SCIENTIFIC ARTICLE
Thoracic Outlet Syndrome in the Pediatric Population: Case Series Anthony T. Vu, MD, Parit A. Patel, MD, Haithem Elhadi, MD, Ann R. Schwentker, MD, Kevin P. Yakuboff, MD
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. (J Hand Surg Am. 2014;39(3):484e487. Copyright Ó 2014 by the American Society for Surgery of the Hand. All rights reserved.) Key words Brachial plexus, pediatric, supraclavicular, thoracic outlet syndrome.
T
HORACIC OUTLET SYNDROME (TOS) is caused by compression of the neurovascular structures as they exit the thoracic outlet and results in a constellation of symptoms including pain, parasthesias, fatigue, pallor, cyanosis, numbness, weakness, limb coldness, heaviness, and motor deficits in the neck, upper extremities, or both.1 Thoracic outlet syndrome is more common in women and typically occurs between the ages of 21 and 50 years. We present 4 children with TOS, including 1 patient who was 4 months of age.
From the Division of Plastic, Reconstructive, and Hand Surgery, University of Cincinnati; and the Division of Plastic Surgery, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH; and the Department of Plastic Surgery, Institute of Reconstructive Surgery, New York University, New York, NY. Received for publication June 4, 2013; accepted in revised form November 14, 2013. All authors listed in this report have followed the principles outlined in the 1975 Declaration of Helsinki. The Cincinnati Children’s Hospital Medical Center Institutional Review Board has approved the research and deemed it to be nonhuman subjects research. No benefits in any form have been received or will be received related directly or indirectly to the subject of this article. Corresponding author: Kevin P. Yakuboff, MD, Division of Plastic, Reconstructive, and Hand Surgery, Department of Surgery, University of Cincinnati, 231 Albert Sabin Way, ML 0558, Cincinnati, OH 45267-0558; e-mail: [email protected]. 0363-5023/14/3903-0012$36.00/0 http://dx.doi.org/10.1016/j.jhsa.2013.11.029
484
r
Ó 2014 ASSH
r
Published by Elsevier, Inc. All rights reserved.
CASE REPORTS Patient 1 A 10-year-old girl presented with numbness and tingling along the lateral aspect of the right arm and weakness with right elbow flexion and shoulder abduction. She had previously had a cervical lymph node biopsy, after which the symptoms worsened; pathology revealed the specimen to have cartilage and bone. On physical examination, she had right-sided sensory abnormalities and decreased tendon reflexes. The Roos, Adson, and Wright tests elicited her symptoms. Magnetic resonance imaging and magnetic resonance angiography demonstrated bilateral cervical ribs without vascular compression. Because of these findings, we thought that the previous biopsy was actually of the cervical rib, and that her symptoms had worsened as a result of the sharp edge of the rib irritating the brachial plexus: in other words, neurogenic TOS. She underwent right supraclavicular brachial plexus exploration with cervical rib resection. The right arm numbness, tingling, and weakness with elbow flexion and shoulder abduction resolved. At a recent follow-up 37 months postoperatively, she had no problems, and all provocative maneuvers were negative. Patient 2 A 4-month-old boy presented with episodes of right arm cyanosis since birth. These occurred when he was
TOS IN THE PEDIATRIC POPULATION
lying prone or on the right arm. On physical examination, there was decreased sensation to pinch testing along the medial aspect of the right arm and lateral shoulder. No palpable masses, bruits, motor, or sensory deficits were noted. Hyperabduction of the arm with the head turned to the left resulted in cyanosis of the right hand without loss of pulses. A chest radiograph revealed a right cervical rib, and an ultrasound examination revealed patency of the subclavian artery and vein. Magnetic resonance imaging and magnetic resonance angiography demonstrated bilateral cervical ribs and compression of the subclavian vein when he was placed in right lateral decubitus and with hyperabduction of the right arm (Fig. 1). The patient was diagnosed with venous TOS and underwent surgical exploration through a supraclavicular approach with right cervical rib resection and anterior scalenotomy. The patient had complete resolution of symptoms including return of sensation with pinch testing and the absence of cyanosis with provocative maneuvers. When the parents were contacted by telephone 13 months later, they expressed satisfaction and had noted no further cyanosis regardless of shoulder position.
FIGURE 1: Magnetic resonance angiograph showing narrowing of the right subclavian vein with the patient placed in a right sideedown position.
Patient 3 A 10-year-old girl presented to the pediatric surgery clinic with a subcutaneous right neck mass that had been present for 3 years. A chest radiograph revealed fused first and second ribs. A computed tomography angiogram revealed an anomalous first rib that fused laterally with the second rib and compression of the subclavian artery that worsened with shoulder abduction. She was referred to the brachial plexus clinic where the patient noted numbness and tingling in the right arm and pain with shoulder abduction, which also caused loss of the pulse distally. The patient was diagnosed with arterial TOS and underwent supraclavicular exploration with right first rib resection. The symptoms resolved and shoulder abduction no longer affected the pulse. Telephone follow-up 3 years after surgery indicated that the patient remained asymptomatic.
shoulder abduction. The costoclavicular compression test led to immediate loss of pulses. The sensory and motor examinations were normal, although there was mild wasting of the right thenar musculature. A chest radiograph revealed a right cervical rib and magnetic resonance imaging and magnetic resonance angiography showed major compression of the right subclavian artery with the arm abducted. The patient was diagnosed with arterial TOS and underwent supraclavicular exploration with cervical rib resection and scalenectomy. Intraoperatively, a fibrous band was found to be compressing the subclavian artery, and was therefore resected. Three months postoperatively, she had complete resolution of symptoms except for some residual right hand weakness. She had a median nerve Tinel sign distal to the antecubital fossa. Provocative maneuvers no longer caused loss of pulses. She no longer had left-sided symptoms. She was prescribed physical therapy; at follow-up 11 months postoperatively, all provocative maneuvers were negative and she no longer reported right arm numbness, tingling, weakness, or pain.
Patient 4 A 9-year-old girl presented with 2 months of numbness, tingling, and aching in the right arm, particularly with shoulder abduction, and progressive right hand weakness and pain when placing her hands above her head. She had similar symptoms on the left, but to a lesser degree. On physical examination, she noted pain with hyperabduction of both hands, right greater than left. She lost wrist pulses with 90 right J Hand Surg Am.
485
DISCUSSION Thoracic outlet syndrome is difficult to diagnose and treat owing to nonspecific symptoms and potential overlap with other pathologic processes. The neurovascular structures within the thoracic outlet can be r
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compressed in 3 regions: the interscalene triangle, the costoclavicular triangle, and the subcoracoid space. Occasionally, cervical ribs, anomalous ribs, or fibrous bands are the offending structures. Thoracic outlet syndrome can be classified into 2 major types: neurogenic and vascular. In adults, the neurogenic type is the most common and comprises more than 90% of all cases.2 Unfortunately, most cases of neurogenic TOS are nonspecific and have no objective findings. Vascular TOS comprises less than 10% of all adult cases and can result from compression of the subclavian artery or vein. 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 ¼ 40) of all patients had vascular TOS. Of those, 30% (n ¼ 23) and 22% (n ¼ 17) were diagnosed with venous TOS and arterial TOS, respectively. Our cases support these findings, with 3 of 4 patients diagnosed with either venous or arterial TOS. Higher rates of vascular TOS in children differ from established rates in adults, which could be because neurologic TOS typically results from upper extremity/neck trauma or overuse, which is less likely to occur in children. Cervical or anomalous first ribs are common and are present in approximately 40% of pediatric TOS cases (Appendix A). 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. In our series, however, we found magnetic resonance imaging and magnetic resonance angiography to be helpful in demonstrating vascular compression with manipulation of the arm during the study. With the help of sedation, patients were placed supine or lateral decubitus with the arm and neck in positions that elicited symptoms on physical examination. Radiographs can also diagnose a cervical or anomalous first rib in cases of suspected TOS. The optimal treatment for TOS is controversial because of the lack of formal criteria for diagnosis and the lack of randomized prospective trials with long-term follow-up. In general, patients are managed conservatively for 6 to 12 months before surgery is considered. Different surgical approaches have been described, including transaxillary, supraclavicular, J Hand Surg Am.
infraclavicular, and posterior. The lead surgeon in our series prefers the supraclavicular approach owing to the wide exposure that allows for resection of all offending structures, minimal morbidity, and lower risk for severe complications. In addition, when vascular reconstruction is required, the supraclavicular approach allows for better proximal control. In a series of 491 patients, there was no difference in success rates when comparing transaxillary first rib resection and scalenectomy versus supraclavicular first rib resection and scalenectomy. Both procedures had over a 90% success rate at 3 months and approximately 70% success rate at 5 to 10 years.17 Although most patients diagnosed with TOS are working-age adults, our series demonstrates that TOS can present in infancy as well, and therefore must remain in the differential diagnosis when a patient presents with unexplained signs of neurovascular compromise to the upper extremity. In an infant, parents’ concerns regarding upper extremity abnormalities should raise the suspicion of TOS. These concerns should be assessed with a detailed physical examination that includes pinch testing for sensation and placing the infant in provocative positions to elicit the symptoms in question. Further workup can include imaging (radiographs, magnetic resonance imaging, and magnetic resonance angiography) to determine the possibility of abnormal anatomy causing compression of the thoracic outlet. Compared with neurogenic TOS, vascular TOS is more common in children. In the workup of pediatric TOS, magnetic resonance arteriography may be helpful in documenting vascular compromise if the arm can be placed in the inciting position during the study. A cervical or anomalous first rib is a common finding and should be evaluated for during the workup of these patients as well. We encountered no intraoperative or postoperative complications in our patients, and with an average follow-up of 2 years, we believe the supraclavicular approach is a safe and effective method for resecting abnormal anatomical structures that commonly cause TOS in children. REFERENCES 1. Teddy PJ, Johnson RD, Cai RR, Wallace D. Surgery for paediatric thoracic outlet syndrome. J Clin Neurosci. 2012;19(2):235e240. 2. Arthur LG, Teich S, Hogan M, Caniano DA, Smead W. Pediatric thoracic outlet syndrome: a disorder with serious vascular complications. J Pediatr Surg. 2008;43(6):1089e1094. 3. Vaksmann G, Noblet D, Dupuis C. Subclavian artery aneurysm secondary to a cervical supernumerary rib in a child. Eur J Pediatr. 1987;146(2):209e210. 4. Yang J, Letts M. Thoracic outlet syndrome in children. J Pediatr Orthop. 1996;16(4):514e517.
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12. Sen S, Discigil B, Boga M, Ozkisacik E, Inci I. Thoracic outlet syndrome with right subclavian artery dilatation in a child—transaxillary resection of the pediatric cervical rib. Thorac Cardiovasc Surg. 2007;55(5):339e341. 13. Maru S, Dosluoglu H, Dryjski M, Cherr G, Curl GR, Harris LM. Thoracic outlet syndrome in children and young adults. Eur J Vasc Endovasc Surg. 2009;38(5):560e564. 14. Dahlin LB, Backman C, Duppe H, et al. Compression of the lower trunk of the brachial plexus by a cervical rib in two adolescent girls: case reports and surgical treatment. J Brachial Plex Peripher Nerve Inj. 2009;4:14. 15. O’Brien PJ, Ramasunder S, Cox MW. Venous thoracic outlet syndrome secondary to first rib osteochondroma in a pediatric patient. J Vasc Surg. 2011;53(3):811e813. 16. Schroeder S, Cannizzaro E, Kellenberger CJ, Saurenmann RK. A 12-year-old girl with absent radial pulse: arterial thoracic outlet syndrome with subclavian artery aneurysm and thrombosis of the brachial artery. Eur J Pediatr. 2012;171(11):1707e1709. 17. Sanders RJ, Pearce WH. The treatment of thoracic outlet syndrome: a comparison of different operations. J Vasc Surg. 1989;10(6): 626e634.
5. Shepard A, Parikshak M. Thoracic outlet syndrome. In: Cameron J, ed. Current Surgical Therapy. 8th ed. Philadelphia, PA: Elsevier and Mosby; 2004:840e845. 6. DiFiore JW, Reid JR, Drummond-Webb J. Thoracic outlet syndrome in a child—transaxillary resection of anomalous first rib. J Pediatr Surg. 2002;37(8):1220e1222. 7. Vercellio G, Baraldini V, Gatti C, Coletti M, Cipolat L. Thoracic outlet syndrome in paediatrics: clinical presentation, surgical treatment, and outcome in a series of eight children. J Pediatr Surg. 2003;38(1): 58e61. 8. Okamoto H, Kawai K, Hattori S, et al. Thoracic outlet syndrome combined with carpal tunnel syndrome and guyon canal syndrome in a child. J Orthop Sci. 2005;10(6):634e640. 9. Swierczynska A, Klusek R, Kroczka S. Neurorehabilitation in children with thoracic outlet syndrom and its assessment. Przegl Lek. 2005;62(11):1308e1313. 10. Cagli K, Ozcakar L, Beyazit M, Sirmali M. Thoracic outlet syndrome in an adolescent with bilateral bifid ribs. Clin Anat. 2006;19(6):558e560. 11. Martins RS, Siqueira MG. Cervical rib fracture: an unusual etiology of thoracic outlet syndrome in a child. Pediatr Neurosurg. 2007;43(4): 293e296.
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Author
Reported Cases of Pediatric TOS Patients, n
Age, y
Vaksmann et al3
1 boy
9
Yang and Letts4
4 girls
Shepherd and Parikshak5
TOS Type
Clinical Details
Procedure/Approach
Outcome
Mean Follow-Up
Managed conservatively
Not reported
Not reported
12e13
Neurogenic
Cervical rib (n ¼ 2)
Transaxillary cervical rib resection with scalenotomy in 2 patients; 2 patients managed conservatively
Complete resolution of symptoms in all 4 patients
Not reported
1 boy
11
Neurogenic
Bilateral cervical ribs
Supraclavicular resection of cervical rib
Complete resolution of symptoms
2 mo
DiFiore et al6
1 boy
6
Arterial
Anomalous first rib, subclavian artery aneurysm
Transaxillary resection of anomalous first rib with scalenotomy, no arterial reconstruction
Complete resolution of symptoms
6 mo
Vercellio et al7
4 boys 4 girls
8e16
Venous (n ¼ 6) Neurogenic (n ¼ 2)
Venous: thrombosed (n ¼ 2), functional intermittent obstruction (n ¼ 4) Neurogenic: cervical rib (n ¼ 2)
Transaxillary first rib resection and scalenotomy in 4 patients with venous TOS (including 2 patients with evidence of thrombosis); supraclavicular and infraclavicular scalenectomy, first rib resection, and venolysis in 1 patient with venous TOS; supraclavicular resection of cervical rib, partial first rib resection, and scalenectomy in both patients with neurogenic TOS; 1 patient managed conservatively
Complete resolution of symptoms in 6 patients; recurrence in 1 patient with venous thrombosis; minor discomfort in 1 patient treated conservatively
18 mo
Okamoto et al8
1 boy
13
Arterial
Anomalous first rib
Transaxillary first rib resection, supraclavicular scalenectomy
Complete resolution of symptoms
Not reported
Swierczynska et al9
3 girls
12e14
Mixed arterial and neurogenic (n ¼ 3)
-
Transaxillary first rib resection in 1 patient; 2 patients managed conservatively
Successful outcomes in all 3 patients
12 mo
Cagli et al10
1 girl
13
Arterial
Bilateral bifid ribs owing to cervical ribs, fibrous bands
Supraclavicular resection of cervical rib and fibrous bands
Complete resolution of symptoms
1 mo
r
Cervical rib, subclavian artery aneurysm
Vol. 39, March 2014
(Continued)
TOS IN THE PEDIATRIC POPULATION
J Hand Surg Am.
Arterial
487.e1
APPENDIX A.
APPENDIX A. Author
Reported Cases of Pediatric TOS (Continued) Patients, n
Age, y
Arthur et al2
7 boys 18 girls
12e18
Martins and Siqueira11
1 girl
Sen et al12
TOS Type
Clinical Details
Procedure/Approach
Outcome
Mean Follow-Up
Transaxillary first rib resection with scalenotomy in 24 patients; cervical rib resection in 2 patients
Limited to 11 patients: complete resolution (n ¼ 6), improvement (n ¼ 1), no change/ recurrence (n ¼ 4)
Not reported
9
Neurogenic
Cervical rib
Supraclavicular resection of cervical rib
Complete resolution of symptoms
24 mo
1 boy
8
Mixed arterial and neurogenic
Cervical rib
Transaxillary first and cervical rib resection with scalenectomy
Complete resolution of symptoms
5y
Maru et al13
4 boys 8 girls
12e20
Arterial (n ¼ 5) Venous (n ¼ 2) Neurogenic (n ¼ 4) Mixed venous and neurogenic (n ¼ 1)
Cervical rib (n ¼ 3)
Supraclavicular resection of first rib, scalenectomy, and resection of cervical rib when present
Limited to 10 patients: complete resolution of symptoms in all patients
26 mo
Dahlin et al14
2 girls
11,16
Neurogenic
Cervical rib (n ¼ 2)
Supraclavicular resection of cervical ribs in both patients
Complete resolution or overall improvement of symptoms in both patients
12 mo
Teddy et al1
3 boys 10 girls
9e17
Arterial (n ¼ 1) Venous (n ¼ 1) Neurogenic (n ¼ 11)
Cervical ribs or elongated C7 transverse process (n ¼ 8); anomalous first rib (n ¼ 1)
Supraclavicular scalenotomy in 17 limbs; transaxillary first rib resection in 3 limbs (2 done for reoperation)
75% successful
34 mo
O’Brien et al15
1 girl
12
Venous
Osteochondroma of first rib
Supraclavicular and infraclavicular first rib resection
Complete resolution of symptoms
6 mo
Schroeder et al16
1 girl
12
Arterial
Anomalous first rib, subclavian artery aneurysm
First rib and subclavian artery aneurysm resection, no mention of approach
Complete resolution of symptoms
Not reported
r
Cervical rib (n ¼ 4)
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TOS IN THE PEDIATRIC POPULATION
J Hand Surg Am.
Venous (n ¼ 11) Arterial (n ¼ 2) Neurogenic (n ¼ 11) Mixed venous and neurogenic (n ¼ 1)
487.e2
Thoracic Outlet Syndrome in the Pediatric Population: Case Series Anthony T. Vu, MD, Parit A. Patel, MD, Haithem Elhadi, MD, Ann R. Schwentker, MD, Kevin P. Yakuboff, MD
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. (J Hand Surg Am. 2014;39(3):484e487. Copyright Ó 2014 by the American Society for Surgery of the Hand. All rights reserved.) Key words Brachial plexus, pediatric, supraclavicular, thoracic outlet syndrome.
T
HORACIC OUTLET SYNDROME (TOS) is caused by compression of the neurovascular structures as they exit the thoracic outlet and results in a constellation of symptoms including pain, parasthesias, fatigue, pallor, cyanosis, numbness, weakness, limb coldness, heaviness, and motor deficits in the neck, upper extremities, or both.1 Thoracic outlet syndrome is more common in women and typically occurs between the ages of 21 and 50 years. We present 4 children with TOS, including 1 patient who was 4 months of age.
From the Division of Plastic, Reconstructive, and Hand Surgery, University of Cincinnati; and the Division of Plastic Surgery, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH; and the Department of Plastic Surgery, Institute of Reconstructive Surgery, New York University, New York, NY. Received for publication June 4, 2013; accepted in revised form November 14, 2013. All authors listed in this report have followed the principles outlined in the 1975 Declaration of Helsinki. The Cincinnati Children’s Hospital Medical Center Institutional Review Board has approved the research and deemed it to be nonhuman subjects research. No benefits in any form have been received or will be received related directly or indirectly to the subject of this article. Corresponding author: Kevin P. Yakuboff, MD, Division of Plastic, Reconstructive, and Hand Surgery, Department of Surgery, University of Cincinnati, 231 Albert Sabin Way, ML 0558, Cincinnati, OH 45267-0558; e-mail: [email protected]. 0363-5023/14/3903-0012$36.00/0 http://dx.doi.org/10.1016/j.jhsa.2013.11.029
484
r
Ó 2014 ASSH
r
Published by Elsevier, Inc. All rights reserved.
CASE REPORTS Patient 1 A 10-year-old girl presented with numbness and tingling along the lateral aspect of the right arm and weakness with right elbow flexion and shoulder abduction. She had previously had a cervical lymph node biopsy, after which the symptoms worsened; pathology revealed the specimen to have cartilage and bone. On physical examination, she had right-sided sensory abnormalities and decreased tendon reflexes. The Roos, Adson, and Wright tests elicited her symptoms. Magnetic resonance imaging and magnetic resonance angiography demonstrated bilateral cervical ribs without vascular compression. Because of these findings, we thought that the previous biopsy was actually of the cervical rib, and that her symptoms had worsened as a result of the sharp edge of the rib irritating the brachial plexus: in other words, neurogenic TOS. She underwent right supraclavicular brachial plexus exploration with cervical rib resection. The right arm numbness, tingling, and weakness with elbow flexion and shoulder abduction resolved. At a recent follow-up 37 months postoperatively, she had no problems, and all provocative maneuvers were negative. Patient 2 A 4-month-old boy presented with episodes of right arm cyanosis since birth. These occurred when he was
TOS IN THE PEDIATRIC POPULATION
lying prone or on the right arm. On physical examination, there was decreased sensation to pinch testing along the medial aspect of the right arm and lateral shoulder. No palpable masses, bruits, motor, or sensory deficits were noted. Hyperabduction of the arm with the head turned to the left resulted in cyanosis of the right hand without loss of pulses. A chest radiograph revealed a right cervical rib, and an ultrasound examination revealed patency of the subclavian artery and vein. Magnetic resonance imaging and magnetic resonance angiography demonstrated bilateral cervical ribs and compression of the subclavian vein when he was placed in right lateral decubitus and with hyperabduction of the right arm (Fig. 1). The patient was diagnosed with venous TOS and underwent surgical exploration through a supraclavicular approach with right cervical rib resection and anterior scalenotomy. The patient had complete resolution of symptoms including return of sensation with pinch testing and the absence of cyanosis with provocative maneuvers. When the parents were contacted by telephone 13 months later, they expressed satisfaction and had noted no further cyanosis regardless of shoulder position.
FIGURE 1: Magnetic resonance angiograph showing narrowing of the right subclavian vein with the patient placed in a right sideedown position.
Patient 3 A 10-year-old girl presented to the pediatric surgery clinic with a subcutaneous right neck mass that had been present for 3 years. A chest radiograph revealed fused first and second ribs. A computed tomography angiogram revealed an anomalous first rib that fused laterally with the second rib and compression of the subclavian artery that worsened with shoulder abduction. She was referred to the brachial plexus clinic where the patient noted numbness and tingling in the right arm and pain with shoulder abduction, which also caused loss of the pulse distally. The patient was diagnosed with arterial TOS and underwent supraclavicular exploration with right first rib resection. The symptoms resolved and shoulder abduction no longer affected the pulse. Telephone follow-up 3 years after surgery indicated that the patient remained asymptomatic.
shoulder abduction. The costoclavicular compression test led to immediate loss of pulses. The sensory and motor examinations were normal, although there was mild wasting of the right thenar musculature. A chest radiograph revealed a right cervical rib and magnetic resonance imaging and magnetic resonance angiography showed major compression of the right subclavian artery with the arm abducted. The patient was diagnosed with arterial TOS and underwent supraclavicular exploration with cervical rib resection and scalenectomy. Intraoperatively, a fibrous band was found to be compressing the subclavian artery, and was therefore resected. Three months postoperatively, she had complete resolution of symptoms except for some residual right hand weakness. She had a median nerve Tinel sign distal to the antecubital fossa. Provocative maneuvers no longer caused loss of pulses. She no longer had left-sided symptoms. She was prescribed physical therapy; at follow-up 11 months postoperatively, all provocative maneuvers were negative and she no longer reported right arm numbness, tingling, weakness, or pain.
Patient 4 A 9-year-old girl presented with 2 months of numbness, tingling, and aching in the right arm, particularly with shoulder abduction, and progressive right hand weakness and pain when placing her hands above her head. She had similar symptoms on the left, but to a lesser degree. On physical examination, she noted pain with hyperabduction of both hands, right greater than left. She lost wrist pulses with 90 right J Hand Surg Am.
485
DISCUSSION Thoracic outlet syndrome is difficult to diagnose and treat owing to nonspecific symptoms and potential overlap with other pathologic processes. The neurovascular structures within the thoracic outlet can be r
Vol. 39, March 2014
486
TOS IN THE PEDIATRIC POPULATION
compressed in 3 regions: the interscalene triangle, the costoclavicular triangle, and the subcoracoid space. Occasionally, cervical ribs, anomalous ribs, or fibrous bands are the offending structures. Thoracic outlet syndrome can be classified into 2 major types: neurogenic and vascular. In adults, the neurogenic type is the most common and comprises more than 90% of all cases.2 Unfortunately, most cases of neurogenic TOS are nonspecific and have no objective findings. Vascular TOS comprises less than 10% of all adult cases and can result from compression of the subclavian artery or vein. 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 ¼ 40) of all patients had vascular TOS. Of those, 30% (n ¼ 23) and 22% (n ¼ 17) were diagnosed with venous TOS and arterial TOS, respectively. Our cases support these findings, with 3 of 4 patients diagnosed with either venous or arterial TOS. Higher rates of vascular TOS in children differ from established rates in adults, which could be because neurologic TOS typically results from upper extremity/neck trauma or overuse, which is less likely to occur in children. Cervical or anomalous first ribs are common and are present in approximately 40% of pediatric TOS cases (Appendix A). 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. In our series, however, we found magnetic resonance imaging and magnetic resonance angiography to be helpful in demonstrating vascular compression with manipulation of the arm during the study. With the help of sedation, patients were placed supine or lateral decubitus with the arm and neck in positions that elicited symptoms on physical examination. Radiographs can also diagnose a cervical or anomalous first rib in cases of suspected TOS. The optimal treatment for TOS is controversial because of the lack of formal criteria for diagnosis and the lack of randomized prospective trials with long-term follow-up. In general, patients are managed conservatively for 6 to 12 months before surgery is considered. Different surgical approaches have been described, including transaxillary, supraclavicular, J Hand Surg Am.
infraclavicular, and posterior. The lead surgeon in our series prefers the supraclavicular approach owing to the wide exposure that allows for resection of all offending structures, minimal morbidity, and lower risk for severe complications. In addition, when vascular reconstruction is required, the supraclavicular approach allows for better proximal control. In a series of 491 patients, there was no difference in success rates when comparing transaxillary first rib resection and scalenectomy versus supraclavicular first rib resection and scalenectomy. Both procedures had over a 90% success rate at 3 months and approximately 70% success rate at 5 to 10 years.17 Although most patients diagnosed with TOS are working-age adults, our series demonstrates that TOS can present in infancy as well, and therefore must remain in the differential diagnosis when a patient presents with unexplained signs of neurovascular compromise to the upper extremity. In an infant, parents’ concerns regarding upper extremity abnormalities should raise the suspicion of TOS. These concerns should be assessed with a detailed physical examination that includes pinch testing for sensation and placing the infant in provocative positions to elicit the symptoms in question. Further workup can include imaging (radiographs, magnetic resonance imaging, and magnetic resonance angiography) to determine the possibility of abnormal anatomy causing compression of the thoracic outlet. Compared with neurogenic TOS, vascular TOS is more common in children. In the workup of pediatric TOS, magnetic resonance arteriography may be helpful in documenting vascular compromise if the arm can be placed in the inciting position during the study. A cervical or anomalous first rib is a common finding and should be evaluated for during the workup of these patients as well. We encountered no intraoperative or postoperative complications in our patients, and with an average follow-up of 2 years, we believe the supraclavicular approach is a safe and effective method for resecting abnormal anatomical structures that commonly cause TOS in children. REFERENCES 1. Teddy PJ, Johnson RD, Cai RR, Wallace D. Surgery for paediatric thoracic outlet syndrome. J Clin Neurosci. 2012;19(2):235e240. 2. Arthur LG, Teich S, Hogan M, Caniano DA, Smead W. Pediatric thoracic outlet syndrome: a disorder with serious vascular complications. J Pediatr Surg. 2008;43(6):1089e1094. 3. Vaksmann G, Noblet D, Dupuis C. Subclavian artery aneurysm secondary to a cervical supernumerary rib in a child. Eur J Pediatr. 1987;146(2):209e210. 4. Yang J, Letts M. Thoracic outlet syndrome in children. J Pediatr Orthop. 1996;16(4):514e517.
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12. Sen S, Discigil B, Boga M, Ozkisacik E, Inci I. Thoracic outlet syndrome with right subclavian artery dilatation in a child—transaxillary resection of the pediatric cervical rib. Thorac Cardiovasc Surg. 2007;55(5):339e341. 13. Maru S, Dosluoglu H, Dryjski M, Cherr G, Curl GR, Harris LM. Thoracic outlet syndrome in children and young adults. Eur J Vasc Endovasc Surg. 2009;38(5):560e564. 14. Dahlin LB, Backman C, Duppe H, et al. Compression of the lower trunk of the brachial plexus by a cervical rib in two adolescent girls: case reports and surgical treatment. J Brachial Plex Peripher Nerve Inj. 2009;4:14. 15. O’Brien PJ, Ramasunder S, Cox MW. Venous thoracic outlet syndrome secondary to first rib osteochondroma in a pediatric patient. J Vasc Surg. 2011;53(3):811e813. 16. Schroeder S, Cannizzaro E, Kellenberger CJ, Saurenmann RK. A 12-year-old girl with absent radial pulse: arterial thoracic outlet syndrome with subclavian artery aneurysm and thrombosis of the brachial artery. Eur J Pediatr. 2012;171(11):1707e1709. 17. Sanders RJ, Pearce WH. The treatment of thoracic outlet syndrome: a comparison of different operations. J Vasc Surg. 1989;10(6): 626e634.
5. Shepard A, Parikshak M. Thoracic outlet syndrome. In: Cameron J, ed. Current Surgical Therapy. 8th ed. Philadelphia, PA: Elsevier and Mosby; 2004:840e845. 6. DiFiore JW, Reid JR, Drummond-Webb J. Thoracic outlet syndrome in a child—transaxillary resection of anomalous first rib. J Pediatr Surg. 2002;37(8):1220e1222. 7. Vercellio G, Baraldini V, Gatti C, Coletti M, Cipolat L. Thoracic outlet syndrome in paediatrics: clinical presentation, surgical treatment, and outcome in a series of eight children. J Pediatr Surg. 2003;38(1): 58e61. 8. Okamoto H, Kawai K, Hattori S, et al. Thoracic outlet syndrome combined with carpal tunnel syndrome and guyon canal syndrome in a child. J Orthop Sci. 2005;10(6):634e640. 9. Swierczynska A, Klusek R, Kroczka S. Neurorehabilitation in children with thoracic outlet syndrom and its assessment. Przegl Lek. 2005;62(11):1308e1313. 10. Cagli K, Ozcakar L, Beyazit M, Sirmali M. Thoracic outlet syndrome in an adolescent with bilateral bifid ribs. Clin Anat. 2006;19(6):558e560. 11. Martins RS, Siqueira MG. Cervical rib fracture: an unusual etiology of thoracic outlet syndrome in a child. Pediatr Neurosurg. 2007;43(4): 293e296.
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Vol. 39, March 2014
Author
Reported Cases of Pediatric TOS Patients, n
Age, y
Vaksmann et al3
1 boy
9
Yang and Letts4
4 girls
Shepherd and Parikshak5
TOS Type
Clinical Details
Procedure/Approach
Outcome
Mean Follow-Up
Managed conservatively
Not reported
Not reported
12e13
Neurogenic
Cervical rib (n ¼ 2)
Transaxillary cervical rib resection with scalenotomy in 2 patients; 2 patients managed conservatively
Complete resolution of symptoms in all 4 patients
Not reported
1 boy
11
Neurogenic
Bilateral cervical ribs
Supraclavicular resection of cervical rib
Complete resolution of symptoms
2 mo
DiFiore et al6
1 boy
6
Arterial
Anomalous first rib, subclavian artery aneurysm
Transaxillary resection of anomalous first rib with scalenotomy, no arterial reconstruction
Complete resolution of symptoms
6 mo
Vercellio et al7
4 boys 4 girls
8e16
Venous (n ¼ 6) Neurogenic (n ¼ 2)
Venous: thrombosed (n ¼ 2), functional intermittent obstruction (n ¼ 4) Neurogenic: cervical rib (n ¼ 2)
Transaxillary first rib resection and scalenotomy in 4 patients with venous TOS (including 2 patients with evidence of thrombosis); supraclavicular and infraclavicular scalenectomy, first rib resection, and venolysis in 1 patient with venous TOS; supraclavicular resection of cervical rib, partial first rib resection, and scalenectomy in both patients with neurogenic TOS; 1 patient managed conservatively
Complete resolution of symptoms in 6 patients; recurrence in 1 patient with venous thrombosis; minor discomfort in 1 patient treated conservatively
18 mo
Okamoto et al8
1 boy
13
Arterial
Anomalous first rib
Transaxillary first rib resection, supraclavicular scalenectomy
Complete resolution of symptoms
Not reported
Swierczynska et al9
3 girls
12e14
Mixed arterial and neurogenic (n ¼ 3)
-
Transaxillary first rib resection in 1 patient; 2 patients managed conservatively
Successful outcomes in all 3 patients
12 mo
Cagli et al10
1 girl
13
Arterial
Bilateral bifid ribs owing to cervical ribs, fibrous bands
Supraclavicular resection of cervical rib and fibrous bands
Complete resolution of symptoms
1 mo
r
Cervical rib, subclavian artery aneurysm
Vol. 39, March 2014
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J Hand Surg Am.
Arterial
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APPENDIX A.
APPENDIX A. Author
Reported Cases of Pediatric TOS (Continued) Patients, n
Age, y
Arthur et al2
7 boys 18 girls
12e18
Martins and Siqueira11
1 girl
Sen et al12
TOS Type
Clinical Details
Procedure/Approach
Outcome
Mean Follow-Up
Transaxillary first rib resection with scalenotomy in 24 patients; cervical rib resection in 2 patients
Limited to 11 patients: complete resolution (n ¼ 6), improvement (n ¼ 1), no change/ recurrence (n ¼ 4)
Not reported
9
Neurogenic
Cervical rib
Supraclavicular resection of cervical rib
Complete resolution of symptoms
24 mo
1 boy
8
Mixed arterial and neurogenic
Cervical rib
Transaxillary first and cervical rib resection with scalenectomy
Complete resolution of symptoms
5y
Maru et al13
4 boys 8 girls
12e20
Arterial (n ¼ 5) Venous (n ¼ 2) Neurogenic (n ¼ 4) Mixed venous and neurogenic (n ¼ 1)
Cervical rib (n ¼ 3)
Supraclavicular resection of first rib, scalenectomy, and resection of cervical rib when present
Limited to 10 patients: complete resolution of symptoms in all patients
26 mo
Dahlin et al14
2 girls
11,16
Neurogenic
Cervical rib (n ¼ 2)
Supraclavicular resection of cervical ribs in both patients
Complete resolution or overall improvement of symptoms in both patients
12 mo
Teddy et al1
3 boys 10 girls
9e17
Arterial (n ¼ 1) Venous (n ¼ 1) Neurogenic (n ¼ 11)
Cervical ribs or elongated C7 transverse process (n ¼ 8); anomalous first rib (n ¼ 1)
Supraclavicular scalenotomy in 17 limbs; transaxillary first rib resection in 3 limbs (2 done for reoperation)
75% successful
34 mo
O’Brien et al15
1 girl
12
Venous
Osteochondroma of first rib
Supraclavicular and infraclavicular first rib resection
Complete resolution of symptoms
6 mo
Schroeder et al16
1 girl
12
Arterial
Anomalous first rib, subclavian artery aneurysm
First rib and subclavian artery aneurysm resection, no mention of approach
Complete resolution of symptoms
Not reported
r
Cervical rib (n ¼ 4)
Vol. 39, March 2014
TOS IN THE PEDIATRIC POPULATION
J Hand Surg Am.
Venous (n ¼ 11) Arterial (n ¼ 2) Neurogenic (n ¼ 11) Mixed venous and neurogenic (n ¼ 1)
487.e2
