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True neurogenic thoracic outlet syndrome: late outcomes from a surgical series

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Abstract

Background

True neurogenic thoracic outlet syndrome (TNTOS) is rare, and evaluation of surgical treatment is limited to a few studies in the literature. The purpose of this study is to present the results from a surgical series of 21 patients with TNTOS.

Methods

Retrospective analysis on 21 patients diagnosed with TNTOS who underwent surgery. Demographic data and neurological status were characterized, and patients were classified in accordance with a pre-established scale for assessing the severity of hand impairment before and after surgery. Neuropathic pain was assessed using a visual analogue scale (VAS) and functional disability was quantified using the QuickDASH questionnaire. The results from before and after surgery were compared using the Wilcoxon test, and the significance level was taken to be 5%.

Results

There was a significant difference in VAS values from before to after the operation (Wilcoxon test: p = 0.0001; r = 0.86). Most patients (90%) improved after surgery, and in 85% of these patients, the VAS improvement was greater than 50%. Improvement in hand function occurred in seven patients (33.3%), and in most of these cases (28.6%), this improvement was classified as mild. Most patients (93.3%) showed moderate to very severe functional disability at the end of the follow-up.

Conclusion

After surgery, only one-third of the cases showed improvement in motor function and most patients had significant functional disability. However, the improvement regarding pain was significant. Surgery to control this symptom should be recommended, even in cases of late presentation and severe motor impairment.

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Abbreviations

DASH:

Disabilities of the Arm, Shoulder and Hand questionnaire

GSH:

Gilliatt-Sumner hand

HNRS:

Hand neurological rating scale

MRI:

Magnetic resonance imaging

NTOS:

Neurogenic thoracic outlet syndrome

TNTOS:

True neurogenic thoracic outlet syndrome

TOS:

Thoracic outlet syndrome

VAS:

Visual analogue scale

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Author information

Authors and Affiliations

  1. Peripheral Nerve Surgery Unit, Division of Neurosurgery, Clinic’s Hospital, University of São Paulo School of Medicine, São Paulo, SP, Brazil

    Roberto Sergio Martins, Monise Zaccariotto, Mario Gilberto Siqueira, Carlos Otto Heise, Hugo Sterman Neto & Manoel Jacobsen Teixeira

Authors
  1. Roberto Sergio Martins
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  2. Monise Zaccariotto
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  3. Mario Gilberto Siqueira
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  4. Carlos Otto Heise
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  5. Hugo Sterman Neto
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  6. Manoel Jacobsen Teixeira
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Corresponding author

Correspondence to Roberto Sergio Martins.

Ethics declarations

Ethics approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the University of São Paulo School of Medicine (research protocol number: 19653) and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Consent to participate

Surgical informed consent was obtained from all individual participants included in the study.

Competing interests

The authors declare no competing interests.

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Comments

With a reported prevalence of 1 in a million patients, true neurogenic thoracic outlet syndrome (TN-TOS) is a rare diagnosis1 . This article by Martins et al. adds importantly to our understanding the surgical management of TN-TOS, providing an excellent overview of the diagnostic work-up, intra-operative findings, and objective outcomes of pain and functional recovery with a supraclavicular approach and decompression without first rib resection.

The diagnosis of TN-TOS is underdiagnosed and underreported. The diagnosis is difficult to establish, especially when patients do not have pain. It is often misdiagnosed as cervical radiculopathy, inflammatory neuropathy, ulnar nerve compression, or severe carpal tunnel syndrome. A comprehensive work-up includes a thorough history, physical examination, electrodiagnostic studies (EDX), and radiographic and vascular imaging. Akin to the four legs of a table, the more positive findings in each of these domains, the more stable the table and certain the diagnosis. Patients commonly report a long history of vague and aching pain in the medial arm and ulnar aspect of the hand. Sensation is usually affected in the ulnar 2 digits; the radial 3 digits, derived from the lateral cord, is usually normal and a helpful differentiating factor between TN-TOS and carpal tunnel syndrome. Due to the often-late diagnosis, patients may exhibit intrinsic muscle atrophy, eponymously named as the Gilliat-Sumner hand2. Clinically, the thenar musculature (greater T1 compared to C8 innervation) is more severely affected compared to the ulnar innervated intrinsic muscles (equal C8 and T1 innervation)3. The T1 ventral rami are more inferiorly positioned and therefore more severely angulated and stretched, resulting in greater injury compared to C8 axons4. Electrodiagnostic features typically demonstrate evidence of axonal loss affecting the lower plexus (T1 more than C8)2. Imaging studies are useful in supporting the diagnosis. The presence of a cervical rib or an elongated C7 transverse process on plain radiographs or CT may be seen in these patients, but are also seen in a small percentage of the normal population (in our opinion, the bony variation changes the relationship of position of the overriding fibromuscular bands which leads to the compression). High resolution MRI may reveal hyperintensity of the lower trunk near the scalenes (but this may be subtle and only identified by experienced radiologists)5. Non-invasive vascular studies often show abnormalities with provocative testing, but must be considered in the right context as they are frequently seen in asymptomatic individuals as well.

Gilliatt’s 1970 report on 9 patients undergoing surgical exploration and division of fibrous band reported pain relief in 8 patients; however, minimal motor recovery and no improvement intrinsic atrophy even at 8 years post-operative2. Tender et al. reported on 33 patients with TN-TOS undergoing surgical decompression with more encouraging results: improvement in 21 of 22 patients with pain, 12 of 14 patients with mild motor deficits, and 14 of 20 patients with severe motor deficits6. Our institutional experience has been more similar to that reported in the current paper, with fairly good motor recovery in extrinsic muscle function but guarded expectations for significant motor recovery in hand intrinsic muscles. The primary goals of surgical intervention for TN-TOS are to alleviate pain, improve positional symptoms, and prevent further motor loss, and, as thoughtfully outlined by Martins et al., should be recommended to patients even with late presentation.

Kitty Y. Wu.

Robert J. Spinner.

Rochester, Minnesota.

References

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2. Gilliatt, R. W., Le Quesne, P. M., Logue, V. & Sumner, A. J. Wasting of the hand associated with a cervical rib or band. J. Neurol. Neurosurg. Psychiatry 33, 615-624 (1970).

3. Tsao, B. E., Ferrante, M. A., Wilbourn, A. J. & Shields, R. W. Electrodiagnostic features of true neurogenic thoracic outlet syndrome. Muscle Nerve 49, 724-727 (2014).

4. Ferrante, M. A. & Ferrante, N. D. The thoracic outlet syndromes: Part 1. Overview of the thoracic outlet syndromes and review of true neurogenic thoracic outlet syndrome. Muscle Nerve 55, 782-793 (2017).

5. Baumer, P. et al. Thoracic outlet syndrome in 3T MR neurography-fibrous bands causing discernible lesions of the lower brachial plexus. Eur. Radiol. 24, 756-761 (2014).

6. Tender, G. C. et al. Gilliatt-Sumner hand revisited: a 25-year experience. Neurosurgery 55, 883-890 (2004).

This article is part of the Topical Collection on Peripheral Nerves

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Martins, R.S., Zaccariotto, M., Siqueira, M.G. et al. True neurogenic thoracic outlet syndrome: late outcomes from a surgical series. Acta Neurochir 164, 2673–2681 (2022). https://doi.org/10.1007/s00701-022-05319-2

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