of T2 intercostal nerve) is noticed on the left side, it can be confused with pain of cardiac or pulmonary origin.[2, 3] o Use some type of rating system to evaluate pain severity or intensity with a degree of objectivity and reproducibility. Different types of pain scales may be used. Numerical scales are more useful and reliable. The Visual Analog Scale (VAS) is a commonly used numerical scale. Arterial TOS often is associated with aching, fatigue, weakness, and pallor due to brachial ischemia. Cold temperature always worsens symptoms Hyperabduction and depression of the shoulder may provoke symptoms in patients with TOS.

Various stress tests or provocative maneuvers are used by the clinician to evaluate TOS. The sensitivity and specificity of these maneuvers have been reported to be low. o Roos maneuver: When in the surrender posture, the patient reports paresthesia and numbness in extremities within 1 minute. This maneuver usually provokes symptoms in lateral cord distribution. o Wright maneuver: This maneuver requires the patient to hold the arms next to the ears. Paresthesias usually are noted down the medial scapular border and into lower trunk distribution. o Elevated-arm stress test: In this test, the patient keeps arms abducted with flexed elbows for 3 minutes while flexing and extending the fingers. Results are considered positive if the patient cannot do this for 3 minutes. o Hyperabduction test: The radial pulse is diminished after elevating the involved arm above the head. o Adson maneuvers: While the patient is in a sitting position, ask the patient to inspire deeply, hold his breath, and extend his neck. Then, turn the patient's head passively as far as possible toward one side and then the other. When the head is turned toward the unaffected side, or sometimes the affected side, obliteration of the radial pulse with a drop in blood pressure in the arm is considered a positive result. While turning the head in either direction, the pulse may disappear on both sides, but, on the affected side, a longer lag occurs in its return. During this maneuver, a bruit may develop that is best heard in the supraclavicular space.[4] o Military maneuver (ie, costoclavicular bracing): This maneuver provokes symptoms when the patient elevates the chin and pulls the shoulder joint behind in an extreme "attention" position.[5] In common neurogenic TOS, physical examination usually does not reveal appreciable sensory loss or motor atrophy in the limb. o Upper trunk involvement results in deltoid, upper arm, and medial scapular border pain. o Lower trunk involvement can cause dull ache in the medial forearm and paresthesias in the fourth and fifth fingers. o Tenderness to palpation over the brachial plexus and paresthesia on percussion may be observed (Tinel sign). Most patients demonstrate hypersensitivity to mechanical compression over the supraclavicular and infraclavicular fossae. o The Spurling sign (ie, pain during direct compression of the foraminal exit areas of cervical nerve roots) may help in making the diagnosis of cervical radiculopathy. o Vasomotor involvement caused by TOS must be differentiated from coexistent or other causes of vasomotor instability (eg, complex regional pain syndrome [reflex sympathetic dystrophy or causalgia]). In classic neurologic TOS, wasting (especially intrinsic hand muscle atrophy) is a characteristic feature. Signs of decreased pain and temperature sensation may be present in the C8 through T1 distribution. In arterial TOS, usual findings include cool and pale extremity. This finding depends on the extent of compression and injury to the subclavian artery. In venous TOS, the affected limb may be swollen and tender. It may exhibit cyanosis (dusky coloration), venous distension, and ischemic changes in the upper extremity. Strenuous physical activities of extremity can evoke these symptoms and signs. Venous thrombosis can develop at the site of compression.Most authors suggest that nonspecific neurologic TOS results from injury to the brachial plexus, by either traction or compression, at some point within the cervicoaxillary canal.

True (classic) neurologic TOS is caused by congenital anomalies and usually includes a taut fibrous band or rudimentary cervical rib. Other anatomic anomalies include elongated transverse process of C7.

Trauma or repetitive activities may produce TOS (eg, motor vehicle accident hyperextension injury, effort vein thrombosis). Certain postures of the body may exacerbate or provoke the symptoms of TOS (eg, hyperabduction with external rotation of arm, depression of shoulder). Differentials

Ankylosing Spondylitis Cervical Spondylosis: Diagnosis and Management Median Neuropathy Metastatic Disease to the Spine and Related Structures Radial Mononeuropathy Reflex Sympathetic Dystrophy Syringomyelia Torticollis Traumatic Peripheral Nerve Lesions Ulnar Neuropathy

Imaging Studies

Radiographs may exhibit bony anomalies. o Chest radiograph may demonstrate cervical or first rib, Pancoast tumor clavicle, or other skeletal deformity. o Cervical spine radiograph may be helpful in showing a cervical rib, an elongated transverse process, or scoliosis. Color flow duplex scanning can identify interruption of blood flow to the affected extremity. Arteriogram can identify blockage of the artery from thrombi or emboli. It also can detect aneurysms that may be compressing the plexus. Venography (subclavian vein) is confirmatory and remains the criterion standard. This study can demonstrate the site of obstruction or the presence of thrombus. CT scan or MRI is useful to exclude cord lesions and radiculopathy and may exhibit plexus distortion.

Other Tests

Electromyography/nerve conduction study may be useful to exclude coexistent abnormalities such as peripheral nerve entrapment or cervical radiculopathy. Sensory evoked potentials are of limited value in making the diagnosis of neurogenic-type TOS and have no established value in vascular-type TOS.

Medical Care Most patients with TOS require only symptomatic treatment and appropriate consultation. Arterial, venous, and neurologic features may coexist; treatment should be directed toward the dominant component.

Common neurologic-type TOS requires conservative management that commonly includes pharmacologic therapy and gentle physiotherapy. o Patients with common neurologic-type TOS may respond to physical therapy, which increases the range of motion of the neck and shoulders, strengthens the rhomboid and trapezius muscles, and induces a more erect posture. o Aggressive physiotherapy, particularly traction, should be avoided, because it may worsen brachial plexus symptoms.

o For true neurologic TOS, sectioning of the congenital band is an appropriate option. Vascular (arterial and venous) TOS is less common and often requires surgical treatment. o Patients with vascular-type TOS need immediate heparinization and vascular surgery consultation. o Anticoagulant therapy (ie, warfarin) may be needed for a minimum of 3 months in vasculartype TOS to prevent recurrent or ongoing thromboembolic occlusion. Analgesic drug therapy for TOS can be divided into the following categories: o Nonopioid analgesics (eg, nonsteroidal anti-inflammatory drugs [NSAIDs], acetaminophen): NSAIDs commonly are used in patients with mild to moderately severe pain. They inhibit inflammatory reactions and pain by decreasing prostaglandin synthesis. Acetaminophen is a safe choice for treatment of pain during pregnancy and breastfeeding. o Opioid analgesics: Opioids are used commonly as an analgesic for many pain syndromes. Opioid therapy can be a safe and effective option in patients with intractable nonmalignant pain and no history of drug abuse.[6] Quang-Cantagrel et al report that failure of one opioid cannot predict the patient's response to another opioid.[7] High doses of tramadol may provide effective and safe relief in neuropathic pain. o Antidepressants: Antidepressant medications play a major role in treatment of neuropathic pain. Tricyclic antidepressants - Amitriptyline (Elavil), nortriptyline (Pamelor) Selective serotonin reuptake inhibitor (SSRI) antidepressants - Paroxetine (Paxil), fluoxetine (Prozac), sertraline (Zoloft) Other antidepressants - Nefazodone (Serzone), venlafaxine (Effexor) o Anticonvulsants Sodium channel antagonists have been used in the management of neuropathic pain for several years. These medications are started slowly and administered as needed. Monitor the patient carefully. Several anticonvulsant drugs (eg, clonazepam, topiramate, gabapentin, lamotrigine, tiagabine, zonisamide) have been tried in treatment of TOS. In studies by Nicholson and Rowbothan, gabapentin has been reported to be effective in the management of chronic neuropathic pain syndromes. Controlled studies for the effect of lamotrigine are not yet available, but Jain noted that the drug has demonstrated effect in neuropathic pain. o Other adjunct analgesics: Muscle relaxants (eg, metaxalone [Skelaxin], cyclobenzaprine [Flexeril], benzodiazepines, tizanidine) may be helpful to decrease spasm and provide pain relief.

Surgical Care Careful evaluation and selection of the patient is very important.

Surgical management of TOS commonly includes supraclavicular and transaxillary approaches for anatomic decompression.[8] For classic neurologic TOS, sectioning of the congenital band with a supraclavicular approach is the appropriate option. If necessary, the tip of the rudimentary cervical rib can be removed. Spinal cord stimulation may be considered carefully for management of severe chronic pain that has been refractory to other conservative modalities.[9] Cherington et al reported on 5 patients who suffered serious injuries after surgery for TOS. These patients had few or no clinical abnormalities on examination prior to the surgery.[10] Other studies, including one of 8 patients who sustained brachial plexus injuries resulting in clinical and electrophysiologic deficits after TOS surgery, have been reported.[11]

ConsultationsConsultation may be needed depending on the type of TOS and pathology, as follows:

Neurology Orthopedic surgery Vascular surgery Physical medicine and rehabilitation

Activity Aggressive physiotherapy, particularly traction, should be avoided, because it may worsen brachial plexus symptoms Nonsteroidal anti-inflammatory agents (NSAIDs) These agents inhibit inflammatory reactions and pain by decreasing activity of cyclooxygenase, which is responsible for prostaglandin synthesis. NSAIDs may provide pain relief in the patient with TOS. Naproxen sodium (Anaprox, Naprelan, Naprosyn)For relief of mild to moderately severe pain; inhibits inflammatory reactions and pain by decreasing activity of cyclooxygenase, which results in decrease of prostaglandin synthesis. Ibuprofen (Motrin, Advil)NSAIDs used commonly for patients with mild to moderately severe pain. Inhibits inflammatory reactions and pain by decreasing prostaglandin synthesis. Analgesics - Class Summary Pain control is essential to quality patient care. Analgesics ensure patient comfort, promote pulmonary toilet, and have sedating properties, which are beneficial for patients who have sustained trauma or injuries. Acetaminophen (Tylenol, Feverall, Tempra, Aspirin Free Anacin)DOC for pain in patients with documented hypersensitivity to aspirin or NSAIDs, with upper GI disease, or who are taking oral anticoagulants. Oxycodone (OxyContin)Long-acting form of opioid currently used commonly for severe pain. Start with small dose and increase gradually. Morphine sulfate (MS Contin, Duramorph, Astramorph)Effective analgesic with good safety profile and ease of reversibility with naloxone. Various IV doses used; commonly titrated until desired effect obtained. Oral morphine sulfate includes Avinza, Kadian, and MS Contin. These medications are available in multiple different strengths (15-120 mg). Fentanyl transdermal patch (Duragesic)Potent narcotic analgesic with much shorter half-life than morphine sulfate. Excellent choice for pain management and sedation with short duration (30-60 min); easy to titrate. Easily and quickly reversed by naloxone. When using transdermal dosage form, most patients' pain controlled with 72-h dosing intervals; however, some patients may require dosing intervals of 48 h Antidepressants This complex group of drugs has central and peripheral anticholinergic effects, as well as sedative effects. They have central effects on pain transmission. They increase synaptic concentration of serotonin and/or norepinephrine in CNS by inhibiting their reuptake by the presynaptic neuronal membrane. Other options include milnacipran (Savella), duloxetine hydrochloride (Cymbalta), venlafaxine (Effexor), and bupropion (Wellbutrin).

Nortriptyline (Pamelor)Has demonstrated effectiveness in treatment of chronic and neuropathic pain. Amitriptyline (Elavil)Analgesic for certain chronic and neuropathic pain. Selective serotonin reuptake inhibitors -These agents may be considered as alternative to TCAs. Fluoxetine (Prozac) Antidepressant with potent specific 5-HT uptake inhibition with fewer anticholinergic and cardiovascular adverse effects than TCAs. Sertraline (Zoloft)Antidepressant with potent specific 5-HT uptake inhibition with fewer anticholinergic and cardiovascular adverse effects than TCAs. Paroxetine (Paxil)Antidepressant with potent specific 5-HT uptake inhibition with fewer anticholinergic and cardiovascular adverse effects than TCAs. Benzodiazepines By binding to specific receptor sites, these agents appear to potentiate the effects of GABA and facilitate inhibitory GABA neurotransmission and other inhibitory transmitters. They may act in the spinal cord to induce muscle relaxation. Clonazepam (Klonopin)Suppresses muscle contractions by facilitating inhibitory GABA neurotransmission and other inhibitory transmitters. Anticonvulsants Use of certain antiepileptic drugs, such as the GABA analogue gabapentin (Neurontin), has proven helpful in some patients with neuropathic pain. Other anticonvulsants (eg, clonazepam, topiramate, lamotrigine, zonisamide, tiagabine) also have been tried in chronic pain. Pregabalin (Lyrica) can be effective, tolerable, and easy to titrate compared to gabapentin. Gabapentin (Neurontin)Has anticonvulsant properties and antineuralgic effects; however, exact mechanism of action unknown. Structurally related to GABA but does not interact with GABA receptors. Prognosis

Neurogenic TOC usually requires chronic pain management. Vascular-type TOC may have better outcome with appropriate management. Patients with profound intrinsic muscle atrophy do not regain their function. Appropriate surgery may arrest the progressive deterioration

Patient Education

Instruct the patient on the following: o Good posture o Job modification o Exercise programs