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CASE REPORT
Year : 2019  |  Volume : 22  |  Issue : 7  |  Page : 1022-1025

Paget–Schroetter syndrome in a teenager after throwing firecrackers – A case report


1 Department Pediatrics I, University of Medicine and Pharmacy, Iuliu Hatieganu, Cluj-Napoca, Romania
2 Department of Radiology and Imaging, Emergency Clinic Pediatric Hospital, Cluj-Napoca, Romania

Date of Acceptance04-Feb-2019
Date of Web Publication11-Jul-2019

Correspondence Address:
Dr. C Lazea
Department Pediatrics I, University of Medicine and Pharmacy Iuliu Hatieganu Cluj-Napoca, 68, Motilor Street, 400370 Cluj-Napoca
Romania
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/njcp.njcp_230_18

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   Abstract 


Paget–Schroetter syndrome (PSS), or effort thrombosis, refers to axillary and/or subclavian vein thrombosis associated with repetitive effort of the superior limbs, and is rare in the pediatric population. We report the case of a previously healthy 15-year-old boy who presented with a painful and swollen right arm after throwing firecrackers. Doppler ultrasound showed extensive right subclavian and axillary vein thrombosis. Anticoagulation therapy was started and had favorable evolution. We emphasize that PSS must be included in the differential diagnosis of a swollen arm.

Keywords: Children, effort thrombosis, paget-Schroetter syndrome


How to cite this article:
Lazea C, Asavoaie C. Paget–Schroetter syndrome in a teenager after throwing firecrackers – A case report. Niger J Clin Pract 2019;22:1022-5

How to cite this URL:
Lazea C, Asavoaie C. Paget–Schroetter syndrome in a teenager after throwing firecrackers – A case report. Niger J Clin Pract [serial online] 2019 [cited 2019 Jul 20];22:1022-5. Available from: http://www.njcponline.com/text.asp?2019/22/7/1022/262515




   Introduction Top


Paget–Schroetter syndrome (PSS), or effort thrombosis, refers to subclavian and/or axillary vein thrombosis and is a rare condition with an incidence of 1 to 2 per 100,000 people per year in Europe, accounting for approximately 1% to 4% of all episodes of venous thrombosis.[1],[2],[3],[4] It usually develops in apparently healthy young adults (mean age = 23 years, range = 9-76) who perform repetitive movements with their dominant arm during sporting activities (wrestling, weightlifting, swimming, rowing, surfing, playing ball, racket games, gymnastics, martial arts, and javelin throwing) or during different jobs (driving, auto repair, cutting, guitar playing, and shooting).[1],[2],[4] No racial or genetic predisposition has been demonstrated. The subclavian vein passes through the anterior part of the thoracic outlet between the costoclavicular ligament and the junction of the first rib and the clavicle. These repetitive movements (hyperabduction, extension, and retroversion) cause straining of the subclavian vein with microtrauma of the endothelium and coagulation cascade activation. Unrecognized, over time, progressive injury of the vein causes endothelial inflammation, fibrosis, and intermittent venous obstruction with clot formation. Another mechanism is represented by scalene muscle hypertrophy, which also appears after repetitive movements of the upper limb and causes compression of the subclavian vein between the ribs and clavicle. Other predisposing factors for PSS include anatomical abnormalities such as cervical rib, congenital bands, hypertrophy of scalenus tendons, and abnormalities of the insertion of the costoclavicular ligament.[1],[2] Smoking and oral contraceptives have been also demonstrated as risk factors.[5] The role of thrombophilic disorders in the development and progression of effort thrombosis is unclear. Undiagnosed PSS can lead to a poor prognosis with respect to pulmonary thromboembolism and post-thrombotic syndrome.[1],[2]

We herein describe a rare case of PSS in a 15-year-old male after throwing firecrackers, and we also present the characteristic features and current management of this disease.


   Case Report Top


A 15-year-old male was admitted into pediatric cardiology department with a 5-week history of painful and swollen right arm and forearm. He had no history of chronic diseases, fever, or rash; no previous medication, surgery, or allergy; and no history of similar episodes. Family history revealed mother's repeated miscarriages.

The patient described a swollen and painful right arm (his dominant upper limb) a day after throwing firecrackers. His family doctor recommended oral non-steroidal anti-inflammatory agent with improvement in arm mobility and pain. He continued treatment and massage for another 2 weeks.

On admission, the right arm was subtly swollen, and the ranges of motion were normal. The capillary refill, radial pulses, blood pressure, and deep tendon reflexes were normal. Thoracic outlet syndrome was diagnosed, and arterial and nerve impingement at the thoracic outlet were excluded after careful clinical evaluation. Clinical history excluded recent trauma or surgery to the right upper limb, central catheter placement, or malignant disorders. The patient has no previous episodes of swelling to suggest lymphedema. The blood count, hepatic function, and renal function were normal [Table 1].
Table 1: Hematological and coagulation parameters at diagnosis

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Normal inflammatory tests excluded rheumatologic musculoskeletal disorders or upper limb soft tissue infections, and chest and right shoulder radiograph excluded thoracic or upper limb bone abnormality. The color Doppler ultrasonography showed complete axillary vein thrombosis [Figure 1], subclavian vein thrombosis (proximal part), and extension into the brachial vein (medial and distal part) [Figure 2]. The standard hypercoagulability workup showed that the patient was negative for lupus anticoagulant, and levels of homocysteine, antithrombin, protein C, and protein S were normal. He carries factor V Leiden G1691A, MTHFR C677T, PAI-1 4G/5G, and factor XIII V34L heterozygous mutations. The patient started with subcutaneous enoxaparin for 7 days, and after the second day of therapy, oral acenocoumarol was added. Oral treatment lasted for 6 months, and the evolution was very good. The size of the thrombus and the diameter of the veins decreased progressively; recanalization of the affected vessels occurred after 6 months, and finally the flow returned to normal [Figure 3] and [Figure 4]. At follow-ups over the next 12 months, he had not experienced any relapse, and D-dimer test remained negative.
Figure 1: (a) Longitudinal view of the right axillary vein. Color Doppler shows complete loss of flow within the vein at diagnosis. The vein appears enlarged, fusiform, incompressible, and with a non-homogeneous content (there are echoic areas in the periphery consistent with an old thrombus). (b) Transverse view of the right axillary vein. Color Doppler shows complete loss of flow within the vein at diagnosis. The caliber of the vein is increased and the walls of the vein appear thickened

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Figure 2: Right brachial vein thrombosis and increased diameter vein at diagnosis (longitudinal view). The vein is incompressible and the content is echoic and inhomogeneous

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Figure 3: Almost complete recanalization of the right axillary vein (a) and brachial vein (b) after 4 months of therapy (Color Doppler). There are small filling defects in the periphery of the vessels

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Figure 4: Normal caliber of axillary vein (a), normal flow (b), and no signs of thrombosis after 6 months of therapy

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   Discussion Top


We have described the case of a 15-year-old boy who developed right axillary and subclavian venous thrombosis following repeated throwing of firecrackers. We found no reports about venous thrombosis occurring after this type of activity.[1],[2],[5]

Effort thrombosis or PSS affects predominately young males because they are more likely to participate in high-risk activities for this disease and occurs more frequently in the dominant upper limb. Endothelial microtrauma precipitates local thrombus formation as a consequence of venous compression during abduction of the upper limb. Common symptoms are edema, pain, and discoloration (including cyanosis and erythema).

PSS is a subset of thoracic outlet syndrome, which also includes nerve, arterial, and venous forms, and each situation requires careful clinical evaluation for differential diagnosis.

Doppler ultrasonography is considered the primary diagnostic tool because of its high specificity and sensitivity.[6],[7] The assessment of the upper limb veins must include the visualization of the veins using B mode and color mode, as well as compression and post-compression evaluation.[6],[7] Filling defects within the vein or complete loss of flow can be demonstrated by using color or Power Doppler. Some of the most important sonographic signs of thrombosis are represented by visualization of thrombus, non-compressible venous segment, filling defects or loss of venous flow, increased or decreased diameter of the vein (acute/chronic), and loss of phasic flow during Valsalva. During the acute phase, the diameter of the vessel may be increased, compression of the affected segment causes pain, and the thrombus is hypoechoic. A chronic thrombus is echoic and retracted from the walls of the vein, and spots of flow may be detected through it and in the periphery.[6],[7]

When ultrasonography is inconclusive, venography, contrast computed tomography (CT) scan is indicated.[2]

In our case report, Doppler ultrasonography revealed a dilated, incompressible right axillary vein with thrombotic, echoic content, and without vascular flow detected by color and spectral Doppler. The thrombus extended into the right subclavian vein and right brachial vein.

Complications of PSS are pulmonary thromboembolism, recurrence of the symptoms, and post-thrombotic syndrome. Recurrence risk is mainly linked with residual vein thrombosis and the presence of D-dimer at the time of anticoagulant treatment discontinuation.[8],[9],[10]

Treatment strategies include both pharmacological and surgical therapy. Catheter-directed thrombolysis is indicated within the first 14 days to restore venous patency and to decrease the risk of complications and is completed with administration of low molecular weight heparin or unfractionated heparin, followed by vitamin K antagonist for 3 to 6 months or more, depending on the etiology.[1],[2],[5],[11],[12] In our patient, because he presented late, 5 weeks after the event, catheter-directed thrombolysis was not indicated. The early initiation of therapy (in the first 2 weeks) leads to better results.

There are dissenting opinion regarding the necessity of thoracic outlet decompression. Many authors suggested that first rib resection which can be accomplished via different protocols involving either the transaxillary or combined infraclavicular and supraclavicular approaches should be considered in severe and persistent cases that are unresponsive to medical therapy, but recently, it was generally accepted that thrombolysis should be followed by surgical decompression of the costoclavicular junction.[2],[5],[12],[13] Physical therapy and rehabilitation program are necessary after surgical therapy for achieving a return to full function. Other treatment modalities are mechanical thrombectomy, stenting, and balloon angioplasty.[2],[5],[11],[12],[13]

As in any rare disease, there are practice variations. A group of experts from Boston Children's Hospital have created an algorithm for diagnosis and treatment of PSS. The first step consists of Doppler ultrasound followed by interventional radiology (venography, catheter-directed thrombolysis), anticoagulation, and vascular surgery for thoracic outlet decompression after 3 to 4 weeks. Postoperative management consists of low-molecular-weight heparin for 6 weeks, activity limitation for 6 weeks, and imaging follow-up at 3 months and 12 months.[2],[5],[11],[12],[13]

Genetic screening for thrombophilia was indicated in the index case based on precocious onset of thrombosis and family history of miscarriages. Heterozygous mutations of factor V Leiden, MTHFR, PAI-1, and factor XIII were discovered. The presence of a heterozygous mutation in factor V Leiden does not alone predispose to deep venous thrombosis, although heterozygous carriers have a threefold to fivefold increased risk of venous thrombotic events throughout life, but it can occur when coupled with another physical or environmental risk factor (repetitive effort during firecrackers throwing in our case). In addition to this, the thrombophilic tendency association (which taken separately are not considered significant risk factors for thrombosis) has been accentuated the possibility of venous thrombosis.[9],[14]

In our patient, anticoagulant treatment was continued for 6 months, because according to the guidelines, there is no evidence of the clinical benefit of extended anticoagulant treatment for carriers of heterozygous factor V Leiden.[15] In addition, negative D-dimer test obtained 1 month after withdrawal of anticoagulant therapy in our patient has strengthened our management decision, because this is a protective factor for recurrence of venous thrombosis. The size of the thrombus and the diameter of the axillary vein decreased, recanalization occurred, and, finally, the flow returned to normal.


   Conclusion Top


PSS is rarely seen in children, but should be considered in the case of upper extremity swelling and pain that appears after exertion. The absence of a correct and precocious diagnosis can lead to severe and disabling complications such as thromboembolism or post-thrombotic syndrome, even at this age.

Consent

Written informed consent was obtained from the patient's family for publication of this case report and its accompanying images.

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.
Alla VM, Natarajan N, Kaushik M, Warrier R, Nair C. Paget-Schroetter syndrome: Review of pathogenesis and treatment of effort thrombosis. West J Emerg Med 2010;11:358-62.  Back to cited text no. 1
    
2.
Illig K, Doyle A. A comprehensive review of Paget-Schroetter syndrome. J Vasc Surg 2010;51:1538-47.  Back to cited text no. 2
    
3.
Lindblad B, Tengborn L, Bergqvist D. Deep vein thrombosis of the axillary-subclavian veins: Epidemiologic data, effects of different types of treatment and late sequelae. Eur J Vasc Surg 1988;2:161-5.  Back to cited text no. 3
    
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Young K, Tunstall O, Mumford A. Subclavian vein thrombosis in an otherwise healthy 9-year-old boy. BMJ Case Rep 2014. doi: 10.1136/bcr-2013-202413.  Back to cited text no. 4
    
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Trenor C, Fisher J, Khan F, Sparks E, Duzan J, Harney K, et al. Paget-Schroetter syndrome in 21 children: Outcomes after multidisciplinary care. J Pediatr 2015;166:1493-7.  Back to cited text no. 5
    
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Chin E, Zimmerman P, Grant E. Sonographic evaluation of upper extremity deep venous thrombosis. J Ultrasound Med 2005;24:829-38.  Back to cited text no. 6
    
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Karande GY, Hedgire SS, Sanchez Y, Baliyan V, Mishra V, Ganguli S, et al. Advanced imaging in acute and chronic deep vein thrombosis. Cardiovasc Diagn Ther 2016;6:493-507.  Back to cited text no. 7
    
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Shimada T, Tounai T, Syoji T, Fukumoto Y. Acute pulmonary embolism due to Paget-Schroetter syndrome. Intern Med 2015;54:1875-9.  Back to cited text no. 8
    
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Prandoni P, Barbar S, Milan M, Campello E, Spiezia L, Piovella C, et al. Optimal duration of anticoagulation Provoked versus unprovoked VTE and role of adjunctive thrombophilia and imaging tests. Thromb Haemost 2014;112:1210-5.  Back to cited text no. 9
    
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Dep A, Concannon E, McHugh SM, Burke P. Paget-Schrotter syndrome and complications of management. BMJ Case Rep 2013;2013:bcr2013008858. doi: 10.1136/bcr-2013-008858.  Back to cited text no. 10
    
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Goss S, Alcantara S, Todd G, Lantis J. Non-operative management of Paget-Schroetter syndrome: A single-center experience. J Invasive Cardiol 2015;27:423-8.  Back to cited text no. 11
    
12.
Vemuri C, Salehi P, Benarroch-Gampel J, McLaughlin LN, Thompson RW. Diagnosis and treatment of effort-induced thrombosis of the axillary subclavian vein due to venous thoracic outlet syndrome. J Vasc Surg Venous Lymphat Disord 2016;4:485-500.  Back to cited text no. 12
    
13.
Engelberger R, Kucher N. Management of deep vein thrombosis of the upper extremity. Circulation 2012;126:768-73.  Back to cited text no. 13
    
14.
Stevens S, Woller S, Bauer K, Kasthuri R, Cushman M, Streiff M, et al. Guidance for the evaluation and treatment of hereditary and acquired thrombophilia. J Thromb Thrombolysis 2016;41:154-64.  Back to cited text no. 14
    
15.
Konstantinides SV, Torbicki A, Agnelli G, Danchin N, Fitzmaurice D, Galiè N, et al. 2014 ESC guidelines on the diagnosis and management of acute pulmonary embolism. Eur Heart J 2014;35:3033-69.  Back to cited text no. 15
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

  [Table 1]



 

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