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Surgery for paediatric thoracic outlet syndrome
Journal of Clinical Neuroscience 19 (2012) 235–240
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Clinical Study
Surgery for paediatric thoracic outlet syndrome P.J. Teddy a,⇑, R.D. Johnson a, R.R. Cai a, D. Wallace a,b a b
Department of Surgery, Royal Melbourne Hospital, Grattan Street, Parkville, Melbourne, Victoria 3050, Australia Royal Childrens’s Hospital, Flemington Road, Parkville, Melbourne, Victoria 3052, Australia
a r t i c l e
i n f o
Article history: Received 5 March 2011 Accepted 25 May 2011
Keywords: Thoracic outlet syndrome Surgery Cervical rib
a b s t r a c t 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. Crown Copyright Ó 2011 Published by Elsevier Ltd. All rights reserved.
1. 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 conten-
⇑ Corresponding author. E-mail address: [email protected] (P.J. Teddy).
tious.13–23 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.
0967-5868/$ - see front matter Crown Copyright Ó 2011 Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.jocn.2011.05.015
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Table 1 Summary of paediatric thoracic outlet (TOS) syndrome patients undergoing surgery, reported in the literature (only patients <18 years of age included) Study
No. of patients
Age (years)
Sex
Clinical details
Procedures
Outcomes
Vaksmann et al. (1987)
1
9
M
Cervical rib with associated subclavian artery aneurysm
None
Not specified
Yang and Letts (1966)
4
12–13
F
Cervical ribs in 2 patients
TARR in 2 cases with cervical rib
Complete resolution of symptoms in all cases
DiFiore et al. (2002)
1
13
M
Neurogenic TOS
–
–
Vercellio et al. (2003)
8
8–16
M:F 1:1
5 patients: venous TOS
TARR in 4 cases of venous TOS
Successful resolution in all cases
2 patients: neurogenic TOS
SCS and partial rib resection in neurogenic TOS
1 patients: both forms of TOS Okamoto et al. (2005)
1
13
M
First rib hypoplasia Evidence of concomitant carpal tunnel and Guyon0 s canal syndromes
TARR, SCS, and decompression of carpal tunnel and Guyon’s canal
Symptoms resolved immediately post-op, but no further follow-up reported
Swierczyn´ska et al. (2005)
3
12–14
F
Mixed vascular and neurogenic TOS in all cases
TARR in 1 case The other cases underwent rehabilitation
Successful outcomes in all cases
Cagli et al. (2006)
1
13
M
Bilateral bifid ribs
Supraclavian resection of bifid ribs
Resolution of symptoms reported
Sen et al. (2007)
1
8
M
Mixed venous and neurogenic TOS in a patient with a cervical rib
TARR and resection of cervical rib
Resolution of symptoms reported
Arthur et al. (2006)
25
12–18 (mean 15.3)
18F, 7M
11 patients: venous TOS 2 patients: arterial TOS 11 patients neurologic TOS 1 patient: both forms of TOS
TARR in 24 cases; 5 patients underwent thrombolysis for subclavian vein thrombosis
Follow-up limited to 11 patients Complete relief in 6 cases Improvement in 1 case No change/recurrence in 4 cases
Maru et al. (2009)
9
12–16 (mean 17.3)
F:M 2:1
5 patients: arterial TOS 2 patients: venous TOS 2 patients neurogenic TOS Cervical ribs in 2 patients (one bilaterally) Subclavian aneurysms in 1 patient
SCS in all patients
Resolution of symptoms in all patients at outpatient follow-up (mean 10.9 weeks)
Dahlin et al. (2009)
2
13.5
F
Cervical ribs
Supraclavicular resection of cervical rib
Complete resolution of symptoms
F = female, M = male, SCS = supraclavicular scalenotomy, TARR = transaxillary resection of the first rib.
2. Methods
2.2. Surgical technique
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.
Two operative procedures were carried out: (i) anterior scalenotomy through a supraclavicular approach; (ii) a transaxillary approach with resection of the first rib (TARR) if scar prominence needed to be minimized or if scalenotomy failed. For supraclavicular scalenotomies (SCS) the patient was in the supine position with the head slightly elevated and rotated contralaterally. An approximately 5 cm transverse incision was made 1 cm above and parallel to the clavicle. Dissection of subcutaneous fat, platysma and deep cervical fascia exposed the clavicular head of the sternocleidomastoid which was then divided. The underlying omohyoid fascia was then divided and the omohyoid muscle retracted, or occasionally divided. The scalene fat pad was also dissected, and other intruding vessels retracted. Scalenotomy was carried out in stages with small fascicles of muscle being coagulated by bipolar diathermy and then divided by microscissors. Complete resection of the muscle was confirmed when the subclavian artery and brachial plexus roots and trunks were not otherwise obscured. Digital palpation excluded any remaining anatomical factors impinging on the artery or plexus. Any bony structures or spaceoccupying lesions were resected and any soft-tissue bands divided. Care was taken to avoid injury to the apex of the lung or Sibson’s fascia medially. When all compressive factors were alleviated, immaculate haemostasis was achieved, and vicryl sutures were used for muscles and nylon subcuticular sutures for the skin.
2.1. Data Information was gathered electronically about the presence, site, quality, and severity of pain pre-operatively, post-operatively and at the current time, and about other TOS symptoms both before surgery and currently. Other data included handedness, general health, and ratings of overall satisfaction outcome. Pain severity was assessed by a visual analogue scale score (VAS); assessment of postural or functional impairment utilized a dropdown list (0 = no pain or impairment; 10 = the most severe pain imaginable or total impairment). A VAS was also used for outcome satisfaction (0 = least; 10 = most favourable). Other symptoms were assessed with a Yes or No selection, with space for elaboration. Pain site was determined by check boxes for each anatomical division of the limb, including each digit and the pectoral girdle.
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For the transaxillary approach the patients were also supine with a large sandbag under the scapula to elevate the ipsilateral side. The arm was suspended from a pulley support system with a variable counterweight (2–5 kg). An incision was made in the most medial axillary skin fold, and the axillary fat and deep fascia traversed with blunt dissection until the first rib was reached. The point at which the subclavian artery and brachial plexus cross the rib was used as the midpoint of the rib resection which was carried out with rongeurs. The neurovascular structures were then visualized or palpated to assess remaining compression. The edges of the resected bone were smoothed out and bone wax applied. After immaculate haemostasis, closure was carried out with vicryl to the deep layers and subcuticular nylon to the skin. Following scalenotomy and TARR, patients were hospitalised for 2–4 days to assess initial results and monitored for early postoperative complications. Skin sutures were removed 7–10 days post-operatively. Follow-up consultations were carried out at 2 months and then several more 6-monthly visits as required to assess residual symptoms.
3. 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. 3.1. Data analysis The data were analysed using the two-tailed sign test for qualitative comparisons. For one-sample numerical data, the t distribution was used as the model and the paired t-test applied. Fisher’s exact test was also used for two-variable data. 3.2. Clinical presentation and investigation Cases were referred from a general practitioner (four cases), specialist physicians (four cases), or other surgeons (five cases).
Two patients had major congenital disorders: one case of Osler-Weber-Rendu disease, and one case of ipsilateral synbrachydactyly. Three patients were involved in motor vehicle accidents prior to presentation, with symptoms being precipitated by the accident in two cases. One patient reported symptom onset after a sports-related injury, whilst four patients reported acute onset without any precipitating event. All but one patient was at school at the time of presentation, the other having left school and being employed in heavy manual work. All patients were involved in at least one sport requiring the use of elevated upper limbs: basketball, netball and volleyball in eight patients; swimming in four patients; tennis in two patients; and cricket in two patients. Symptom frequency was ascertained from medical records and from questionnaires. From the medical records it was determined that the mean time between onset of symptoms and first consultation with the operating surgeon was 28 months. The predominant symptom was pain, followed by paraesthesia, numbness, weakness, limb ‘coldness’, and heaviness (Fig. 1A). Only two patients reported cyanosis, one bilaterally, and one patient reported hand tremor. Two patients reported sensing a mass in the supraclavicular fossa. More symptoms were reported in the questionnaire, with paraesthesia being the commonest, followed by weakness, pain, and numbness (Fig. 1B). Pallor and swelling were also more commonly reported in the questionnaire than in the medical records. The distribution of pain was usually widespread in the upper limb, with the neck and digits being the commonest sites (Fig. 1C). Postural symptom provocation was reported by all but one patient in the questionnaire, the elevated position being most important, with severity ranging from 4 to 10 (mean 7.8). Functional deficits were also reported with the affected limb pulled downwards (range 6–10, mean 7.1), out in front (range 5–10, mean 6.2), or extended rearward (range 3–10, mean 5.4). All patients reported impairments in functional capabilities, with sport and recreation being the most affected in all patients (range 4–10, mean 7.5). Impairment was also reported in basic daily living (17 limbs in 12 patients, mean 6.8), and study (14 limbs in 11 patients, mean 5.8). Overall functional impairment ranged from 4 to 10 (mean 7.6). Examination findings were variable. The EAST result correlated positively with the affected limb(s) in 11 patients, although the
Table 2 List of participants and operations performed on 13 paediatric patients with thoracic outlet syndrome. Patient
Sex
Age at first consultation (years)
Onset before presentation (months)
Dominant hand
Side(s) affected
Side operated
Surgical approach
1 2 3 4
F M M F
17 11 16 14
15 25 18 24
R R R R
R L RL L>R
5
F
15
48
R
RL
6 7
F F
15 16
6 18
R R
R L>R
8
F
9
60
L
Equal
9
F
13
24
R
L>R
10 11 12
F F F
15 17 15
7 31 48
R R R
L L L>R
13
M
14
39
R
RL
R L R L R R R R L R L R L R L L L L R R
SCS SCS SCS SCS SCS SCS TARR SCS SCS SCS SCS SCS SCS SCS TARR TARR SCS SCS SCS SCS
Three patients suffered significant contralateral symptoms (all on the non-dominant side), but did not elect for surgery. F = female, L = left, M = male, R = right, SCS = supraclavicular scalenotomy; TARR = transaxillary resection of he first rib.
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Fig. 1. (A) Symptomatology recorded in the medical notes. (B) Symptomatology reported at questionnaire follow-up. (C) Distribution of pain.
less-affected limbs in bilateral cases were negative. One case recorded an outright negative result, while a bilateral case was equivocal. Motor deficits were found in seven patients (nine limbs) including substantial weakness and wasting of the interossei and muscles of the thenar and hypothenar eminences in one patient. Mild weakness of triceps was seen in four patients (five limbs), and of finger extension in three (four limbs). Sensory impairment was found in four patients (five limbs): one was diffuse in the forearm, two in the C8 dermatome, and the other within the C4 distribution (two limbs). Palpation revealed tender supraclavicular
protuberances as reported by the patients, and the tip of a cervical rib in another case. Restricted cervical movement was found in one patient, while another experienced pain on neck flexion. X-ray imaging was carried out in all 13 patients, MRI in nine, and CT in five. Cervical ribs or an elongated C7 transverse process were discovered in eight patients. One of these was unilateral, and occurred on the contralateral side. There were cervical ribs of equal size in two cases (one set complete, the other incomplete), and both were operated on bilaterally. Bilateral elongated C7 transverse processes were found in two cases, and asymmetric cervical
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ribs in two, but all four underwent unilateral operations. One patient with bilateral symptoms was found to have a complete cervical rib on the most affected side, and an elongated C7 transverse process on the other. In one patient a hypoplastic first rib was fused anteriorly with the second. The palpable clavicular deformity was associated with mild hypoplasia and synchondrosis of the two upper ribs. The other noteworthy congenital malformation was a partial butterfly vertebra at C5-6, with fusion and a single large foramen on the affected side. A clavicular callus from a previous fracture was noted on the less affected side in the same patient. No brachial plexus impingement was found on imaging. One patient underwent magnetic resonance angiography and was found to have ipsilateral subclavian artery nominal irregularity at rest and significant constriction with shoulder abduction. Another patient underwent computed-tomography angiography and was found to have considerable superior vena cava thrombosis extending to the right subclavian vein which was deemed to be likely the result of compression of the vein. Only three patients underwent neurophysiological testing and in only one of these were findings suggestive of TOS.
Table 3 Effect of surgery on severity of symptoms produced by postural provocation by shoulder movement, as assessed by questionnaire at follow-up Postural provocation
Elevation Flexion Extension Depression
QFU 95% CI
6.2* 5.0* 4.8* 5.3*
4.7–7.6 3.8–6.3 3.0–3.9 3.9–6.8
1.6 1.2 0.6 1.8
QFU = questionnaire follow-up. p < 0.001.
*
Table 4 Change and status of activity impairment according to questionnaire follow-up Activity
Basic daily living Work/housework Study Sport/recreation Overall
3.3. Operative findings and outcomes All patients underwent operative management. Ten had a trial of conservative management for a minimum of 1 month after surgical assessment with a course of shoulder ‘shrugging and bracing’ exercises. Other conservative measures included shoulder bracing or postural taping (seven cases), massage (three cases), osteopathy (two cases), and Botox injections (one case). Although most reported the effects of these conservative measures favourably (satisfaction 2 to 9, mean 5.9), all patients required surgical intervention because of continuing, or worsening symptoms. Five patients underwent bilateral procedures, and there were two separate re-operations, yielding a total of 20 procedures. Age range at first operation was 10–19 years (mean 15), with five patients operated on before their 15th birthday. All patients elected for surgery before the age of 18, and the majority of procedures were undertaken at the Royal Children’s Hospital, Melbourne. SCS was performed in 17 cases and TARR in three (two being re-operations). In bilateral cases, the second side was operated on 2–9 months after the first (mean 5.8 months). The two re-operations occurred 13 and 55 months following the primary procedure. In the case re-operated at 55 months the patient was bedridden and immobile under the care of the paediatric medical team with illness, attributed to chronic fatigue syndrome, for 2 years post scalenectomy. Inpatient stay ranged from 1 to 4 nights (mean 2.6). Cervical ribs were resected in four cases, as were fused ribs where these occurred. The anterior scalene muscle was divided in every supraclavicular procedure, and in seven SCS procedures a fibrous band was found and divided. In six SCS procedures the anterior scalene was reported to be thick, fibrous, or otherwise impinging upon the neurovascular structures, with visible distortion of the subclavian artery in one case. In the single primary TARR case the first rib was broad and difficult to resect, and the middle trunk of the brachial plexus oedematous and swollen. A further case featured anomalous vasculature with the subclavian artery not being visualized. No major complications occurred. Minor temporary complications with SCS included three instances of significant post-operative pain, one of hypersensitivity around the scar, and one of C8 paraesthesia. In the latter two cases the pleural cavity had been breached, although no clinically or radiologically apparent pneumothorax ensued. These complications correlated strongly with bone resection (p = 0.0097) and the only complication with simple scalenotomy was a temporary increase in shoulder pain postoperatively.
Change Mean
Change
QFU
Mean
95% CI
5.8* 3.9* 4.2* 6.2* 5.7*
4.5–7.1 2.5–5.3 2.5–6.0 4.7–7.6 4.6–6.8
1.0 1.4 1.5 1.7 1.9
QFU = questionnaire follow-up. p < 0.001.
*
Surgical outcomes in terms of pain relief were variable. The overall mean change in pain in the first post-operative week was a decrease in severity of 2.6 (p = 0.003, 95% CI 1.0–4.2). Pain relief in the first post-operative week occurred in 17 patients; in three patients who reported increased pain, the pain continued until the questionnaire follow-up, with two patients regressing to their pre-operative states. This is in contrast to four patients (6 limbs) who experienced substantial (between 5 and 8) improvement in the same period. At questionnaire follow-up, pain relief from all 20 operations varied widely, from 0 to 9 (mean 4.2, p < 0.001, 95% CI 2.5–5.8). Both re-operated patients experienced long-term pain relief (changes of 5 and 7) and total pain relief occurred in seven patients (10 limbs) long term, although four patients reported significant residual pain at questionnaire follow-up (range 6–8). Sensory symptoms were better resolved, with nine patients (11 limbs) free of numbness and ten patients (14 limbs) free of paraesthesia, representing cure rates of 79% (p = 0.044), and 78% (p = 0.023), respectively. Results in terms of motor deficit were mediocre, with six patients (8 limbs) restored to full strength, a cure rate of 53%. Spasm or cramping proved more intractable, with only two out of six limbs cured. The lone case of tremor persisted. More positively, scalenotomies eradicated fatigue, heaviness, swelling, and cyanosis. One case of ‘coldness’ and pallor remained. Functional and postural outcomes also improved with surgery. Postural provocation improvements (Table 3) ranged from a mean of 4.8 for shoulder extension to 6.2 for arm elevation (p < 0.001), the greatest improvement in activities (Table 4) being seen in sporting activities (mean 6.2, p < 0.001, CI 4.7–7.6). Slight deterioration in sport was seen in only one patient. Overall patient satisfaction with surgical outcome was high. For the primary procedures this ranged from 5 to 10 on the VAS (mean 8.4, p < 0.001, 95% CI 7.6–9.3), while both re-operations scored 9 (and the original procedures 6). For all operations combined, the mean was 8.5 (p < 0.001, 95% CI 7.7–9.3). Of the 20 operations, 75% scored 8 or more and can be considered unequivocally successful at 6–96 months post-operatively (p = 0.03). With one exception, all patients said they would elect to undergo surgery were the choice to be given again (p < 0.001).
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4. Discussion This study, though retrospective and lacking a conservativelymanaged comparison group, represents the largest operated group of paediatric TOS patients reported to date. It made use of singlesurgeon 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 occurrence of posture provocation. Sporting participation was high and it may be that the repetitive use of the upper limbs, especially in outstretched or elevated positions, contributes to TOS. However, given the nationality and age of the patient population, participation in sporting activities may have been expected. The prevalence of cervical ribs or an elongated C7 transverse process (72% of the operated limbs) was higher than in the general population and in adult studies. The presence of other malformations, including fused upper ribs and synbrachydactyly, puts the total of limbs with an ipsilateral anomaly in this series at 83%. Neurophysiological testing was not carried out in the majority of patients in the series. The operating surgeon (DW) has long held the view that electrophysiological testing would be most useful if carried out with the arm in the elevated position. As neurophysiological testing is routinely carried out with the arm by the patient’s side it has been found by the operating surgeon to be of little help in diagnosing TOS. Sensory symptoms of numbness and paraesthesia showed greater improvements with surgery than did pain and motor deficits. Almost all vascular symptomatology resolved with surgery, possibly reflecting the lack of truly severe vascular compromise in this series (most cases would be classified as neurogenic, with some instances of mild-to-moderate vascular involvement), although it may also reflect a greater resilience of vascular structures compared to neural structures.13,16,17,21–23 None of the factors examined for predictability of surgical outcome, including age at presentation, gender, and provocative postures, was found to be significant. Both EAST and Allen’s manoeuvre had poor predictive value, though in eight patients post-operative EAST testing correlated with improvement or recurrence of symptoms. Operative outcomes were generally similar or superior to those of earlier studies.13–23,26 Despite residual symptoms in most of the operated limbs (67%), the majority of operations were deemed successful by both patients and observers. This may represent the significant improved mobility of these limbs post-operatively which was more important to the patients’ quality of life. 5. 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 early onset of TOS. References 1. Peet RM, Henriksen JD, Anderson TP, et al. Thoracic outlet syndrome: evaluation of a therapeutic exercise program. Proceedings of the staff meetings. Mayo Clin 1956;31:281–7. 2. Lindgren KA, Oksala I. Long-term outcome of surgery for thoracic outlet syndrome. Am J Surg 1995;169:358–60. 3. Atasoy E. Thoracic outlet compression syndrome. Orthop Clin North Am 1996;27:265–303. 4. Watson LA, Pizzari T, Balster S. Thoracic outlet syndrome part 1: clinical manifestations, differentiation and treatment pathways. Man Ther 2009;14:586–95. 5. Riddell DH, Smith BM. Thoracic and vascular aspects of thoracic outlet syndrome. Clin Orthop Relat Res 1986;207:31–6. 6. Shukla PC, Carlton FB. Diagnosis of thoracic outlet syndrome in the emergency department. South Med J 1996;89:212–7. 7. Sheth RN, Belzberg AJ. Diagnosis and treatment of thoracic outlet syndrome. Neurosurg Clin N Am 2001;15:32–6. 8. Wilbourn AJ. The thoracic outlet syndrome is over diagnosed. Arch Neurol 1990;47:328–30. 9. Cakmur R, Idiman F, Akalin E, et al. Dermatomal and mixed somatosensory evoked potentials in the diagnosis of neurogenic thoracic outlet syndrome. Electroencenpalogr Clin Neurophysiol 1998;108:423–34. 10. Yanaka K, Asakawa H, Matsumaru Y, et al. Diagnosis of vascular compression at the thoracic outlet using magnetic resonance angiography. Eur Neurol 2004;51:122–3. 11. Povlsen B, Belzberg A, Hansson T, et al. Treatment for thoracic outlet syndrome. Cochrane Database Syst Rev 2010;1:CD007218. 12. Edwards DP, Mulkern E, Raja AN, et al. Trans-axillary first rib excision for thoracic outlet syndrome. J R Coll Surg Edinburgh 1999;6:362–5. 13. Vaksmann G, Noblet D, Dupuis C. Subclavian artery aneurysm secondary to a cervical supernumerary rib in a child. Eur J Paediatr 1987;146:209–10. 14. Yang J, Letts M. Thoracic outlet syndrome in children. J Paediatr Orthop 1996;16:514–7. 15. DiFiore JW, Reid JR, Drummond-Webb J. Thoracic outlet syndrome in a child– transaxillary resection of anomalous first rib. J Pediatr Surg 2002;37:1220–2. 16. Reid JR, Morrison SC, DiFiore JW. Thoracic outlet syndrome with subclavian aneurysm in a very young child: the complimentary value of MRA and 3D-CT in diagnosis. Pediatr Radiol 2002;32:22–4. 17. Vercellio G, Baraldini V, Gatti C, et al. Thoracic outlet syndrome in paediatrics: clinical presentation, surgical treatment and outcome in a series of eight patients. J Paediatr Surg 2003;38:58–61. 18. 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;10:634–40. 19. Swierczyn´ska A, Khisek R, Kroczka S. Neurorehabilitation in children with thoracic outlet syndrome and its assessment. Przegl Lek 2005;62:1308–13. 20. Cagli K, Ozcakar L, Beyazit M. Thoracic outlet syndrome in an adolescent with bilateral bifid ribs. Clin Anat 2006;19:558–60. 21. Sen S, Disßçigil B, Boga M, et al. Thoracic outlet syndrome with right subclavian artery dilatation in a child – transaxillary resection of the pediatric cervical rib. Thorac Cardiovasc Surg 2007;55:339–41. 22. Maru S, Dosluoglu D, Dryski M, et al. Thoracic outlet syndrome in children and young adults. Eur J Vasc Endovasc Surg 2009;38:560–4. 23. Arthur LG, Teich S, Hogan M, et al. Pediatric thoracic outlet syndrome: a disorder with serious vascular complications. J Pediatr Surg 2009;43:1089–94. 24. Casbas L, Chauffour X, Cau J, et al. Post-traumatic thoracic outlet syndromes. Ann Vasc Surg 2005;19:25–8. 25. Abe M, Ichinie K, Nishida J. Diagnosis, treatment and complications of thoracic outlet syndrome. J Orthop Sci 1999;2:66–9. 26. 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 Plexus Peripher Nerv Inj 2009;4:14–20.
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Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Clinical Study
Surgery for paediatric thoracic outlet syndrome P.J. Teddy a,⇑, R.D. Johnson a, R.R. Cai a, D. Wallace a,b a b
Department of Surgery, Royal Melbourne Hospital, Grattan Street, Parkville, Melbourne, Victoria 3050, Australia Royal Childrens’s Hospital, Flemington Road, Parkville, Melbourne, Victoria 3052, Australia
a r t i c l e
i n f o
Article history: Received 5 March 2011 Accepted 25 May 2011
Keywords: Thoracic outlet syndrome Surgery Cervical rib
a b s t r a c t 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. Crown Copyright Ó 2011 Published by Elsevier Ltd. All rights reserved.
1. 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 conten-
⇑ Corresponding author. E-mail address: [email protected] (P.J. Teddy).
tious.13–23 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.
0967-5868/$ - see front matter Crown Copyright Ó 2011 Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.jocn.2011.05.015
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Table 1 Summary of paediatric thoracic outlet (TOS) syndrome patients undergoing surgery, reported in the literature (only patients <18 years of age included) Study
No. of patients
Age (years)
Sex
Clinical details
Procedures
Outcomes
Vaksmann et al. (1987)
1
9
M
Cervical rib with associated subclavian artery aneurysm
None
Not specified
Yang and Letts (1966)
4
12–13
F
Cervical ribs in 2 patients
TARR in 2 cases with cervical rib
Complete resolution of symptoms in all cases
DiFiore et al. (2002)
1
13
M
Neurogenic TOS
–
–
Vercellio et al. (2003)
8
8–16
M:F 1:1
5 patients: venous TOS
TARR in 4 cases of venous TOS
Successful resolution in all cases
2 patients: neurogenic TOS
SCS and partial rib resection in neurogenic TOS
1 patients: both forms of TOS Okamoto et al. (2005)
1
13
M
First rib hypoplasia Evidence of concomitant carpal tunnel and Guyon0 s canal syndromes
TARR, SCS, and decompression of carpal tunnel and Guyon’s canal
Symptoms resolved immediately post-op, but no further follow-up reported
Swierczyn´ska et al. (2005)
3
12–14
F
Mixed vascular and neurogenic TOS in all cases
TARR in 1 case The other cases underwent rehabilitation
Successful outcomes in all cases
Cagli et al. (2006)
1
13
M
Bilateral bifid ribs
Supraclavian resection of bifid ribs
Resolution of symptoms reported
Sen et al. (2007)
1
8
M
Mixed venous and neurogenic TOS in a patient with a cervical rib
TARR and resection of cervical rib
Resolution of symptoms reported
Arthur et al. (2006)
25
12–18 (mean 15.3)
18F, 7M
11 patients: venous TOS 2 patients: arterial TOS 11 patients neurologic TOS 1 patient: both forms of TOS
TARR in 24 cases; 5 patients underwent thrombolysis for subclavian vein thrombosis
Follow-up limited to 11 patients Complete relief in 6 cases Improvement in 1 case No change/recurrence in 4 cases
Maru et al. (2009)
9
12–16 (mean 17.3)
F:M 2:1
5 patients: arterial TOS 2 patients: venous TOS 2 patients neurogenic TOS Cervical ribs in 2 patients (one bilaterally) Subclavian aneurysms in 1 patient
SCS in all patients
Resolution of symptoms in all patients at outpatient follow-up (mean 10.9 weeks)
Dahlin et al. (2009)
2
13.5
F
Cervical ribs
Supraclavicular resection of cervical rib
Complete resolution of symptoms
F = female, M = male, SCS = supraclavicular scalenotomy, TARR = transaxillary resection of the first rib.
2. Methods
2.2. Surgical technique
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.
Two operative procedures were carried out: (i) anterior scalenotomy through a supraclavicular approach; (ii) a transaxillary approach with resection of the first rib (TARR) if scar prominence needed to be minimized or if scalenotomy failed. For supraclavicular scalenotomies (SCS) the patient was in the supine position with the head slightly elevated and rotated contralaterally. An approximately 5 cm transverse incision was made 1 cm above and parallel to the clavicle. Dissection of subcutaneous fat, platysma and deep cervical fascia exposed the clavicular head of the sternocleidomastoid which was then divided. The underlying omohyoid fascia was then divided and the omohyoid muscle retracted, or occasionally divided. The scalene fat pad was also dissected, and other intruding vessels retracted. Scalenotomy was carried out in stages with small fascicles of muscle being coagulated by bipolar diathermy and then divided by microscissors. Complete resection of the muscle was confirmed when the subclavian artery and brachial plexus roots and trunks were not otherwise obscured. Digital palpation excluded any remaining anatomical factors impinging on the artery or plexus. Any bony structures or spaceoccupying lesions were resected and any soft-tissue bands divided. Care was taken to avoid injury to the apex of the lung or Sibson’s fascia medially. When all compressive factors were alleviated, immaculate haemostasis was achieved, and vicryl sutures were used for muscles and nylon subcuticular sutures for the skin.
2.1. Data Information was gathered electronically about the presence, site, quality, and severity of pain pre-operatively, post-operatively and at the current time, and about other TOS symptoms both before surgery and currently. Other data included handedness, general health, and ratings of overall satisfaction outcome. Pain severity was assessed by a visual analogue scale score (VAS); assessment of postural or functional impairment utilized a dropdown list (0 = no pain or impairment; 10 = the most severe pain imaginable or total impairment). A VAS was also used for outcome satisfaction (0 = least; 10 = most favourable). Other symptoms were assessed with a Yes or No selection, with space for elaboration. Pain site was determined by check boxes for each anatomical division of the limb, including each digit and the pectoral girdle.
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For the transaxillary approach the patients were also supine with a large sandbag under the scapula to elevate the ipsilateral side. The arm was suspended from a pulley support system with a variable counterweight (2–5 kg). An incision was made in the most medial axillary skin fold, and the axillary fat and deep fascia traversed with blunt dissection until the first rib was reached. The point at which the subclavian artery and brachial plexus cross the rib was used as the midpoint of the rib resection which was carried out with rongeurs. The neurovascular structures were then visualized or palpated to assess remaining compression. The edges of the resected bone were smoothed out and bone wax applied. After immaculate haemostasis, closure was carried out with vicryl to the deep layers and subcuticular nylon to the skin. Following scalenotomy and TARR, patients were hospitalised for 2–4 days to assess initial results and monitored for early postoperative complications. Skin sutures were removed 7–10 days post-operatively. Follow-up consultations were carried out at 2 months and then several more 6-monthly visits as required to assess residual symptoms.
3. 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. 3.1. Data analysis The data were analysed using the two-tailed sign test for qualitative comparisons. For one-sample numerical data, the t distribution was used as the model and the paired t-test applied. Fisher’s exact test was also used for two-variable data. 3.2. Clinical presentation and investigation Cases were referred from a general practitioner (four cases), specialist physicians (four cases), or other surgeons (five cases).
Two patients had major congenital disorders: one case of Osler-Weber-Rendu disease, and one case of ipsilateral synbrachydactyly. Three patients were involved in motor vehicle accidents prior to presentation, with symptoms being precipitated by the accident in two cases. One patient reported symptom onset after a sports-related injury, whilst four patients reported acute onset without any precipitating event. All but one patient was at school at the time of presentation, the other having left school and being employed in heavy manual work. All patients were involved in at least one sport requiring the use of elevated upper limbs: basketball, netball and volleyball in eight patients; swimming in four patients; tennis in two patients; and cricket in two patients. Symptom frequency was ascertained from medical records and from questionnaires. From the medical records it was determined that the mean time between onset of symptoms and first consultation with the operating surgeon was 28 months. The predominant symptom was pain, followed by paraesthesia, numbness, weakness, limb ‘coldness’, and heaviness (Fig. 1A). Only two patients reported cyanosis, one bilaterally, and one patient reported hand tremor. Two patients reported sensing a mass in the supraclavicular fossa. More symptoms were reported in the questionnaire, with paraesthesia being the commonest, followed by weakness, pain, and numbness (Fig. 1B). Pallor and swelling were also more commonly reported in the questionnaire than in the medical records. The distribution of pain was usually widespread in the upper limb, with the neck and digits being the commonest sites (Fig. 1C). Postural symptom provocation was reported by all but one patient in the questionnaire, the elevated position being most important, with severity ranging from 4 to 10 (mean 7.8). Functional deficits were also reported with the affected limb pulled downwards (range 6–10, mean 7.1), out in front (range 5–10, mean 6.2), or extended rearward (range 3–10, mean 5.4). All patients reported impairments in functional capabilities, with sport and recreation being the most affected in all patients (range 4–10, mean 7.5). Impairment was also reported in basic daily living (17 limbs in 12 patients, mean 6.8), and study (14 limbs in 11 patients, mean 5.8). Overall functional impairment ranged from 4 to 10 (mean 7.6). Examination findings were variable. The EAST result correlated positively with the affected limb(s) in 11 patients, although the
Table 2 List of participants and operations performed on 13 paediatric patients with thoracic outlet syndrome. Patient
Sex
Age at first consultation (years)
Onset before presentation (months)
Dominant hand
Side(s) affected
Side operated
Surgical approach
1 2 3 4
F M M F
17 11 16 14
15 25 18 24
R R R R
R L RL L>R
5
F
15
48
R
RL
6 7
F F
15 16
6 18
R R
R L>R
8
F
9
60
L
Equal
9
F
13
24
R
L>R
10 11 12
F F F
15 17 15
7 31 48
R R R
L L L>R
13
M
14
39
R
RL
R L R L R R R R L R L R L R L L L L R R
SCS SCS SCS SCS SCS SCS TARR SCS SCS SCS SCS SCS SCS SCS TARR TARR SCS SCS SCS SCS
Three patients suffered significant contralateral symptoms (all on the non-dominant side), but did not elect for surgery. F = female, L = left, M = male, R = right, SCS = supraclavicular scalenotomy; TARR = transaxillary resection of he first rib.
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Fig. 1. (A) Symptomatology recorded in the medical notes. (B) Symptomatology reported at questionnaire follow-up. (C) Distribution of pain.
less-affected limbs in bilateral cases were negative. One case recorded an outright negative result, while a bilateral case was equivocal. Motor deficits were found in seven patients (nine limbs) including substantial weakness and wasting of the interossei and muscles of the thenar and hypothenar eminences in one patient. Mild weakness of triceps was seen in four patients (five limbs), and of finger extension in three (four limbs). Sensory impairment was found in four patients (five limbs): one was diffuse in the forearm, two in the C8 dermatome, and the other within the C4 distribution (two limbs). Palpation revealed tender supraclavicular
protuberances as reported by the patients, and the tip of a cervical rib in another case. Restricted cervical movement was found in one patient, while another experienced pain on neck flexion. X-ray imaging was carried out in all 13 patients, MRI in nine, and CT in five. Cervical ribs or an elongated C7 transverse process were discovered in eight patients. One of these was unilateral, and occurred on the contralateral side. There were cervical ribs of equal size in two cases (one set complete, the other incomplete), and both were operated on bilaterally. Bilateral elongated C7 transverse processes were found in two cases, and asymmetric cervical
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ribs in two, but all four underwent unilateral operations. One patient with bilateral symptoms was found to have a complete cervical rib on the most affected side, and an elongated C7 transverse process on the other. In one patient a hypoplastic first rib was fused anteriorly with the second. The palpable clavicular deformity was associated with mild hypoplasia and synchondrosis of the two upper ribs. The other noteworthy congenital malformation was a partial butterfly vertebra at C5-6, with fusion and a single large foramen on the affected side. A clavicular callus from a previous fracture was noted on the less affected side in the same patient. No brachial plexus impingement was found on imaging. One patient underwent magnetic resonance angiography and was found to have ipsilateral subclavian artery nominal irregularity at rest and significant constriction with shoulder abduction. Another patient underwent computed-tomography angiography and was found to have considerable superior vena cava thrombosis extending to the right subclavian vein which was deemed to be likely the result of compression of the vein. Only three patients underwent neurophysiological testing and in only one of these were findings suggestive of TOS.
Table 3 Effect of surgery on severity of symptoms produced by postural provocation by shoulder movement, as assessed by questionnaire at follow-up Postural provocation
Elevation Flexion Extension Depression
QFU 95% CI
6.2* 5.0* 4.8* 5.3*
4.7–7.6 3.8–6.3 3.0–3.9 3.9–6.8
1.6 1.2 0.6 1.8
QFU = questionnaire follow-up. p < 0.001.
*
Table 4 Change and status of activity impairment according to questionnaire follow-up Activity
Basic daily living Work/housework Study Sport/recreation Overall
3.3. Operative findings and outcomes All patients underwent operative management. Ten had a trial of conservative management for a minimum of 1 month after surgical assessment with a course of shoulder ‘shrugging and bracing’ exercises. Other conservative measures included shoulder bracing or postural taping (seven cases), massage (three cases), osteopathy (two cases), and Botox injections (one case). Although most reported the effects of these conservative measures favourably (satisfaction 2 to 9, mean 5.9), all patients required surgical intervention because of continuing, or worsening symptoms. Five patients underwent bilateral procedures, and there were two separate re-operations, yielding a total of 20 procedures. Age range at first operation was 10–19 years (mean 15), with five patients operated on before their 15th birthday. All patients elected for surgery before the age of 18, and the majority of procedures were undertaken at the Royal Children’s Hospital, Melbourne. SCS was performed in 17 cases and TARR in three (two being re-operations). In bilateral cases, the second side was operated on 2–9 months after the first (mean 5.8 months). The two re-operations occurred 13 and 55 months following the primary procedure. In the case re-operated at 55 months the patient was bedridden and immobile under the care of the paediatric medical team with illness, attributed to chronic fatigue syndrome, for 2 years post scalenectomy. Inpatient stay ranged from 1 to 4 nights (mean 2.6). Cervical ribs were resected in four cases, as were fused ribs where these occurred. The anterior scalene muscle was divided in every supraclavicular procedure, and in seven SCS procedures a fibrous band was found and divided. In six SCS procedures the anterior scalene was reported to be thick, fibrous, or otherwise impinging upon the neurovascular structures, with visible distortion of the subclavian artery in one case. In the single primary TARR case the first rib was broad and difficult to resect, and the middle trunk of the brachial plexus oedematous and swollen. A further case featured anomalous vasculature with the subclavian artery not being visualized. No major complications occurred. Minor temporary complications with SCS included three instances of significant post-operative pain, one of hypersensitivity around the scar, and one of C8 paraesthesia. In the latter two cases the pleural cavity had been breached, although no clinically or radiologically apparent pneumothorax ensued. These complications correlated strongly with bone resection (p = 0.0097) and the only complication with simple scalenotomy was a temporary increase in shoulder pain postoperatively.
Change Mean
Change
QFU
Mean
95% CI
5.8* 3.9* 4.2* 6.2* 5.7*
4.5–7.1 2.5–5.3 2.5–6.0 4.7–7.6 4.6–6.8
1.0 1.4 1.5 1.7 1.9
QFU = questionnaire follow-up. p < 0.001.
*
Surgical outcomes in terms of pain relief were variable. The overall mean change in pain in the first post-operative week was a decrease in severity of 2.6 (p = 0.003, 95% CI 1.0–4.2). Pain relief in the first post-operative week occurred in 17 patients; in three patients who reported increased pain, the pain continued until the questionnaire follow-up, with two patients regressing to their pre-operative states. This is in contrast to four patients (6 limbs) who experienced substantial (between 5 and 8) improvement in the same period. At questionnaire follow-up, pain relief from all 20 operations varied widely, from 0 to 9 (mean 4.2, p < 0.001, 95% CI 2.5–5.8). Both re-operated patients experienced long-term pain relief (changes of 5 and 7) and total pain relief occurred in seven patients (10 limbs) long term, although four patients reported significant residual pain at questionnaire follow-up (range 6–8). Sensory symptoms were better resolved, with nine patients (11 limbs) free of numbness and ten patients (14 limbs) free of paraesthesia, representing cure rates of 79% (p = 0.044), and 78% (p = 0.023), respectively. Results in terms of motor deficit were mediocre, with six patients (8 limbs) restored to full strength, a cure rate of 53%. Spasm or cramping proved more intractable, with only two out of six limbs cured. The lone case of tremor persisted. More positively, scalenotomies eradicated fatigue, heaviness, swelling, and cyanosis. One case of ‘coldness’ and pallor remained. Functional and postural outcomes also improved with surgery. Postural provocation improvements (Table 3) ranged from a mean of 4.8 for shoulder extension to 6.2 for arm elevation (p < 0.001), the greatest improvement in activities (Table 4) being seen in sporting activities (mean 6.2, p < 0.001, CI 4.7–7.6). Slight deterioration in sport was seen in only one patient. Overall patient satisfaction with surgical outcome was high. For the primary procedures this ranged from 5 to 10 on the VAS (mean 8.4, p < 0.001, 95% CI 7.6–9.3), while both re-operations scored 9 (and the original procedures 6). For all operations combined, the mean was 8.5 (p < 0.001, 95% CI 7.7–9.3). Of the 20 operations, 75% scored 8 or more and can be considered unequivocally successful at 6–96 months post-operatively (p = 0.03). With one exception, all patients said they would elect to undergo surgery were the choice to be given again (p < 0.001).
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4. Discussion This study, though retrospective and lacking a conservativelymanaged comparison group, represents the largest operated group of paediatric TOS patients reported to date. It made use of singlesurgeon 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 occurrence of posture provocation. Sporting participation was high and it may be that the repetitive use of the upper limbs, especially in outstretched or elevated positions, contributes to TOS. However, given the nationality and age of the patient population, participation in sporting activities may have been expected. The prevalence of cervical ribs or an elongated C7 transverse process (72% of the operated limbs) was higher than in the general population and in adult studies. The presence of other malformations, including fused upper ribs and synbrachydactyly, puts the total of limbs with an ipsilateral anomaly in this series at 83%. Neurophysiological testing was not carried out in the majority of patients in the series. The operating surgeon (DW) has long held the view that electrophysiological testing would be most useful if carried out with the arm in the elevated position. As neurophysiological testing is routinely carried out with the arm by the patient’s side it has been found by the operating surgeon to be of little help in diagnosing TOS. Sensory symptoms of numbness and paraesthesia showed greater improvements with surgery than did pain and motor deficits. Almost all vascular symptomatology resolved with surgery, possibly reflecting the lack of truly severe vascular compromise in this series (most cases would be classified as neurogenic, with some instances of mild-to-moderate vascular involvement), although it may also reflect a greater resilience of vascular structures compared to neural structures.13,16,17,21–23 None of the factors examined for predictability of surgical outcome, including age at presentation, gender, and provocative postures, was found to be significant. Both EAST and Allen’s manoeuvre had poor predictive value, though in eight patients post-operative EAST testing correlated with improvement or recurrence of symptoms. Operative outcomes were generally similar or superior to those of earlier studies.13–23,26 Despite residual symptoms in most of the operated limbs (67%), the majority of operations were deemed successful by both patients and observers. This may represent the significant improved mobility of these limbs post-operatively which was more important to the patients’ quality of life. 5. 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 early onset of TOS. References 1. Peet RM, Henriksen JD, Anderson TP, et al. Thoracic outlet syndrome: evaluation of a therapeutic exercise program. Proceedings of the staff meetings. Mayo Clin 1956;31:281–7. 2. Lindgren KA, Oksala I. Long-term outcome of surgery for thoracic outlet syndrome. Am J Surg 1995;169:358–60. 3. Atasoy E. Thoracic outlet compression syndrome. Orthop Clin North Am 1996;27:265–303. 4. Watson LA, Pizzari T, Balster S. Thoracic outlet syndrome part 1: clinical manifestations, differentiation and treatment pathways. Man Ther 2009;14:586–95. 5. Riddell DH, Smith BM. Thoracic and vascular aspects of thoracic outlet syndrome. Clin Orthop Relat Res 1986;207:31–6. 6. Shukla PC, Carlton FB. Diagnosis of thoracic outlet syndrome in the emergency department. South Med J 1996;89:212–7. 7. Sheth RN, Belzberg AJ. Diagnosis and treatment of thoracic outlet syndrome. Neurosurg Clin N Am 2001;15:32–6. 8. Wilbourn AJ. The thoracic outlet syndrome is over diagnosed. Arch Neurol 1990;47:328–30. 9. Cakmur R, Idiman F, Akalin E, et al. Dermatomal and mixed somatosensory evoked potentials in the diagnosis of neurogenic thoracic outlet syndrome. Electroencenpalogr Clin Neurophysiol 1998;108:423–34. 10. Yanaka K, Asakawa H, Matsumaru Y, et al. Diagnosis of vascular compression at the thoracic outlet using magnetic resonance angiography. Eur Neurol 2004;51:122–3. 11. Povlsen B, Belzberg A, Hansson T, et al. Treatment for thoracic outlet syndrome. Cochrane Database Syst Rev 2010;1:CD007218. 12. Edwards DP, Mulkern E, Raja AN, et al. Trans-axillary first rib excision for thoracic outlet syndrome. J R Coll Surg Edinburgh 1999;6:362–5. 13. Vaksmann G, Noblet D, Dupuis C. Subclavian artery aneurysm secondary to a cervical supernumerary rib in a child. Eur J Paediatr 1987;146:209–10. 14. Yang J, Letts M. Thoracic outlet syndrome in children. J Paediatr Orthop 1996;16:514–7. 15. DiFiore JW, Reid JR, Drummond-Webb J. Thoracic outlet syndrome in a child– transaxillary resection of anomalous first rib. J Pediatr Surg 2002;37:1220–2. 16. Reid JR, Morrison SC, DiFiore JW. Thoracic outlet syndrome with subclavian aneurysm in a very young child: the complimentary value of MRA and 3D-CT in diagnosis. Pediatr Radiol 2002;32:22–4. 17. Vercellio G, Baraldini V, Gatti C, et al. Thoracic outlet syndrome in paediatrics: clinical presentation, surgical treatment and outcome in a series of eight patients. J Paediatr Surg 2003;38:58–61. 18. 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;10:634–40. 19. Swierczyn´ska A, Khisek R, Kroczka S. Neurorehabilitation in children with thoracic outlet syndrome and its assessment. Przegl Lek 2005;62:1308–13. 20. Cagli K, Ozcakar L, Beyazit M. Thoracic outlet syndrome in an adolescent with bilateral bifid ribs. Clin Anat 2006;19:558–60. 21. Sen S, Disßçigil B, Boga M, et al. Thoracic outlet syndrome with right subclavian artery dilatation in a child – transaxillary resection of the pediatric cervical rib. Thorac Cardiovasc Surg 2007;55:339–41. 22. Maru S, Dosluoglu D, Dryski M, et al. Thoracic outlet syndrome in children and young adults. Eur J Vasc Endovasc Surg 2009;38:560–4. 23. Arthur LG, Teich S, Hogan M, et al. Pediatric thoracic outlet syndrome: a disorder with serious vascular complications. J Pediatr Surg 2009;43:1089–94. 24. Casbas L, Chauffour X, Cau J, et al. Post-traumatic thoracic outlet syndromes. Ann Vasc Surg 2005;19:25–8. 25. Abe M, Ichinie K, Nishida J. Diagnosis, treatment and complications of thoracic outlet syndrome. J Orthop Sci 1999;2:66–9. 26. 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 Plexus Peripher Nerv Inj 2009;4:14–20.