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To explore patient-reported outcome measures of pediatric paradoxical vocal fold motion through a multi-institutional study of geographically diverse United States medical facilities to assess long-term management and outcomes.Eligible participants8 years of age diagnosed with PVFM over a 10-year period from 7 tertiary pediatric hospitals were invited to complete a survey addressing study objectives.65 participants completed the survey, of whom 80% were female, 75% reported a 3.5 grade point average or better, and 75% identified as competitive athletes or extremely athletic individuals. Participants rated their perceived efficacy of 13 specific treatments. Only five treatments were considered effective by a majority of the participants who tried them. The treatments that participants tried most often were breathing exercises (89.2%), bronchodilator treatments (45%), and allergy medications (35.4%). 78.8% of participants reported receiving more than one treatment and 25% reported receiving a combination of bronchodilators, anticholinergics, and steroids. At the time of PVFM diagnosis, 38% of participants had no idea when their symptoms would completely resolve. 23.3% of participants did not experience symptom resolution until greater than 1 year after diagnosis.Traditional management tools such as breathing exercises and biofeedback treatments may not provide the long-term benefit that providers anticipate. In addition to these commonly used management strategies, highly efficacious techniques such as counseling and lifestyle management should be incorporated into the long-term management of patients whose symptoms are refractory to traditional care.4 Laryngoscope, 2022.

Following injury, the oral mucosa undergoes a complex sequences of biological healing processes to restore homeostasis. While general similarities exist, there are marked differences in the genomics and kinetics of wound healing between the oral cavity and cutaneous epithelium. The lack of successful therapy for oral mucosal wounds has influenced clinicians to explore alternative treatments and potential autotherapies to enhance intraoral healing. The present in-depth review discusses current gold standards for oral mucosal wound healing and compares endogenous factors that dictate the quality of tissue remodeling. We conducted a review of the literature on in vivo oral wound healing models and emerging regenerative therapies published during the past twenty years. Studies were evaluated by injury models, therapy interventions, and outcome measures. The success of therapeutic approaches was assessed, and research outcomes were compared based on current hallmarks of oral wound healing. By leveraging therapeutic advancements, particularly within in cell-based biomaterials and immunoregulation, there is great potential for translational therapy in oral tissue regeneration.

Lateral medullary syndrome/Wallenberg syndrome is a stroke in the lateral medulla with symptoms often including dysphagia and dysphonia. In adults, this stroke is the most common brainstem stroke, but it is rare in the pediatric population. Insults to the medulla can involve the "swallowing centers," the nucleus ambiguus and nucleus tractus solitarius, and the cranial nerves involved in swallowing, namely IX (glossopharyngeal) and X (vagus). These individuals can develop severe dysphagia with an inability to trigger a swallow due to pharyngeal weakness and impaired mechanical opening of the upper esophageal sphincter (UES) which can result in aspiration. We present a 7-year-old male with 22q11.2 deletion syndrome (velocardiofacial syndrome) and velopharyngeal insufficiency who underwent pharyngeal flap surgery at an outside hospital whose post-operative course was complicated by adenovirus, viral myocarditis, and dorsal medullary stroke. He required a tracheostomy and gastrostomy tube. He was discharged from that hospital and readmitted to our hospital 4 months later for increased oxygen requirement, requiring a 5 month admission in the intensive care units. His initial VFSS revealed absent UES opening with the entire bolus remaining in the pyriform sinuses resulting in aspiration. His workup over the course of his admission included multiple videofluoroscopic swallow studies (VFSS), flexible endoscopic evaluation of swallowing (FEES), and pharyngeal and esophageal manometry. Intervention included intensive speech therapy, cricopharyngeal Botox® injection, and cricopharyngeal myotomy. Nineteen months after his stroke, he transitioned to oral intake of solids and liquids with adequate movement of the bolus through the pharynx and UES and no aspiration on his VFSS.

To illustrate how quality improvement can produce unexpected positive outcomes.We compared a retrospective review of perioperative management and outcomes of baseline 122 pediatric total thyroidectomies to 121 subsequent total thyroidectomies managed by an Electronic Medical Record protocol in a large, free-standing children's healthcare system. Process measures included serum calcium measurement 6-12 hours postoperatively; parathyroid hormone measurement 6 hours postoperatively; preoperative iodine for Graves disease, and postoperative prophylactic calcium carbonate administration. In addition, we completed 4 Plan-Do-Study-Act (PDSA) cycles, focusing on implementation, refinement, usage, education, and postoperative calcitriol administration. The primary outcome included transient hypocalcemia during admission.All perioperative process measures improved over PDSA cycles. Measurement of postoperative serum calcium increased from 42% at baseline to 100%. Measurement of postoperative PTH increased from 11% to 97%. Preoperative iodine administration for Graves disease surgeries improved from 72% to 94%. Postoperative calcium carbonate administration increased from 36% to 100%. There was a trend toward lower rates of severe hypocalcemia during admission over the subsequent PDSA cycles starting at 11.6% and improving to 3.4%. With the regular review of outcomes, surgical volume consolidated among high-volume providers, associated with a decrease in a permanent hypoparathyroid rate of 20.5% at baseline to 10% by the end of monitoring.In standardizing care at 1 large pediatric institution, implementing a focused quality improvement project involving the perioperative management of transient hypocalcemia in total thyroidectomy pediatric patients resulted in additional, unanticipated improvements in patient care.

The Clinical Assessment Score-15 (CAS-15) has been validated as an office-based assessment for pediatric sleep-disordered breathing in otherwise healthy children. Our objective was to determine the generalizability of the CAS-15 in a multi-institutional fashion.Five hundred and thirty children from 13 sites with suspected sleep-disordered breathing were recruited, and the investigators completed the CAS-15. Based on decisions made in the course of clinical care, investigators recommended overnight polysomnography, observation, medical therapy, and/or surgery. Two hundred and forty-seven subjects had a follow-up CAS-15.Mean age was 5.1 (2.6) years; 54.2% were male; 39.1% were white; and 37.0% were African American. Initial mean (standard deviation [SD]) CAS-15 was 37.3 (12.7), n = 508. Spearman correlation between the initial CAS-15 and the initial apnea-hypopnea index (AHI) was 0.41 (95% confidence interval [CI], 0.29, 0.51), n = 212, P .001. A receiver-operating characteristic curve predicting positive polysomnography (AHI 2) had an area under the curve of 0.71 (95% CI, 0.63, 0.80). A score ≥ 32 had a sensitivity of 69.0% (95% CI, 61.7, 75.5), a specificity of 63.4% (95% CI, 47.9, 76.6), a positive predictive value of 88.7% (95% CI, 82.1, 93.1), and a negative predictive value of 32.9% (95% CI, 23.5, 44.0) in predicting positive polysomnography. Among children who underwent surgery, the mean change (SD) score was 30.5 (12.6), n = 201, t = 36.85, P .001, effect size = 3.1.This study establishes the generalizability of the CAS-15 as a useful office tool for the evaluation of pediatric sleep-disordered breathing.2B Laryngoscope, 130:2256-2262, 2020.

Purpose This article aims to provide a set of guiding principles for interdisciplinary team care of velopharyngeal inadequacy (VPI) for speech, regardless of the etiology. Method A working group of practitioners with advanced training and experience in the management of patients with cleft palate/velopharyngeal disorders, including representatives from speech-language pathology, otolaryngology, and plastic surgery, was formed. Pertinent literature was reviewed, and practical suggestions for clinicians were developed through consensus discussion. Results Seven key principles were identified as being integral to the provision of interdisciplinary team care for VPI. Conclusion Collaborative interdisciplinary team care for persons with velopharyngeal disorders is key to optimal management and outcomes. Practical suggestions for implementing an interdisciplinary team care model for management of cleft-related and noncleft VPI are described.

Oral cavity wound healing occurs in an environment that sustains ongoing physical trauma and is rich in bacteria. Despite this, injuries to the mucosal surface often heal faster than cutaneous wounds and leave less noticeable scars. Patients undergoing cleft palate repair have a high degree of wound healing complications with up to 60% experiencing oronasal fistula (ONF) formation. In this study, we developed a mouse model of hard palate mucosal injury, to study the endogenous injury response during oral cavity wound healing and ONF formation. Immunophenotyping of the inflammatory infiltrate following hard palate injury showed delayed recruitment of non-classical LY6Clo monocytes and failure to resolve inflammation. To induce a pro-regenerative inflammatory response, delivery of FTY720 nanofiber scaffolds following hard palate mucosal injury promoted complete ONF healing and was associated with increased LY6Clo monocytes and pro-regenerative M2 macrophages. Alteration in gene expression with FTY720 delivery included increased Sox2 expression, reduction in pro-inflammatory IL-1, IL-4 and IL-6 and increased pro-regenerative IL-10 expression. Increased keratinocyte proliferation during ONF healing was observed at day 5 following FTY720 delivery. Our results show that local delivery of FTY720 from nanofiber scaffolds in the oral cavity enhances healing of ONF, occurring through multiple immunomodulatory mechanisms. STATEMENT OF SIGNIFICANCE: Wound healing complications occur in up to 60% of patients undergoing cleft palate repair where an oronasal fistula (ONF) develops, allowing food and air to escape from the nose. Using a mouse model of palate mucosal injury, we explored the role of immune cell infiltration during ONF formation. Delivery of FTY720, an immunomodulatory drug, using a nanofiber scaffold into the ONF was able to attract anti-inflammatory immune cells following injury that enhanced the reepithelization process. ONF healing at day 5 following FTY720 delivery was associated with altered inflammatory and epithelial transcriptional gene expression, increased anti-inflammatory immune cell infiltration, and increased proliferation. These findings demonstrate the potential efficacy of immunoregenerative therapies to improve oral cavity wound healing.

Langerhans Cell Histiocytosis (LCH) is a sporadic myeloid neoplasm with proliferation of dendritic cells.1 In the United States, LCH has an estimated annual incidence of approximately 1 per million children.2 Controversy exists regarding management of LCH in head and neck: debridement, chemotherapy, or both. Few reports describe LCH in the OMS literature. The purpose of this study was to present experience with LCH of head and neck in children. This was a retrospective review of children with LCH at Children's Healthcare of Atlanta from 2009 to 2020. A patient was included when he or she: 1) was 18 years old or younger, 2) had LCH in the head and neck, and 3) had complete medical records. Medical records were reviewed for demographic information, lesion characteristics, and clinical presentations. Radiographic images were reviewed for specific findings characteristic of LCH (e.g., osteolytic changes, lymphadenopathy). Treatment regimen (debridement, debridement with chemotherapy, or chemotherapy only) and length of follow-up were recorded. The outcome variable was disease reactivation defined as: 1) a new LCH lesion or 2) an increase in size/recurrence of a lesion after treatment. Data were collected using a standardized collection form. Descriptive statistics were calculated. Overall, 67 patients (42 males) with a mean age of 6.5 years old met inclusion criteria. According to clinical, radiographic, and histological features, there were 3 presentations of LCH in the head and neck: 1) lesions in 1 system without CNS risk (i.e., single system without CNS risk, SS-), 2) lesions in 1 system with CNS risk (i.e., single system with CNS risk, SS+), or 3) multisystem (> 2 systems, MS) presentations. As a group, length of follow-up was on average 3 years (3 months-10.5 years). There were 24 patients (17 males) with an average age of 10 years old (19 months to 18 years) with SS- LCH. Lesions appeared as persistent local swelling (n = 18, 75%) and/or pain (n = 8, 33.3%). Lesions were predominately located in calvaria (n = 15, 62.5%) and mandible (n = 7, 29.2%). Radiographically, most patients had osteolytic changes (n = 18, 78.3%). Patients were treated via debridement (n = 16, 72.7%), chemotherapy (n = 5, 22.7%), or debridement with chemotherapy (n = 2, 9.1%). Three patients (12.5%) experienced reactivation. There were 30 patients (18 males) with an average age of 6 years old (9 month to 15 years) with SS+ LCH. Patients presented with persistent local swelling (n = 21, 70%), recurrent ear infection (n = 5, 16.7%), localized pain (n = 4, 13.3%), or systemic symptoms (n = 2, 6.5%). Radiographic findings were osteolytic changes (n = 24, 77.4%) and/or soft-tissue mass (15, 48.4%). All except 2 patients completed treatment consist of debridement (n = 1, 3.3%), chemotherapy (n = 17, 56.7%), or debridement with chemotherapy (n = 11, 36.7%). One patient (3.3%) who was treated with chemotherapy had disease reactivation. Thirteen patients (9 males) with an average age of 2 years old (1 day to 7 years) had MS LCH. LCH was found in skin (n = 9, 69.2%), lymphatic system (n = 6, 46.2%), and at least 1 bone (range 1-5). In this group, patients presented with extracranial visceral organ involvement on imaging (e.g., hepatosplenomegaly, pulmonary nodules). Patients were treated with chemotherapy (n = 12, 92.3%) or debridement with chemotherapy (n = 1, 7.7%). Six patients (40%) experienced reactivation, and 1 patient passed away during treatment. There are 3 separate categories of LCH in the head and neck. Oral and maxillofacial surgeons who provide care to children would benefit from approaching head and neck LCH based on these categories. A referral to hematology/oncology is necessary for appropriate diagnosis and management.

Children with orofacial deformity may require repeated imaging of the facial skeleton.To test the feasibility and accuracy of "black bone" magnetic resonance imaging (MRI) for assessing facial deformity in children.Three-dimensional (3-D) black bone gradient echo sequences (flip angle 5°, submillimetre spatial resolution) from 10 children (median age: 13 years, range: 2-16 years), who underwent MRI of the temporomandibular joints, were evaluated with multiplanar reconstruction and 3-D rendering tools. Intra- and inter-reader agreement was investigated for measuring the height of the mandibular ramus and condyle, basal length of the mandible, gonion angle and mandibular inclination angle by intraclass correlation coefficient (ICC) and Bland-Altman analysis. Absolute percentage error was calculated with the average of all measurements serving as reference.Sixty linear and 40 angle measurements were obtained on reformatted multiplanar black bone images with excellent inter-reader agreement (ICC 0.99, agreement bias 1.4 mm/ 1.5°) and small error (median absolute error 3%). The black bone images required inversion of the signal intensity and removal of air before they could be processed with standard volume rendering tools. The diagnostic utility of 3-D views for assessing the facial skeleton was sufficient except for assessing dental relationship.Morphometric measurements of the mandible can be obtained from black bone MRI with comparable inter-rater agreement to that reported for cone beam computed tomography (CT). With improvements of 3-D rendering techniques and software, black bone MRI may become a radiation-free alternative to CT in children with facial deformities.

Objective The coronavirus disease 2019 (COVID-19) pandemic caused delays in medical and surgical interventions in most health care systems worldwide. Oral and maxillofacial surgeons (OMSs) delayed operations to protect themselves, patients, and staff. This article (1) presents one institution's experience in the management of pediatric craniomaxillofacial trauma during the COVID-19 pandemic and (2) suggests recommendations to decrease transmission. Methods This was a retrospective review of children aged 18 years or younger who underwent surgery at Children's Healthcare of Atlanta in Atlanta, GA, between March and August 2020. Patients (1) were aged 18 years old or younger, (2) had one or more maxillofacial fractures, and (3) underwent surgery performed by an OMS, otolaryngologist, or plastic surgeon. Medical records were reviewed regarding (1) fracture location, (2) COVID-19 status, (3) timing, (4) personal protective equipment, and (5) infection status. Descriptive statistics were computed. Results Fifty-eight children met the inclusion criteria. The most commonly injured maxillofacial location was the nose. Operations were performed 50.9 hours after admission. Specific prevention perioperative guidelines were used with all patients, with no transmission occurring from a patient to a health care worker. Conclusions With application of our recommendations, there was no transmission to health care workers. We hope that these guidelines will assist OMSs during the COVID-19 pandemic.

Current strategies for regeneration of large bone fractures yield limited clinical success mainly due to poor integration and healing. Multidisciplinary approaches in design and development of functional tissue engineered scaffolds are required to overcome these translational challenges. Here, a new generation of hyperelastic bone (HB) implants, loaded with superparamagnetic iron oxide nanoparticles (SPIONs), are 3D bioprinted and their regenerative effect on large non-healing bone fractures is studied. Scaffolds are bioprinted with the geometry that closely correspond to that of the bone defect, using an osteoconductive, highly elastic, surgically friendly bioink mainly composed of hydroxyapatite. Incorporation of SPIONs into HB bioink results in enhanced bacteriostatic properties of bone grafts while exhibiting no cytotoxicity. In vitro culture of mouse embryonic cells and human osteoblast-like cells remain viable and functional up to 14 days on printed HB scaffolds. Implantation of damage-specific bioprinted constructs into a rat model of femoral bone defect demonstrates significant regenerative effect over the 2-week time course. While no infection, immune rejection, or fibrotic encapsulation is observed, HB grafts show rapid integration with host tissue, ossification, and growth of new bone. These results suggest a great translational potential for 3D bioprinted HB scaffolds, laden with functional nanoparticles, for hard tissue engineering applications.

Objective To describe the implementation of telemedicine in a pediatric otolaryngology practice during the coronavirus disease 2019 (COVID-19) global pandemic. Methods A descriptive paper documenting the development and application of telemedicine in a tertiary academic pediatric otolaryngology practice. Results A total of 51 established patients were seen via telemedicine within the first 2 weeks of telemedicine implementation. Seven (7) patients were no shows to the appointment. The median patient age was 5 years old, with 55% male patients. Common diagnoses for the visits included sleep disordered breathing/obstructive sleep apnea (25%) and hearing loss (19.64%). Over half (50.98%) of visits were billed at level 4 visit code. Discussion The majority (88%) of visits during the first 2 weeks of telemedicine implementation in our practice were completed successfully. Reasons that patients did not schedule telemedicine appointments included preference for in person appointments, and lack of adequate device at home to complete telemedicine visit. Limitations to our telemedicine practice included offering telemedicine only to patients who had home internet service, were established patients, and English-speaking. Trainees were not involved in this initial implementation of telemedicine. Conclusions COVID-19 has driven the rapid adoption of telemedicine in outpatient medicine. Our group was able to institute an effective telemedicine practice during this time.

To assess the effect of 3-dimensional (3D)-printed surgical simulators used in an advanced pediatric otolaryngology fellowship preparatory course on trainee education.Quasi-experimental pre/postsurvey.Multicenter collaborative course conducted at a contract research organization prior to a national conference.A 5-station, 7-simulator prep course was piloted for 9 pediatric otolaryngology fellows and 17 otolaryngology senior residents, with simulators for airway graft carving, microtia ear framework carving, and cleft lip/palate repair. Prior to the course, trainees were provided educational materials electronically along with presurveys rating confidence, expertise, and attitude around surgical simulators. In October 2018, surgeons engaged in simulation stations with direction from 2 attending faculty per station, then completed postsurveys for each simulator.Statistically significant increases (We demonstrate the benefit of high-fidelity, 3D-printed simulators in exposing trainees to advanced procedures, allowing them hands-on practice in a zero-risk environment. In the future, we hope to refine this course design, develop standardized tools to assess their educational value, and explore opportunities for integration into use in milestone assessment and accreditation.

Cleft lip and palate are common craniofacial deformities. The etiology underlying these deformities is complex and multifactorial and they can occur as part of one of many chromosomal syndromes, Mendelian single gene disorders, teratogenic effects, and as yet uncharacterized syndromes. Our paper will provide an overview of the multiple genes and molecular pathways that have been implicated in palatal fusion. We believe that understanding the molecular mechanisms of cleft formation can help clinicians anticipate which patients may have difficulties healing and in the future allow them to make surgical and medical treatment decisions based on genetic information.

INTRODUCTION: Obstructive sleep apnea (OSA) is prevalent and may be more severe in children with Sickle Cell Disease (SCD) compared to the general pediatric population. OBJECTIVES: The objective of this study was to describe the therapeutic effects and complications of tonsillectomy and adenoidectomy (T&A) for treatment of OSA in children with SCD. METHODS: A comprehensive database of pediatric SCD patients was reviewed to identify all patients who underwent T&A between 2010 and 2016. An IRB-approved, retrospective review of laboratory values, perioperative course, pre- and post-T&A hospital utilization, and polysomnography was conducted. RESULTS: There were 132 SCD children (108 HbSS) who underwent T&A. Mean age was 7.6 ± 4.6 years. The mean baseline hemoglobin of these patients was 9.3 ± 1.4g/dL; 72.7% of patients had pre-operative transfusion, such that the mean Hb at time of T&A was 11.4 ± 1.0 g/dL. The average admission length surrounding T&A was 3.5 ±1.2 days. Complications were documented in 11.4% of operative cases. Polysomnography was available in 104 pre-T&A and 45 post-T&A. The Apnea-Hypopnea Index decreased on post-T&A polysomnogram (7.6 ± 8.7 vs. 1.3 ± 1.9, p = 0.0001). The O2 nadir improved on post-T&A polysomnogram (81.2 ± 10.8 vs. 89.3 ± 7, p = 0.0003). Emergency room visits (mean events per year) decreased post-operatively (2.6 ± 2.8 vs. 1.8 ± 2.2, p = 0.0002). CONCLUSIONS: T&A can be a safe and effective option to treat OSA in pediatric patients with SCD and was significantly associated with reduced AHI and fewer ER visits post-operatively.

Despite humanitarian otolaryngology groups traveling in record numbers to resource-limited areas treating pediatric otolaryngology disease processes and training local providers, there remains a large burden of unmet needs. There is a meager amount of published information that comes from the developing world from an otolaryngology standpoint. As would be expected, the little information that does comes involves some of the most common pediatric otolaryngology diseases and surgical burdens including childhood hearing loss, otitis media, adenotonsillectomies, airway obstructions requiring tracheostomies, foreign body aspirations, and craniomaxillofacial surgeries, including cleft lip and palate.

Osteoblast commitment and differentiation are controlled by multiple growth factors including members of the Notch signaling pathway. JAGGED1 is a cell surface ligand of the Notch pathway that is necessary for murine bone formation. The delivery of JAGGED1 to induce bone formation is complicated by its need to be presented in a bound form to allow for proper Notch receptor signaling. In this study, we investigate whether the sustained release of JAGGED1 stimulates human mesenchymal cells to commit to osteoblast cell fate using polyethylene glycol malemeide (PEG-MAL) hydrogel delivery system. Our data demonstrated that PEG-MAL hydrogel constructs are stable in culture for at least three weeks and maintain human mesenchymal cell viability with little cytotoxicity in vitro. JAGGED1 loaded on PEG-MAL hydrogel (JAGGED1-PEG-MAL) showed continuous release from the gel for up to three weeks, with induction of Notch signaling using a CHO cell line with a Notch1 reporter construct, and qPCR gene expression analysis in vitro. Importantly, JAGGED1-PEG-MAL hydrogel induced mesenchymal cells towards osteogenic differentiation based on increased Alkaline phosphatase activity and osteoblast genes expression including RUNX2, ALP, COL1, and BSP. These results thus indicated that JAGGED1 delivery in vitro using PEG-MAL hydrogel induced osteoblast commitment, suggesting that this may be a viable in vivo approach to bone regeneration. © 2017 Wiley Periodicals, Inc. J Biomed Mater Res Part A: 106A: 552-560, 2018.

Introduction Long-term immunosuppressants form an integral part of therapy for post-transplantation patients. Immunosuppressants may also have an anticoagulant effect, and little is known about their effects on bleeding risk after adenotonsillectomy. Our objective was to investigate whether there is an increased observed rate of post-tonsillectomy hemorrhage in a population of pediatric patients on long-term immunosuppressants after solid organ transplantation, compared to healthy controls. Methods This was a retrospective chart review of pediatric patients with a history of renal or heart transplant undergoing adenotonsillectomy at our institution between 2000 and 2014. All patients underwent tonsillectomy with monopolar electrocautery. Retrieved data included perioperative medications, occurrence of post-operative bleeding and associated treatment. For comparison, we obtained a population of age-matched controls with no history of immunosuppression who underwent the same procedure. Results A total of 34 patients meeting criteria were identified, of which 3 (8.82%) suffered a postoperative bleed. Forty-seven controls were obtained, with a total of 2 (4.26%) postoperative hemorrhages (p = 0.65). Two of the post-transplantation patients who bled postoperatively required cauterization in the operating room. None of the controls required surgical treatment. The incidences of postoperative bleeding requiring surgical treatment were 5.88% and 0%, respectively (p = 0.17). Conclusion We failed to demonstrate an increased risk of bleeding after undergoing adenotonsillectomy in our cohort of post-transplantation pediatric patients on chronic immunosuppression. Future research, likely requiring a multi-institutional effort, could stratify by immunosuppressive agent to elucidate bleeding risk with specific medications.

PurposePediatric temporomandibular joint (TMJ) reconstruction occurs as a result of acquired, developmental, and/or congenital conditions. Current pediatric reconstruction options are limited. The aim of this project was to develop a proof of principle porcine model for a load bearing, customized 3-dimensional (3D) printed and BMP2-coated scaffold implanted in a pedicled (temporal) flap as a regenerative approach to pediatric TMJ mandibular condyle reconstruction.Materials and MethodsScaffolds were custom designed and 3D printed based on porcine computed tomography and absorbed with BMP2. Two operations occured: (1) implantation of scaffold in temporalis muscle to establish vascularity, and six weeks later, (2) unilateral condylectomy and rotation of the vascularized scaffold (with preservation of superficial temporal artery) onto mandibular ramus defect and secured with titanium screws. At 6 months post-implantation, the pigs were sacrified. The experimental side (muscle-scaffold) and the control side (unoperated condyle) were individually harvested at 6 months and evaluated by clinical, mechanical, radiographic, and qualitative/histologic methods.ResultsScaffolds maintained physical properties similar in appearance to unoperated condyles. The vascularized scaffolds had bone formation at edges and adjacent to scaffold-bone interface. New bone was visible in scaffold. Condyle height on the reconstructed side was 68% and 78% of the control side. Reconstructed condyle stiffness was between 20% and 45% of the control side.ConclusionIn our porcine model, customized 3D printed TMJ scaffolds impregnanted with BMP2 and implanted in and pedicled on temporalis muscle has the ability to (1) reconstruct a TMJ defect model, (2) maintain appropriate condylar height and upper airway diameter, and (3) generate new bone, without impacting functional outcomes.

Objective To develop an expert-based consensus of recommendations for the diagnosis and management of pediatric obstructive sleep apnea. Methods A two-iterative Delphi method questionnaire was used to formulate expert recommendations by the members of the International Pediatric Otolaryngology Group (IPOG). Results Twenty-six members completed the survey. Consensus recommendations (>90% agreement) are formulated for 15 different items related to the clinical evaluation, diagnosis, treatment, postoperative management and follow-up of children with OSA. Conclusion The recommendations formulated in this IPOG consensus statement may be used along with existing clinical practice guidelines to improve the quality of care and to reduce variation in care for children with OSA.

Advanced practice providers (APPs), including nurse practitioners and physician assistants, have been deployed in children's hospital-based academic pediatric otolaryngology practices for many years. However, this relationship in terms of prevalence, roles, financial consequences and satisfaction has not been examined. The objective of this study is to explore how APPs impact healthcare delivery in this setting.Pediatric otolaryngology chiefs of all academic children's hospitals in the US were electronically surveyed about the ways APPs intersected clinically and financially in their respective practice.A total of 29 of 36 children's hospital-based pediatric otolaryngology practices completed the survey, of which 26 practices (90%) utilized APP. There were large variances within the APP practice cohort in faculty size (mean/median/range = 9.4/8.5/3-29); annual patient visits (mean/median = 18,373/17,600); number of practice site (mean/median/range = 4.3/4/2-9) and number of outpatient APP (mean/median/range = 6.3/5/1-30). No factors (faculty size, annual visits and number of practice sites) differentiated between the APP and non-APP practices. Among APP practices, significant correlation (p.00001) was observed between size of APP cohort to faculty size and annual visits. 69% of the practices did not differentiate job functions of nurse practitioners and physician assistants. 85% of the practices utilized APPs in all practice sites and 19% utilized APPs in the operating room. 77% of APPs billed independently and 46% had on-site supervision. The most prevalent APP salary bracket based on 0-5, 6-10 and 11 years of tenure were $76-100K (65%), $100-150K (77%) and $100-150K (86%), respectively. In 46% of the practices, APPs were able to generate enough revenue to cover more than 75% of their salary and 23% of practices generated a profit. 81% of the chiefs ranked the effectiveness of APPs as high (4 and 5) on a 5-point Likert scale.The majority of academic pediatric otolaryngology practices employed APPs. Despite the diversity seen in practice complexity, APP functionality and financial impact, most found the APP model to be beneficial in improving patient care, patient access and faculty productivity.

Cleft lip and palate are common craniofacial deformities. The etiology underlying these deformities is complex and multifactorial and they can occur as part of one of many chromosomal syndromes, Mendelian single gene disorders, teratogenic effects, and as yet uncharacterized syndromes. Our paper will provide an overview of the multiple genes and molecular pathways that have been implicated in palatal fusion. We believe that understanding the molecular mechanisms of cleft formation can help clinicians anticipate which patients may have difficulties healing and in the future allow them to make surgical and medical treatment decisions based on genetic information.

Objectives/Hypothesis Determine predictive patient characteristics that guide the decision to proceed with surgical management of tongue-based airway obstruction (TBAO) in Pierre Robin sequence (PRS) patients. Study Design Retrospective review of PRS patients between 2005 and 2014 requiring observation in the neonatal intensive care unit (NICU). Methods Patient charts were reviewed for prenatal diagnoses, clinical course, and need for surgical intervention (tracheotomy or mandibular distraction osteogenesis), and the nonsurgical and surgical group were compared with a logistic regression model. Results Thirty-eight PRS patients who were identified with TBAO and required NICU observation had an average follow-up of 5.4 years. Associated anomalies identified in the PRS patients included neurologic disease (n = 6), renal abnormalities (n = 5), limb abnormalities (n = 4), and cardiac abnormalities (n = 12). Nonsurgical management of PRS TBAO included side (n = 12)/stomach positioning (n = 15), oral airway (n = 6), nasopharyngeal airway (NPA) (n = 14), and intubation (n = 12). Surgical intervention occurred in 13 patients, with tracheotomy in eight due to unstable airway, and mandible distraction in five due to NPA dependence. Factors with significant uncontrolled correlations with the need for surgical airways included presence of cardiac disease (P = .03), cardiac disease severity (P = .03), neurologic disease (P = .01), and continuous positive airway pressure (CPAP)/bilevel positive airway pressure (BiPAP) use (P = .006). Further, stepwise regression showed strong predictive value for CPAP/BiPAP use (odds ratio [OR]: 10.43) and presence of neurological disease (OR: 9.16). PRS TBAO patients required multiple modalities of noninvasive surgical intervention to stabilize their airway. Conclusions This study identified patient characteristics predictive of progression to a surgical airway to address TBAO, which may decrease healthcare utilization and improve quality of life for these families. Level of Evidence 4 Laryngoscope, 127:945–949, 2017

Objectives/Hypothesis Superior pediatric orbital subperiosteal abscesses (SPAs) are less common than medial ones, and clinical features specific to patients with superior SPAs have not been well defined. Clinical characteristics between patients with superior and medial SPAs are compared to determine whether superior location is a risk factor for surgical intervention. Study Design Retrospective cohort study. Methods The target population consisted of patients diagnosed with an SPA and seen by the pediatric otolaryngology service at a tertiary children's hospital between January 2010 and October 2014. Imaging characteristics including proptosis, hypoglobus, intraorbital air, and abscess volume as well as treatment interventions were reviewed. Results Forty patients between 5 and 17 years of age treated for an orbital SPA were identified. Thirteen patients were identified as having superior SPAs; 27 had medial SPAs. The average ages in the two groups were 10.92 and 9.26 years, respectively. The odds ratio for surgical treatment per each increasing year of age was 1.5 (P = .004). The proportion of patients requiring surgery was significantly different between the groups (12/13 superior vs. 13/27 medial, P = .01). The predominant organism group cultured in surgical patients was Streptococcus anginosus (8/24, 29.17%). Superior SPA patients had significantly more proptosis, hypoglobus, and abscess volume on computed tomography scan. Conclusions Patients with superior SPAs may present with more advanced disease, leading to a higher rate of characteristics such as proptosis, hypoglobus, and intraorbital air, factors that would predispose to surgical drainage. We found that abscess volume was the most predictive of surgery. Level of Evidence 4 Laryngoscope, 2016

Objectives To provide recommendations to otolaryngologists, pulmonologists, and allied clinicians for tracheostomy decannulation in pediatric patients. Methods An iterative questionnaire was used to establish expert recommendations by the members of the International Pediatric Otolaryngology Group. Results Twenty-six members completed the survey. Recommendations address patient criteria for decannulation readiness, airway evaluation prior to decannulation, decannulation protocol, and follow-up after both successful and failed decannulation. Conclusion Tracheostomy decannulation recommendations are aimed at improving patient-centered care, quality and safety in children with tracheostomies.

Objective To make recommendations on the identification, routine evaluation, and management of fetuses at risk for airway compromise at delivery. Methods Recommendations are based on expert opinion by members of the International Pediatric Otolaryngology Group (IPOG). A two-iterative Delphi method questionnaire was distributed to all members of the IPOG and responses recorded. The respondents were given the opportunity to comment on the content and format of the survey, which was modified for the second round. “Consensus” was defined by >80% respondent affirmative responses, “agreement” by 51–80% affirmative responses, and “no agreement” by 50% or less affirmative responses. Results Recommendations are provided regarding etiologies of perinatal airway obstruction, imaging evaluation, adjunct evaluation, multidisciplinary team and decision factors, micrognathia management, congenital high airway obstruction syndrome management, head and neck mass management, attended delivery procedure, and delivery on placental support procedure. Conclusions Thorough evaluation and thoughtful decision making are required to optimally balance fetal and maternal risks/benefits.

Assess interrater agreement of endoscopic assessment of velopharyngeal (VP) function before and after viewing the video instruction tool (VIT). We hypothesized improvement in interrater agreement using the Golding-Kushner scale (GKS) after viewing the VIT.Prospective study.Multi-institutional.Sixteen fellowship-trained pediatric otolaryngologists who treat velopharyngeal insufficiency (VPI) rated 50 video segments using the GKS before and after watching the VIT. Raters assessed gap size percentage and lateral pharyngeal wall (LPW), soft palate (SP), and posterior pharyngeal wall (PPW) movement. Intraclass correlation coefficient was estimated for these continuous measures. Raters also indicated the presence of a palatal notch, Passavant's ridge, and aberrant pulsations (categorical variables). Fleiss κ coefficient was used for categorical variables. Wilcoxon signed-rank test was performed on the difference between the pre/post individual video ratings.Reliability improved for all continuous variables after watching the instructional video. The improvement was significant for PPW (0.22-0.30, P.001), SP (left: 0.63-0.68, P.001 and right: 0.64-0.68, P = .001), and LPW (left: 0.49-0.54, P = .01 and right: 0.49-0.54, P = .09) but not significant for gap size (0.65-0.69, P = .36). Among categorical variables, agreement on Passavant's ridge significantly improved (0.30-0.36, P = .03).Exposure to a video instruction tool improves interrater agreement of endoscopic assessment of VP function. Significant improvement was observed in our primary end points, specifically posterior pharyngeal wall movement, soft palate movement, and lateral pharyngeal wall movement. There was less impact of the VIT on the interrater agreement of the categorical variables, palatal notch, Passavant's ridge, and aberrant pulsations.

Introduction Sensorineural hearing loss (SNHL) has been reported to occur at increased frequency in the pediatric sickle cell disease (SCD) population, likely secondary to ototoxic medication regimens and repeat sickling events that lead to end organ damage. Risk and protective factors of SNHL in this population are not fully characterized. The objective of this study was to describe audiology results in children with SCD and the prevalence and sequelae of SNHL. Methods A comprehensive clinical database of 2600 pediatric SCD patients treated at 1 institution from 2010-16 was retrospectively reviewed to identify all patients who were referred for audiologic testing. Audiologic test results, patient characteristics, and SCD treatments were reviewed. Results 181 SCD children (97 male, 153 HbSS) underwent audiologic testing, with 276 total audiology encounters, ranging 1–9 per patient. Mean age at first audiogram was 8.9 ± 5.2 years. 29.8% had prior cerebrovascular infarct and an additional 25.4% had prior abnormal transcranial Doppler screens documented at time of first audiogram. Overall, 13.3% had documented hearing loss, with 6.6% SNHL. Mean pure tone average (PTA) among patients with SNHL ranged from mild to profound hearing loss (Right: 43.3 ± 28.9, Left: 40.8 ± 29.7), sloping to more severe hearing loss at higher frequencies. Conclusions Hearing loss was identified in a significant subset of children with SCD and the hearing loss ranged from normal to profound. Though the overall prevalence of SNHL in SCD patients was low, baseline audiology screening should be considered.

During craniofacial development, cranial neural crest (CNC) cells migrate into the developing face and form bone through intramembranous ossification. Loss of JAGGED1 (JAG1) signaling in the CNC cells is associated with maxillary hypoplasia or maxillary bone deficiency (MBD) in mice and recapitulates the MBD seen in humans with Alagille syndrome. JAGGED1, a membrane-bound NOTCH ligand, is required for normal craniofacial development, andJagged1mutations in humans are known to cause Alagille Syndrome, which is associated with cardiac, biliary, and bone phenotypes and these children experience increased bony fractures. Previously, we demonstrated deficient maxillary osteogenesis inWnt1-cre;Jagged1f/f(Jag1CKO) mice by conditional deletion ofJagged1in maxillary CNC cells. In this study, we investigated the JAG1 signaling pathways in a CNC cell line. Treatment with JAG1 induced osteoblast differentiation and maturation markers,Runx2andOcn, respectively, Alkaline Phosphatase (ALP) production, as well as classic NOTCH1 targets,Hes1andHey1.While JAG1-inducedHes1andHey1expression levels were predictably decreased after DAPT (NOTCH inhibitor) treatment, JAG1-inducedRunx2andOcnlevels were surprisingly constant in the presence of DAPT, indicating that JAG1 effects in the CNC cells are independent of the canonical NOTCH pathway. JAG1 treatment of CNC cells increased Janus Kinase 2 (JAK2) phosphorylation, which was refractory to DAPT treatment, highlighting the importance of the non-canonical NOTCH pathway during CNC cells osteoblast commitment. Pharmacologic inhibition of JAK2 phosphorylation, with and without DAPT treatment, upon JAG1 induction reduced ALP production and,Runx2andOcngene expression. Collectively, these data suggest that JAK2 is an essential component downstream of a non-canonical JAG1-NOTCH1 pathway through which JAG1 stimulates expression of osteoblast-specific gene targets in CNC cells that contribute to osteoblast differentiation and bone mineralization.

Background Pediatric surgical risk assessment tools use patient- and procedure-specific variables to predict postoperative complications. These tools assist clinicians in preoperative counseling and surgical decision-making. The objective of this systematic literature review was to compile and compare existing pediatric surgical risk tools that are broadly applicable across pediatric surgical specialties. Methods A systematic literature review was performed following the Preferred Reporting Items for Systematic Reviews (PRISMA) guidelines. Relevant publications were identified and screened based on predefined eligibility criteria: (1) a preoperative risk assessment tool predicting postoperative complications or mortality, (2) applicable across various surgical specialties, and (3) pertinent to the pediatric population. Studies with specialty- or procedure-specific risk scores and validation studies were excluded. Included articles were assessed for quality and risk of bias by using the Newcastle–Ottawa Scale. Results Four studies met inclusion criteria. Risk factors were evaluated across the models as proxies for operative suitability of patients before surgery. Risk factors common to all studies were the presence of cardiovascular or neurological diseases and history of prematurity. Three of the four included studies defined most risk factors in binary terms, whereas one study used a scale of severity of organ system disease when defining preoperative risk. Generated risk score models provided good to strong concordance with inpatient mortality or postoperative complications, with c-statistic values ranging from 0.77 to 0.98. Conclusions Each study reported an assessment of a novel, generally applicable pediatric surgical risk assessment tool for risk-stratifying children preoperatively for complications that rise after surgery. More studies are needed to assess generalizability in all populations and procedures.

This article focuses on the embryologic development and growth of the temporomandibular joint (TMJ) and touches on the development and growth of surrounding structures. Aberrations in structures surrounding the TMJ can affect its development as well. The normal adult anatomy of the TMJ is described as well as common malformations. The clinical ramifications of a malformed TMJ are also discussed in order to understand future necessary consultants involved in the care of these patients.

Congenital high airway obstruction syndrome (CHAOS) encompasses a heterogeneous group of pathologies leading to poor lung development and difficulty oxygenating the newborn after delivery. While previously uniformly fatal, the ex utero intrapartum therapy (EXIT) procedure has provided a method to treat these patients and provide an airway to potentiate survival. We present a patient diagnosed prenatally with CHAOS secondary to tracheal atresia complicated by severe intra-uterine growth restriction (IUGR) who was successfully delivered via an EXIT procedure at 33-weeks. Multidisciplinary care and planning is paramount.

Objective The purpose of this study was to determine the prevalence and characterize the types of hearing loss in pediatric patients with 22q11.2 deletion syndrome (22q11DS). Methods Fifty-eight patients were identified via retrospective chart review performed of patients with 22q11DS between 1996 and 2014. Patient demographics, pertinent family history, associated comorbidities, and degree and type of hearing loss were gathered for each patient. A literature review of the National Library of Medicine's database with a focus on hearing loss and 22q11DS was performed. Results 22 patients (38%) were found to have hearing impairment: 68% with conductive hearing loss, 14% with sensorineural hearing loss, and 18% with mixed hearing loss. Patients with hearing loss regardless of type had a higher prevalence of developmental delay (55%), cleft palate (23%), articulation disorders (77%), and a greater need for tympanostomy tubes (73%) compared to patients with normal hearing. Temporal bone computed tomography scans of 5 patients revealed a variety of abnormalities in the middle and/or inner ears. Conclusion Hearing impairment occurs in up to 38% of 22q11DS patients of both conductive and sensorineural types, with the conductive type being the most common. These patients have a greater need for tympanostomy tubes and a higher prevalence of developmental delay and speech articulation disorders. Early hearing screening and treatment is warranted in this population.

Velopharyngeal dysfunction (VPD) is a condition that can greatly impact a child's quality of life. The initial evaluation and workup of patients presenting with VPD is generally straightforward, consisting of history taking and physical examination, perceptual speech analysis, and objective techniques such as nasometry and speech endoscopy. However, there is no standardized treatment option. Multiple surgical and nonsurgical techniques have been described, all with varying approaches to correction of the defect and similarly, with varying risks and outcomes. We aim to provide an overview of this condition and available treatment options, as well as highlight recent updates in management, including the use of cine magnetic resonance imaging, new injectable options for pharyngeal augmentation, and the evolving role of robotic surgery. We also discuss techniques to facilitate teaching during oropharyngeal surgery and our approach to revision surgical planning.

Superior pediatric orbital subperiosteal abscesses (SPAs) are less common than medial ones, and clinical features specific to patients with superior SPAs have not been well defined. Clinical characteristics between patients with superior and medial SPAs are compared to determine whether superior location is a risk factor for surgical intervention.Retrospective cohort study.The target population consisted of patients diagnosed with an SPA and seen by the pediatric otolaryngology service at a tertiary children's hospital between January 2010 and October 2014. Imaging characteristics including proptosis, hypoglobus, intraorbital air, and abscess volume as well as treatment interventions were reviewed.Forty patients between 5 and 17 years of age treated for an orbital SPA were identified. Thirteen patients were identified as having superior SPAs; 27 had medial SPAs. The average ages in the two groups were 10.92 and 9.26 years, respectively. The odds ratio for surgical treatment per each increasing year of age was 1.5 (P = .004). The proportion of patients requiring surgery was significantly different between the groups (12/13 superior vs. 13/27 medial, P = .01). The predominant organism group cultured in surgical patients was Streptococcus anginosus (8/24, 29.17%). Superior SPA patients had significantly more proptosis, hypoglobus, and abscess volume on computed tomography scan.Patients with superior SPAs may present with more advanced disease, leading to a higher rate of characteristics such as proptosis, hypoglobus, and intraorbital air, factors that would predispose to surgical drainage. We found that abscess volume was the most predictive of surgery.4 Laryngoscope, 127:735-740, 2017.

Objective The purpose of this study was to identify prognostic factors associated with improved speech outcomes following surgical correction for velopharyngeal insufficiency (VPI) in pediatric patients with 22q11.2 deletion syndrome (22q11DS). Methods Eighteen patients were identified via retrospective chart review of patients with 22q11DS between 2005 and 2014. Patient characteristics, medical histories, associated comorbidities, surgical procedures, and pre- and postoperative perceptual hypernasality (subjectively rated 1–5 with 5 being the most severe) were gathered for each patient. Results 12 patients (67%) experienced improvement in hypernasality following corrective surgery for VPI. Higher severity of hypernasality preoperatively was found to be indicative of a lower chance of improvement with VPI surgery. Of 8 patients with a preoperative hypernasality score of 5, 3 (38%) showed improvement in hypernasality postoperatively, while 9 out of 10 (90%) of patients with a preoperative hypernasality score less than 5 showed postoperative improvement. Females were also found to have worse speech outcomes compared to males. Conclusion 22q11DS patients presenting with severely hypernasal speech preoperatively are less likely to show improvement in hypernasality following corrective surgery for VPI. Those patients with moderate hypernasality are most likely to benefit from surgery.