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Background: The aim of our study was to investigate the prevalence of coexisting conditions and exposures in children with nodular tracheobronchitis diagnosed by flexible bronchoscopy., Methods: We conducted a single-center retrospective review of 100 children diagnosed with nodular tracheobronchitis by flexible bronchoscopy between 2012 and 2023., Results: Common coexisting diagnoses included gastroesophageal reflux disease (GERD, 50%), dysphagia/aspiration (40%), asthma (30%), recurrent croup (30%), tracheostomy dependence (19%) and eosinophilic esophagitis (EOE) (12%). Bronchoalveolar lavage (BAL) demonstrated cellular inflammation with elevated proportions of neutrophils in 63%, and lymphocytes in 24%. Among 88 patients in whom bacterial cultures were performed, 52% were positive, with Moraxella, Haemophilus, Streptococcal and Pseudomonas species predominating. Among 30 patients who underwent viral testing, 57% were positive, with rhinovirus (82%) and adenovirus (29%) predominating. Patients with neutrophilic inflammation were more likely to have a positive respiratory bacterial culture and/or viral polymerase chain reaction (p = 0.003, 0.005). Evaluation of the gastrointestinal tract included 79 patients with a history of esophagogastroduodenoscopy, 45 patients with a videofluoroscopic swallow study (VFSS), and 45 patients with multi-channel intraluminal impedance and pH testing. The majority of VFSS were abnormal (60%) demonstrating either laryngeal penetration (33%) or intratracheal aspiration (27%). Median pH reflux and impedance proximal reflux indices were 3.8% and 0.5% respectively., Conclusion: Potential contributing factors in the pathophysiology of nodular tracheobronchitis include bacterial and viral infections, GERD, dysphagia/aspiration, and EOE. When nodular tracheobronchitis is observed during bronchoscopy, further evaluation to assess for these conditions should be considered., (© 2024 Wiley Periodicals LLC.)

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 T H - 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 - CD25 hi 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 CD25 hi Tfh cells all localized outside of germinal centers. In CVID+AIC lymph nodes, cellular positions were similar but CD25 hi 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., (Copyright © 2024 American Academy of Allergy, Asthma & Immunology. All rights reserved.)

Objective: To provide detailed guidance on the administration of systemic bevacizumab in patients with recurrent respiratory papillomatosis (RRP) based on a detailed review of the scientific literature and a consensus of experts with real-world clinical experience., Methods: A bevacizumab consensus working group (N = 10) was composed of adult and pediatric otolaryngologists, adult and pediatric oncologists, and a representative from the RRP Foundation (RRPF), all with experience administering systemic bevacizumab in patients with RRP. After extensive review of the medical literature, a modified Delphi method-based survey series was utilized to establish consensus on the following key areas: clinical and patient characteristics ideal for treatment candidacy, patient perspective in treatment decisions, treatment access, initial dosing, monitoring, guidelines for tapering and discontinuation, and reintensifying therapy., Results: Seventy-nine statements were identified across nine critical domains, and 45 reached consensus [clinical benefits of bevacizumab (3), patient and disease characteristics for treatment consideration (7), contraindications for treatment (3), shared decision-making (incorporating the patient perspective) (5), treatment access (3), initial dosing and administration (8), monitoring (7), tapering and discontinuation (6), and reintensification (3)]., Conclusion: This consensus statement provides the necessary guidance for clinicians to initiate systemic administration of bevacizumab and represents a potential paradigm shift toward nonsurgical treatment options for patients with RRP., Level of Evidence: 5 Laryngoscope, 134:5041-5046, 2024., (© 2024 The American Laryngological, Rhinological and Otological Society, Inc.)

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., Competing Interests: Declaration of competing interest None declared, (Copyright © 2023 Elsevier B.V. All rights reserved.)

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 T reg -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.

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., Competing Interests: Declaration of competing interest The authors have no conflicts of interest or financial relationships relevant to this article to disclose., (Copyright © 2023 Elsevier B.V. All rights reserved.)

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., (© 2022 The American Laryngological, Rhinological and Otological Society, Inc.)

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., (© 2021 The American Laryngological, Rhinological and Otological Society, Inc.)

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.

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., (Copyright © 2021 Elsevier B.V. All rights reserved.)

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., (Copyright © 2021 Elsevier B.V. All rights reserved.)

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.

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., (© 2020 American Laryngological, Rhinological and Otological Society Inc, "The Triological Society" and American Laryngological Association (ALA).)

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., (© 2020 The American Laryngological, Rhinological and Otological Society, Inc.)

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.

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., (Copyright © 2020 Elsevier B.V. All rights reserved.)

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., (Copyright © 2020 Elsevier B.V. All rights reserved.)

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., (Copyright © 2020 Elsevier B.V. All rights reserved.)

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., (© 2019 The American Laryngological, Rhinological and Otological Society, Inc.)

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.

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., Competing Interests: Declaration of competing interest The authors have no conflicts of interest., (Copyright © 2020 Elsevier B.V. All rights reserved.)

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.

Objectives: To determine the rate of significant respiratory events following adenoidectomy in young patients and to identify factors that would prompt inpatient admission postoperatively., Methods: A retrospective chart review was performed of consecutive adenoidectomy surgeries at a high-volume, tertiary-care children's hospital between 2016 and 2018. Children under 3.5 years of age who had surgery for obstructive symptoms were included. Patients were grouped by age (youngest ≤1.5 years, middle 1.6-2.5 years, and oldest 2.6-3.5 years). We excluded patients having revision surgery, a concomitant tonsillectomy, or additional major surgical procedure., Results: There were 353 patients that met inclusion criteria. The three age groups were similar with respect to all characteristics except age (p < .001), body mass index (p < .001), and percentage of Black or African American children (p = .02). Patients under 1.5 years more often had preoperative polysomnography (p = .02) with a lower oxygen saturation nadir (p = .04), and were more likely to have surgery for obstructive sleep apnea (p < .001). No differences were found between age groups with respect to recovery room issues, nurse triage calls, or readmissions within 30 days of surgery. An elective admission rate in the cohort was 35.1%, and this was age-group dependent with 79.5% of the youngest group being admitted (p < .001). On admission, 16.9% of all patients had admission events requiring positive pressure support, intensive care unit admission, or prolonged hospitalization, which was similar across all age groups (p = .67). Events were more common in younger patients (17 mos. vs 20 mos., p = .07), those with more comorbidities (74.8% vs 51.5%, p = .06) and significantly higher in those with severe preoperative polysomnogram variables (p < .001). Based on multivariate regression analysis, younger children (OR: 13.7, 95% CI: 6.5 - 29.0, p < .001) or children with an AHI over 5 events/hr (OR: 32.3, 95% CI: 3.4 - 303.2, p = .005) were more likely to have significant events on admission., Conclusions: Significant respiratory events are uncommon after adenoidectomy for obstructive symptoms, even in very young children. However, for children under 1.5 years of age or those with AHI scores above 5 events/hr, postoperative admission for monitoring is recommended. Clinical judgement should be used when considering outpatient surgery for older children or those with comorbidities., Competing Interests: Declaration of competing interest The authors have no conflicts of interest to report., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Objectives: To develop consensus recommendations for the evaluation and management of juvenile-onset recurrent respiratory papillomatosis (JORRP) in pediatric patients., Methods: Expert opinion by the members of the International Pediatric Otolaryngology Group (IPOG). The mission of the IPOG is to develop expertise-based consensus recommendations for the management of pediatric otolaryngologic disorders with the goal of improving patient care. The consensus recommendations herein represent the first publication by the group., Results: Consensus recommendations including diagnostic considerations, surgical management, systemic adjuvant therapies, postoperative management, surveillance, and voice evaluation. These recommendations are based on the collective opinion of the IPOG members and are targeted for otolaryngologists, primary care providers, pulmonologists, infectious disease specialists, and any other health care providers that manage patients with JORRP., Conclusions: Pediatric JORRP consensus recommendations are aimed at improving care and outcomes in this patient population., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Objectives/hypothesis: Pediatricians are the first physicians to see a dysphonic child (DC), yet there are limited data on their proficiency in caring for them. The objective of this study was to understand how pediatricians' experience and their comfort in recognizing/diagnosing voice disorders affects their referral patterns and use of basic treatment options., Study Design: Survey study., Methods: A 13-question survey was sent to pediatricians in the Children's Hospital of Philadelphia's primary care network; 45/216 were returned. Statistical analyses were performed using the Student t test, linear/logistic regression model, Fisher exact test, Kruskal-Wallis test, and Spearman's correlation test., Results: Pediatricians practicing longer are more comfortable recognizing dysphonia (P = .0022). They are significantly more likely to refer a DC, even without subjective complaints of hoarseness by the family/patient or compounding medical issues. For each year in practice, the probability of referring increases by 1.55% (P = .0017). Pediatricians with a higher percentage of dysphonic children in their practice are more likely to trust their own perceptual recognition when deciding to refer (P = .0496). Nearly all pediatricians (40/45) would refer to a pediatric otolaryngologist. None would refer to a laryngologist or a voice therapist. No factors significantly affected treatment options., Conclusions: Veteran pediatricians feel more comfortable diagnosing a voice disorder and are more likely to refer a DC, regardless of patient/parent complaints or compounding factors. Pediatricians are most likely to refer to a pediatric otolaryngologist versus a voice specialist. These findings suggest that education of younger, less-experienced pediatricians about recognizing voice disorders and options for referral is needed. This may improve the overall care of the DC., Level of Evidence: 4 Laryngoscope, 129:1756-1762, 2019., (© 2018 The American Laryngological, Rhinological and Otological Society, Inc.)

22q11.2 deletion syndrome (22q11.2DS) is a disorder caused by recurrent, chromosome-specific, low copy repeat (LCR)-mediated copy-number losses of chromosome 22q11. The Children's Hospital of Philadelphia has been involved in the clinical care of individuals with what is now known as 22q11.2DS since our initial report of the association with DiGeorge syndrome in 1982. We reviewed the medical records on our continuously growing longitudinal cohort of 1,421 patients with molecularly confirmed 22q11.2DS from 1992 to 2018. Most individuals are Caucasian and older than 8 years. The mean age at diagnosis was 3.9 years. The majority of patients (85%) had typical LCR22A-LCR22D deletions, and only 7% of these typical deletions were inherited from a parent harboring the deletion constitutionally. However, 6% of individuals harbored other nested deletions that would not be identified by traditional 22q11.2 FISH, thus requiring an orthogonal technology to diagnose. Major medical problems included immune dysfunction or allergies (77%), palatal abnormalities (67%), congenital heart disease (64%), gastrointestinal difficulties (65%), endocrine dysfunction (>50%), scoliosis (50%), renal anomalies (16%), and airway abnormalities. Median full-scale intelligence quotient was 76, with no significant difference between individuals with and without congenital heart disease or hypocalcemia. Characteristic dysmorphic facial features were present in most individuals, but dermatoglyphic patterns of our cohort are similar to normal controls. This is the largest longitudinal study of patients with 22q11.2DS, helping to further describe the condition and aid in diagnosis and management. Further surveillance will likely elucidate additional clinically relevant findings as they age., (© 2018 Wiley Periodicals, Inc.)

Vocal fold motion abnormalities in children are the second most common form of laryngeal pathology seen in children, and often present in the first 24 months of life. A thorough evaluation of the aerodigestive tract will include an examination of the vocal folds, and a proper diagnosis is essential in order to decipher the etiology of swallowing, voicing and breathing abnormalities. This article reviews the workup, management options and clinical outcomes of unilateral and bilateral vocal fold motion impairment in the pediatric population., (Copyright © 2018 Mosby, Inc. All rights reserved.)

Aerodigestive programs provide coordinated interdisciplinary care to pediatric patients with complex congenital or acquired conditions affecting breathing, swallowing, and growth. Although there has been a proliferation of programs, as well as national meetings, interest groups and early research activity, there is, as of yet, no consensus definition of an aerodigestive patient, standardized structure, and functions of an aerodigestive program or a blueprint for research prioritization. The Delphi method was used by a multidisciplinary and multi-institutional panel of aerodigestive providers to obtain consensus on 4 broad content areas related to aerodigestive care: (1) definition of an aerodigestive patient, (2) essential construct and functions of an aerodigestive program, (3) identification of aerodigestive research priorities, and (4) evaluation and recognition of aerodigestive programs and future directions. After 3 iterations of survey, consensus was obtained by either a supermajority of 75% or stability in median ranking on 33 of 36 items. This included a standard definition of an aerodigestive patient, level of participation of specific pediatric disciplines in a program, essential components of the care cycle and functions of the program, feeding and swallowing assessment and therapy, procedural scope and volume, research priorities and outcome measures, certification, coding, and funding. We propose the first consensus definition of the aerodigestive care model with specific recommendations regarding associated personnel, infrastructure, research, and outcome measures. We hope that this may provide an initial framework to further standardize care, develop clinical guidelines, and improve outcomes for aerodigestive patients., Competing Interests: POTENTIAL CONFLICT OF INTEREST: Drs Deboer, Prager, Friedlander, and Deterding are co-founders (Secretary, Treasurer, President, and Vice-President, respectively) and board members of Triple Endoscopy, Inc. They are also co-inventors with University of Colorado on Patent Cooperation Treaty application “Pediatric Nasal Endoscope PCT/US16/39352.” They have not received funding from these entities for this project or at the time of the writing of this manuscript; Dr Rutter serves as consultant to Bryan Medical, which produces and markets an airway balloon of his patent. This is unrelated to the current study and he receives no revenue from this stent; Dr Gold has received grants and funding from Johnson and Johnson, Takeda Pharmaceuticals, Nestle Nutrition, Nutricia Nutrition, and Mead Johnson (unrelated to the current study); Dr Putnam has received payment for lectures including service on speaker bureaus for Abbott Nutrition (unrelated to the current study); the other authors have indicated they have no potential conflicts of interest to disclose., (Copyright © 2018 by the American Academy of Pediatrics.)

Pediatric aspiration is a multifactorial process that is often complex to manage. Recurrent laryngeal nerve (RLN) injury can cause glottic insufficiency and aspiration. We describe three cases of unilateral vocal fold paralysis resulting in aspiration and the successful use of the RLN reinnervation for its treatment. The theory for utilizing the reinnervation procedure is that when glottic closure improves and a less breathy vocalization occurs, then the larynx is better equipped to protect the lower airway and avoid aspiration. Our cases demonstrate stronger voice and improved swallow function, with normalization of modified barium swallow evaluation, at approximately 6-months post reinnervation., (Copyright © 2017. Published by Elsevier B.V.)

Introduction: The diagnosis and management of type I laryngeal clefts can be controversial and varies across centers and surgeons. Using existing peer-reviewed literature to develop an expert-based consensus will help guide physicians in the treatment of these patients as well as develop research hypotheses to further study this condition., Objective: To provide recommendations for the diagnosis and management of type I laryngeal clefts., Methods: Determination of current expert- and literature-based recommendations, via a survey of the International Pediatric Otolaryngology Group, using a modified Delphi method., Setting: Multinational, multi-institutional, tertiary pediatric hospitals., Results: Consensus recommendations include diagnostic workup, medical management, pre-operative, intra-operative and post-operative considerations for type I laryngeal clefts., Conclusions: This guide on the diagnosis and management of patients with type I laryngeal clefts is aimed at improving patient care and promoting future hypothesis generation and research to validate the recommendations made here., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Objectives/hypothesis: Aggressive laryngeal, tracheal, and pulmonary papilloma is an extremely challenging clinical problem without proven treatment options. A recent German report documented promising results with systemic bevacizumab. The objective of this study is to report the initial experience of this novel treatment in the United States for recurrent respiratory papillomatosis (RRP)., Study Design: Cases series., Methods: Electronic survey of the RRP Task Force of the American Society of Pediatric Otolaryngology, American Broncho-Esophagological Association, and physicians known to the authors to have used systemic bevacizumab for RRP., Results: Eleven completed surveys were obtained. In three cases, systemic bevacizumab was considered clinically but not administered. Eight patients were treated with systemic bevacizumab, all for aggressive papillomatosis uncontrolled by surgical and adjuvant therapy, including seven of eight with pulmonary disease. Treatment dosing ranged from 5 to 10 mg/kg every 2 to 4 weeks, with all patients responding (7/8 partial response, 1/8 complete response). In four patients who had postbevacizumab chest imaging, three demonstrated improvement of disease and one stabilization. Treatment interval could be lengthened in seven patients and clinical response maintained. One patient with long-standing pulmonary disease (>10 years) was diagnosed with malignant transformation while on treatment, and bevacizumab was discontinued in lieu of other chemotherapeutic agents. All other patients continue on systemic bevacizumab with minimal complications (hemoptysis n = 1, proteinuria n = 1)., Conclusions: Systemic bevacizumab appears to have significant promise in the most treatment-resistant and aggressive forms of papillomatosis with a low complication profile. These results suggest bevacizumab should be studied in a formal clinical trial for RRP., Level of Evidence: 4. Laryngoscope, 127:2225-2229, 2017., (© 2017 The American Laryngological, Rhinological and Otological Society, Inc.)

Recurrent laryngeal papillomatosis (RRP) can be a devastating condition for a child to endure, and pulmonary involvement may have terminal consequences. Adjuvant therapies have been trialed and reported over the years; however, these chemotherapy options have not been successful. Bevacizumab (Avastin, Genetech Inc., South San Francisco, CA) is a vascular endothelial factor (VEGF) inhibitor that has shown promise in the management of papillomatosis. Most research has focused on intralesional injections of this antiangiogenic drug. The systemic use of bevacizumab is not as well described. This is a case report of a 12-year-old female diagnosed with severe laryngotracheal papillomatosis near birth who underwent a tracheostomy tube placement at 1 year of age. She required weekly debridements to prevent tracheal obstruction. When lung involvement was diagnosed at 1 year of age, cidofovir was started intravenously. Over the course of the past 10 years, the patient was managed with celecoxib (Celebrex, Pfizer, New York, NY), anti-reflux medications, zithromycin, propranolol, Gardasil (Merck and Co., Kenilworth, NJ), and a 7-year course of interferon-alpha. Intravenous bevacizumab was started when the patient's pulmonary status deteriorated. There was remarkable improvement in her laryngotracheal disease within 6 weeks of therapy. Following 3 months of bevacizumab, the patient's disease was completely resolved at the laryngeal level and nearly gone in the trachea, and she was decannulated. A computed scan was performed following 5 months of intravenous bevacizumab, and the pulmonary RRP nodules completely resolved. The patient had no major or minor complication from the chemotherapy to date. Systemic Bevacizumab is a promising modality of adjuvant therapy for significant papillomatosis. Laryngoscope, 127:1538-1542, 2017., (© 2016 The American Laryngological, Rhinological and Otological Society, Inc.)

Introduction: 22q11.2 deletion syndrome (22q11.2DS) presents with complex but variable symptoms, including cardiac, immune, palatal, endocrine, cognitive, and psychiatric issues. However, an association of 22q11.2DS with structural airway abnormalities has not been formally described. The aim of this study was to document the frequency of this association., Methods: We retrospectively reviewed medical records of patients with 22q11.2DS evaluated in the 22q and You Center at the Children's Hospital of Philadelphia between 1999 and 2015 referred to otolaryngology for an airway assessment. Type of airway abnormality and presence of comorbidities, such as congenital heart disease, tracheostomy, and association with prenatal symptomatology such as polyhydramnios, were noted., Results: Of the 104 patients who underwent an otolaryngology procedure (microlaryngoscopy or bronchoscopy), 71% (n = 74) had airway abnormalities. Patients with airway abnormalities ranged in age from 5 months to 37 years, with similar prevalence among males and females. Observed airway abnormalities included tracheomalacia (36%), subglottic stenosis (28%), laryngomalacia (26%), glottic web (21%), and bronchomalacia (16%). Most patients with airway abnormalities (91%) had an associated congenital heart defect, with ventricular septal defect and Tetralogy of Fallot being the most prevalent. Importantly, 30% of patients required a tracheostomy, and overall polyhydramnios was noted in 16% of pregnancies., Conclusion: Airway abnormalities are a common feature of 22q11.2DS, leading to substantial morbidity, particularly when combined with complex cardiac disease. Polyhydramnios may be an important prenatal clue to both the diagnosis of 22q11.2DS and airway anomalies. Postnatal assessment of airway structure and function among patients with 22q11.2DS is an important component of overall evaluation and will help guide long-term management., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Objectives/hypothesis: To establish the benefit of ansa cervicalis-recurrent laryngeal nerve reinnervation (ANSA-RLN) for the management of dysphonia secondary to unilateral vocal cord paralysis (UVCP) in children. Children treated with ANSA-RLN for the management of dysphonia secondary to unilateral vocal fold immobility will have superior acoustic, perceptual, and stroboscopic outcomes compared to injection laryngoplasty and observation., Study Design: Retrospective case-series chart review., Methods: Laryngeal, perceptual, and acoustic analysis of dysphonia was performed in 33 children (age 2-16 years) diagnosed with UVCP. Comparison of pre-post function for treatment groups (no treatment, injection laryngoplasty, ANSA-RLN) with additional comparison between gestational ages, age at initial evaluation, and gender were examined. Perceptual measures included Pediatric Voice Handicap Index (pVHI) and Grade, Roughness, Breathiness, Asthenia, Strain (GBRAS) perceptual rating. Objective measures included semitone (ST) range, jitter%, shimmer%, noise-to-harmonic ratio, voicing, and maximum phonation time., Results: Post-treatment, pVHI, jitter%, and ST were significantly improved for ANSA-RLN subjects compared to injection subjects. Improved function (laryngeal diadochokinesis, pVHI, GRBAS, and/or acoustic) was observed in all ANSA-RLN subjects who had vocal fold paralysis as the only laryngeal diagnosis., Conclusions: This study presents one of the largest studies of pediatric vocal fold paralysis diagnosis and treatment. The study looks at the spectrum of function in patients with UVCP and looks at the outcomes of options: no treatment, injection laryngoplasty, and ANSA-RLN. Although surgical outcomes vary, both injection laryngoplasty and ANSA-RLN show benefit in laryngeal function, voice stability, voice capacity, perceptual rating, and pVHI scores. Both injection laryngoplasty and ANSA-RLN showed improvements post-treatment, and should be considered for management of pediatric UVCP. However, the ANSA-RLN group showed better and longer-lasting perceptual and acoustic parameters in comparison with the injection and control groups. Reinnervation, even long term after the onset of vocal fold paralysis, should be considered a viable permanent treatment for pediatric UVCP., (© 2015 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: This study sought to determine the impact of voice disability on children with elevated vocal fold lesions (nodules, cysts, polyps). The Pediatric Voice Handicap Index (pVHI) was used to assess the impact on functional, physical, and emotional aspects of voice and oral communication. The degree of talkativeness and overall severity of dysphonia were also determined., Study Design: Case series with chart review of children evaluated at a voice clinic from 2007 to 2011., Setting: Tertiary specialized children's hospital., Subjects and Methods: Medical records of 33 children (11 girls, 22 boys) diagnosed with an elevated vocal fold lesion by a pediatric laryngologist were reviewed for voice disability using the pVHI. The pVHI is a parental proxy of perceived voice handicap in functional, physical, and emotional aspects and includes a talkativeness rating scale and visual analog overall severity rating of voice (VAS). Data were examined for young children (age 2-5 years), children (age 6-10 years), and adolescents (age 11-17 years)., Results: The VAS was significantly correlated with pVHI-total. There was no significant difference between age groups for overall pVHI (mean, 29.3) or talkativeness, but all groups had significantly higher functional disability (mean, 16) compared with physical (mean, 8.2) or emotional (mean, 5.1) disability., Conclusions: Children across all age groups with elevated vocal fold lesions have significant voice disability, greatest for functional aspects of voice. Voice care team professionals must recognize the functional impact of dysphonia on the pediatric patient.

OBJECTIVES To confirm interrater reliability using blinded evaluation of a skills-assessment instrument to assess the surgical performance of resident and fellow trainees performing pediatric direct laryngoscopy and rigid bronchoscopy in simulated models. DESIGN Prospective, paired, blinded observational validation study. SUBJECTS Paired observers from multiple institutions simultaneously evaluated residents and fellows who were performing surgery in an animal laboratory or using high-fidelity manikins. The evaluators had no previous affiliation with the residents and fellows and did not know their year of training. INTERVENTIONS One- and 2-page versions of an objective structured assessment of technical skills (OSATS) assessment instrument composed of global and a task-specific surgical items were used to evaluate surgical performance. RESULTS Fifty-two evaluations were completed by 17 attending evaluators. The instrument agreement for the 2-page assessment was 71.4% when measured as a binary variable (ie, competent vs not competent) (κ = 0.38; P = .08). Evaluation as a continuous variable revealed a 42.9% percentage agreement (κ = 0.18; P = .14). The intraclass correlation was 0.53, considered substantial/good interrater reliability (69% reliable). For the 1-page instrument, agreement was 77.4% when measured as a binary variable (κ = 0.53, P = .0015). Agreement when evaluated as a continuous measure was 71.0% (κ = 0.54, P < .001). The intraclass correlation was 0.73, considered high interrater reliability (85% reliable). CONCLUSIONS The OSATS assessment instrument is an effective tool for evaluating surgical performance among trainees with acceptable interrater reliability in a simulator setting. Reliability was good for both the 1- and 2-page OSATS checklists, and both serve as excellent tools to provide immediate formative feedback on operational competency.

Objectives/hypothesis: In patients undergoing laryngotracheal reconstruction (LTR), malacic segments of trachea can pose challenges to successful reconstruction. Malacic segments may inadequately support cartilage grafts used in augmentation surgery, sometimes requiring cricotracheal or tracheal resections. We describe a novel technique of LTR with resorbable microplate buttressing of malacic lateral tracheal segments., Study Design: Retrospective case series., Methods: Review of technique, treatment outcomes, and complications of seven children with subglottic stenosis and tracheomalacia requiring a microplate-augmented LTR technique., Results: Seven infants ranging from 26 months to 9 years of age successfully underwent LTR for subglottic stenosis. Six children had a grade III subglottic stenosis. The seventh child had grade II subglottic stenosis, bilateral vocal fold paralysis, an elliptical cricoid, and an obstructing giant suprastomal fibroma. Five children underwent a double-stage LTR with resorbable microplates sutured bilaterally to support severely malacic lateral tracheal segments. A cricotracheal resection would not have been feasible in one child due to the resection length and inadequate tracheal mobilization. Two children underwent a single-stage LTR with unilateral application of a microplate. Six children were decannulated within 3 months and continue without airway symptoms or complications. One child, who is just over 2 months from reconstructive surgery, is being setup for decannulation. No complications were encountered., Conclusions: LTR with resorbable microplate buttressing of malacic lateral tracheal segments is technically feasible, safe, and can avoid more extensive surgery requiring tracheal resection. Further experience may support the use of this technique in challenging airway reconstructions., (Copyright © 2012 The American Laryngological, Rhinological, and Otological Society, Inc.)

Objective: To determine whether the multidisciplinary approach to the management of vocal cord dysfunction (VCD), which combines patient education and behavioral intervention in the same session that VCD is diagnosed, provides long-term therapeutic benefits., Methods: Chart review and telephone interviews of patients treated for VCD at The Children's Hospital of Philadelphia were performed in this retrospective nonrandomized study. All forty patients diagnosed with VCD from October 2007 to April 2009 were included. Patients were evaluated with a multidisciplinary team approach, including speech therapy assessment, otolaryngology exam and flexible laryngoscopy. Patients with VCD were educated about their condition and instructed about breathing techniques in the same session., Results: Twenty-two patients were available for a phone interview. Mean age of patients was 13.4 ± 3.0 years. Sixteen patients were female. Mean number of clinic visits was 1.3 ± 0.8. Average time between phone interview and first clinical encounter was 14.0 ± 7.2 months. Compliance rate to demonstrate breathing exercises was 90.9%. Nineteen out of 22 patients (86.4%) reported improvement of their symptoms in frequency and/or severity. Six patients (27.3%) sought additional medical advice related to their respiratory symptoms. Twenty-one patients (95.5%) were able to maintain or increase their level of physical activity following clinic visit., Conclusion: Combining the diagnostic encounter with multidisciplinary behavioral intervention in a single visit is an efficacious approach for the long-term management of VCD in the pediatric population., (Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.)

There has been a dramatic evolution in the treatment of laryngotracheal hemangiomas during the past decade and recent accounts and case reports of propranolol treatment have been encouraging. The purpose of the study is to determine the clinical course and outcomes of treating laryngotracheal hemangiomas at The Children's Hospital of Philadelphia in the last 8 years with the various modalities. We review with contemporary surgical techniques, including propranolol, and determine the results, limitations and complications. The study was a retrospective review of all patients referred to the Otolaryngology service at The Children's Hospital of Philadelphia with symptomatic laryngotracheal hemangiomas between January 2002 and December 2010. The study consisted of 30 infants, ranging in age from 1 to 18 months at time of diagnosis. Surgical interventions included open surgical excision, laser surgery, microdebrider excision and/or propranolol therapy. The main outcome measures include improvement in symptoms, decannulation, number of required treatments and airway size. All but two patients underwent an initial trial of steroids. Thirteen patients underwent open surgical excision, 9 requiring cartilage grafts and 12 were done in a single stage. Twelve surgical patients remained asymptomatic. One patient with diffuse mediastinal disease experiencing postoperative airway symptoms despite a normal appearing airway improved on propranolol. Two patients underwent at least 2 laser ablations, 4 responded to systemic steroids alone, and 1 had microdebrider resection. In the last 14 months, 12 patients have had propranolol therapy at a dose of 2mg/kg divided every 8h. Eight patients improved clinically within 1 week of initiating treatment. Four patients failed to respond to propranolol therapy; 1 patient subsequently underwent open excision and the other continued with a tracheostomy for 18 months and finally was decannulated. A third patient had a partial response, but remains relatively asymptomatic. A fourth patient has had no response at all. There were no major complications from propranolol; minor complications included diarrhea and decreased appetite. This study gives an overview of the evolution of hemangioma treatment at our institution over the last 8 years. Surgical excision remains an effective treatment for subglottic hemangiomas. Carefully administered, propranolol may demonstrate efficacy as a first-line agent in most cases avoiding surgery, tracheostomy, prolonged steroids, or as treatment of diffuse and unresectable disease. However, some lesions may be resistant to propranolol and require surgery or long-term steroids., (Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.)

We are reporting the case of a female child presenting for the management of symptomatic extensive tracheal hemangiomas who was successfully treated with multiple ablative procedures using KTP laser. This is the first description in the English literature of the use of KTP laser as primary treatment modality for the endoscopic ablation of tracheal hemangiomas in a child. We review treatment recommendations for symptomatic airway hemangiomas in the pediatric population, highlighting the advantages of KTP laser for tracheal lesions., (Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.)

We describe a medically complex 4-year-old child with a history of chronic unilateral myringotomy tube otorrhea and laryngopharyngeal reflux who was diagnosed with a patulous eustachian tube. The diagnosis was confirmed by nasopharyngoscopy, retrieval of food from the affected ear after oral challenges with green dye, and computed tomography. The child was effectively treated with an endoscopic transoral injection of calcium hydroxylapatite (Radiesse Voice) in the lateral pharyngeal wall and torus tubarius. She initially received a test injection with a temporary gel of synthetic polymers (Radiesse Voice Gel) with benefit lasting several months; she subsequently underwent a longer term injection with the hydroxylapatite and tube removal. She had complete resolution of otorrhea for 18 months with no subsequent ear infections or food matter in the ear despite persistent significant gastroesophageal reflux. The patulous eustachian tube is rarely identified in children and, when found, is a challenging condition to manage. Many current surgical treatments involve permanent occlusion of the eustachian tube. Our endoscopic transoral technique is a shorter-term alternative for management of complications related to this anatomic variation, and it allows time for eustachian tube growth., (Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.)

Objective: To compare single-stage laryngotracheal reconstruction (ssLTR) and double-stage LTR (dsLTR)., Design: Retrospective medical record review., Setting: Tertiary care children's hospital., Patients: Seventy-one patients underwent 84 procedures (22 ssLTRs and 62 dsLTRs)., Intervention: Review of preoperative disease severity and surgical outcomes for patients who underwent ssLTR vs dsLTR., Main Outcome Measure: Operation-specific and overall decannulation rates., Results: Regarding ssLTRs, the mean grade of subglottic stenosis was 2.1 and the overall and operation-specific decannulation rates were 100% and 91%, respectively. The mean grade of subglottic stenosis for double-stage procedures was 2.9, and the overall and operation-specific decannulation rates were 93% and 68%, respectively. Patients who underwent ssLTR and dsLTR were further divided into early and late groups based on whether the posterior graft was sutured in place (early) or not (late). Overall and operation-specific decannulation rates were 100% and 89%, respectively, for the early single-stage group and 100% and 92% for the late group. Regarding the dsLTR group, overall and operation-specific decannulation rates were 88% and 42%, respectively, for the early group and 95% and 79% for the late group. Among all groups, there was no significant difference in overall decannulation rates (P > .05). Single-stage LTR offered an increased rate of operation-specific decannulation over dsLTR (P < .05). However, that difference was not significant between the late ssLTR and the late dsLTR groups (P > .05)., Conclusion: Careful assessment of preoperative disease severity and overall medical status will help surgeons choose between ssLTR and dsLTR, maximizing patient outcomes for both modalities.

Objective: To systematically evaluate the diagnosis of eosinophilic esophagitis (EE)., Methods: A retrospective review of 657 patients seen at the EE center of a tertiary care children's hospital between 1994 and 2007 was performed. Charts were reviewed for the 144 patients who were also seen by the otolaryngology service., Results: One hundred forty-four patients received 193 otolaryngology-related diagnoses. Eustachian tube dysfunction (27.5%) and sleep disordered breathing (24.9%) were the most common, followed by dysphagia (13.0%), rhinosinusitis/nasal congestion (9.3%) and airway stenosis (5.2%). Seventy-nine patients (54.9%) had a pre-existing diagnosis of EE at the time of their otolaryngology consultation. Twenty-one patients (14.6%) were referred to the gastroenterology service for evaluation for EE. Forty-four patients (30.5%) remained undiagnosed. Twenty-five of these patients presented with dysphagia, 16 of whom were not previously diagnosed with EE; only 4 of these 16 patients were referred for evaluation for EE. In one case, a child with moderate sized tonsils underwent adenotonsillectomy for dysphagia and failure to thrive; this patient was diagnosed with EE 1 month post-operatively., Conclusions: Twenty percent of patients with EE may require care by an otolaryngologist for a myriad of complaints. Even experienced pediatric otolaryngologists may not recognize this condition. Otolaryngologists should consider EE in patients presenting with dysphagia. A careful gastroenterology review of symptoms may also allow otolaryngologists to identify EE in patients with allergy mediated nasal complaints, or laryngeal/airway disorders.

Airway foreign body aspiration most commonly occurs in young children and is associated with a high rate of airway distress, morbidity, and mortality. The presenting symptoms of foreign body aspiration range from none to severe airway obstruction, and may often be innocuous and nonspecific. In the absence of a choking or aspiration event, the diagnosis may be delayed for weeks to months and contribute to worsening lung disease. Radiography and high resolution CT scan may contribute to the eventual diagnosis. Bronchoscopy is used to confirm the diagnosis and retrieve the object. The safest method of removing an airway foreign body is by utilizing general anesthesia. Communication between anesthesiologist and surgeon is essential for optimal outcome. The choice between maintenance of spontaneous and controlled ventilation is often based on personal preference and does not appear to affect the outcome of the procedure. Complications are related to the actual obstruction and to the retrieval of the impacted object. The localized inflammation and irritation that result from the impacted object can lead to bronchitis, tracheitis, atelectasis, and pneumonia.