The common variable immunodeficiency IgM repertoire narrowly recognizes erythrocyte and platelet glycans.

BACKGROUND: Autoimmune cytopenias (AICs) regularly occur in profoundly IgG-deficient patients with common variable immunodeficiency (CVID). The isotypes, antigenic targets, and origin(s) of their disease-causing autoantibodies are unclear. OBJECTIVE: We sought to determine reactivity, clonality, and provenance of AIC-associated IgM autoantibodies in patients with CVID. METHODS: We used glycan arrays, patient erythrocytes, and platelets to determine targets of CVID IgM autoantibodies. Glycan-binding profiles were used to identify autoreactive clones across B-cell subsets, specifically circulating marginal zone (MZ) B cells, for sorting and IGH sequencing. The locations, transcriptomes, and responses of tonsillar MZ B cells to different TH- cell subsets were determined by confocal microscopy, RNA-sequencing, and cocultures, respectively. RESULTS: Autoreactive IgM coated erythrocytes and platelets from many CVID patients with AICs (CVID+AIC). On glycan arrays, CVID+AIC plasma IgM narrowly recognized erythrocytic i antigens and platelet i-related antigens and failed to bind hundreds of pathogen- and tumor-associated carbohydrates. Polyclonal i antigen-recognizing B-cell receptors were highly enriched among CVID+AIC circulating MZ B cells. Within tonsillar tissues, MZ B cells secreted copious IgM when activated by the combination of IL-10 and IL-21 or when cultured with IL-10/IL-21-secreting FOXP3-CD25hi T follicular helper (Tfh) cells. In lymph nodes from immunocompetent controls, MZ B cells, plentiful FOXP3+ regulatory T cells, and rare FOXP3-CD25+ cells that represented likely CD25hi Tfh cells all localized outside of germinal centers. In CVID+AIC lymph nodes, cellular positions were similar but CD25hi Tfh cells greatly outnumbered regulatory cells. CONCLUSIONS: Our findings indicate that glycan-reactive IgM autoantibodies produced outside of germinal centers may contribute to the autoimmune pathogenesis of CVID.

International pediatric otolaryngology group (IPOG) consensus on approach to aspiration.

OBJECTIVE: To provide recommendations for a comprehensive management approach for infants and children presenting with symptoms or signs of aspiration. METHODS: Three rounds of surveys were sent to authors from 23 institutions worldwide. The threshold for the critical level of agreement among respondents was set at 80 %. To develop the definition of "intractable aspiration," each author was first asked to define the condition. Second, each author was asked to complete a 5-point Likert scale to specify the level of agreement with the definition derived in the first step. RESULTS: Recommendations by the authors regarding the clinical presentation, diagnostic considerations, and medical and surgical management options for aspiration in children. CONCLUSION: Approach to pediatric aspiration is best achieved by implementing a multidisciplinary approach with a comprehensive investigation strategy and different treatment options.

Human T follicular helper clones seed the germinal center-resident regulatory pool.

The mechanisms by which FOXP3+ T follicular regulatory (Tfr) cells simultaneously steer antibody formation toward microbe or vaccine recognition and away from self-reactivity remain incompletely understood. To explore underappreciated heterogeneity in human Tfr cell development, function, and localization, we used paired TCRVA/TCRVB sequencing to distinguish tonsillar Tfr cells that are clonally related to natural regulatory T cells (nTfr) from those likely induced from T follicular helper (Tfh) cells (iTfr). The proteins iTfr and nTfr cells differentially expressed were used to pinpoint their in situ locations via multiplex microscopy and establish their divergent functional roles. In silico analyses and in vitro tonsil organoid tracking models corroborated the existence of separate Treg-to-nTfr and Tfh-to-iTfr developmental trajectories. Our results identify human iTfr cells as a distinct CD38+, germinal center-resident, Tfh-descended subset that gains suppressive function while retaining the capacity to help B cells, whereas CD38- nTfr cells are elite suppressors primarily localized in follicular mantles. Interventions differentially targeting specific Tfr cell subsets may provide therapeutic opportunities to boost immunity or more precisely treat autoimmune diseases.

Tracheostomy in the extremely premature neonate - Long term outcomes in a multi-institutional study.

OBJECTIVES: To describe the long-term outcomes related to breathing, feeding, and neurocognitive development in extremely premature infants requiring tracheostomy. STUDY DESIGN: Pooled cross-sectional survey. SETTING: Multi-institutional academic children's hospitals. METHODS: Extremely premature infants who underwent tracheostomy between January 1, 2012, and December 31, 2019, at four academic hospitals were identified from an existing database. Information was gathered from responses to a questionnaire by caregivers regarding airway status, feeding, and neurodevelopment 2-9 years after tracheostomy. RESULTS: Data was available for 89/91 children (96.8%). The mean gestational age was 25.5 weeks (95% CI 25.2-25.7) and mean birth weight was 0.71 kg (95% CI 0.67-0.75). Mean post gestational age at tracheostomy was 22.8 weeks (95% CI 19.0-26.6). At time of the survey, 18 (20.2%) were deceased. 29 (40.8%) maintained a tracheostomy, 18 (25.4%) were on ventilatory support, and 5 (7%) required 24-h supplemental oxygen. Forty-six (64.8%) maintained a gastrostomy tube, 25 (35.2%) had oral dysphagia, and 24 (33.8%) required a modified diet. 51 (71.8%) had developmental delay, 45 (63.4%) were enrolled in school of whom 33 (73.3%) required special education services. CONCLUSIONS: Tracheostomy in extremely premature neonates is associated with long term morbidity in the pulmonary, feeding, and neurocognitive domains. At time of the survey, about half are decannulated, with a majority weaned off ventilatory support indicating improvement in lung function with age. Feeding dysfunction is persistent, and a significant number will have some degree of neurocognitive dysfunction at school age. This information may help caregivers regarding expectations and plans for resource management.

Systemic Bevacizumab Treatment for Recurrent Respiratory Papillomatosis: Long-Term Follow-Up.

OBJECTIVES/HYPOTHESIS: Systemic bevacizumab is a new adjuvant therapy for recurrent respiratory papillomatosis (RRP) that has shown promising preliminary results in children. The objective of this study was to report the largest series to date that includes long-term follow-up data on bevacizumab treatment. STUDY DESIGN: Retrospective chart review. METHODS: Retrospective review of seven pediatric patients treated within the past 6 years with systemic bevacizumab for RRP refractory to traditional debridement. RESULTS: All seven patients had a significant reduction in disease burden after initiation of systemic bevacizumab. There have been no major complications associated with systemic therapy so far. Median duration of bevacizumab treatment was 2.13 years. Three of the seven patients have been on treatment for over 3 years with the longest duration of treatment in our first patient now at 5.5 years. One patient experienced significant disease recurrence on two occasions when therapy was temporarily discontinued and was recently diagnosed with squamous cell carcinoma of the lung. CONCLUSION: Systemic bevacizumab is an effective therapy for cases of severe RRP with promising results both in short-term and long-term follow-up. Side effects are minimal. Patients must be followed closely to determine appropriate dosing intervals to control disease and to screen for disease progression. LEVEL OF EVIDENCE: 4 Laryngoscope, 132:2071-2075, 2022.

Intubation-Related Laryngeal Deficiency and Vocal Fold Immobility in Pediatric Premature Patients.

OBJECTIVES/HYPOTHESIS: We report a posterior laryngeal rating system and measures of voice disability in pediatric patients undergoing phonosurgery for vocal fold paralysis. Posterior glottic deficiency may account for persistent voice disability. STUDY DESIGN: Retrospective Study. METHODS: Retrospective analyses of 66 subjects with primary unilateral vocal fold paralysis were reviewed for the status of posterior glottis and voice disability (Pediatric Voice Handicap Index [pVHI]). Gestation age (GA), weight, and medical/surgical history were reviewed. The width, length, and depth of the larynx were analyzed to create a reproducible rating scale. RESULTS: Mean GA was 29 weeks, with an intubation history for all subjects, with 90% having a left vocal fold immobility. Cardiac surgery was performed in 92% of subjects. A progressive rating (type 0-3) Benjamin Defect Severity Scale (BDSS) was developed to rate the absence or presence of a posterior abnormality. BDSS-2 and BDSS-3 subjects were more likely to have low birth weight. Extremely preterm GA was more likely to be associated with BDSS-1 (mild) or BDSS-2. History of multiple and prolonged intubations were seen more frequently in BDSS-2 or BDSS-3. Post-op pVHI reduced an average of 15 points for BDSS-0 to BDSS-2, but only 3 points for BDSS-3. Post-op pVHI matched normal values for preintervention dysphonic children. CONCLUSIONS: The presence of a persistent breathy voice after intervention for unilateral vocal fold immobility is potentially associated with posterior glottic defects. Low birth weight with multiple/prolonged intubation is more likely to be present with higher-grade BDs, whereas low GA is more likely to be associated with BDSS-1 to BDSS-2. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:2550-2557, 2021.

Tracheostomy on infants born in the periviable period: Outcomes at discharge from the neonatal intensive care unit (NICU).

OBJECTIVES: Advances in neonatal intensive care have allowed successful resuscitation of children born at the border of viability. However, there has been little change in the incidence of bronchopulmonary dysplasia (BPD) and anatomical upper airway obstruction which may require a tracheostomy in that group. The benefits of the procedure are accompanied by sequelae that impact outcomes. Information about these issues can assist caregivers in making decisions and planning care after discharge from the neonatal intensive care unit (NICU). The objectives of this study were to describe the clinical characteristics of neonates born in the periviable period (≤25 weeks gestation) requiring tracheotomy and to highlight their hospital course, complications and status upon NICU discharge. METHODS: Retrospective analysis at four tertiary care academic children's hospitals. Medical records of neonates born ≤25 weeks gestation who required tracheotomy between January 1, 2012 and December 31, 2018 were reviewed. Demographics, medical comorbidities, and tracheostomy related complications were studied. Feeding, ventilation, and neurodevelopmental outcomes at time of transfer from NICU were evaluated. RESULTS: Fifty-two patients were included. The mean gestational age was 24.3 (95% confidence interval, 24.1 to 24.5) weeks. The mean birth weight was 635 (95% CI: 603 to 667) grams and 50 (96.2%) children had BPD. At time of discharge from the NICU, 47 (90.4%) required mechanical ventilation, four (7.7%) required supplemental oxygen and one (1.9%) was weaned to room air. Forty-two (80.8%) were discharged with a gastrostomy tube, seven (28%) with a nasogastric tube, and three (5.8%) were on oral feeds. Two (3.8%) suffered hypoxic ischemic encephalopathy, 27 (51.9%) had neurodevelopmental delay, seven (13.5%) were diagnosed with another anomaly, and 16 (30.8%) were considered normal. Complications related to the procedure were observed in 28 (53.8%) neonates. Granulation tissue was seen in 17 (32.7%), wound break down or cellulitis in three (5.8%), one (1.9%) with tracheostomy plugging, three (5.8%) with dislodgement of the tracheostomy tube and four (7.7%) developed tracheitis. CONCLUSIONS: Tracheostomy in infants born in the periviable period is primarily performed for BPD and portends extended ventilatory dependence. It is associated with non-oral alimentation at the time of discharge from the NICU and developmental delay. Mortality directly related to the procedure is rare. Minor complications are common but do not require surgical intervention. These data may aid in counseling caregivers about the procedure in this vulnerable population.

Drug delivery to the pediatric upper airway.

Pediatric upper airway disorders are frequently life-threatening and require precise assessment and intervention. Targeting these pathologies remains a challenge for clinicians due to the high complexity of pediatric upper airway anatomy and numerous potential etiologies; the most common treatments include systemic delivery of high dose steroids and antibiotics or complex and invasive surgeries. Furthermore, the majority of innovative airway management technologies are only designed and tested for adults, limiting their widespread implementation in the pediatric population. Here, we provide a comprehensive review of the most recent challenges of managing common pediatric upper airway disorders, describe the limitations of current clinical treatments, and elaborate on how to circumvent those limitations via local controlled drug delivery. Furthermore, we propose future advancements in the field of drug-eluting technologies to improve pediatric upper airway management outcomes.

Tracheocutaneous Fistula After Pediatric Open Airway Reconstruction.

OBJECTIVES: Tracheocutaneous fistula (TCF) is a common occurrence after pediatric tracheostomy decannulation. However, the persistence of TCF after staged reconstruction of the pediatric airway is not well-described. The primary objective was to determine the rate of persistent TCF after successful decannulation in children with staged open airway reconstruction. METHODS: A case series with chart review of children who underwent decannulation after double-stage laryngotracheal reconstruction between 2017 and 2019. RESULTS: A total of 26 children were included. The most common open airway procedure was anterior and posterior costal cartilage grafting (84.6%, 22/26). Median age at decannulation was 3.4 years (IQR: 2.8-4.3) and occurred 7.0 months (IQR: 4.3-10.4) after airway reconstruction. TCF persisted in 84.6% (22/26) of children while 15.4% (4/26) of stomas closed spontaneously. All closures were identified by the one-month follow-up visit. There was no difference in age at tracheostomy (P = .86), age at decannulation (P = .97), duration of tracheostomy (P = .43), or gestational age (P = .23) between stomas that persisted or closed. Median diameter of stent used at reconstruction was larger in TCFs that persisted (7.0 mm vs 6.5 mm, P = .03). Tracheostomy tube diameter (P = .02) and stent size (P < .01) correlated with persistence of TCF on multivariable logistic regression analysis. There were 16 surgical closure procedures, which occurred at a median of 14.4 months (IQR: 11.4-15.4) after decannulation. Techniques included 56.3% (9/16) by primary closure, 18.8% (3/16) by secondary intention and 25% (4/16) by cartilage tracheoplasty. The overall success of closure was 93.8% (15/16) at latest follow-up. CONCLUSIONS: Persistent TCF occurs in 85% of children who are successfully decannulated after staged open airway reconstruction. Spontaneous closure could be identified by 1 month after decannulation and was more likely when smaller stents and tracheostomy tubes were utilized. Surgeons should counsel families on the frequency of TCF and the potential for additional procedures needed for closure.

International Pediatric Otolaryngology Group (IPOG) management recommendations: Pediatric tracheostomy decannulation.

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.

Competency-Based Assessment Tool for Pediatric Esophagoscopy: International Modified Delphi Consensus.

OBJECTIVES/HYPOTHESIS: Create a competency-based assessment tool for pediatric esophagoscopy with foreign body removal. STUDY DESIGN: Blinded modified Delphi consensus process. SETTING: Tertiary care center. METHODS: A list of 25 potential items was sent via the Research Electronic Data Capture database to 66 expert surgeons who perform pediatric esophagoscopy. In the first round, items were rated as "keep" or "remove" and comments were incorporated. In the second round, experts rated the importance of each item on a seven-point Likert scale. Consensus was determined with a goal of 7 to 25 final items. RESULTS: The response rate was 38/64 (59.4%) in the first round and returned questionnaires were 100% complete. Experts wanted to "keep" all items and 172 comments were incorporated. Twenty-four task-specific and 7 previously-validated global rating items were distributed in the second round, and the response rate was 53/64 (82.8%) with questionnaires returned 97.5% complete. Of the task-specific items, 9 reached consensus, 7 were near consensus, and 8 did not achieve consensus. For global rating items that were previously validated, 6 reached consensus and 1 was near consensus. CONCLUSIONS: It is possible to reach consensus about the important steps involved in rigid esophagoscopy with foreign body removal using a modified Delphi consensus technique. These items can now be considered when evaluating trainees during this procedure. This tool may allow trainees to focus on important steps of the procedure and help training programs standardize how trainees are evaluated. LEVEL OF EVIDENCE: 5. Laryngoscope, 131:1168-1174, 2021.

Reflux as a Risk Factor for Morbidity after Pediatric Tonsillectomy: A National Cohort of Inpatients.

OBJECTIVE: Gastroesophageal reflux (GER) has been identified as a risk factor for complications following pediatric tonsillectomy. The primary objective of this study was to examine outcomes after tonsillectomy among children with GER using a nationwide database. Secondary objectives included analyzing duration of hospitalization and total charges after admission. METHODS: A cross-sectional review of the Healthcare Cost and Utilization Project (HCUP) Kids' Inpatient Databases (KID) from 2012 and 2016. RESULTS: A total of 21,232 children had a tonsillectomy with or without adenoidectomy, with 1683 (7.9%) diagnosed with GER. Average age for patients with reflux was 4.4 years and for those without was 5.6 years (P < .001). The rate of primary hemorrhage was not statistically different between groups (P = .87). Patients with reflux were more likely to have respiratory complications (P = .03), aspiration pneumonitis (P < .001), and hypoxemia (P < .001) during their hospital course. Noninvasive ventilation and reintubation also occurred more frequently in this population (P < .001). Children with reflux had a longer duration of postoperative admission (3.8 vs. 2.3 days, P < .001) and higher total hospital charges ($47,129 vs. $27,584, P < .001). Multivariable regression analysis determined that reflux remained a statistically significant indicator of aspiration pneumonitis, hypoxemia, invasive and non-invasive ventilation, as well as length of admission. CONCLUSION: Children with GER were significantly more likely to experience inpatient complications following tonsillectomy. Further, length of admission was higher compared to children without reflux. These results suggest that children with GER experience poorer outcomes after tonsillectomy and highlight the role for appropriate preoperative counseling and planning in this patient population. Laryngoscope, 131:907-910, 2021.

Adenoidectomy Without Tonsillectomy for Pediatric Obstructive Sleep Apnea.

OBJECTIVE: The primary objective was to determine if obstructive sleep apnea (OSA) can improve after adenoidectomy. STUDY DESIGN: Case series with chart review. SETTING: Tertiary children's hospital between 2016 and 2018. METHODS: The study included children under 3.5 years with small (1+ or 2+) palatine tonsils, large (3+ or 4+) adenoids, and documented OSA on polysomnogram (PSG). RESULTS: Seventy-one children were included. Age at adenoidectomy was 2.0 years (95% CI, 1.8-2.2) and 71.8% were male. Mean follow-up was 2.5 years (95% CI, 2.3-2.7). Twenty-six children (36.6%) obtained a repeat PSG at a mean of 9.7 months (95% CI, 6.3-13.2) after adenoidectomy. Among those with a postoperative PSG, apnea-hypopnea index decreased in 77.0% (mean, -3.2 events/h; 95% CI, -14.1 to 7.6), and the proportion with moderate to severe OSA decreased from 65.4% to 30.8% (P = .03). Six children (23.1%) had a normal PSG after adenoidectomy. Tonsillectomy was performed in 14.1% of children at 12.1 months (95% CI, 7.5-16.7) after adenoidectomy. Despite similar preoperative PSG variables, younger children (1.5 vs 2.1 years, P = .02) were more likely to require tonsillectomy. Substantial adenoid regrowth was identified in 1 child at the time of tonsillectomy. CONCLUSION: Adenoidectomy may improve OSA in young children with large adenoids and small tonsils. However, younger age predicted the need for subsequent tonsillectomy. Prospective studies with additional PSG data are necessary to corroborate these findings.

Recorded Flexible Nasolaryngoscopy for Neonatal Vocal Cord Assessment in a Prospective Cohort.

OBJECTIVE: Assessing vocal cord mobility by flexible nasolaryngoscopy (FNL) can be difficult in neonates. To date, prospective studies evaluating the incidence and diagnostic accuracy of vocal cord paralysis (VCP) after surgical patent ductus arteriosus (PDA) ligation are limited. It is unknown whether video FNL improves diagnosis in this population. This study compared video recordings with bedside evaluation for diagnosis of VCP and determined inter-rater reliability of the diagnosis of VCP in preterm infants after PDA ligation. METHODS: Prospective cohort of preterm neonates undergoing bedside FNL within two weeks of extubation following PDA ligation. In a subset, FNL was recorded. Two pediatric otolaryngologists, blinded to the initial diagnosis, reviewed the FNL video recordings. RESULTS: Eighty infants were enrolled and 37 with a recorded FNL were included in the cohort. Average gestational age at birth was 25.2 weeks (SD: 1.2) and postmenstrual age at FNL was 37.0 weeks (SD: 4.5), which was 9.5 days (SD: 14.7) after extubation following PDA repair. There were 6 diagnosed with left VCP (16.2%; 95% CI: 4.3-28.1%) at bedside, and 9 diagnosed by video review (24.3%; 95% CI: 10.5-38.1%) (P = .56). Videos confirmed all 6 VCP diagnosed initially, but also identified 3 additional cases. Though imperfect, reviewing FNL by video showed substantial reliability (kappa = .75), with 91.9% agreement. CONCLUSION: Video recorded FNL most often confirms a bedside diagnosis of VCP, but may also identify discrepancies. Physicians should consider the limitations of diagnosis especially when infants persist with symptoms such as weak voice or signs of postoperative aspiration. LEVEL OF EVIDENCE: 2b.

International Pediatric Otolaryngology Group (IPOG) consensus recommendations: Management of suprastomal collapse in the pediatric population.

INTRODUCTION: Suprastomal Collapse (SuStCo) is a common complication of prolonged tracheostomy in children. There is a paucity of literature on this subject, especially regarding how to manage significant suprastomal collapse that prevents safe decannulation. OBJECTIVE: Provide a definition, classification system, and recommend management options for significant suprastomal collapse in children with tracheostomy. METHODS: Members of the International Pediatric Otolaryngology Group (IPOG) who are experts in pediatric airway conditions were surveyed and results were refined using a modified Delphi method. RESULTS: Consensus was defined as > 70% agreement on a subject. The experts achieved consensus: CONCLUSION: This consensus statement provides recommendations for medical specialists who manage infants and children with tracheostomies with significant Suprastomal Collapse. It provides a classification system to facilitate diagnosis and treatment options for this condition.

International Pediatric Otolaryngology group (IPOG) consensus on the diagnosis and management of pediatric obstructive sleep apnea (OSA).

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.

Algorithm-Based Pediatric Otolaryngology Management During the COVID-19 Global Pandemic: A Children's Hospital of Philadelphia Clinical Consensus.

OBJECTIVE: The coronavirus disease 2019 (COVID-19) pandemic requires clinicians to explore alternatives to routine patient management. Otolaryngologists caring for children commonly depend on physical examination, laboratory data, and ambulatory surgical procedures. Limiting patient care, mindful allocation of resources, and concern for safety have challenged all aspects of our health care system. This evidence-based clinical consensus is designed to guide practitioners of pediatric otolaryngology for common scenarios during this time. DATA SOURCES: Peer-reviewed literature, published reports, institutional guidelines, and expert consensus. REVIEW METHODS: A clinical consensus on 6 common scenarios in pediatric otolaryngology developed with evidence-based strategies. CONCLUSIONS: Providers should suspend all in-person nonessential office visits and elective surgical procedures. An emphasis on medical management and caregiver education will provide reasonable approaches to many of the common outpatient concerns. Surgery for chronic otitis media, obstructive sleep apnea, and acute rhinosinusitis should occur only in response to severe complications or failure of medical regimens. The approach to the pediatric neck mass focuses on timely management for oncologic etiologies and cautious surgical intervention for abscess drainage or tissue sampling. Finally, epistaxis and otorrhea must be triaged and addressed without the usual ambulatory procedures. IMPLICATIONS FOR PRACTICE: Adaptation of practice patterns during this unprecedented moment for our health care system requires thoughtful planning. The strategies described allow for safe handling of common pediatric otolaryngology diagnoses. Ultimately, otolaryngologists must be stewards of our global health community while advocating for the care of individual pediatric patients.

Pediatric laryngopharyngeal reflux: Perceptual, acoustic, and laryngeal findings.

OBJECTIVES: Describe acoustic and laryngeal findings in pediatric patients with subjective dysphonia attributed to laryngopharyngeal reflux (LPR). Determine the impact of LPR on perceived voice quality using the pediatric Voice Handicap Index (pVHI). Compare these findings with age-matched normative values as well as data on pediatric patients with dysphonia due to other etiologies. METHODS: Retrospective case series of pediatric patients (age 2-17 years) evaluated at a specialty pediatric voice clinic at a tertiary care children's hospital from January 1 2007 to December 31 2017 in whom LPR in whom LPR was deemed to be the most significant contributing factor for dysphonia based on physical examination and history. Patients with structural laryngeal abnormalities unrelated to LPR, such as raised lesions, stenosis, papillomatosis, or vocal fold immobility were excluded. RESULTS: 163 out of 1195 evaluable patients met inclusion criteria. Of these, 87% had pVHI and 83% had acoustic data available from their first appointment for analysis. Mean total pVHI score was 24 (range: 0-81). Perturbation measures were elevated in both females (jitter 1.38%, shimmer 4.16%) and males (jitter 2.01%, shimmer 5.62%). Laryngologic assessment revealed: vocal fold changes including erythema and/or pre-nodules in 72% of patients. Cobblestoning of any portion of the pharynx was present in 67% with hypopharyngeal cobblestoning the most common, present in 64% of patients. CONCLUSION: Pediatric patients with clinically diagnosed LPR have pVHI, jitter, and shimmer scores that are comparable to previously reported patients with raised lesions, scar and immobility, and values that are significantly higher than published normative data. Dysphonic children should be assessed for LPR and treated when indicated. LEVEL OF EVIDENCE: 4.

Tracheostomy in the Extremely Premature Neonate: A Multi-Institutional Study.

OBJECTIVE: (1) To describe characteristics associated with tracheostomy placement and (2) to describe associated in-hospital morbidity in extremely premature infants. STUDY DESIGN: Pooled retrospective analysis of charts. SETTING: Academic children's hospitals. SUBJECTS AND METHODS: The patient records of premature infants (23-28 weeks gestational age) who underwent tracheostomy between January 1, 2012, and December 31, 2017, were reviewed from 4 academic children's hospitals. Demographics, procedural morbidity, feeding, respiratory, and neurodevelopmental outcomes at the time of transfer from the neonatal intensive care unit (NICU) were obtained. The contribution of baseline characteristics to mortality, neurodevelopmental, and feeding outcomes was also assessed. RESULTS: The charts of 119 infants were included. The mean gestational age was 25.5 (95% confidence interval, 25.2-25.7) weeks. The mean birth weight was 712 (671-752) g. Approximately 50% was African American. The principal comorbidity was chronic lung disease (92.4%). Overall, 60.5% of the infants had at least 1 complication. At the time of transfer, most remained mechanically ventilated (94%) and dependent on a feeding tube (90%). Necrotizing enterocolitis increased the risk of feeding impairment (P = .002) and death (P = .03). CONCLUSIONS: Tracheostomy in the extremely premature neonate is primarily performed for chronic lung disease. Complications occur frequently, with skin breakdown being the most common. Placement of a tracheostomy does not seem to mitigate the systemic morbidity associated with extreme prematurity.

Competency-Based Assessment Tool for Pediatric Tracheotomy: International Modified Delphi Consensus.

OBJECTIVES/HYPOTHESIS: Create a competency-based assessment tool for pediatric tracheotomy. STUDY DESIGN: Blinded, modified, Delphi consensus process. METHODS: Using the REDCap database, a list of 31 potential items was circulated to 65 expert surgeons who perform pediatric tracheotomy. In the first round, items were rated as "keep" or "remove," and comments were incorporated. In the second round, experts were asked to rate the importance of each item on a seven-point Likert scale. Consensus criteria were determined a priori with a goal of 7 to 25 final items. RESULTS: The first round achieved a response rate of 39/65 (60.0%), and returned questionnaires were 99.5% complete. All items were rated as "keep," and 137 comments were incorporated. In the second round, 30 task-specific and seven previously validated global rating items were distributed, and the response rate was 44/65 (67.7%), with returned questionnaires being 99.3% complete. Of the Task-Specific Items, 13 reached consensus, 10 were near consensus, and 7 did not achieve consensus. For the 7 previously validated global rating items, 5 reached consensus and two were near consensus. CONCLUSIONS: It is feasible to reach consensus on the important steps involved in pediatric tracheotomy using a modified Delphi consensus process. These items can now be considered to create a competency-based assessment tool for pediatric tracheotomy. Such a tool will hopefully allow trainees to focus on the important aspects of this procedure and help teaching programs standardize how they evaluate trainees during this procedure. LEVEL OF EVIDENCE: 5 Laryngoscope, 130:2700-2707, 2020.