Age and sex Differences in Pediatric Neuropathic Pain and Complex Regional Pain Syndrome: A Scoping Review.

BACKGROUND: Age and sex differences may exist in the frequency (incidence, prevalence) or symptoms of neuropathic pain (NP) and complex regional pain syndrome (CRPS) due to biopsychosocial factors (e.g., neurodevelopment, physiological and hormonal changes, psychosocial differences) that evolve through childhood and adolescence.2 Age and sex differences may have implications for evaluating screening and diagnostic tools and treatment interventions. OBJECTIVE: To map the existing literature on pediatric NP and CRPS with respect to age and sex distributions, and age and sex differences in symptomology and frequency. METHODS: A scoping literature review was conducted. Databases were searched from inception to January 2023. Data were collected on study design, setting, demographics, and age and sex differences in frequency and symptoms. RESULTS: Eighty-seven studies were included. Distribution of participants with CRPS (n=37 studies) was predominantly early adolescence (10-14 years) and female sex, while NP (n=42 studies) was most commonly reported throughout adolescence (10-19 years) in both sexes. Forty-one studies examined age and sex differences in frequency; 6 studies reported higher frequency in adolescence. Very few studies (n=11) examined differences in symptomology. DISCUSSION: Large epidemiological studies are required to further understand age and sex differences in frequency of pediatric NP and CRPS. Age and sex differences must be considered when evaluating screening and diagnostic tools and treatment interventions to ensure relevance and validity to both sexes and across ages. Validated tools will improve understanding of age- and sex-dependent differences in symptoms, pathophysiology, and psychosocial impact of pediatric NP and CRPS.

Prevalence and predictors of elbow flexion contractures during early childhood following brachial plexus birth injury.

BACKGROUND: Elbow flexion contracture development in school-age children with a brachial plexus birth injury (BPBI) is common. Reports indicate onset between 2 and 4 years; however, little is known about early childhood prevalence, development, and trajectory of these contractures. PURPOSE: To determine the prevalence and predictors of BPBI elbow flexion contractures during early childhood. STUDY DESIGN: A retrospective cross-sectional study. METHODS: Demographic, diagnostic, treatment, and elbow contracture data were collected for children with a BPBI <4 years between 2015 and 2019 from a prospectively collected database. Spinal root motor contributions and injury were determined using Active Movement Scale (AMS) scores at 6 weeks of age and used to predict contracture development. RESULTS: Of the 171 children that met inclusion criteria, 87% (n = 149) had upper plexus injuries. The mean age at the time of evaluation for an elbow contracture was 21.4 ± 12.7 months. The prevalence of elbow flexion contractures was 22% (n = 38), with mean onset at 13.4 ± 11.0 months. Mean contracture degree was -10.8 ± -6.9 degrees with 76% (n = 29) <-10 degrees. AMS shoulder abduction, flexion, and external rotation; elbow flexion; forearm supination; and wrist extension scores at a mean 2.3 ± 1.4 months were significantly lower in children who developed elbow flexion contractures (p < 0.001). Logistic regression found that low AMS elbow flexion with high elbow extension scores were a significant (p < 0.003) predictor of elbow contracture development. CONCLUSIONS: The prevalence of elbow flexion contractures in early childhood is greater than previously understood. These findings indicate that C5-C6 injury affecting elbow flexion with relative preservation of elbow extension is a predictor of contracture development. Further research is needed to investigate the nature and sequelae of C5-C6 injury and its effects on elbow flexion contracture development.

Evaluating the feasibility of implementing the Sup-ER protocol for infants with brachial plexus birth injury.

BACKGROUND: The Sup-ER protocol involves a repositioning program for infants with brachial plexus birth injury to position the shoulder in external rotation (ER) to address progressive loss in passive range of motion (PROM). The British Columbia Children's Hospital (BCCH) eligibility criteria for this protocol are infants aged 4-8 weeks with decreased shoulder ER PROM and/or Active Movement Scale (AMS) shoulder ER and/or supination scores ≤2. The resources needed to implement this protocol in large clinics have not been studied. PURPOSE: This study aims to evaluate the BCCH criteria that are used to identify appropriate candidates for the Sup-ER protocol. STUDY DESIGN: A retrospective cohort study was conducted to identify the percentage of infants who would have been recommended the Sup-ER protocol based on their PROM and AMS scores between 4 and 8 weeks of age. METHODS: A sensitivity and specificity evaluation was used to describe the BCCH criteria's ability to identify infants in this historical cohort who went on to have incomplete shoulder function (ie, true positive) vs infants who had functional shoulder outcome at 9 months of age (ie, false positive). RESULTS: At a mean of 5.8 weeks (95% confidence interval [CI] 5.3, 6.3), 46 of the 87 (53%) infants satisfied the BCCH Sup-ER protocol criteria. Forty-four (51%) were female, half (n = 45) were left side affected, and 88% had upper plexus injury. The BCCH Sup-ER protocol criteria had sensitivity of 100% and specificity of 71% to identify infants with incomplete shoulder function. Removing the AMS supination ≤2 score criterion from the criteria improves the specificity to 84%, while sensitivity remains high (97%). CONCLUSIONS: Modifying the BCCH criteria to all infants aged 4-8 weeks with AMS shoulder ER ≤2 and/or decreased shoulder ER PROM improves the precision of identifying infants who would benefit from the Sup-ER protocol.

Surgery for internal rotation contracture in infancy may obviate the need for brachial plexus nerve reconstruction: early experience.

BACKGROUND: Shoulder internal rotation contracture and subluxation in the first year of life has long been recognized in some patients with brachial plexus birth injury (BPBI). Surgical management of shoulder pathology has traditionally been undertaken following nerve reconstruction as necessary. In some patients; however, shoulder pathology may impair or obscure functional neuromuscular recovery of the upper extremity. As a proof of concept, we report a highly selected subset of patients with BPBI in whom shoulder surgery undertaken before one year of age obviated the need for neuroma resection and nerve grafting. METHODS: A retrospective review was performed of all patients with upper trunk BPBI who underwent shoulder surgery before one year of age from 2015 to 2018. Upper extremity motor function was evaluated with preoperative and postoperative Active Movement Scale scores, Cookie tests, and the requirement for subsequent neuroma resection and nerve grafting. RESULTS: Fifteen patients with BPBI meeting the inclusion criteria underwent shoulder surgery (including a subscapularis slide and tendon transfers of the teres major and latissimus dorsi muscles) before 1 year of age. Preoperatively, no patients of the appropriate age passed the Cookie test for elbow flexion. Thirteen patients either passed the Cookie test or scored Active Movement Scale score 7 for elbow flexion at or before the last available follow-up undertaken at a median age of 3.4 [1.4, 5.2] years. One of those 13 patients underwent single fascicular distal nerve transfer to improve elbow flexion before subsequently passing the Cookie test. Two patients did not have sufficient follow-up to assess elbow flexion. CONCLUSION: Although the exact role of shoulder surgery in infancy for BPBI remains to be defined, the findings from this study provide proof of concept that early, targeted surgical treatment of the shoulder may obviate the need for brachial plexus nerve reconstruction in a highly selected group of infants with BPBI.

Mental health and psychosocial support for children with upper limb musculoskeletal conditions.

This study describes mental health and psychosocial screening processes, access to care and interventions provided to children with upper limb musculoskeletal conditions. A cross-sectional e-survey study was conducted of 107 healthcare professionals who work with children with congenital hand and upper limb differences and brachial plexus birth injuries. Of them, 41 (38%) reported that they routinely screen for mental health and psychosocial concerns. Few (12%) reported the use of standardized outcome measures. In total, 51 (48%) healthcare professionals reported that there was a waiting list for mental health services at their institution. Collectively, healthcare professionals were unsatisfied with the staffing, access to care and types of interventions available. Reported barriers to care included the growing need for mental health support, lack of resources and poor continuity of care after referrals. Future research should focus on identifying and validating a mental health screening tool and investigating the processes affecting access to mental health care.Level of evidence: IV.

Management of Brachial Plexus Birth Injury: The SickKids Experience.

This article describes the approach utilized by the multidisciplinary team at Sick Kids Hospital to evaluate and treat patients with brachial plexus birth injury (BPBI). This approach has been informed by more than 30 years of experience treating over 1,800 patients with BPBI and continues to evolve over time. The objective of this article is to provide readers with a practical overview of the Sick Kids approach to the management of infants with BPBI.

Elucidating children's understanding of brachial plexus birth injury.

Facilitating children's understanding of their medical condition can improve health outcomes and psychosocial well-being. To inform how medical information is delivered, an interpretive qualitative approach was used to explore children's understanding of their brachial plexus birth injury. In-depth interviews of children with brachial plexus birth injuries (n = 8) and their caregivers (n = 10) were conducted individually and as a child-caregiver dyad. Thematic analysis of interview data found that children primarily understood their injury through lived experiences of functional and psychosocial concerns related to movement and appearance of the affected limb, rather than medical information. Children's ability to learn about diagnostic and prognostic information was influenced by age, emotional readiness, and background knowledge. In receiving information about their medical condition, children needed greater support in understanding their prognosis and its implications on their future. These narratives indicate the importance of addressing the primary functional and psychosocial concerns to contextualize medical information and ascertain the emotional readiness of children with brachial plexus birth injuries in information delivery approaches.

Recent Advances in the Treatment of Brachial Plexus Birth Injury.

LEARNING OBJECTIVES: After studying this article, the participant should be able to: 1. Describe methods of clinical evaluation for neurologic recovery in brachial plexus birth injury. 2. Understand the role of different diagnostic imaging modalities to evaluate the upper limb. 3. List nonsurgical strategies and surgical procedures to manage shoulder abnormality. 4. Explain the advantages and disadvantages of microsurgical nerve reconstruction and distal nerve transfers in brachial plexus birth injury. 5. Recognize the prevalence of pain in this population and the need for greater sensory outcomes evaluation. SUMMARY: Brachial plexus birth injury (BPBI) results from closed traction injury to the brachial plexus in the neck during an infant's vertex passage through the birth canal. Although spontaneous upper limb recovery occurs in most instances of BPBI, some infants do not demonstrate adequate motor recovery within an acceptable timeline and require surgical intervention to restore upper limb function. This article reviews major advances in the management of BPBI in the past decade that include improved understanding of shoulder pathology and its impact on observed motor recovery, novel surgical techniques, new insights in sensory function and pain, and global efforts to develop standardized outcomes assessment scales.

Two congenital cases of pigmented epithelioid melanocytoma with unique clinical and genetic features.

Pigmented epithelioid melanocytomas (PEM) are intermediate-grade melanocytic lesions with frequent lymph node involvement and rare metastases that tend to follow an indolent course with a favorable outcome. We report two unique cases of congenital PEM with PRKCA fusion transcripts: a multifocal PEM with an aggressive incompletely resectable scalp tumor and a solitary palmar PEM with newly reported ITGB5-PRKCA fusion. Through these case reports and a summary of previously reported cases, we outline the spectrum of disease of PEM and highlight the key clinical and histopathologic features associated with PEM with PRKCA fusion transcripts. We also discuss the treatment options and suggest that surgical excision without further adjuvant systemic treatment is reasonable first-line therapy given the favorable prognosis.

Mental health risk and protective factors associated with brachial plexus birth injuries: a scoping review.

PURPOSE: The purpose of this scoping review is to synthesize the current evidence on the risk and protective factors associated with mental health in children with brachial plexus birth injury (BPBI) and associated interventions. MATERIALS AND METHODS: MEDLINE, EMBASE, and Cochrane databases were searched for reports on mental health in children with BPBI between 10 and 18 years. Risk and protective factors were charted using the VicHealth review and the Person-Environment-Occupation model. RESULTS: Of 732 records found, 133 full text reports were reviewed and 16 reports were included. Multiple mental health risks associated with BPBI were identified, while protective factors were largely unexplored. Person-related risks were most common including negative coping strategies (n = 8, 50%) and pain (n = 6, 38%). Most frequently reported environment and occupation risk factors were social difficulties (n = 9, 56%) and challenges with upper limb function and daily activities (n = 6, 38%). Good self-determination and/or self-concept (n = 5, 30%) was the most common protective factor. CONCLUSIONS: Research using a strengths-based approach is needed to elucidate protective factors and further understanding of the intersection of person and socio-cultural risk factors of mental health in children with BPBI.

Evidence on mental health in children with brachial plexus birth injury largely comes from studies of quality of life and functional-status outcomes, while direct reports of mental health outcome in these children are lacking.Mental health risk factors in children with brachial plexus birth injury are largely understood by clinicians and researchers as person-related factors, while the multidimensional nature of these risks is relatively unexplored.Further investigation of mental health protective factors, such as resiliency and self-determination, will foster a strengths-based approach to rehabilitation in children with brachial plexus birth injury.

Leeches and Caudal Analgesia After Replantation for Glans Amputation During Neonatal Circumcision.

Neonatal circumcision is one of the most commonly performed surgical procedures in the world. As with all surgeries, it carries risks, including rare, but devastating complications, such as glans, and/or penile shaft amputation. Complete glans amputation with successful replantation has been previously reported. Herein we report a case of complete amputation at the penile midshaft in a 28-day-old neonate with a unique approach to postoperative care including the use of leech therapy, topical heparin, and caudal analgesia following successful microvascular replantation.

Developing a decision aid for youth with brachial plexus birth injuries facing treatment decisions for an elbow flexion contracture.

BACKGROUND: A common sequela of brachial plexus birth injury (BPBI) is an elbow flexion contracture. Youth with BPBI and their families face rehabilitation or surgical decisions that ideally entail careful deliberation of the risks and benefits of treatment within the context of the child's and family's functional and appearance-related goals. OBJECTIVE: To develop a patient decision aid (PtDA) following International Patient Decision Aid Standards to help these youth and their families make treatment decisions. DESIGN: Mixed-methods study. SETTING: Brachial plexus clinic in a pediatric tertiary care center. PARTICIPANTS: Five young adults (21-24 years), 14 youth (8-19 years) and their parents, 15 families (children 2-16 years), and 19 clinicians from a brachial plexus clinic participated in the PtDA development. Seventeen other youth (8-18 years) facing treatment decisions regarding their elbow contracture field tested the PtDA prototype. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: In-depth interviews and participant observation to conduct a decisional needs assessment of young adults, youth, parents, and health care professionals involved in these shared decisions. Cognitive interviews to field test the PtDA prototype. RESULTS: Evidence from previously conducted knowledge synthesis and the youth-focused decisional needs assessment qualitative data informed the development of new PtDA prototype, which included a questionnaire-based values clarification method for youth to rate what matters most to them regarding their elbow function, appearance, psychosocial impact, and treatment options. The majority (90%) of youth >11 years who field tested the PtDA prototype understood its content, whereas only 29% of those between 8 and 11 years demonstrated independent comprehension. The majority (69%) responded that they would prefer to use the PtDA on their own either at home or in the clinic waiting room. CONCLUSIONS: This new PtDA can be used to help youth with BPBI and their families to make a personal value-based informed decision regarding treatment options for their elbow flexion contracture.

Development of a Screening Tool for Pediatric Neuropathic Pain and Complex Regional Pain Syndrome: Pediatric PainSCAN.

OBJECTIVE: Neuropathic pain (NP) and complex regional pain syndrome (CRPS) in children can result in significant disability and emotional distress. Early assessment and treatment could potentially improve pain, function, quality of life, and reduce costs to the health care system. Currently, there are no screening tools for pediatric NP and CRPS. This research aimed to develop and establish the content validity of a screening tool for pediatric NP and CRPS using a phased approach. MATERIALS AND METHODS: Phase I surveyed clinical experts using a modified Delphi procedure to elicit disease concepts for inclusion. In phase II, a consensus conference including clinicians, researchers, and people with lived experience, informed the initial item pool. Consensus for item inclusion was achieved using a nominal group technique for voting. Phase III used iterative rounds of cognitive interviews with children aged 8 to 18 years with CRPS or NP to evaluate the tool's comprehensiveness and individual item relevance and comprehensibility. Descriptive statistics were used to describe participant characteristics. Content analysis was used to analyze patient interviews. RESULTS: Phase I (n=50) generated an initial item pool (22 items). Phase II generated a comprehensive item pool (50 items), after which an initial version of the screening tool was drafted. Following phase III (n=26) after item revision and elimination, 37 items remained. DISCUSSION: The Pediatric PainSCAN is a novel screening tool that has undergone rigorous development and content validity testing. Further research is needed to conduct item reduction, determine scoring, and test additional measurement properties.

Shared decision making in youth with brachial plexus birth injuries and their families: A qualitative study.

OBJECTIVE: The objective of this study is to elucidate the decision-making experiences of youth with brachial plexus birth injuries who face preference-sensitive decisions regarding treatment options for a persistent elbow contracture. METHODS: Transcripts, research-created drawings, and field notes from in-depth interviews with 5 young adults and 14 youth-parent dyads, and 15 participant observation sessions of families and clinicians in the clinic setting were deductively and inductively coded and thematically analyzed. RESULTS: Youth's shared decision making was influenced by relational processes with their clinicians and parents. Youth's trust in their clinicians' recommendations for pursuing treatment and pressure from their parents to pursue or continue rehabilitation treatment affected how their voices were expressed and heard. Parental emotional adjustment to the birth injury played a role in parental perceptions of what was deemed their youth's best interest in the shared decision. CONCLUSIONS: The decision-making experiences of youth with brachial plexus birth injury illustrate the complexity of paediatric preference-sensitive decisions and the significance of social and emotional factors on these shared decisions. PRACTICE IMPLICATIONS: Opportunity for youth to express their voice without external pressure during shared decision making is needed to make well-informed decisions based on their own values. Interventions such as decision coaching and decision support tools may help youth and parents to formally identify and discuss these relational processes.

Surgical Reconstruction of Isolated Upper Trunk Brachial Plexus Birth Injuries in the Presence of an Avulsed C5 or C6 Nerve Root.

BACKGROUND: Avulsion of either the C5 or C6 root with intact middle and lower trunks in brachial plexus birth injury is rare. In these cases, only 1 proximal root is available for intraplexal reconstruction. The purpose of the present study was to determine the outcomes of these patients when single-root reconstruction was balanced across the anterior and posterior elements of the upper trunk. METHODS: We performed a retrospective cohort study of prospectively collected data for patients with brachial plexus birth injury who underwent primary nerve reconstruction between 1993 and 2014. Patients were included who had isolated upper-trunk injuries with intact middle and lower trunks. The study group had avulsion of either the C5 or C6 root. The control group had neuroma-in-continuity or ruptures of the upper trunk. Outcomes were assessed with use of the Active Movement Scale and the Brachial Plexus Outcome Measure. The Wilcoxon signed-rank test was utilized to evaluate changes across treatment. RESULTS: Ten patients with brachial plexus birth injury were included in the avulsion cohort. Surgical reconstruction entailed neuroma resection and nerve grafting from the single available root balanced across all distal targets with or without spinal accessory-to-suprascapular nerve transfer. Significant improvements were observed across treatment for both the avulsion and control groups in terms of shoulder abduction, shoulder flexion, external rotation, elbow flexion, and supination. At a mean follow-up of 54.5 ± 8.8 months, patients in the avulsion group achieved Active Movement Scale scores of 6.8 ± 0.4 for elbow flexion and 6.5 ± 0.9 for shoulder flexion and abduction, with lesser recovery observed in external rotation (3.3 ± 2.8). All patients available for Brachial Plexus Outcome Measure assessments demonstrated functional movement. CONCLUSIONS: In the setting of avulsion of 1 upper-trunk root, nerve reconstruction by grafting of the upper trunk from the other upper-trunk root provides improved movement, high Active Movement Scale scores, and satisfactory function according to the Brachial Plexus Outcome Measure. These data provide support for a strategy that ensures the entire upper trunk is adequately reconstructed in the setting of upper-trunk lesions. LEVEL OF EVIDENCE: Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.

Surgical Reconstruction of Isolated Upper Trunk Brachial Plexus Birth Injuries in the Presence of an Avulsed C5 or C6 Nerve Root.

BACKGROUND: Avulsion of either the C5 or C6 root with intact middle and lower trunks in brachial plexus birth injury is rare. In these cases, only 1 proximal root is available for intraplexal reconstruction. The purpose of the present study was to determine the outcomes of these patients when single-root reconstruction was balanced across the anterior and posterior elements of the upper trunk. METHODS: We performed a retrospective cohort study of prospectively collected data for patients with brachial plexus birth injury who underwent primary nerve reconstruction between 1993 and 2014. Patients were included who had isolated upper-trunk injuries with intact middle and lower trunks. The study group had avulsion of either the C5 or C6 root. The control group had neuroma-in-continuity or ruptures of the upper trunk. Outcomes were assessed with use of the Active Movement Scale and the Brachial Plexus Outcome Measure. The Wilcoxon signed-rank test was utilized to evaluate changes across treatment. RESULTS: Ten patients with brachial plexus birth injury were included in the avulsion cohort. Surgical reconstruction entailed neuroma resection and nerve grafting from the single available root balanced across all distal targets with or without spinal accessory-to-suprascapular nerve transfer. Significant improvements were observed across treatment for both the avulsion and control groups in terms of shoulder abduction, shoulder flexion, external rotation, elbow flexion, and supination. At a mean follow-up of 54.5 ± 8.8 months, patients in the avulsion group achieved Active Movement Scale scores of 6.8 ± 0.4 for elbow flexion and 6.5 ± 0.9 for shoulder flexion and abduction, with lesser recovery observed in external rotation (3.3 ± 2.8). All patients available for Brachial Plexus Outcome Measure assessments demonstrated functional movement. CONCLUSIONS: In the setting of avulsion of 1 upper-trunk root, nerve reconstruction by grafting of the upper trunk from the other upper-trunk root provides improved movement, high Active Movement Scale scores, and satisfactory function according to the Brachial Plexus Outcome Measure. These data provide support for a strategy that ensures the entire upper trunk is adequately reconstructed in the setting of upper-trunk lesions. LEVEL OF EVIDENCE: Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.

Dissemination and Implementation Science in Plastic and Reconstructive Surgery: Perfecting, Protecting, and Promoting the Innovation That Defines Our Specialty.

SUMMARY: Plastic and reconstructive surgery has an illustrious history of innovation. The advancement, if not the survival, of the specialty depends on the continual development and improvement of procedures, practices, and technologies. It follows that the safe adoption of innovation into clinical practice is also paramount. Traditionally, adoption has relied on the diffusion of new knowledge, which is a consistent but slow and passive process. The emerging field of dissemination and implementation science promises to expedite the spread and adoption of evidence-based interventions into clinical practice. The field is increasingly recognized as an important function of academia and is a growing priority for major health-related funding institutions. The authors discuss the contemporary challenges of the safe implementation and dissemination of new innovations in plastic and reconstructive surgery, and call on their colleagues to engage in this growing field of dissemination and implementation science.