Citations

OBJECTIVE Intracranial facial nerve schwannomas (FNS) requiring treatment are frequently recommended for surgery or stereotactic radiosurgery (SRS). The objective of this study was to compare facial nerve function outcomes between these two interventions for FNS via a systematic review and meta-analysis. METHODS A search of the Ovid EMBASE, PubMed, SCOPUS, and Cochrane databases from inception to July 2019 was conducted following PRISMA guidelines. Articles were screened against prespecified criteria. Facial nerve outcomes were classified as improved, stabilized, or worsened by last follow-up. Incidence was pooled by random-effects meta-analysis of proportions. RESULTS Thirty-three articles with a pooled cohort of 519 patients with FNS satisfied all criteria. Twenty-five articles described operative outcomes in 407 (78%) patients; 10 articles reported SRS outcomes in 112 (22%). In the surgical cohort, facial nerve function improved in 23% (95% CI 15%–32%), stabilized in 41% (95% CI 32%–50%), and worsened in 30% (95% CI 21%–40%). In the SRS cohort, facial nerve function was improved in 20% (95% CI 9%–34%), stable in 66% (95% CI 54%–78%), and worsened in 9% (95% CI 3%–16%). Compared with SRS, microsurgery was associated with a significantly lower incidence of stable facial nerve function (p < 0.01) and a significantly higher incidence of worsened facial nerve function (p < 0.01). Tumor progression and complication rates were comparable. Outcome certainty assessments were very low to moderate for all parameters. CONCLUSIONS Unfavorable facial nerve function outcomes are associated with surgical treatment of intracranial FNS, whereas stable facial nerve function outcomes are associated with SRS. Therefore, SRS should be recommended to patients with FNS who require treatment, and surgery should be reserved for patients with another indication, such as decompression of the brainstem. Further study is required to definitively optimize and validate management strategies for these rare skull base tumors.

The COVID-19 pandemic has accelerated many changes in medicine including the transition from providing care in person to providing care via technology enabled telemedicine. The benefits of telemedicine visits with a Pediatric Rehabilitation Medicine (PRM) provider, also known as telerehabilitation medicine visits, are numerous. Telerehabilitation medicine provides an opportunity to deliver timely, patient and family-centric rehabilitation care while maintaining physical distance and reducing potential COVID-19 exposure for our patients, their caregivers and medical providers. Telerehabilitation medicine also allows for access to PRM care in rural areas or areas without medical specialty, virtual in-home equipment evaluation, and reduced travel burden. Because of these and many other benefits, telerehabilitation medicine will likely become part of our ongoing model of care if barriers to telemedicine continue to be lowered or removed. This paper is intended to establish a foundation for pediatric telerehabilitation medicine visit efficiency and effectiveness in our current environment and into the future.

AbstractBackgroundLittle is known about skin‐related complications in Klippel–Trenaunay syndrome (KTS), a complex vascular anomaly defined by capillary malformation (CM), venous malformation (VM) ± lymphatic malformation (LM) and limb overgrowth. Reported skin‐related complications of KTS include ulceration, vascular ectasias (blebs), bleeding and infection.ObjectiveTo determine the spectrum, prevalence and predictors of skin‐related complications in KTS.MethodsA retrospective review of 410 patients fulfilling KTS criteria was performed to assess for the presence of skin‐related complications.ResultsSkin‐related complications were present in 45% of patients. Most prevalent were CM‐related complications including blebs, bleeding, thickening (25%), cellulitis (22%) and ulceration (21%). Features positively associated with skin‐related complications were presence of LM (OR 17.17; P < 0.001), VM on the buttocks/perineum/genitalia (OR 1.92; P = 0.009), CM on the feet (OR 1.77; P = 0.039) and male sex (OR 1.63; P = 0.034). Features negatively associated with skin‐related complications were CM on the trunk (OR 0.59; P = 0.029) and tissue hypertrophy of the hands (OR 0.27; P = 0.025).ConclusionSkin‐related complications affect nearly half of patients with KTS. Those with lymphatic involvement or malformation presence in the undergarment area or feet are most at risk.

Background: The rate of elbow medial ulnar collateral ligament (MUCL) injury and surgery continues to rise steadily. While authors have failed to reach a consensus on the optimal graft or anchor configuration for MUCL reconstruction, the vast majority of the literature is focused on the young, elite athlete population utilizing autograft. These studies may not be as applicable for the “weekend warrior” type of patient or for young kids playing on high school leagues or recreationally without the intent or aspiration to participate at an elite level. Purpose: To investigate the clinical outcomes and complication rates of MUCL reconstruction utilizing only allograft sources in nonelite athletes. Study Design: Case series; Level of evidence, 4. Methods: Patient records were retrospectively analyzed for individuals who underwent allograft MUCL reconstruction at a single institution between 2000 and 2016. A total of 25 patients met inclusion criteria as laborers or nonelite (not collegiate or professional) athletes with a minimum of 2 years of postoperative follow-up. A review of the medical records for the included patients was performed to determine survivorship free of reoperation, complications, and clinical outcomes with use of the Summary Outcome Determination (SOD) and Timmerman-Andrews scores. Statistical analysis included a Wilcoxon rank-sum test to compare continuous variables between groups with an alpha level set at .05 for significance. Subgroup analysis included comparing outcome scores based on the allograft type used. Results: Twenty-five patients met all inclusion and exclusion criteria. The mean time to follow-up was 91 months (range, 25-195 months), and the mean age at the time of surgery was 25 years (range, 12-65 years). There were no revision operations for recurrent instability. The mean SOD score was 9 (range, 5-10) at the most recent follow-up, and the Timmerman-Andrews scores averaged 97 (range, 80-100). Three patients underwent subsequent surgical procedures for ulnar neuropathy (n = 2) and contracture (n = 1), and 1 patient underwent surgical intervention for combined ulnar neuropathy and contracture. Conclusion: Allograft MUCL reconstruction in nonelite athletes demonstrates comparable functional scores with many previously reported autograft outcomes in elite athletes. These results may be informative for elbow surgeons who wish to avoid autograft morbidity in common laborers and nonelite athletes with MUCL incompetency.

Objective: To evaluate adverse events and feasibility of performing 1.5-T MRI in patients with cochlear implants (CI) and auditory brainstem implants (ABI). Setting: Single tertiary academic referral center. Patients: CI and ABI recipients undergoing 1.5-T MRI without internal magnet removal. Intervention(s): MRI after tight headwrap application. Main Outcome Measures: Adverse events, patient tolerance. Results: A total of 131 MR studies in 79 patients were performed, with a total of 157 study ears. Sixty-one patients (77%) had unilateral devices. Four patients (5%) underwent MRI with ABI magnets in place. Sixteen patients (20%) had MRI-compatible devices that did not require a head wrap. There were no instances of device stimulation, device malfunction, or excessive heating of the receiver-stimulator package. Magnet tilt requiring manual repositioning occurred during seven studies (4.5%) and magnet displacement requiring operative intervention occurred during seven studies (4.5%). Significant pain where imaging had to be discontinued occurred during three episodes (2%). No adverse events were noted among patients who underwent MRI with an MRI-compatible magnet. Conclusions: MRI with CI or ABI magnets in place is associated with a low prevalence of adverse events when performed in a controlled setting. Many partial magnet displacements can be corrected with firm manual pressure. Devices with magnets that align with the field within their housing were not associated with any adverse events and do not require immobilization of the magnet during the scan. These may be valuable in patients with known or anticipated need for MRI.

PurposeMore than 460 million people are impacted by disabling hearing loss worldwide. Hearing loss has negative impacts across the life span and varies by individual. Specifically, in children, hearing loss has been shown to affect learning outcomes, behavioral and cognitive growth, and psychosocial development. In adults, hearing loss has been shown to affect quality of life, social isolation, depression, and anxiety. The purpose of this review is to advance the understanding of the latest research in the development of new treatments of hearing loss, including gene therapy, stem cell therapy, and pharmacotherapies. We address the use of animals to model human hearing loss and the importance of animals in previous discoveries and developing new therapies. We highlight the importance of early identification for better speech and developmental outcomes specifically in pediatric individuals. Lastly, we review new possibilities in the prevention of hearing loss and the path to translation into practice.ConclusionsResearch and development of new therapies to treat hearing loss is an ever growing field with many notable successes. However, clinical translation still poses many challenges. Many discoveries related to gene therapy, stem cell therapy, and pharmacotherapy are in the early phases of clinical trials and hold promise. This review empowers audiologists to be aware of these most recent developments and be conscious of the evolving future landscape of treatment and management of hearing loss.

Abstract Zinc-finger protein 384 (ZNF384) gene fusions with EP300 have recently been described as a recurrent fusion in B-cell acute lymphoblastic leukemia (B-ALL) with a good response to conventional chemotherapy, suggesting a favorable prognosis. Herein, we report on a female patient aged 12 years with uninformative conventional chromosome and B-ALL panel fluorescence in situ hybridization studies with chromosomal microarray showing multiple copy number gains, including relative gains in the ZNF384 (12p13.31) and EP300 (22q13.2) gene regions, suggesting a cryptic EP300/ZNF384 fusion. Ultimately, a next-generation sequencing assay, mate pair sequencing, was utilized to confirm EP300/ZNF384 fusion in this B-ALL clone, which may confer a favorable overall prognosis and potential targeted therapy.

Background: When revision elbow arthroplasty is required in the presence of structural proximal ulnar bone loss and triceps insufficiency, structural ulnar bone-grafting and triceps reconstruction are both required to reconstruct the skeleton and to restore active extension. We have developed a technique utilizing a structural proximal ulnar allograft with its attached triceps as an allograft-prosthetic composite (APC) to provide reconstruction of the proximal ulnar bone and deficient extensor mechanism with the same allograft. Methods: Between 2010 and 2017, the senior author performed 10 revision elbow arthroplasties using a proximal ulnar allograft with its intact triceps tendon allograft. The allograft ulna was combined with an ulnar component in an APC fashion, whereas the remaining triceps was repaired to the triceps allograft. Indications for a revision surgical procedure included aseptic loosening in 4 elbows, periprosthetic ulnar fracture with component loosening in 2 elbows, and the second stage of a 2-stage reimplantation in 4 elbows. Two elbows also required humeral APCs due to associated structural humeral bone loss. Postoperatively, all elbows were immobilized in extension for 6 weeks. The mean follow-up time was 45 months (range, 24 to 76 months). Results: Revision elbow arthroplasty resulted in pain improvement in all elbows in a 10-point visual analog scale. The mean flexion-extension arc was 95°, the mean Mayo Elbow Performance Score was 76 points (range, 45 to 95 points), and the mean triceps strength score was 4. There were 6 reoperations: 3 for humeral loosening, 1 for deep infection, 1 for fracture of the ulnar allograft, and 1 for wound debridement and closure. For the 8 elbows with an intact ulnar reconstruction, no ulnar components were radiographically loose, and the ulnar graft was considered radiographically intact and healed in 8 elbows. Conclusions: Reconstruction of the proximal part of the ulna and triceps in failed elbow arthroplasties with structural ulnar bone loss can be effectively accomplished using a structural proximal ulnar allograft as an APC with the preserved triceps tendon for the extensor mechanism reconstruction, but the procedure is associated with an expected high reoperation rate, as is the case in complex revision elbow arthroplasty. Level of Evidence: Therapeutic Level IV . See Instructions for Authors for a complete description of levels of evidence.

Background While there is a long history of measuring death and disability from injuries, modern research methods must account for the wide spectrum of disability that can occur in an injury, and must provide estimates with sufficient demographic, geographical and temporal detail to be useful for policy makers. The Global Burden of Disease (GBD) 2017 study used methods to provide highly detailed estimates of global injury burden that meet these criteria. Methods In this study, we report and discuss the methods used in GBD 2017 for injury morbidity and mortality burden estimation. In summary, these methods included estimating cause-specific mortality for every cause of injury, and then estimating incidence for every cause of injury. Non-fatal disability for each cause is then calculated based on the probabilities of suffering from different types of bodily injury experienced. Results GBD 2017 produced morbidity and mortality estimates for 38 causes of injury. Estimates were produced in terms of incidence, prevalence, years lived with disability, cause-specific mortality, years of life lost and disability-adjusted life-years for a 28-year period for 22 age groups, 195 countries and both sexes. Conclusions GBD 2017 demonstrated a complex and sophisticated series of analytical steps using the largest known database of morbidity and mortality data on injuries. GBD 2017 results should be used to help inform injury prevention policy making and resource allocation. We also identify important avenues for improving injury burden estimation in the future.