Pediatric thoracic outlet syndrome: a disorder with serious vascular complications

doi:10.1016/j.jpedsurg.2008.02.038

Journal of Pediatric Surgery

Volume 43, Issue 6, June 2008, Pages 1089-1094

https://doi.org/10.1016/j.jpedsurg.2008.02.038 Get rights and content

Abstract

Background

Thoracic outlet syndrome (TOS), caused by compression of the neurovascular structures between the clavicle and scalene muscles, typically presents with neurologic symptoms in adults. We reviewed our experience with 25 adolescents and propose a diagnostic/treatment algorithm for pediatric TOS.

Methods

From 1993 to 2005, 25 patients were treated with TOS. A retrospective chart review was performed with institutional review board approval. Demographics, clinical presentation, diagnostic studies, and treatment were evaluated.

Results

Seven male (28%) and 18 female (72%) patients presented between the ages of 12 to 18 years. Thirteen (52%) had vascular TOS (11 venous, 2 arterial), 11 (44%) had neurologic TOS, and 1 had both. Vascular TOS included subclavian vein thrombosis (7), venous impingement (4), and arterial impingement (2). Three patients had hypercoagulable disorders, and 6 had effort thrombosis. Venography was diagnostic in 10 cases. Neurogenic TOS was diagnosed by clinical symptoms. Five patients with subclavian vein thrombosis underwent thrombolysis, with 3 maintaining long-term patency. Of 25 patients, 24 underwent transaxillary first rib resection.

Conclusion

Vascular complications are more common in adolescents with TOS than in adults. A diagnostic/treatment algorithm includes urgent venography and thrombolysis for venous TOS and a workup for hypercoagulability. Neurogenic TOS is diagnosed clinically, whereas other studies are rarely beneficial.

Section snippets

Materials and methods

From 1993 to 2005, 25 patients (Fig. 1) were treated for TOS between the ages of 12 and 18 years (mean, 15.3 years). A retrospective chart review was performed with institutional review board approval (institutional review board study #2004E0118) that focused on patient risk factors, clinical presentation, diagnostic workup, and treatment.

Of 11 patients, 9 with venous TOS and one of the 2 arterial TOS patients had angiograms. Nine of these angiograms were available for independent review by a

Results

Of the 25 patients in our review, 11 (44%) had venous manifestations of TOS. The sex of these adolescents with venous TOS was almost equally divided, with 6 females (55%) and 5 males (45%). Seven patients (64%) had subclavian vein thrombosis with 6 of these patients having a history consistent with thrombosis related to physical activity. One female patient had a spontaneous thrombosis of unknown etiology. The other 4 patients (36%) presented with upper extremity swelling and pain and were

Discussion

Thoracic outlet syndrome is a well-established disease process in adults that manifests with neurologic symptomatology more than 90% of the time. By contrast, TOS has not been well studied in children. Our review of the literature found only 3 case series and 1 case report of children with TOS. The first review by Yang et al [1], in 1996, reported 4 cases of neurogenic TOS in the adolescent population. Swiercynska et al [2] also reported on 3 adolescents with TOS with primarily neurologic

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Venous thoracic outlet syndrome and Paget-Schroetter syndrome
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Venous thoracic outlet syndrome represents a relatively rare but important diagnosis in the adolescent population with increasing recognition. Compression of the subclavian vein within the costoclavicular space can lead to episodic venous outlet obstruction in the upper extremity, with edema, rubor and functional symptoms. Over time, cumulative injury and compression can lead to thrombosis of the vein, referred to as “effort thrombosis” or the Paget-Schroetter syndrome. This progression can lead to the need for acute management of the venous thromboembolism, requirement for thoracic outlet decompression surgery and the potential for long-term sequelae such as post-thrombotic syndrome. Management is focused on clot minimization, anticoagulation during the period of endothelial injury and inflammation and surgical decompression via first rib resection, anterior scalenectomy and venolysis to remove external compression of the vein. This manuscript reviews the diagnosis, evaluation and treatment of venous thoracic outlet syndrome and Paget-Schroetter syndrome.
Treatment-Related Outcomes in Paget–Schroetter Syndrome—A Cross-Sectional Investigation
2019, Journal of Pediatrics
To investigate treatment-related outcomes, namely radiological clot resolution, post-thrombotic syndrome (PTS), and health related quality-of-life (HRQoL) scores, in children with Paget–Schroetter syndrome (PSS) undergoing multidisciplinary management, including anticoagulation and decompressive rib-resection surgery, with or without thrombolytic therapy.
We identified all patients treated for PSS at our institution between the years 2010 and 2017. Baseline clinical and radiologic data were abstracted from medical records. Two validated survey instruments to quantify PTS and HRQoL were mailed to eligible patients. Standard statistical methods were used to summarize these measures.
In total, 22 eligible patients were identified; 10 were treated with thrombolysis followed by anticoagulation and rib resection, and 12 were treated with anticoagulation and rib resection alone. Nineteen patients responded to the survey instruments. Median age at deep vein thrombosis diagnosis and survey completion were 16.3 and 20.4 years, respectively. Nineteen of 22 patients had thrombus resolution on radiologic follow-up. Fourteen of 19 survey respondents reported signs/symptoms of PTS of which the majority (12/14) reported mild PTS. Aggregate total, physical, and psychosocial HRQoL scores reported were 90.6, 96.7, and 93.3, respectively. Thrombolytic therapy was not associated with a significant improvement in radiologic, clinical or HRQoL outcomes.
Most patients with PSS had complete thrombus resolution on imaging. Only 11% of survey respondents reported moderate PTS. The entire cohort reported excellent HRQoL scores. The role for thrombolytic therapy in the management of childhood PSS remains incompletely elucidated.
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.
Paget-Schroetter syndrome in 21 children: Outcomes after multidisciplinary care
2015, Journal of Pediatrics
Citation Excerpt :
This study aimed to review the presentation, treatment, and outcomes in a cohort of 21 children with PSS and to present a unified, multidisciplinary treatment algorithm developed for all patients with PSS at our pediatric institution, regardless of referral stream. Few investigations have specifically examined children with PSS.7-9,15 With the peak incidence of this type of thoracic outlet syndrome occurring during the second and third decades of life, many larger series include only small numbers of adolescents.5,6,12,19-25
To review the presentation, management, and outcomes of Paget-Schroetter syndrome (PSS) in children and propose a multidisciplinary treatment algorithm involving pediatric and vascular surgery, interventional radiology, and hematology.
Patients with PSS presenting between 2003 and 2013 were reviewed. Demographics, symptoms, therapies, and functional outcomes were noted. Data from early patients informed the development of a multidisciplinary treatment algorithm applied to later patients.
Of 21 patients, mean ± SD age was 16 ± 1.6 years and 11 (52%) were male. Of patients with complete presentation data, common symptoms were edema (84%), discoloration (58%), and pain (58%). Thrombophilia workup revealed one heterozygote for factor V Leiden, 2 patients with factor VIII elevation and 1 patient with mildly low antithrombin. The most recent 8 patients were treated according to an algorithm developed by a multidisciplinary working group through experience with the first 13 cases. All patients underwent a venogram, endovascular intervention (including 15 receiving catheter-directed thrombolysis), and operative ipsilateral thoracic outlet decompression (first rib resection, anterior scalenectomy, and venolysis). Postoperative complications included hemothorax (2), pneumothorax (1), and recurrent thrombosis (2). Follow up duration was 12 ± 9.5 months. Symptoms recurred transiently in 1 patient.
Pediatric patients with PSS can be treated successfully using a multidisciplinary treatment algorithm including anticoagulation, catheter-directed thrombolysis, and operative decompression of the thoracic outlet. Early outcomes are promising.
Thoracic outlet syndrome in the pediatric population: Case series
2014, Journal of Hand Surgery
Citation Excerpt :
In children, however, the incidence of vascular TOS is much higher. In a review of published cases of pediatric TOS (Appendix A, available on the Journal's Web site at www.jhandsurg.org), there were 76 patients (53 female and 23 male) with ages ranging between 6 and 20 years.1–16 A total of 52% (n = 40) of all patients had vascular TOS.
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
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: Presented at the 59th Annual Meeting of the Section on Surgery, American Academy of Pediatrics, San Francisco, CA, October 25-27, 2007.

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Pediatric thoracic outlet syndrome: a disorder with serious vascular complications

Abstract

Background

Methods

Results

Conclusion

Section snippets

Materials and methods

Results

Discussion

J Pediatr Surg

J Pediatr Surg

J Vasc Surg

Ann Vasc Surg

Thoracic outlet syndrome in children

J Pediatr Orthop