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Table of Contents    
BRIEF REPORT
Year : 2021  |  Volume : 69  |  Issue : 6  |  Page : 1777-1780

A Rare Case of Stroke in an Adolescent Violinist Due to Thoracic Outlet Syndrome


1 Department of Pediatrics, Division of Pediatric Hematology-Oncology, Shawn Jenkins Children's Hospital, Medical University of South Carolina, Charleston, SC, United States
2 Department of Pediatrics, Division of Pediatric Neurology, Shawn Jenkins Children's Hospital; Department of Neurology, Medical University of South Carolina, Charleston, SC, United States
3 Department of Pediatrics, Division of Pediatric Neurology, Shawn Jenkins Children's Hospital, Medical University of South Carolina, Charleston, SC, United States

Date of Submission16-Jan-2021
Date of Decision06-Jul-2021
Date of Acceptance12-Jul-2021
Date of Web Publication23-Dec-2021

Correspondence Address:
Dr. Sonal Bhatia
Department of Pediatrics, Division of Pediatric Neurology, Shawn Jenkins Children's Hospital, 125 Doughty Street, Suite 550 E, Charleston, SC 29425
United States
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.333506

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 » Abstract 


Thoracic outlet syndrome (TOS), a rare condition, results from the compression of neurovascular structures traversing from the neck through the thoracic outlet into the axilla. It can develop from chronic repetitive activities of the upper extremities, commonly reported in athletes playing sports involving vigorous use of arms and shoulders. While symptoms of neurovascular compression can occur, stroke due to TOS in children is not commonly reported. We describe a rare case of a healthy 14-year-old boy, a competitive violinist, with acute limb ischemia from extensive occlusive thrombi involving several arteries in the right upper extremity as well as the right vertebral artery, which ultimately caused infarcts in the bilateral posterior circulation. The etiology was determined to be TOS leading to impingement of the right subclavian artery by a fused cervical rib aggravated by patient's prolonged violin practice. This case represents the first description of stroke from TOS in an adolescent violinist.


Keywords: Adolescent, stroke, thoracic outlet syndrome, violinist
Key Message: Consider TOS as a differential diagnosis of unexplained upper extremity ischemia and posterior circulation strokes in a child and enquire about the use of string instruments such as a violin or indulgence in competitive sports involving strenuous use of arms.


How to cite this article:
Kuril S, Chopade PR, Mandava M, Bhatia S. A Rare Case of Stroke in an Adolescent Violinist Due to Thoracic Outlet Syndrome. Neurol India 2021;69:1777-80

How to cite this URL:
Kuril S, Chopade PR, Mandava M, Bhatia S. A Rare Case of Stroke in an Adolescent Violinist Due to Thoracic Outlet Syndrome. Neurol India [serial online] 2021 [cited 2022 Jan 18];69:1777-80. Available from: https://www.neurologyindia.com/text.asp?2021/69/6/1777/333506




Thoracic outlet syndrome (TOS) results from compression of neurovascular structures as they exit the thorax. It is more prevalent in women, often of young age with poor musculoskeletal development and/or poor posture. Symptoms from TOS are often related to arm position and can be aggravated by sustained activities involving the arms and the shoulder girdles – therefore, athletes or those participating in sports are usually susceptible. Interestingly, it also a common occurrence in those involved in the use of high-string instruments such as violinists. Compression of the subclavian artery usually results from a bony structure such as cervical rib and is rarely associated with thromboembolic strokes – especially in the pediatric population.[1],[2],[3],[4],[5] Herein, we present a unique and hitherto unreported case of a pediatric patient who developed both acute right upper extremity (RUE) ischemia and infarcts involving the bilateral posterior circulation. He required extensive surgical intervention to relieve the compression of the subclavian artery with right cervical rib resection. The patient in this report was not an athlete but a competitive violinist who was practicing vigorously, approximately four hours a day for a few years.


 » Case Report Top


A 14-year-old, right-handed, Caucasian male with past medical history of migraines, but otherwise healthy, presented with severe, acute onset, generalized, throbbing headache, preceded by an aura of a scintillating scotoma. Headache was associated with transient pain and weakness in upper extremities and right lower extremity, slurred speech and gait abnormalities lasting for 30 min. After a dose of oral analgesia headache subsided. Two hours later, he developed acute onset, severe, throbbing, RUE pain with duskiness of the right forearm, upon which he seeked medical attention. On admission, the patient was hemodynamically stable. On physical exam, he had no palpable pulsation of the right radial, ulnar, and brachial arteries. Right forearm was tender on palpation and mottled, but he had intact motor and sensory function. He was oriented in time, place, and person, had no cranial or focal neurological deficits on examination, and was normoreflexic with no evidence of ataxia or dysmetria.

RUE ultrasound showed a multifocal acute occlusive thrombus extending distally from the right subclavian artery to the right axillary, brachial, radial, and ulnar arteries. A computed tomography angiogram (CTA) of the RUE corresponded with findings of RUE ultrasound [Figure 1] and [Figure 2]. Computed tomography head showed hypodensities involving bilateral cerebellar lobes. Magnetic resonance imaging (MRI) brain with and without contrast confirmed multiple ischemic infarcts in the bilateral cerebellar and occipital lobes as well as the left thalamus [Figure 3]. CTA of head and neck revealed right intracranial vertebral artery occlusion, with collateral flow from the left vertebral artery and anterior circulation. Thrombolysis with tissue plasminogen activator was not recommended due to onset of symptoms greater than four and half hours. Initial laboratory work-up revealed normal complete blood count, serum electrolytes, renal and hepatic function, coagulation panel, lipid and thyroid profile, glycosylated HbA1c (5.2%, normal 4.6–5.6%), vitamin B12 (511, normal 211–911 pg/mL), erythrocyte sedimentation rate (2 mm/h), C-reactive protein (0.58, normal 0.0–1.0 mg/dL), negative antineutrophil and antineutrophilic cytoplasmic antibodies, and normal creatinine kinase (85, normal 30–260 U/L). Electrocardiogram and echocardiogram were normal. Transcranial Doppler study showed no evidence of microemboli in the basilar artery. Bilateral lower extremity and left upper extremity Doppler studies did not show any evidence of deep vein thromboses.
Figure 1: CT angiogram with evidence of right subclavian occlusion (arrow)

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Figure 2: RUE CT angiogram with evidence of right subclavian occlusion in the region of the cervical rib and occlusion/severe stenosis of axillary, brachial, and radial arteries

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Figure 3: (a and b): Diffusion-weighted brain MRI with evidence of bilateral, acute ischemic infarcts involving the posterior circulation

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The patient was an avid violinist and practiced playing violin for up to 4 hours daily for the past few years, using the bow in his right hand. Since he started playing violin, approximately 4 years before the acute event, patient noted occasional numbness and tingling in his fingers bilaterally sometimes associated with transient color changes at the finger tips; this was attributed to possible Raynaud's phenomenon. He had no prior history of clots, embolic events, drug usage, and was not on any medications.

On admission, the patient immediately underwent decompression fasciotomy of extensor and flexor compartment of right forearm and wrist along with carpel tunnel release without any debridement as all the forearm muscles were viable. Simultaneously, open thrombectomy of right subclavian, axillary, and ulnar arteries were performed. Postthrombectomy, anticoagulation with unfractionated heparin, and aspirin were started with a goal-activated partial thromboplastin time of 65–100. After the procedure, perfusion and color in his right hand improved; but 3 days later, suddenly he was noticed to have a cold and clammy right hand. To further assess the bony anatomy, a chest radiograph was done which revealed bilateral cervical ribs. On correlation with the initial CTA of the neck, impingement of bilateral subclavian arteries and a proximal right subclavian aneurysm was found. He subsequently underwent immediate resection of a right cervical rib, right subclavian aneurysm repair with subclavian-to-axillary artery bypass with prosthesis, and a repeat thrombectomy upon which he had complete resolution of pain with emergence of strong distal pulses in the RUE.

Subsequent thrombophilia work-up with serum homocysteine, antithrombin, cardiolipin, beta-2-glycoprotein, and lupus anticoagulant antibodies; protein C and S, Dilute Russell's Viper Venom Time, and Factor V Leiden levels were negative. He was found to have clinically insignificant isolated heterozygous methylenetetrahydrofolate reductase mutation. The patient was discharged with continued anticoagulation with low-molecular-weight heparin for 3 months and antiplatelet therapy with aspirin 81 mg daily. The resection of the asymptomatic left cervical rib was scheduled for a later date to prevent recurrence of symptoms and potentially cerebral infarcts.


 » Discussion Top


Thoracic outlet boundaries are defined by the scalene muscles, first rib, and the clavicle.[5] Any bony or soft tissue structural abnormalities within this outlet can compress various neurovascular structures resulting in symptoms. These include anomalous cervical ribs, hypoplastic first thoracic ribs, exostoses (bony outgrowths) of first ribs or clavicle, malformations or misalignment of the clavicle, hypertrophic muscles – especially scalenes, anomalous fibrous muscular bands, and space-occupying lesions.[6] Activities that require repetitive movements resulting in prolonged or extreme abduction and external rotation of the shoulders are common risk factors; these include sports such as competitive swimming, especially butterfly stroke and backstroke, baseball, tennis, and archery as well as playing high-string musical instruments.[7],[8] Variable presentations of TOS have been noted based on whether the compression is primarily neurogenic or vascular (venous or arterial), or both. Symptoms resulting from neurogenic compression are painless atrophy of intrinsic muscles of the hand, sensory loss or paresthesias, and occasionally mild pain. Common symptoms resulting from vascular compression are upper extremity swelling, diffuse arm or forearm pain, loss of pulse, and color change leading to limb ischemia.[9] In most instances, a combination of vascular and neurogenic symptoms is present.

Arterial TOS is the least common form of TOS and is reported in only 1–3% of patients.[10] Stroke as a direct result of arterial TOS is therefore even rare, especially in children.[11] [Table 1] illustrates cases of pediatric posterior circulation stroke due to TOS. Ischemia involving the bilateral posterior circulation secondary to arterial TOS with symptom exacerbation by competitive violin playing in the pediatric age group is hitherto unreported.[11],[12],[13],[14]
Table 1: Clinical summary of reported cases of posterior circulation stroke due to TOS in children

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In our patient, the etiology for these infarcts as well as associated symptoms of limb ischemia due to RUE thrombosis was determined to be TOS with impingement of right subclavian artery by a fused cervical rib, which was compounded by prolonged violin practice. In light of patient's chronic, transient symptoms of numbness and tingling in bilateral fingers with associated worsening acrocyanosis with violin practice, there was a concern for chronic impingement of the RUE vasculature due to prolonged arm abduction to 90° during violin practice. It is likely that the right arm arterial thrombosis originated at the point where the subclavian artery was being impinged by the right cervical rib, which was consistent with TOS. Infarctions on MRI clearly pointed to an embolic process. A probable mechanism of the limb ischemia was embolization of an intramural clot from a subclavian artery aneurysm or because of thrombosis of the subclavian artery aneurysm itself. The clot in the subclavian artery can have a retrograde propagation extending toward the origin of the vertebral artery and embolize via the posterior circulation resulting in strokes.

The low prevalence of ischemic infarcts in pediatric patients necessitates a thorough work-up to determine the etiology. In patients with embolic infarcts, the possibility of TOS causing arterial thrombi and retrograde embolization should be considered. This is especially true in patients who undertake activities that require prolonged immobilization of upper extremities at acute angles such as playing musical instruments or sports that involve extreme hyperactivity of upper extremities.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 » References Top

1.
Vemuri C, McLaughlin LN, Abuirqeba AA, Thompson RW. Clinical presentation and management of arterial thoracic outlet syndrome. J Vasc Surg 2017;65:1429-39.  Back to cited text no. 1
    
2.
Strzelecka J, Skadorwa T, Franckiewicz M, Jóźwiak S. A case of symmetric retrograde thromboembolic cerebral infarction in an 8-year-old child due to arterial thoracic outlet syndrome. Childs Nerv Syst 2018;34:2503-7.  Back to cited text no. 2
    
3.
Smith KF. The thoracic outlet syndrome: A protocol of treatment. J Orthop Sports Phys Ther 1979;1:89-99.  Back to cited text no. 3
    
4.
Stewman C, Vitanzo PC Jr, Harwood MI. Neurologic thoracic outlet syndrome: Summarizing a complex history and evolution. Curr Sports Med Rep 2014;13:100-6.  Back to cited text no. 4
    
5.
Meumann EM, Chuen J, Fitt G, Perchyonok Y, Pond F, Dewey HM. Thromboembolic stroke associated with thoracic outlet syndrome. J Clin Neurosci 2014;21:886-9.  Back to cited text no. 5
    
6.
Rivera-Vega A, Peguero L, Colon E, Saavedra FM, Sosa I, Ramos E. Fibrotendinous band causing neurogenic Thoracic Outlet syndrome in adolescent with bilateral cervical ribs. J Pediatr Rehabil Med 2011;4:149-54.  Back to cited text no. 6
    
7.
Chandra V, Little C, Lee JT. Thoracic outlet syndrome in high-performance athletes. J Vasc Surg 2014;60:1012-8.  Back to cited text no. 7
    
8.
Demaree CJ, Wang K, Lin PH. Thoracic outlet syndrome affecting high-performance musicians playing bowed string instruments. Vascular 2017;25:329-32.  Back to cited text no. 8
    
9.
Ohman JW, Thompson RW. Thoracic outlet syndrome in the overhead athlete: Diagnosis and treatment recommendations. Curr Rev Musculoskelet Med 2020;13:457-71.  Back to cited text no. 9
    
10.
De Mooij T, Duncan AA, Kakar S. Vascular injuries in the upper extremity in athletes. Hand Clinics 2015;31:39-52.  Back to cited text no. 10
    
11.
Mackay MT, Prabhu SP, Coleman L. Childhood posterior circulation arterial ischemic stroke. Stroke 2010;41:2201-09.  Back to cited text no. 11
    
12.
Bains RD, Platt J, MacGregor D, Borschel GH. Atypical thoracic outlet syndrome and reverse flow thromboembolism. Pediatr Neurol 2014;51:453-6.  Back to cited text no. 12
    
13.
Kataria R, Sharma A, Srivastava T, Bagaria H, Sharma A. Cervical Rib, a rare cause of recurrent stroke in the young. Neurologist 2012;18:321-3.  Back to cited text no. 13
    
14.
Blank RH, Connar RG. Arterial complications associated with thoracic outlet compression syndrome. Ann Thorac Surg 1974;17:315-24.  Back to cited text no. 14
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
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