Surgery for paediatric thoracic outlet syndrome

doi:10.1016/j.jocn.2011.05.015

Journal of Clinical Neuroscience

Volume 19, Issue 2, February 2012, Pages 235-240

https://doi.org/10.1016/j.jocn.2011.05.015 Get rights and content

Abstract

The effectiveness of operative treatment of paediatric thoracic outlet syndrome (TOS) has been analysed, and an attempt made to improve the definition of the condition in terms of presentation, aetiology and diagnosis. A retrospective review of postoperative pain, functional capability and overall outcome was carried out on 13 patients (<18 years) treated by a single surgeon. In 20 operations, 17 were scalenotomies, and three were transaxillary rib resections (TARRs). Follow-up was 6–96 months post-operatively. Surgery alleviated many TOS symptoms, especially vascular compromise, although pain resolution was inconsistent and that of motor deficit poor. Mean functional improvement was good, and overall operative outcomes excellent. Therefore, surgery was successful for paediatric TOS in this series. Anatomical anomalies and sport participation may be related to early onset of TOS in many paediatric patients.

Introduction

The thoracic outlet syndrome (TOS),¹ characterised by pain, paraesthesia, and motor deficits in the upper limb which are aggravated by elevation of the arms or by exaggerated movements of the head and neck,² is related to compression of elements of the brachial plexus or subclavian vessels as they pass in close relation to the first rib into the upper limb. Because identification of TOS as vascular or neurological³ can be difficult, with patients often showing symptoms characteristic of both,⁴ the condition has been surrounded by controversy,⁵ some believing it to be a much overlooked and misdiagnosed condition6, 7 whilst others question its existence.⁸ Diagnostic tests used are: (i) the elevated arm stress test (EAST) in which the patient keeps the arms elevated with the elbows flexed, the test being positive if the patient is unable to flex and extend the fingers for 3 minutes because of pain or weakness; and (ii) Adson’s and Allen’s test, in which the radial pulse is examined whilst the patient tilts the head back contralaterally and either takes a deep breath (Adson’s) or raises the affected arm (Allen’s). Electrophysiological tests and imaging modalities are, however, often normal.9, 10

Though a recent review found no accepted diagnostic criteria for TOS and no randomized evidence to support surgical intervention,¹¹ TOS continues to be diagnosed, with a reported prevalence of 10 per 100,000.¹² The relative paucity of reports of paediatric TOS makes the condition in this age group particularly contentious.13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23 The few studies of TOS in paediatric patients that have been reported in the literature are summarized in Table 1.

The anatomical area of interest, extending from the inter-scalene triangle medially to the pectoralis minor insertion laterally, contains three parts important to the syndrome’s purported aetiology, that is, the scalene triangle, the costoclavicular region, and the subcoracoid tunnel. The tight anatomical configuration of the neurovascular structures at these three sites may make them vulnerable to anatomical impingement.

Congenital anatomical variation is the most obvious form of impingement. A large cervical rib can protrude into the scalene triangle, but the protrusion is often an enlarged C7 transverse process with a soft-tissue extension (cervical band) inserting at the first (thoracic) rib. Both bone and its soft tissue analogue can compress the lower trunk and nerve roots of the brachial plexus. Malformations of the upper thoracic ribs can also cause compression.

The significance of soft-tissue compressive factors remains controversial as they are found only on surgical exploration. Clavicular fractures and poor posture have been postulated to cause compression in the costoclavicular region by wedging the brachial plexus and vessels against the first rib. In addition to an anatomical factor, an environmental trigger, such as faulty posture, a depressed shoulder, acute trauma, cumulative stress, or pre-existing musculo-skeletal ailments, may be required for TOS to develop.²⁴ Indeed, as people with normal anatomy may apparently develop TOS,²⁵ an anatomical anomaly may sometimes be unnecessary.

We report a retrospective observational analysis of a series of cases of paediatric TOS with the aim of examining the efficacy of surgery for this condition, and clarifying aspects of its presentation, aetiology and diagnosis.

Section snippets

Methods

The study was conducted on a single-surgeon series (DW). Medical records and interactive electronic questionnaire results were analysed. The study was approved by the Ethics in Human Research Committee of the Royal Children’s Hospital, Melbourne, Australia (EHRC 26166 B&C). The two primary inclusion criteria were: age <18 years at first presentation; and operative management.

Results

Over the period 1997–2007, 13 patients presented (10 female, 3 male; 9–17 years, mean 14.4 years). Follow-up was between 9 and 25 months from the first consultation (mean 34 months) and 6 to 96 months from the time of operation (mean = 34 months). The participants and the operative procedures are summarized in Table 2.

Discussion

This study, though retrospective and lacking a conservatively-managed comparison group, represents the largest operated group of paediatric TOS patients reported to date. It made use of single-surgeon data, but unlike most surgical TOS studies did not involve the operating surgeon in the assessment, analysis and reporting of the outcome. The patient characteristics were similar to those of previously reported series, including female preponderance, widely variable symptomatology, and the common

Conclusion

Surgery was used successfully to treat TOS in the paediatric patients in our series. Determining the usefulness of surgery in its treatment is, however, limited by the variability of clinical presentation and the unreliability of diagnostic and follow-up investigation. These considerations, coupled with our relatively small sample size, mean we cannot unequivocally validate the procedure or the suggestion that the presence of anatomical anomalies and participation in sport may contribute to the

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Cited by (13)

Surgical treatment of thoracic outlet syndrome in pediatrics
2022, Journal of Pediatric Surgery
Citation Excerpt :
However, the role and extent of bone resection in surgical treatment for TOS is debatable. One series reported that more complications were associated with bone resection [23], leading some surgeons to advocate for soft-tissue resection alone, even in cases with cervical ribs [24]. In a series of 54 adolescents, in which first rib resection was performed in 28, Ransom found no difference in outcomes of patients treated with rib resection versus those in whom it was left intact [25].
Thoracic outlet syndrome (TOS) is a condition that occurs when the cervical neuro-vascular bundle becomes compressed at one of the three narrow areas of the thoraco-cervico-axillary region. Conservative management is the first line of treatment. Patients who do not respond to conservative management should be treated surgically. The aim of this review is to present our experience with the surgical management of TOS in pediatric patients.
We retrospectively reviewed the outcomes of all patients with TOS operated at our Hospital between 2001 and 2020. We collected all demographic data, clinical features, imaging data, type of operation performed, intraoperative findings, complications and recurrence.
We operated 9 patients within the study period. The median age at surgery was 14 (7 to 17) years. A transaxillary approach was used in 7 patients and a supraclavicular approach in 2. There was only one minor intraoperative complication (violation of the pleural space). There were no postoperative complications. The median length of stay was 3 (2 to 4) days. All patients were extubated in the operating room. Two patients developed symptoms on the contralateral side. One of these underwent a successful contralateral transaxillary Roos operation. The follow-up was 4 months to 20 years. All patients are asymptomatic.
We believe that the Roos operation is a safe and effective treatment with excellent long-term outcomes for children with TOS that fail conservative management.
Cervical Rib Prevalence and its Association with Thoracic Outlet Syndrome: A Meta-Analysis of 141 Studies with Surgical Considerations
2018, World Neurosurgery
Cervical ribs (CR) are supernumerary ribs that arise from the seventh cervical vertebra. In the presence of CR, the boundaries of the interscalene triangle can be further constricted and result in neurovascular compression and thoracic outlet syndrome (TOS). The aim of our study was to provide a comprehensive evidence-based assessment of CR prevalence and their association with TOS as well as surgical approach to excision of CR and surgical patients' characteristics.
A thorough search of major electronic databases was conducted to identify any relevant studies. Data on the prevalence, laterality, and side of CR were extracted from the eligible studies for both healthy individuals and patients with TOS. Data on the type of TOS and surgical approach to excision of CR were extracted as well.
A total of 141 studies (n = 77,924 participants) were included into the meta-analysis. CR was significantly more prevalent in patients with TOS than in healthy individuals, with pooled prevalence estimates of 29.5% and 1.1%, respectively. More than half of the patients had unilateral CR in both the healthy and the TOS group. The analysis showed that 51.3% of the symptomatic patients with CR had vascular TOS, and 48.7% had neurogenic TOS. Most CR were surgically excised in women using a supraclavicular approach.
CR ribs are frequent findings in patients with TOS. We recommended counseling asymptomatic patients with incidentally discovered CR on the symptoms of TOS, so that if symptoms develop, the patients can undergo prompt and appropriate workup and treatment.
Radiculopathies and Plexopathies
2015, Neuromuscular Disorders of Infancy, Childhood, and Adolescence: A Clinician's Approach
Radiculopathies are very rare in childhood and adolescence. Most pediatric radiculopathies predominantly involve the lumbosacral nerve roots. Cervical radiculopathies are uncommon, but other conditions, such as congenital spinal stenosis, can mimic nerve root lesions in the cervical spine. Plexopathies are more common than radiculopathies in children. Neonatal brachial plexus palsy continues to be a challenge in the setting of complicated labor and childbirth. Most brachial and lumbar plexopathies in older children result from trauma, although occasionally cases are familial (caused by hereditary neuralgic amyotrophy or hereditary neuropathy with a tendency to pressure palsies) or postinfectious.
Thoracic outlet syndrome in the pediatric population: Case series
2014, Journal of Hand Surgery
Citation Excerpt :
Our series supports these findings: 3 patients had cervical ribs and 1 had an anomalous first rib. Maneuvers such as the Roos elevated arm stress test, Adson test, Wright test, costoclavicular compression, and neck tilting have poor predictive value in diagnosing TOS.1 Adjunctive studies such as electromyography, nerve conduction velocities, and all imaging modalities have not been shown to be helpful.
We present 4 patients, 4 months to 10 years of age, with thoracic outlet syndrome. All were referred to the brachial plexus clinic. Three patients were diagnosed with vascular thoracic outlet syndrome after clinical evaluation and diagnostic imaging. Three had a cervical rib and 1 had an anomalous first rib. All patients were treated surgically through a supraclavicular approach and had resolution of the symptoms. No postoperative complications were noted.
Clinical presentation of cervical ribs in the pediatric population
2013, Journal of Pediatrics
Supraclavicular decompression for neurogenic thoracic outlet syndrome in adolescent and adult populations
2013, Journal of Vascular Surgery
Citation Excerpt :
This reinforces the notion that surgical treatment of NTOS is effective in the adolescent age group and helps to identify clinical factors that may be associated with more favorable outcomes in other patients. Several reports have been published concerning TOS in pediatric and adolescent patients.18-23 Although these studies have provided valuable insights, the relatively low numbers of patients with NTOS prevents meaningful conclusions to be drawn regarding optimal management of this condition in children and young adults.
This study was conducted to better define clinical results and understand factors determining responsiveness to surgical treatment for neurogenic thoracic outlet syndrome (NTOS) in adolescent and adult populations.
A retrospective review was conducted for 189 patients with disabling NTOS who underwent primary supraclavicular decompression (scalenectomy, brachial plexus neurolysis and first rib resection, with or without pectoralis minor tenotomy) from April 2008 to December 2010. Clinical characteristics were compared between 35 adolescent patients (aged <21 years) and 154 adults (aged >21 years). Functional outcome measures were assessed before surgery and at 3- and 6-month follow-up using a composite NTOS Index combining the Disabilities of the Arm, Shoulder and Hand (DASH) survey, the Cervical-Brachial Symptom Questionnaire (CBSQ), and a 10-point visual analog scale (VAS) for pain.
Adolescent and adult patients were not significantly different with respect to sex (overall 72.5% female), side affected (58.7% right, 60.3% dominant limb), bony anomalies (23.3%), previous injury (55.6%), coexisting pain disorders (11.1%), and positive responses to scalene muscle anesthetic blocks (95.6%). Compared with adults, adolescent patients had a significantly (P < .05) lower incidence of depression (11.4% vs 41.6%), motor vehicle injury (5.7% vs 20.1%), previous operations (11.4% vs 29.9%), preoperative use of opiate medications (17.1% vs 44.8%), and symptom duration >2 years (24.2% vs 50.0%). Mean preoperative NTOS Index (scale 0-100) was significantly lower in adolescent vs adult patients (46.5 ± 3.6 vs 58.5 ± 1.7; P = .009), and hospital length of stay was 4.4 ± 0.2 vs 4.9 ± 0.1 days (P = .03), but the rate of postoperative complications was no different (overall, 4.2%). Although both groups exhibited significant improvement in functional outcome measures at 3 and 6 months, adolescent patients had significantly lower NTOS Index (10.4 ± 3.1 vs 39.3 ± 3.3; P < .001) and use of opiate medications (11.4% vs 47.4%; P < .001) compared with adults.
Adolescents undergoing supraclavicular decompression for NTOS had more favorable preoperative characteristics and enhanced 3-month and 6-month functional outcomes than adults. Further study is needed to delineate the age-dependent and independent factors that promote optimal surgical outcomes for NTOS.

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Clinical StudySurgery for paediatric thoracic outlet syndrome

Abstract

Introduction

Section snippets

Methods

Results

Discussion

Conclusion

Am J Surg

Orthop Clin North Am

Man Ther

Electroencenpalogr Clin Neurophysiol

J Pediatr Surg

J Paediatr Surg

J Orthop Sci

Eur J Vasc Endovasc Surg

Ann Vasc Surg