Thoracic Outlet Syndrome in the Pediatric and Young Adult Population.

PURPOSE: This study aimed to assess both nonsurgical and operative treatment outcomes of pediatric and young adult patients with thoracic outlet syndrome (TOS) at a tertiary care pediatric hospital. METHODS: A retrospective chart review of patients diagnosed with TOS, who were seen between January 2010 and August 2022 at a tertiary care pediatric hospital, was conducted. Collected pre- and postoperative data included symptoms, provocative testing (ie, Roo's, Wright's, and Adson's tests), participation in sports or upper-extremity activities, additional operations, and surgical complications. Assessment of operative treatment efficacy was based on pre- and post-provocative testing, pain, venogram results, alleviation of symptoms, and return to previous activity level 6 months after surgery. RESULTS: Ninety-six patients, (70 females and 26 males) with an average age at onset of 15 ± 4 (4-25) years, met the inclusion criteria for TOS. Among them, 27 had neurogenic TOS, 29 had neurogenic and vasculogenic TOS, 20 had vasculogenic TOS, 19 had Paget-Schroetter Syndrome, and one was asymptomatic. Twenty-six patients were excluded because of less than 6 months of follow-up. Of the remaining 70, 6 (8.6%) patients (4 bilateral and 2 unilateral) underwent nonoperative management with activity modification and physical therapy only, and one was fully discharged because of complete relief of symptoms. Sixty-four (90.1%) patients (45 bilateral and 19 unilateral) underwent surgery. A total of 102 operations were performed. Substantial improvements were observed in provocative maneuvers after surgery. Before surgery, 79.7% were involved in sports or playing musical instruments with repetitive overhead activity, and after surgery, 86.2% of these patients returned to their previous activity level. CONCLUSIONS: Few patients were successfully managed with nonoperative activity modification and physical therapy. In those requiring surgical intervention, first or cervical rib resection with scalenectomy using a supraclavicular approach provided resolution of symptoms with 86.2% of patients being able to return to presymptom sport or activity level. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Brachial plexus birth injury and cerebral palsy lead to a common contracture phenotype characterized by reduced functional muscle length and strength.

Introduction: Brachial plexus birth injury (BPBI) and cerebral palsy (CP) both cause disabling contractures for which no curative treatments exist, largely because contracture pathophysiology is incompletely understood. The distinct neurologic nature of BPBI and CP suggest different potential contracture etiologies, although imbalanced muscle strength and insufficient muscle length have been variably implicated. The current study directly compares the muscle phenotype of elbow flexion contractures in human subjects with BPBI and CP to test the hypothesis that both conditions cause contractures characterized by a deficit in muscle length rather than an excess in muscle strength. Methods: Subjects over 6 years of age with unilateral BPBI or hemiplegic CP, and with elbow flexion contractures greater than 10 degrees on the affected side, underwent bilateral elbow flexion isokinetic strength testing to identify peak torque and impulse, or area under the torque-angle curve. Subjects then underwent needle microendoscopic sarcomere length measurement of bilateral biceps brachii muscles at symmetric joint angles. Results: In five subjects with unilateral BPBI and five with hemiplegic CP, peak torque and impulse were significantly lower on the affected versus unaffected sides, with no differences between BPBI and CP subjects in the percent reduction of either strength measurement. In both BPBI and CP, the percent reduction of impulse was significantly greater than that of peak torque, consistent with functionally shorter muscles. Similarly, in both conditions, affected muscles had significantly longer sarcomeres than unaffected muscles at symmetric joint angles, indicating fewer sarcomeres in series, with no differences between BPBI and CP subjects in relative sarcomere overstretch. Discussion: The current study reveals a common phenotype of muscle contracture in BPBI and CP, with contractures in both conditions characterized by a similar deficit in muscle length rather than an excess in muscle strength. These findings support contracture treatments that lengthen rather than weaken affected muscles. Moreover, the discovery of a common contracture phenotype between CP and BPBI challenges the presumed dichotomy between upper and lower motor neuron lesions in contracture pathogenesis, instead revealing the broader concept of "myobrevopathy", or disorder of short muscle, warranting increased investigation into the poorly understood mechanisms regulating muscle length.

Brachial plexus birth injuries and the association between pre-procedure and post-procedure pediatric outcomes data collection instrument scores and narakas classification.

BACKGROUND: The brachial plexus is a network of nerves exiting the spinal cord through the fifth, sixth, seventh, and eighth cervical nerves (C5-C8) as well as the first thoracic nerve (T1) to conduct signals for motion and sensation throughout the arm. Brachial plexus birth injuries (BPBI) occur in 1.5 per 1,000 live births. The purpose of this study was to determine the perceived change in musculoskeletal health-related quality of life of brachial plexus patients utilizing the Pediatric Outcomes Data Collection Instrument (PODCI). PODCI scores were examined along with the patient's procedure history (surgical or Botulinum Toxin), extent of involvement and demographics. PATIENTS: A total of 81 patients from two to eighteen years of age from nine different states met the inclusion criteria of having a pre-procedure and post-procedure PODCI score along with a Narakas score from 2002-2017. These patients were seen at the Brachial Plexus Center, which is an interdisciplinary clinic at a large academic medical centerMETHODS: This retrospective study utilized PODCI data collected annually during their regular brachial plexus clinic visits. Upper extremity (UE) and global functioning (GFx) scores pre- and post-procedure were stratified by Narakas Classification. Data were analyzed using paired t-test and ANOVA testing. RESULTS: Patients with a Brachial Plexus Birth Injury (BPBI) had lower PODCI scores for UE and GFx when compared with the pediatric normative scores for age-matched healthy children. Scores in both UE and GFx domains were higher after procedure in the groups of Narakas I and IV. There was significant correlation between UE and GFx scores and documented first PODCI score (2 years of age) and age at intervention (5 years of age). CONCLUSION: Procedures increased the perceived quality of life for children with a BPBI and increased their overall PODCI scores for both UE and GFx.

The impact of pediatric brachial plexus injury on families.

PURPOSE: To determine the impact of brachial plexus injuries on families to best meet their clinical and social needs. METHODS: Our cross-sectional study included families with children between the ages of 1 and 18 years with birth or non-neonatal brachial plexus injuries (BPIs). The consenting parent or guardian completed a demographic questionnaire and the validated Impact on Family Scale during a single assessment. Total scores can range from 0 to 100, with the higher the score indicating a higher impact on the family. Factor analysis and item-total correlations were used to examine structure, individual items, and dimensions of family impact. RESULTS: A total of 102 caregivers participated. Overall, families perceived various dimensions of impact on having a child with a BPI. Total family impact was 43. The 2 individual items correlating most strongly with the overall total score were from the financial dimension of the Impact on Family Scale. The strongest demographic relationship was traveling nationally for care and treatment of the BPI. Severity of injury was marginally correlated with impact on the family. Parent-child agreement about the severity of the illness was relatively high. CONCLUSIONS: Caretakers of children with a BPI perceived impact on their families in the form of personal strain, family/social factors, financial stress, and mastery. A multidisciplinary clinical care team should address the various realms of impact on family throughout the course of treatment. TYPE OF STUDY/LEVEL OF EVIDENCE: Prognostic II.

Factors related to the psychosocial functioning of youth with neonatal brachial plexus injuries.

OBJECTIVE: Owing to the possible visible nature and functional impairments associated with neonatal brachial plexus injuries (NBPI), the current study investigated the relations of injury severity, social support, and coping strategies to social difficulties and self-concept in youth with NBPI. METHODS: 88 children (aged 10-17 years) with NBPI and their parent(s) were recruited from a national organization and two brachial plexus clinics. Participants completed a variety of questionnaires during their scheduled clinic visits. RESULTS: More social support from classmates was associated with better self-concept and fewer social difficulties. Less frequent use of negative coping strategies was associated with better self-concept and fewer social difficulties and was a significant moderator of the relation between injury severity and self-concept. CONCLUSIONS: Clinicians who work with children with NBPI should consider peer support and coping strategies when promoting the psychosocial functioning of these youth.

Use of botulinum toxin type A in the management of neonatal brachial plexus palsy.

OBJECTIVE: To evaluate functional outcomes and the impact on surgical interventions after the use of botulinum neurotoxin type A (BoNT-A) for muscle imbalance, cocontractions, or contractures with neonatal brachial plexus palsy. DESIGN: A retrospective cohort study. SETTING: A brachial plexus center in a tertiary children's hospital. PARTICIPANTS: Fifty-nine patients with neonatal brachial plexus palsy (75 injection procedures, 91 muscles and/or muscle groups) received BoNT-A injections (mean age at injection, 36.2 months; range, 6-123 months; 31 boys; 30 right-sided injuries, 28 left-sided injuries, 1 bilateral injury). METHODS: Data collected retrospectively from medical records, from procedure notes and clinic visits before BoNT-A use, at ≤6 months follow-up (BoNT-A active [BA]) and at ≥7 months follow-up (BoNT-A not active [BNA]) included demographics, injection indication, side, and site(s), previous surgical history, occupational therapy and/or physical therapy plan, and outcome measurements. MAIN OUTCOME MEASUREMENTS: Outcomes assessed before and after injections included active and passive range of motion, Mallet and Toronto scores, parent comments about arm function, preinjection surgical considerations, and postinjection surgical history. RESULTS: Injection procedures included 51 to shoulder internal rotators, 15 triceps, 15 pronator teres, 9 biceps, and 1 flexor carpi ulnaris. Active and passive shoulder external rotation (SER) range of motion improved after shoulder internal rotator injections (P = .0003 and P = .002, respectively), as did Mallet scores with BA; the latter were sustained with BNA. Surgical intervention was averted, modified, or deferred after BoNT-A in 45% (n = 20) under surgical consideration before BoNT-A. Active elbow flexion improved in 67% (P = .005), sustained BNA (P = .004) after triceps injections; 2 of 7 patients averted surgery. Active supination improved with BA (P = .002), with gains sustained BNA (P = .016). Passive elbow extension improved after biceps injections by an average 17° (P = .004) BA, although not sustained BNA. CONCLUSIONS: BoNT-A is an effective adjunct to therapy and surgery in managing muscle imbalance, cocontractions, and contractures in neonatal brachial plexus palsy. Use of BoNT-A can result in averting, modifying, or deferring surgical interventions in a number of affected children.

Arthroscopically assisted Sever-L'Episcopo procedure improves clinical and radiographic outcomes in neonatal brachial plexus palsy patients.

BACKGROUND: Muscle pathology resulting in internal rotation contractures in children with neonatal brachial plexus palsy places abnormal stresses on the glenohumeral joint and limits global shoulder function. The objective of this study was to assess the clinical and radiographic outcomes in children treated with an arthroscopic release with or without tendon transfer, the so-called arthroscopically assisted Sever-L'Episcopo procedure. METHODS: Fifty children with an average age of 5.1 years who underwent an arthroscopic release with or without tendon transfer were retrospectively reviewed. Clinical outcomes were assessed using Mallet classification scores, whereas glenoid retroversion and posterior humeral head subluxation were measured on magnetic resonance images to quantify radiographic outcomes. Mean clinical follow-up was 30 months (range: 24 to 65 mo) and mean radiographic follow-up was 24 months (range: 11 to 42 mo). RESULTS: Aggregate Mallet score improved significantly from 12.6 to 16.3 (P<0.0001), with shoulder abduction from 3.4 to 3.8 (P=0.0007), shoulder external rotation from 2.2 to 3.3 (P<0.0001), hand-to-neck from 2.3 to 3.2 (P<0.0001), and hand-to-mouth from 2.3 to 3.3 (P<0.0001). Hand-to-spine Mallet score did not significantly change from preoperative (2.4) to postoperative (2.6) (P=0.1348), although 4 patients experienced a loss in internal rotation function. Forty-eight percent of children improved by at least 4 points on the total Mallet score. Glenoid retroversion improved from 25 to 14.1 degrees (P<0.0001) and percent humeral head anterior to the central axis of the scapula increased from 30.5% to 38.8% (P=0.0001). Sixty-seven percent of patients demonstrated glenohumeral joint remodeling on magnetic resonance imaging. No child exhibited a worsening of glenohumeral anatomy. CONCLUSIONS: An arthroscopic release with or without tendon transfer is effective in reducing internal rotation contractures and increasing global shoulder function. Both clinical and radiographic outcomes were significantly improved at 2-year follow-up. Furthermore, in the majority of children, aggregate, abduction, and external rotation Mallet scores all increased without sacrificing internal rotation. LEVEL OF EVIDENCE: Therapeutic Level IV.