Novel treatment for hypotonic airway obstruction and severe obstructive sleep apnea using a nasopharyngeal airway device with 3D printing innovation.

STUDY OBJECTIVES: Pediatric obstructive sleep apnea impacts child and familial well-being. Airway management in patients with hypotonic pharyngeal conditions is complex. Some patients may benefit from continuous positive airway pressure or bilevel positive airway pressure, others may require further invasive measures for treatment. There is critical need for treatment alternatives for patients with pharyngeal hypotonia. METHODS: This is a retrospective case series. Collaboratively with patients, families, biomedical engineers, and medical professionals, a long-term nasopharyngeal airway (NPA) was created to bypass upper airway obstruction. Two patients used a safety pin and tape attachment, and two patients used a novel 3D-printed, self-supporting nasal securement. All 4 patients had polysomnography before and during NPA use. Paired 1-tailed t-tests were conducted to compare apnea-hypopnea index, hypopnea index, obstructive index, and oxygen nadir. RESULTS: Compared to baseline polysomnography, repeat polysomnography with the NPA in place demonstrated statistically significant improvement for apnea-hypopnea index (75.8 ± 36.6 events/h to 8.9 ± 2.9 events/h, P = .03), hypopnea index (45.4 ± 25.8 events/h to 7.7 ± 3.2 events/h, P = .04), and oxygen saturation nadir (60.3 ± 13.0% to 79.3 ± 8.7%, P = .01). The NPA had been used for over 1 year in 3 of the 4 children. Those using the safety pin and tape did report skin irritation due to adhesive required to keep device in place. CONCLUSIONS: Current management of severe upper airway obstruction and obstructive sleep apnea in hypotonic pharyngeal conditions requires a team-based approach to care. A long-term NPA device may be an alternative or temporizing option to continuous positive airway pressure, upper airway surgery, or tracheostomy in children with pharyngeal hypotonia and severe obstructive sleep apnea. Larger studies of this approach are underway to assess efficacy in a range of obstructive sleep apnea severity in this population. CITATION: Powell AR, Srinivasan S, Helman JL, et al. Novel treatment for hypotonic airway obstruction and severe obstructive sleep apnea using a nasopharyngeal airway device with 3D printing innovation. J Clin Sleep Med. 2022;18(10):2497-2502.

A Review of Prenatal Care Delivery to Inform the Michigan Plan for Appropriate Tailored Healthcare in Pregnancy Panel.

OBJECTIVE: To perform a literature review of key aspects of prenatal care delivery to inform new guidelines. DATA SOURCES: A comprehensive review of Ovid MEDLINE, Elsevier's Scopus, Google Scholar, and ClinicalTrials.gov. METHODS OF STUDY SELECTION: We included studies addressing components of prenatal care delivery (visit frequency, routine pregnancy assessments, and telemedicine) that assessed maternal and neonatal health outcomes, patient experience, or care utilization in pregnant individuals with and without medical conditions. Quality was assessed using the RAND/UCLA Appropriateness Methodology approach. Articles were independently reviewed by at least two members of the study team for inclusion and data abstraction. TABULATION, INTEGRATION, AND RESULTS: Of the 4,105 published abstracts identified, 53 studies met inclusion criteria, totaling 140,150 participants. There were no differences in maternal and neonatal outcomes among patients without medical conditions with reduced visit frequency schedules. For patients at risk of preterm birth, increased visit frequency with enhanced prenatal services was inconsistently associated with improved outcomes. Home monitoring of blood pressure and weight was feasible, but home monitoring of fetal heart tones and fundal height were not assessed. More frequent weight measurement did not lower rates of excessive weight gain. Home monitoring of blood pressure for individuals with medical conditions was feasible, accurate, and associated with lower clinic utilization. There were no differences in health outcomes for patients without medical conditions who received telemedicine visits for routine prenatal care, and patients had decreased care utilization. Telemedicine was a successful strategy for consultations among individuals with medical conditions; resulted in improved outcomes for patients with depression, diabetes, and hypertension; and had inconsistent results for patients with obesity and those at risk of preterm birth. CONCLUSION: Existing evidence for many components of prenatal care delivery, including visit frequency, routine pregnancy assessments, and telemedicine, is limited.

The Michigan Plan for Appropriate Tailored Healthcare in Pregnancy Prenatal Care Recommendations.

OBJECTIVE: To describe MiPATH (the Michigan Plan for Appropriate Tailored Healthcare in pregnancy) panel process and key recommendations for prenatal care delivery. METHODS: We conducted an appropriateness study using the RAND Corporation and University of California Los Angeles Appropriateness Method, a modified e-Delphi process, to develop MiPATH recommendations using sequential steps: 1) definition and scope of key terms, 2) literature review and data synthesis, 3) case scenario development, 4) panel selection and scenario revisions, and 5) two rounds of panel appropriateness ratings with deliberation. Recommendations were developed for average-risk pregnant individuals (eg, individuals not requiring care by maternal-fetal medicine specialists). Because prenatal services (eg, laboratory tests, vaccinations) have robust evidence, panelists considered only how services are delivered (eg, visit frequency, telemedicine). RESULTS: The appropriateness of key aspects of prenatal care delivery across individuals with and without common medical and pregnancy complications, as well as social and structural determinants of health, was determined by the panel. Panelists agreed that a risk assessment for medical, social, and structural determinants of health should be completed as soon as individuals present for care. Additionally, the panel provided recommendations for: 1) prenatal visit schedules (care initiation, visit timing and frequency, routine pregnancy assessments), 2) integration of telemedicine (virtual visits and home devices), and 3) care individualization. Panelists recognized significant gaps in existing evidence and the need for policy changes to support equitable care with changing practices. CONCLUSION: The MiPATH recommendations offer more flexible prenatal care delivery for average-risk individuals.

Symptom Prevalence and Genotype-Phenotype Correlations in Patients With TANGO2-Related Metabolic Encephalopathy and Arrhythmias (TRMEA).

BACKGROUND: TANGO2-related metabolic encephalopathy and arrhythmias (TRMEA) is a rare, phenotypically heterogeneous, neurological disease affecting children. METHODS: We conducted a chart review of five children with molecularly confirmed TRMEA diagnosed at our institution and compiled pathogenic variant frequency and symptom prevalence from cases previously reported in the literature. RESULTS: Including those patients in our case series, 76 patients with TRMEA have been described. Developmental delay (93%) and/or regression (71%), spasticity (78%), and seizures (57%) are common in TRMEA and frequently precede life-threatening symptoms such as metabolic decompensation with lactic acidosis (83%), cardiomyopathy (38%), and cardiac arrhythmias (68%). Deletion of exons 3 to 9 is the most common pathogenic variant (39% of alleles). The majority of reported intragenic variants (17 of 27) result in disruption of the reading frame, and no clear genotype-phenotype correlations could be identified for those variants wherein the reading frame is maintained, highlighting instead the variable expressivity of the disease. CONCLUSIONS: Patients with TRMEA frequently experience life-threatening complications that are preceded by common neurological symptoms underscoring the need for pediatric neurologists to be familiar with this condition. Additional work pertaining to disease pathophysiology and potential therapeutics is needed.

Simulation of Cerebrospinal Fluid Leak Repair Using a 3-Dimensional Printed Model.

BACKGROUND: Acquiring proficiency for the repair of a cerebrospinal fluid (CSF) leak is challenging in great part due to its relative rarity, which offers a finite number of training opportunities. OBJECTIVE: The purpose of this study was to evaluates the use of a 3-dimensional (3D) printed, anatomically accurate model to simulate CSF leak closure. METHODS: Volunteer participants completed two simulation sessions. Questionnaires to assess their professional qualifications and a standardized 5-point Likert scale to estimate the level of confidence, were completed before and after each session. Participants were also queried on the overall educational utility of the simulation. RESULTS: Thirteen otolaryngologists and 11 neurosurgeons, met the inclusion criteria. A successful repair of the CSF leak was achieved by 20/24 (83.33%), and 24/24 (100%) during the first and second simulation sessions respectively (average time 04:04 ± 1.39 and 02:10 ± 01:11). Time-to-close-the-CSF-leak during the second session was significantly shorter than the first (p < 0.001). Confidence scores increased across the training sessions (3.3 ± 1.0, before the simulation, 3.7 ± 0.6 after the first simulation, and 4.2 ± 0.4 after the second simulation; p < 0.001). All participants reported an increase in confidence and believed that the model represented a valuable training tool. CONCLUSIONS: Despite significant differences with varying clinical scenarios, 3D printed models for cerebrospinal leak repair offer a feasible simulation for the training of residents and novice surgeons outside the constrictions of a clinical environment.

Development of a novel mandibular distraction osteogenesis simulator using Computer Aided Design and 3D printing.

INTRODUCTION: Micrognathia, a component of Robin Sequence, can cause glossoptosis, failure of palatal fusion, and critical obstruction of the airway. Mandibular distraction osteogenesis (MDO) is at times offered to anteriorly translate the mandible and tongue, relieving airway obstruction. MDO is an intricate reconstructive procedure that may be ideal for teaching using a high-fidelity educational simulator, allowing early hands-on experience in a zero-risk environment. OBJECTIVES: To design a novel, low-cost, high-fidelity neonatal MDO simulator that can be used for trainee education and refinement of surgical technique. METHODS: A novel MDO simulator was developed using additive manufacturing techniques. Three experts in MDO surgery completed a 20-item survey, rating the simulator's physical attributes, the realism of experience, the simulator's value, its relevance to practice and the surgeon's ability to perform tasks on a 4-point Likert scale. RESULTS: Computer Aided Design (CAD) and 3D printing allowed for the production of a realistic surgical simulator that emulates important aspects of MDO surgery. This preliminary evaluation indicated adequate means across the five domains relevant to the simulator's fidelity and usability (M = 3.33 to 3.75) out of a maximum of 4 points. Lowest rated items were consistent with expert comments allowing future refinement on subsequent iterations. Consumable material costs per model were $9.39 USD. CONCLUSIONS: The MDO model demonstrated adequate fidelity and holds promise as a skill-development tool for surgeons in training. Further studies are planned to determine its utility as a training and assessment tool.

Simulation of Pediatric Anterior Skull Base Anatomy Using a 3D Printed Model.

OBJECTIVE: The pediatric skull base may present anatomic challenges to the skull base surgeon, including limited sphenoid pneumatization and a narrow nasal corridor. The rare nature of pediatric skull base pathology makes it difficult to gain experience with these anatomic challenges. The objective of this study was to create a 3-dimensionally (3D) printed model of the pediatric skull base and assess its potential as a training tool. METHODS: Twenty-eight participants at various stages of training and practice were included in our study. They completed a pre- and postdissection questionnaire assessing challenges with endoscopic endonasal skeletonization of the carotid arteries and sella face using the 3D printed model. RESULTS: The majority of participants had completed a skull base surgery fellowship (60.7%), were <5 years into practice (60.7%), and had <10 cases of pediatric skull base experience (82.1%). Anticipated challenges included limitation of maneuverability of instruments (71.4%), narrow nasal corridor and nonpneumatized bone (57.1%). On a scale of 0-10, 10 being very difficult, the average participant expected level of difficulty with visualization was 6.89 and expected level of difficulty with instrumentation was 7.3. On postdissection assessment, there was a nonstatistically significant change to 6.93 and 7.5, respectively. Participants endorsed on a scale of 0-10, 10 being very realistic, an overall model realism of 7.0 and haptic realism of 7.1. CONCLUSIONS: A 3D printed model of the pediatric skull base may provide a realistic model to help participants gain experience with anatomic limitations characteristic of the pediatric anterior skull base.

Three-dimensional modeling of the scapular tip for anterolateral and lateral mandibular defects.

OBJECTIVE: Three-dimensional (3D) computational modeling represents an invaluable surgical tool for complex reconstructive cases. There exists limited data on 3D imaging to evaluate the use of the scapular tip free flap in complex, anterolateral mandibular reconstructions. The purpose of this study is to radiographically assess the scapular tip free flap in anterolateral defects of the mandible. METHODS: A retrospective case series of 50 patients was performed on head and neck cancer patients treated at Michigan Medicine from 2013 to 2018. Patients who had pre-operative computed tomography neck and thorax for review were included. RESULTS: The scapular tip was able to cover defects from the ipsilateral mandibular angle to the symphysis in all cases. On average, the bone extended to cover defects from the mandibular angle to 17 mm (SD ± 3.6 mm) beyond the mandibular symphysis, which equates to 70% (95% CI 0.66-0.74) of the distance from the symphysis to the contralateral mental foramen. CONCLUSION: Use of the scapular tip as a donor site for reconstruction of the mandibular body can be evaluated pre-operatively utilizing 3D imaging. All patients were able to achieve posterior coverage to the vertical plane of the mandibular condyle and contralateral extension across the symphysis on average extends almost three-fourths of the distance to the mandibular foramen. The extent of contralateral coverage can be more accurately delineated utilizing the patient's pre-operative imaging.

Development and Multidisciplinary Preliminary Validation of a 3-Dimensional-Printed Pediatric Airway Model for Emergency Airway Front-of-Neck Access Procedures.

BACKGROUND: Pediatric-specific difficult airway guidelines include algorithms for 3 scenarios: unanticipated difficult tracheal intubation, difficult mask ventilation, and cannot intubate/cannot ventilate. While rare, these instances may require front-of-neck access (FONA) to secure an airway until a definitive airway can be established. The aim of this study was to develop a pediatric FONA simulator evaluated by both anesthesiology and otolaryngology providers, promoting multidisciplinary airway management. METHODS: A 3-dimensional-printed tracheal model was developed using rescaled, anatomically accurate dimensions from a computerized tomography scan using computer-aided design software. The medical grade silicone model was incorporated into a mannequin to create a low-cost, high-fidelity simulator. A multidisciplinary team of anesthesiology, otolaryngology, and simulation experts refined the model. Experts in airway management were recruited to rate the realism of the model's characteristics and features and their own ability to complete specific FONA-related tasks. RESULTS: Six expert raters (3 anesthesiology and 3 otolaryngology) were identified for multidisciplinary evaluation of model test content validity. Analysis of response data shows null variance within 1 or both specialties for a majority of the content validity tool elements. High and consistent absolute ratings for each domain indicate that the tested experts perceived this trainer as a realistic and highly valuable tool in its current state. CONCLUSIONS: The ability to practice front-of-neck emergency airway procedures safely and subsequently demonstrate proficiency on a child model has great implications regarding both quality of physician training and patient outcomes. This model may be incorporated into curricula to teach needle cricothyroidotomy and other FONA procedures to providers across disciplines.

Development of a high fidelity subglottic stenosis simulator for laryngotracheal reconstruction rehearsal using 3D printing.

INTRODUCTION: Laryngotracheal reconstruction (LTR) with cartilage graft augmentation is an effective treatment for subglottic stenosis and a critical advanced procedure for Pediatric Otolaryngologists. Trainees almost exclusively learn this procedure intraoperatively on children due to the lack of adequate pediatric training models. An enhanced and accelerated educational experience may be possible if trainees can rehearse the key portions of the procedure on a simulation model. OBJECTIVE: To design and manufacture a low-cost, high fidelity surgical simulation model of subglottic stenosis for LTR. METHODS: This simulator is composed of two component models: rib cartilage and trachea. Additive manufacturing techniques, including Computer Aided Design and Three Dimensional (3D) printing, were utilized to create the simulator. Three expert Pediatric Otolaryngologists rated the functionality and realism of the simulator using Likert scale survey data. RESULTS: The use of CAD and 3D printing techniques allowed for realistic, reproducible surgical simulation of key aspects of LTR. The validation evidence indicated good to excellent means across the five domains relevant to the simulator's fidelity and usability (M = 3.47 to 4.00) out of a maximum of 4 points. Lowest rated items were consistent with expert comments suggesting minor simulator improvements. Time of production is approximately 20 h from print to post-processing, and consumable material costs per model are $2.60 USD. CONCLUSIONS: This subglottic stenosis airway simulator facilitated Laryngotracheal Reconstruction rehearsal and is a promising training tool for pediatric otolaryngologists. Our methods allow patient-specific, pre-surgical rehearsal for complex airway scenarios that could benefit the experienced airway surgeon and trainees alike. Future research aims to validate this device's utility for accelerating attainment of proficiency and improving surgical outcomes.

Computer-Aided Design, 3-D-Printed Manufacturing, and Expert Validation of a High-fidelity Facial Flap Surgical Simulator.

IMPORTANCE: Facial flap procedures may be difficult for surgical trainees to conceptualize and challenging for supervising surgeons to allow entrustment early in training. Simulation outside of the operating room may accelerate and enhance the surgical education experience. OBJECTIVE: To design and manufacture a 3-dimensional (3-D)-printed, multilayer, anatomically accurate facial flap model for use in surgical education. DESIGN, SETTING, AND PARTICIPANTS: In this multicenter validation study, a 3-D-printed facial flap simulator was designed from a computed tomographic (CT) scan and manufactured for low-cost, high-fidelity simulation. Expert otolaryngology-head and neck surgeon feedback was acquired through surgical rehearsal and performance of 8 local facial flap procedures on the facial flap simulator by 7 otolaryngologists fellowship trained in facial plastic surgery. MAIN OUTCOMES AND MEASURES: Likert scale surveys were made based on evaluation criteria categorized into domains of realism, experience, and applicability of the simulator. Measures of central tendency, variability, and confidence intervals were generated to evaluate the outcomes. RESULTS: Seven expert otolaryngology-head and neck surgeons completed a Likert scale evaluation survey containing quantitative analysis of 6 questions on physical attributes, 12 questions on realism, 8 questions on experience, and 4 questions on the applicability of the simulator. All expert surgeons were additionally fellowship trained in facial plastic surgery with their mean years in practice being 11.9. Overall evaluation demonstrated valuable ability of the simulator for medical education with suggestions for future directions. Importantly, the simulator was rated on a scale of 1 (no value) to 4 (great value) as 3.86 as a training tool, 3.57 as a competency evaluation tool, and 3.43 as a rehearsal tool. CONCLUSIONS AND REVELANCE: Expert experience with the local facial flap simulator was rated highly for realism, experience, performance, and usefulness. With slight refinement, the model has strong potential for broad use in training in otolaryngology-head and neck surgery and facial plastic surgery. LEVEL OF EVIDENCE: NA.