Altered axonal excitability properties in facial palsy.

INTRODUCTION: Axonal excitability measures give insight into the biophysical properties of peripheral nerve axons. In this study we applied these techniques to the study of facial palsy. METHODS: Thirty patients with established facial palsy due to unresolved Bell's palsy or herpes zoster (>6 months duration), tumor invasion of the facial nerve, or traumatic facial nerve injury were assessed using facial nerve excitability techniques. RESULTS: Full recordings were obtained in 23 patients (15 unrecovered Bell's palsy or herpes zoster, 5 trauma, 3 tumor-related). Compared with normal controls, the facial palsy group demonstrated changes in stimulus response properties, threshold electrotonus, refractoriness, superexcitability, and I/V slope. Depolarizing threshold electrotonus distinguished between viral and non-viral etiologies on subgroup analysis. DISCUSSION: In this cross-sectional study, established facial palsy demonstrated findings similar to those seen in studies of regenerated axons. The improved understanding of underlying axonal characteristics offered by the technique may guide future treatment. Muscle Nerve 57: 268-272, 2018.

Children's experiences of congenital heart disease: a systematic review of qualitative studies.

We aimed to describe the experiences of children and adolescents with congenital heart disease (CHD). Electronic databases were searched until August 2016. Qualitative studies of children's perspectives on CHD were included. Data was extracted using thematic synthesis. From 44 studies from 12 countries involving 995 children, we identified 6 themes: disrupting normality (denying the diagnosis, oscillating between sickness and health, destabilizing the family dynamic), powerlessness in deteriorating health (preoccupation with impending mortality, vulnerability to catastrophic complications, exhaustion from medical testing), enduring medical ordeals (traumatized by invasive procedures, disappointed by treatment failure, displaced by transition, valuing empathy and continuity in care, overcoming uncertainty with information), warring with the body (losing stamina, distressing inability to participate in sport, distorted body image, testing the limits), hampering potential and goals (feeling disabled, unfair judgment and exclusion, difficulties with academic achievement, limiting attainment and maintenance of life milestones), and establishing one's own pace (demarcating disease from life, determination to survive, taking limitations in their stride, embracing the positives, finding personal enrichment, relying on social or spiritual support). CONCLUSION: Children with CHD feel vulnerable and burdened by debilitating physical symptoms, unpredictable complications, and discrimination. Clinicians may support patients by sharing recognition of these profound psychosocial consequences. What is Known: • CHD is associated with difficulties in learning and attention, school absenteeism, decreased endurance, poor body image, and peer socialization • What is lesser known is how young patients cope with the symptoms, prognostic uncertainty, and treatment burden What is New: • We found that children are challenged by lifestyle restrictions, fear of invasive procedures, impaired body image, discrimination, and uncertainty about the future. Feelings of disempowerment are intensified by the unpredictability of disease progression • Thus, strategies to improve outcomes include improved patient education on disease and lifestyle management and partnership with school teachers and counselors for unique psychosocial support.

Range and Heterogeneity of Outcomes in Randomized Trials of Pediatric Chronic Kidney Disease.

OBJECTIVE: To determine the range and heterogeneity of outcomes reported in randomized controlled trials of interventions for children with chronic kidney disease (CKD). STUDY DESIGN: The Cochrane Kidney and Transplant Specialized Register was searched to March 2016. Randomized trials involving children across all stages of CKD were selected. All outcome domains and measurements were extracted from included trials. The frequency and characteristics of the outcome domains and measures were evaluated. RESULTS: From 205 trials included, 6158 different measurements of 100 different outcome domains were reported, with a median of 22 domains per trial (IQR 13-41). Overall, 52 domains (52%) were surrogate, 38 (38%) were clinical, and 10 (10%) were patient-reported. The 5 most commonly reported domains were blood pressure (76 [37%] trials), relapse/remission (70 [34%]), kidney function (66 [32%]), infection (61 [30%]), and height/pubertal development (51 [25%]). Mortality (14%), cardiovascular disease (4%), and quality of life (1%) were reported infrequently. The 2 most frequently reported outcomes, blood pressure and relapse/remission, had 56 and 81 different outcome measures, respectively. CONCLUSIONS: The outcomes reported in clinical trials involving children with CKD are extremely heterogeneous and are most often surrogate outcomes, rather than clinical and patient-centered outcomes such as cardiovascular disease and quality of life. Efforts to ensure consistent reporting of outcomes that are important to patients and clinicians will improve the value of trials to guide clinical decision-making. In our study, non-English articles were excluded.

Experiences and Perspectives of Transgender Youths in Accessing Health Care: A Systematic Review.

Importance: Transgender and nonbinary youths have a higher incidence of a range of health conditions and may paradoxically face limited access to health care. Objective: To describe the perspectives and needs of transgender youths in accessing health care. Evidence Review: MEDLINE, Embase, PsycInfo, and the Cumulative Index to Nursing and Allied Health Literature were searched from inception to January 2021. Qualitative studies of transgender youths' perspectives on accessing health care were selected. Results from primary studies were extracted. Data were analyzed using thematic synthesis. Findings: Ninety-one studies involving 884 participants aged 9 to 24 years across 17 countries were included. We identified 6 themes: experiencing pervasive stigma and discrimination in health care, feeling vulnerable and uncertain in decision-making, traversing risks to overcome systemic barriers to transitioning, internalizing intense fear of consequences, experiencing prejudice undermining help-seeking efforts, and experiencing strengthened gender identity and finding allies. Each theme encapsulated multiple subthemes. Conclusions and Relevance: This review found that transgender youths contend with feelings of gender incongruence, fear, and vulnerability in accessing health care, which are compounded by legal, economic, and social barriers. This can lead to disengagement from care and resorting to high-risk and unsafe interventions. Improving access to gender-affirming care services with a cultural humility lens and addressing sociolegal stressors may improve outcomes in transgender and nonbinary youths.

Dual chimeric innervated vastus lateralis free flap for single stage blink and midface reanimation.

BACKGROUND: The immediate reconstruction of the face in the setting of radical parotidectomy for malignancy represents a particular challenge. We present a novel technique using 2 sections of the vastus lateralis muscle as a chimeric flap in combination with the anterolateral thigh (ALT) fasciocutaneous flap to achieve midface reanimation and eye closure after radical parotidectomy. METHODS: The harvest technique in view of variations in chimeric muscle components, vascular pedicles, and neural coaptations is described herein. RESULTS: On postoperative facial nerve examination, we observed encouraging results in middle-aged patients even in the context of radiotherapy. CONCLUSION: Although reanimation using the chimeric vastus lateralis free flap is yet to be objectively determined in a large series, our experience is that this flap adds the potential of dynamic midface movement and eye closure to our current reconstructive approach with excellent postoperative results in selected cases.

Single innervated segmented vastus lateralis for midfacial reanimation during radical parotidectomy.

BACKGROUND: Innervated free muscle transfer using the gracilis muscle or temporalis myoplasty has been successfully utilized for facial reanimation in chronic facial palsy. These techniques are less suited to immediate facial reconstruction in the setting of radical parotidectomy, in which the complexity of the defect, patient age, postoperative radiotherapy, and limited life expectancy pose particular challenges. METHODS: We present a novel description of the use of a chimeric anterolateral thigh (ALT) flap and innervated vastus lateralis to achieve midface static suspension and dynamic reanimation in the setting of radical parotidectomy. RESULTS: The technique is described in detail along with a video demonstrating the early and medium-term results in an example case. CONCLUSION: Although outcomes using the vastus lateralis free flap for midface reanimation need to be objectively determined, the flap adds the potential of dynamic midface movement in patients undergoing radical parotidectomy who would otherwise not be afforded this opportunity. © 2017 Wiley Periodicals, Inc. Head Neck 39: 602-604, 2017.

Validation of the Clinician-Graded Electronic Facial Paralysis Assessment.

BACKGROUND: Facial paralysis remains a debilitating condition despite advances in medical, surgical, and adjunctive interventions. Established grading systems used to assess facial paralysis and interventional outcomes have well-described limitations. The Electronic Facial Paralysis Assessment, a clinician-graded zone-based facial function scale, has recently emerged as a grading tool that may provide greater sensitivity when assessing incomplete paralysis and postsurgical improvement. The authors perform the first comprehensive validation of this tool. METHODS: Video recordings of 83 facial paralysis patients were assessed. Grading was performed in two sittings by three individuals with varying degrees of experience in assessing facial paralysis. Interobserver reliability; intraobserver reliability; administration time; and agreement with the Facial Disability Index, House-Brackmann, Sunnybrook, and Sydney facial grading systems were assessed. RESULTS: The Electronic Facial Paralysis Assessment demonstrated high intra observer and interobserver reliability (intraclass correlation coefficient, 0.84 to 0.91 and 0.81 to 0.83, respectively). It correlated well with the House-Brackmann, Sunnybrook, and Sydney facial grading systems (Spearman rho, 0.73, 0.77 and 0.77, respectively). In subdomain analysis, it correlated well with the Sunnybrook and Sydney systems in dynamic movement (Spearman rho, 0.90 and 0.89, respectively) and synkinesis (Spearman rho, range 0.74 and 0.72, respectively). It had poor agreement with the Facial Disability Index (Spearman rho, 0.25). The mean time to complete the tool was 116 ± 61 seconds. CONCLUSIONS: The Electronic Facial Paralysis Assessment is a valid facial assessment tool with high reliability and correlation with the established facial paralysis grading systems. It also provides an efficient and detailed analysis of paralysis according to each facial zone. CLINICAL QUESTION/LEVEL OF EVIDENCE: Diagnostic, II.