Thoracic Outlet Syndrome: A Case and Current Diagnostic and Management Principles

This article is an educational revision of a surgical case report. It is not personal medical advice and should not be used as a substitute for evaluation by clinicians experienced in vascular, neurologic, musculoskeletal, and peripheral nerve disorders.

Abstract

A case of thoracic outlet syndrome (TOS) treated at the University Hospital of the West Indies, Jamaica, is presented, followed by an updated discussion of diagnosis and management. The case includes cervical-rib-related subclavian artery compression and neurologic findings, making it better understood as vascular TOS (VTOS) with possible concomitant lower brachial plexus involvement. Current practice classifies TOS into neurogenic, venous, and arterial subtypes, each with distinct diagnostic and treatment pathways (Illig et al., 2016).

Introduction

Thoracic outlet syndrome is a group of disorders caused by compression of neurovascular structures as they pass from the neck and thorax into the upper limb. The Society for Vascular Surgery reporting standards classify TOS according to the compressed structure: neurogenic TOS (NTOS) involves the brachial plexus, venous TOS (VTOS) involves the subclavian vein, and arterial TOS (ATOS) involves the subclavian artery (Illig et al., 2016).

Neurogenic TOS is the most common subtype, while venous and arterial TOS are less common. Recent reviews estimate NTOS accounts for most diagnosed cases, VTOS for a small minority, and ATOS for the smallest proportion; exact frequency varies by diagnostic criteria, referral setting, and whether nonspecific upper-limb pain syndromes are included (Rizzo et al., 2024; Camporese et al., 2022).

The observation that TOS can be missed is important. Current consensus emphasizes that diagnosis depends on a careful history, physical examination, exclusion of more common mimics, and subtype-specific testing rather than a single provocative maneuver or imaging test (Chim and Hagan, 2024; Hock et al., 2024).

Case Report

A 15-year-old female presented with a long history of painful swelling on the left side of the neck, cramps, and numbness in the left upper limb. On physical examination there was a 2 cm bony swelling in the left supraclavicular fossa. The left radial pulse was weaker than the right. The small muscles of the left hand were wasted and there was diminished sensibility to fine touch over the outer aspect of the left arm. Chest radiographs showed a left cervical rib, and arteriography demonstrated subclavian arterial stenosis and post-stenotic dilatation anterior to the tip of the cervical rib.

TOS was diagnosed and the transaxillary approach was used to resect the first thoracic rib, cervical rib, and scalenus anticus on the affected side. Mild stenosis of the subclavian artery did not require resection. The pleural cavity was inadvertently entered and a thoracostomy tube was inserted.

After operation the left radial pulse was normal and equal to the right in volume. Recovery was uneventful and the patient was discharged one week after surgery. At three months follow-up she had complete resolution of pain and cramps and normal sensation in the left upper limb.

In current terminology, this case has ATOS features because of cervical-rib-associated subclavian artery stenosis and post-stenotic dilatation. The hand wasting and sensory symptoms also suggest severe lower brachial plexus involvement, which may coexist with vascular compression when a bony anomaly narrows the thoracic outlet (Camporese et al., 2022; Khabyeh-Hasbani et al., 2023).

Discussion

Anatomy and classification

Pain, paresthesia, weakness, swelling, color change, or ischemic symptoms in the neck and upper limb have a broad differential diagnosis. Important alternatives include cervical radiculopathy, peripheral nerve entrapment, shoulder disease, brachial neuritis, Raynaud disease, Pancoast tumor, neuropathy, and vascular disease. Neurogenic TOS is usually a diagnosis of exclusion, and consensus criteria require that symptoms not be satisfactorily explained by another condition (Chim and Hagan, 2024; Khabyeh-Hasbani et al., 2023).

Compression can occur in the scalene triangle, costoclavicular space, subpectoral or pectoralis minor space. The scalene triangle is a common site for brachial plexus and subclavian artery compression, the costoclavicular space is important in venous compression, and the subpectoral space can contribute to pectoralis minor syndrome (Camporese et al., 2022; Rizzo et al., 2024).

Anatomic contributors include cervical ribs, first-rib anomalies, prominent C7 transverse processes, fibrous bands, clavicle or first-rib fractures, scalene muscle abnormalities, posture, repetitive overhead activity, and trauma. Cervical ribs are uncommon, often quoted around 1%-2% of the general population, and they are particularly important when associated with arterial compression or lower brachial plexus symptoms (Camporese et al., 2022).

Clinical presentation

Neurogenic TOS commonly presents with pain in the neck, shoulder, upper back, arm, or hand, along with numbness, paresthesia, weakness, vasomotor symptoms, or, in severe cases, intrinsic hand wasting. International Neurogenic Thoracic Outlet Syndrome (INTOS) consensus supports modified Society for Vascular Surgery criteria in which NTOS requires local findings, peripheral neurologic findings, and absence of another reasonably likely diagnosis; Consortium for Research and Education on Thoracic Outlet Syndrome (CORE-TOS) criteria also require upper-extremity symptoms beyond a single nerve-root or peripheral-nerve distribution and symptoms not satisfactorily explained by another condition (Chim and Hagan, 2024).

Venous TOS typically presents with effort thrombosis or focal subclavian-vein stenosis, causing upper-limb swelling, pain, reduced range of motion, and visible superficial collateral veins over the chest or upper arm. When subclavian-vein thrombosis is diagnosed, anticoagulation is started to limit clot propagation while definitive evaluation and treatment are considered (Davies and Hart, 2024).

Arterial TOS is rare but clinically important because it can cause subclavian artery stenosis, post-stenotic dilatation or aneurysm, thrombosis, distal embolization, claudication, pallor, ischemia, ulceration, or gangrene. Pulse diminution during provocative positioning can support suspicion, but pulse change alone is not sufficiently specific to establish the diagnosis (Rizzo et al., 2024).

Physical examination and provocative tests

A detailed history and physical examination remain the foundation of diagnosis. Provocative maneuvers can reproduce symptoms, but they are best interpreted as supportive findings rather than definitive tests because clinical testing has limited sensitivity and specificity and there is no single gold-standard diagnostic test (Hock et al., 2024).

The elevated arm stress test (EAST), also called the Roos test, remains a commonly used maneuver for suspected NTOS. In this test, the shoulders are abducted and externally rotated while the patient repeatedly opens and closes the hands for up to three minutes; reproduction of pain, paresthesia, weakness, or early fatigue supports but does not prove TOS (Chim and Hagan, 2024; Rizzo et al., 2024).

Symptom reproduction with palpation or percussion over the scalene triangle is described as a supraclavicular Tinel sign or Morley-type sign suggestive of NTOS. An important mimic of NTOS is cervical radiculopathy. Spurling’s maneuver is commonly used to rule-in the diagnosis of cervical radiculopathy. In the Spurling’s maneuver, the patient’s cervical spine is extended, and laterally flexed with the head rotated towards the affected limb while gentle downward pressure is applied to the head by the examiner.

Other provocative maneuvers, including Adson, Wright, costoclavicular, upper-limb tension, and vascular positional tests, may help characterize symptoms. Current consensus supports using the EAST/Roos test and supraclavicular Tinel/Morley-type findings for NTOS, while vascular symptoms should prompt duplex ultrasound, CT/MR angiographic or venographic imaging, and vascular specialist evaluation as appropriate (Chim and Hagan, 2024; Rizzo et al., 2024).

Imaging and electrodiagnostic testing

Plain chest or cervical spine radiographs remain useful to identify cervical ribs, prominent C7 transverse processes, clavicular abnormalities, and other bony causes of compression. INTOS consensus recommends routine cervical spine or chest radiography in NTOS workup to evaluate for a cervical rib (Chim and Hagan, 2024).

Imaging should be selected according to suspected subtype. For ATOS, CT angiography, MR angiography, Doppler ultrasound, and arteriography may identify stenosis, aneurysm, thrombosis, embolization, or dynamic compression. For VTOS, duplex ultrasound, CT venography, MR venography, catheter venography, and dynamic venography can identify subclavian-vein thrombosis, stenosis, collateral veins, and positional compression (Rizzo et al., 2024; Davies and Hart, 2024).

For NTOS, MRI may help identify cervical spine disease, mass lesions, brachial plexus abnormalities, or anatomic compression, and electrodiagnostic studies may document lower trunk or C8-T1 plexopathy or help exclude mimics such as cervical radiculopathy, carpal tunnel syndrome, and cubital tunnel syndrome. Electrodiagnostic studies can be normal in many patients with NTOS, so a normal study does not necessarily exclude the diagnosis when clinical criteria are otherwise met (Chim and Hagan, 2024; Khabyeh-Hasbani et al., 2023).

Treatment

Treatment should be subtype-specific. For most patients with NTOS, first-line treatment is conservative management with structured rehabilitation, activity modification, posture and scapular mechanics training, nerve-gliding exercises, stretching, and progressive strengthening. INTOS consensus recommends conservative treatment for 3-6 months before surgery for most NTOS patients, but objective muscle atrophy or weakness can justify earlier surgical evaluation (Chim and Hagan, 2024; Camporese et al., 2022).

Rehabilitation is also important after VTOS surgery and for return of function, but venous and arterial TOS are not managed by rehabilitation alone when thrombosis, aneurysm, embolization, stenosis, or ischemia is present. Acute or subacute VTOS with subclavian-vein thrombosis may require anticoagulation, catheter-directed thrombolysis or thrombectomy in selected patients, decompression, venolysis, and staged endovascular management depending on timing and anatomy (Davies and Hart, 2024; Mota et al., 2024).

Arterial TOS with significant stenosis, post-stenotic dilatation, aneurysm, thrombosis, embolization, or limb ischemia requires vascular evaluation. Surgical decompression may need cervical-rib or first-rib resection, scalenectomy, and arterial repair or reconstruction depending on residual arterial disease after decompression (Rizzo et al., 2024).

Surgical approaches and outcomes

Surgical intervention involves primarily thoracic outlet decompression. Depending on subtype and anatomy, decompression may include scalenectomy, first-rib resection, cervical-rib resection, fibrous-band excision, brachial plexus neurolysis, pectoralis minor tenotomy, venolysis, or arterial reconstruction. The approach may be supraclavicular, infraclavicular, transaxillary, paraclavicular, transmanubrial, thoracoscopic, or robotic depending on exposure needs and surgeon expertise (Rizzo et al., 2024; Khabyeh-Hasbani et al., 2023).

For NTOS, INTOS consensus recommends an anterior supraclavicular approach for exposure of the supraclavicular brachial plexus, with infraclavicular exposure or pectoralis minor tenotomy considered when examination or imaging suggests pectoralis minor involvement (Chim and Hagan, 2024). For VTOS, modern series describe infraclavicular decompression and venolysis as a way to access the costoclavicular space and subclavian vein directly, though evidence remains limited and approach selection varies by center (Mota et al., 2024).

Outcome estimates should be interpreted cautiously. Surgical results vary by subtype, diagnostic certainty, operation, surgeon experience, duration of symptoms, and outcome definition. A recent NTOS review reports more variable outcomes for NTOS than for VTOS, while a recent VTOS decompression cohort reported high subclavian-vein patency and symptom-free rates at follow-up but was retrospective and single-center (Khabyeh-Hasbani et al., 2023; Mota et al., 2024).

Persistent or recurrent symptoms after surgery are not always due to incomplete first-rib resection alone. Potential causes include an incorrect or incomplete diagnosis, coexisting cervical radiculopathy or distal nerve entrapment, residual scalene or fibrous-band compression, pectoralis minor syndrome, scar-related tethering, recurrent vascular stenosis or thrombosis, and incomplete decompression. Current consensus therefore emphasizes standardized diagnostic criteria, careful exclusion of mimics, appropriate imaging, patient selection, and postoperative rehabilitation (Chim and Hagan, 2024; Hock et al., 2024).

References

Camporese, Giuseppe, Enrico Bernardi, Andrea Venturin, Alice Pellizzaro, Alessandra Schiavon, Francesca Caneva, Alessandro Strullato, et al. “Diagnostic and Therapeutic Management of the Thoracic Outlet Syndrome. Review of the Literature and Report of an Italian Experience.” Frontiers in Cardiovascular Medicine 9 (2022): 802183. https://doi.org/10.3389/fcvm.2022.802183. Link: https://pmc.ncbi.nlm.nih.gov/articles/PMC8983020/.

Chim, Harvey, Robert R. Hagan, and the INTOS Workgroup. “Consensus Recommendations for Neurogenic Thoracic Outlet Syndrome from the INTOS Workgroup.” Plastic and Reconstructive Surgery Global Open 12, no. 8 (2024): e6107. https://doi.org/10.1097/GOX.0000000000006107. Link: https://pmc.ncbi.nlm.nih.gov/articles/PMC11357692/.

Davies, Mark G., and Joseph P. Hart. “Endovascular Management of Acute and Subacute Venous Thoracic Outlet Syndrome.” Frontiers in Surgery 11 (2024): 1302568. https://doi.org/10.3389/fsurg.2024.1302568. Link: https://pmc.ncbi.nlm.nih.gov/articles/PMC10909919/.

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Khabyeh-Hasbani, Nathan, Katherine Connors, Joshua R. Buksbaum, and Steven K. Koehler. “Current Concepts in the Management of Neurogenic Thoracic Outlet Syndrome: A Review.” Plastic and Reconstructive Surgery Global Open 11, no. 3 (2023): e4829. https://doi.org/10.1097/GOX.0000000000004829. Link: https://pmc.ncbi.nlm.nih.gov/articles/PMC9984160/.

Mota, Lucas, John N. Tomeo, Sai Divya Yadavalli, Andy Lee, Patric Liang, Allen D. Hamdan, Mark C. Wyers, Marc L. Schermerhorn, and Lars Stangenberg. “Management and Outcomes of Venous Thoracic Outlet Decompression: A Transition to the Infraclavicular Approach.” Journal of Vascular Surgery: Venous and Lymphatic Disorders 12, no. 6 (2024): 101959. https://doi.org/10.1016/j.jvsv.2024.101959. Link: https://pmc.ncbi.nlm.nih.gov/articles/PMC11523382/.

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