Pediatric Aerodigestive Medicine: Advancing Collaborative Care for Children With Oropharyngeal Dysphagia.

OBJECTIVES: Aerodigestive disorders encompass various pathological conditions affecting the lungs, upper airway, and gastrointestinal tract in children. While advanced care has primarily occurred in specialty centers, many children first present to general pediatric gastroenterologists with aerodigestive symptoms necessitating awareness of these conditions. At the 2021 Annual North American Society for Pediatric Gastroenterology, Hepatology and Nutrition meeting, the aerodigestive Special Interest Group held a full-day symposium entitled, Pediatric Aerodigestive Medicine: Advancing Collaborative Care of Children with Aerodigestive Disorders. The symposium aimed to underline the significance of a multidisciplinary approach to achieve better outcomes for these complex patients. METHODS: The symposium brought together leading experts to highlight the growing aerodigestive field, promote new scientific and therapeutic strategies, share the structure and benefits of a multidisciplinary approach in diagnosing common and rare aerodigestive disorders, and foster multidisciplinary discussion of complex cases while highlighting the range of therapeutic and diagnostic options. In this article, we showcase the diagnostic and therapeutic approach to oropharyngeal dysphagia (OPD), one of the most common aerodigestive conditions, emphasizing the role of a collaborative model. CONCLUSIONS: The aerodigestive field has made significant progress and continues to grow due to a unique multidisciplinary, collaborative model of care for these conditions. Despite diagnostic and therapeutic challenges, the multidisciplinary approach has enabled and greatly improved efficient, high-quality, and evidence-based care for patients, including those with OPD.

Anesthesia for laryngotracheal reconstruction in children: A narrative review.

Laryngotracheal stenosis, congenital or acquired, is a common cause of pediatric airway obstruction. Acquired subglottic stenosis frequently results from prolonged neonatal intubation. The clinical presentation of subglottic stenosis is variable, ranging from biphasic stridor and frequent upper respiratory infections to acute airway compromise. Optimal patient care requires clinical coordination within a multidisciplinary subspecialty team. Medical management includes optimizing respiratory status, gastroesophageal reflux, speech, feeding, nutrition therapies, and providing psychosocial support. If surgical intervention is required, the otolaryngologist, anesthesiologist, and perioperative team must collaborate closely to ensure successful operative outcomes. This narrative review of laryngotracheal stenosis will discuss the pathophysiology, clinical evaluation, medical management, and surgical interventions, and focus on the perioperative anesthetic considerations for children undergoing laryngotracheal reconstruction.

FUT2 Variants Confer Susceptibility to Familial Otitis Media.

Non-secretor status due to homozygosity for the common FUT2 variant c.461G>A (p.Trp154∗) is associated with either risk for autoimmune diseases or protection against viral diarrhea and HIV. We determined the role of FUT2 in otitis media susceptibility by obtaining DNA samples from 609 multi-ethnic families and simplex case subjects with otitis media. Exome and Sanger sequencing, linkage analysis, and Fisher exact and transmission disequilibrium tests (TDT) were performed. The common FUT2 c.604C>T (p.Arg202∗) variant co-segregates with otitis media in a Filipino pedigree (LOD = 4.0). Additionally, a rare variant, c.412C>T (p.Arg138Cys), is associated with recurrent/chronic otitis media in European-American children (p = 1.2 × 10-5) and US trios (TDT p = 0.01). The c.461G>A (p.Trp154∗) variant was also over-transmitted in US trios (TDT p = 0.01) and was associated with shifts in middle ear microbiota composition (PERMANOVA p < 10-7) and increased biodiversity. When all missense and nonsense variants identified in multi-ethnic US trios with CADD > 20 were combined, FUT2 variants were over-transmitted in trios (TDT p = 0.001). Fut2 is transiently upregulated in mouse middle ear after inoculation with non-typeable Haemophilus influenzae. Four FUT2 variants-namely p.Ala104Val, p.Arg138Cys, p.Trp154∗, and p.Arg202∗-reduced A antigen in mutant-transfected COS-7 cells, while the nonsense variants also reduced FUT2 protein levels. Common and rare FUT2 variants confer susceptibility to otitis media, likely by modifying the middle ear microbiome through regulation of A antigen levels in epithelial cells. Our families demonstrate marked intra-familial genetic heterogeneity, suggesting that multiple combinations of common and rare variants plus environmental factors influence the individual otitis media phenotype as a complex trait.

A2ML1 and otitis media: novel variants, differential expression, and relevant pathways.

A genetic basis for otitis media is established, however, the role of rare variants in disease etiology is largely unknown. Previously a duplication variant within A2ML1 was identified as a significant risk factor for otitis media in an indigenous Filipino population and in US children. In this report exome and Sanger sequencing was performed using DNA samples from the indigenous Filipino population, Filipino cochlear implantees, US probands, Finnish, and Pakistani families with otitis media. Sixteen novel, damaging A2ML1 variants identified in otitis media patients were rare or low-frequency in population-matched controls. In the indigenous population, both gingivitis and A2ML1 variants including the known duplication variant and the novel splice variant c.4061 + 1 G>C were independently associated with otitis media. Sequencing of salivary RNA samples from indigenous Filipinos demonstrated lower A2ML1 expression according to the carriage of A2ML1 variants. Sequencing of additional salivary RNA samples from US patients with otitis media revealed differentially expressed genes that are highly correlated with A2ML1 expression levels. In particular, RND3 is upregulated in both A2ML1 variant carriers and high-A2ML1 expressors. These findings support a role for A2ML1 in keratinocyte differentiation within the middle ear as part of otitis media pathology and the potential application of ROCK inhibition in otitis media.

Transnasal Endoscopy in Unsedated Children With Eosinophilic Esophagitis Using Virtual Reality Video Goggles.

BACKGROUND & AIMS: Evaluation and treatment of children with eosinophilic esophagitis (EoE) requires serial endoscopic, visual, and histologic assessment by sedated esophagogastroduodenoscopy (EGD). Unsedated transnasal endoscopy (TNE) was reported to be successful in a pilot study of children. We evaluated video goggle and virtual reality-based unsedated TNE in children with EoE, collecting data on rates of completion, adverse events, and adequacy of visual and histologic findings. METHODS: We performed a retrospective study of 190 children and young adults (age, 3-22 y) who underwent video goggle or virtual reality-based unsedated TNE from January 2015 through February 2018. We analyzed data on patient demographics, procedure completion, endoscope type, adverse events, visual and histologic findings, estimated costs, and duration in the facility. Esophageal biopsies from the first 173 subjects who underwent TNE were compared with those from previous EGD evaluations. RESULTS: During 300 attempts, 294 TNEs were performed (98% rate of success). Fifty-four patients (age, 6-18 y) underwent multiple TNEs for dietary or medical management of EoE. There were no significant adverse events. Visual and histologic findings were adequate for assessment of EoE. TNE reduced costs by 53.4% compared with EGD (TNE $4393.00 vs EGD $9444.33). TNE was used increasingly from 2015 through 2017, comprising 31.8% of endoscopies performed for EoE. The total time spent in the clinic (front desk check-in to check-out) in 2018 was 71 minutes. CONCLUSIONS: In a retrospective study of 190 children and young adults (age, 3-22 y) who underwent video goggle or virtual reality-based unsedated TNE, TNE was safe and effective and reduced costs of EoE monitoring. Advantages of TNE include reduced risk and cost associated with anesthesia as well as decreased in-office time, which is of particular relevance for patients with EoE, who require serial EGDs.

Pediatric tracheomalacia and the perioperative anesthetic management of thoracoscopic posterior tracheopexy.

Tracheomalacia is a broad term used to describe an abnormally compliant trachea that can lead to exaggerated collapse and obstruction with expiration. We describe the perioperative management of a complex pediatric patient undergoing a posterior tracheopexy which is a relatively new surgical treatment, with a novel surgical approach-thoracoscopy. This procedure has competing surgical and anesthetic needs and presents unique challenges to the physicians involved in caring for these patients. We also review the current literature on pediatric tracheomalacia and examine the newest treatment options to highlight the potential anesthetic challenges and pitfalls associated with management.

Unsedated transnasal esophagoscopy for monitoring therapy in pediatric eosinophilic esophagitis.

BACKGROUND AND AIMS: Unsedated transnasal endoscopy (TNE) is safer and less costly than sedated EGD. The aim of this study was to evaluate the performance of TNE with biopsies in monitoring the esophageal mucosa of pediatric patients with eosinophilic esophagitis. METHODS: Patients between 8 and 17 years of age with eosinophilic esophagitis and their parents were enrolled. Unsedated TNE was performed. A 2.8-mm (1.2-mm channel) or a 4-mm flexible bronchoscope (2-mm channel) was used, and esophageal biopsy specimens were obtained. Biopsy specimen analysis, duration, adverse events, and billing charges of TNE were assessed. Immediately after TNE and a minimum of 2 weeks later, a modified Group Health Association of America 9 survey and a preference questionnaire were completed, respectively. RESULTS: Twenty-one of 22 enrolled patients underwent TNE. TNE was performed with no serious adverse events. Histopathological analysis revealed 0 eosinophils per high-power field (n = 12), fewer than 15 eosinophils per high-power field (n = 4), and more than 15 eosinophils per high-power field (n = 5). The total epithelial surface area of mucosal biopsy samples from either TNE Forceps (1.2 mm or 2 mm biopsy channel forceps) compared with those obtained during the subject's previous EGD by using standard endoscopic forceps was not statistically different (P = .308 [1.2 mm]/P = .492 [2 mm]). All parents and 76.2% of subjects would undergo the TNE again. TNE was preferred over EGD by 85.7% of parents and 52.4% of subjects. The modified Group Health Association of America 9 survey revealed a high degree of satisfaction (average, 43.19 ± 2.6; maximum score, 45). Charges associated with TNE were 60.1% lower than for previous EGDs. CONCLUSIONS: Unsedated TNE is an effective, lower-cost procedure for monitoring the esophageal mucosa of children with eosinophilic esophagitis.

Airway management in laryngotracheal injuries from blunt neck trauma in children.

Pediatric laryngotracheal injuries from blunt neck trauma are extremely rare, but can be potentially catastrophic. Early diagnosis and skillful airway management is critical in avoiding significant morbidity and mortality associated with these cases. We present a case of a patient who suffered a complete tracheal transection and cervical spine fracture following a clothesline injury to the anterior neck. A review of the mechanisms of injury, clinical presentation, initial airway management, and anesthetic considerations in laryngotracheal injuries from blunt neck trauma in children are presented.

Ex utero intrapartum treatment to resection of a bronchogenic cyst causing airway compression.

We present a case report of a 28-year-old primigravida with a singleton pregnancy complicated by a fetal bronchogenic cyst compressing the left mainstem bronchus with resultant hyperinflation of the entire left lung and rightward mediastinal shift. An ex utero intrapartum treatment to resection of the fetal bronchogenic cyst via a fetal thoracotomy was performed at 36 weeks' gestational age, circumventing a potentially complicated neonatal airway emergency at birth.

Social support moderates the relationship between burnout and job satisfaction in aerodigestive team members.

OBJECTIVES: Burnout among healthcare workers is a public health crisis. Burnout is associated with elevated cynicism, emotional exhaustion, and low job satisfaction. Methods to combat burnout have been challenging to identify. Based on positive experiences of pediatric aerodigestive team members, we hypothesized that social support in multidisciplinary aerodigestive teams moderates the effects of burnout on job satisfaction. METHODS: Using a survey of the Aerodigestive Society, members of Aerodigestive teams (N = 119) completed demographics, the Maslach Burnout Inventory, and measures of job satisfaction, emotional, and instrumental social support. In addition to assessing relationships between components of burnout and job satisfaction, six tests were conducted using PROCESS to ascertain the degree to which social support moderated these relationships. RESULTS: Similar to US healthcare base rates, burnout scores in this sample suggest that a third-to-half felt Emotionally Exhausted and Burned Out from work "A few times a month"-to-"Every Day." Simultaneously, however, the majority in sample (60.6%) noted feeling that they "positively impact others' lives" with 33.3% endorsing "Every Day." Job satisfaction was strikingly high at 89%, with most reporting Aerodigestive team affiliation related to higher job satisfaction. Both Emotional and Instrumental social support moderated the effect of Cynicism and Emotional Exhaustion on Job Satisfaction, with higher Job Satisfaction scores in conditions of high support. CONCLUSIONS: These results support the hypothesis that social support from a multidisciplinary aerodigestive team moderates the effect of burnout in its team members. Further work is needed to understand if membership in other interprofessional healthcare teams can help combat the negative effects of burnout.

A multicenter study analyzing the impact of pre-existing comorbidities on laryngotracheal reconstruction (LTR) outcomes.

INTRODUCTION: Comorbidities such as chronic lung disease and gastroesophageal reflux (GERD), prematurity, and numerous other conditions may impact the success of LTR. Single-center studies are limited in terms of patient numbers and may be underpowered. OBJECTIVES: To analyze the impact of specific comorbidities on the operation-specific and overall surgical success of LTR in a large multicenter cohort and validate a predictive model for surgical success. METHODS: A large retrospective multicenter 10-year review was undertaken to validate the data of a previous single-center study (Wertz et al. Laryngoscope 2020) which identified specific predictive comorbidities which impacted LTR outcomes. A Monte Carlo simulation based on the previous data set suggested that 300-400 cases would be needed to optimize the statistical power of a Bayesian model developed from the single-center data to predict surgical success. An IRB-approved data-sharing agreement was executed for 4 large U.S. CENTERS: A virtual REDCap® data entry form inquired about patient characteristics that best predicted surgical success in the single-center model. These included demographics, surgical approaches, cardiac, airway, genetic, endocrine, musculoskeletal, gastrointestinal, and pulmonary comorbidities; details of the surgical procedures, and results of esophagogastroduodenoscopy (EGD), esophageal pH/impedance and flexible bronchoscopy with bronchioalveolar lavage (BAL) were included. Surgical success defined as successful decannulation or resolution of airway symptoms was recorded as single surgery success and overall success following open surgical revision surgery. Multivariate Bayesian analysis, logistical regression, and Kaplan-Meier analysis were performed. RESULTS: 542 patients were identified, including 165 from the single-center study and an additional 377 patients from the multicenter group. The median age was 36 months at the time of the most recent surgery. 70.9% of the LTRs were double-staged procedures. The overall success rate was 86.4% and operation-specific success rate was 69.2%. The specific comorbidities and aerodigestive test results that impacted success based on univariate analysis included staging, bronchiectasis, pulmonary hypertension, GERD, ASD, PDA, grade of stenosis, advanced levels of stenosis, Trisomy 21, MRSA, prior open surgery at another hospital, and gross appearance on EGD. Bayesian model averaging with backward selection was used to validate and refine a predictive model for surgical success with favorable receiver operating curve characteristics - AUC values of 0.827 for single surgery success and 0.797 for overall success. DISCUSSION: With over 500 patients reviewed, this was the largest multicenter study of LTR to date, which elucidated the impact of comorbidities on success with LTR and was able to improve upon the predictive modeling based on single-center data. Patient factors are most critical in the outcome of LTR. Stage and levels of stenosis, as well as pulmonary and GI conditions most strongly impact the likelihood of success. Future prospective case-control studies will be performed to further optimize the current model for outcome prediction and patient management.

Prophylactic inhaled corticosteroids for the management of recurrent croup.

OBJECTIVES: Croup is characterized by a barky cough, inspiratory stridor, hoarseness and varying degrees of respiratory distress. Acute croup episodes are often treated with oral, inhaled, or intravenous corticosteroids. Recurrent croup, defined as more than 2-3 episodes of acute croup in the same patient, can mimic asthma. We hypothesized that inhaled corticosteroids (ICS) given at the first sign of a respiratory viral prodrome can be a safe treatment to reduce the frequency of recurrent croup episodes in children without fixed airway lesions. METHODS: A retrospective chart review of patients being treated over an 18-month period was performed at a large tertiary care pediatric hospital following Institutional Review Board (IRB) approval. Patients under 21 years old referred to Pediatric Pulmonology, Otolaryngology, or Gastroenterology for recurrent croup were analyzed for their demographics, medical history, evaluation, treatment and clinical improvement. A Fisher's two-tailed exact test was used to compare the number of croup episodes before and after interventions. RESULTS: 124 patients were included in our analysis: 87 male and 34 female with a mean age of 54 months. Of these, 78 had >5 episodes of croup, 45 had 3-5, and 3 had 2 episodes prior to their first visit for recurrent croup. Operative direct laryngoscopy/bronchoscopy was performed in 35 patients (27.8%), with 60% showing a normal exam without fixed lesions. Ninety-two patients (74.2%) were treated with ICS, 24 were lost to follow up. Of the remaining 68 treated patients, 59 (86.7%) saw improvement with reduced severity and overall number of episodes of croup. Additionally, patients with >5 episodes of croup (47) as compared to <5 (12) were more likely to improve with ICS, (p = 0.003). There were no adverse reactions reported with ICS treatment. CONCLUSION: The novel initiation of ICS at the earliest sign of a viral upper respiratory infection shows promise as a safe preventative treatment to mitigate the frequency of recurrent croup episodes.

Otitis Media in Children with Down Syndrome Is Associated with Shifts in the Nasopharyngeal and Middle Ear Microbiotas.

Background: Otitis media (OM) is defined as middle ear (ME) inflammation that is usually due to infection. Globally, OM is a leading cause of hearing loss and is the most frequently diagnosed disease in young children. For OM, pediatric patients with Down syndrome (DS) demonstrate higher incidence rates, greater severity, and poorer outcomes. However, to date, no studies have investigated the bacterial profiles of children with DS and OM. Method: We aimed to determine if there are differences in composition of bacterial profiles or the relative abundance of individual taxa within the ME and nasopharyngeal (NP) microbiotas of pediatric OM patients with DS (n = 11) compared with those without DS (n = 84). We sequenced the 16S rRNA genes and analyzed the sequence data for diversity indices and relative abundance of individual taxa. Results: Individuals with DS demonstrated increased biodiversity in their ME and NP microbiotas. In children with OM, DS was associated with increased biodiversity and higher relative abundance of specific taxa in the ME. Conclusion: Our findings suggest that dysbioses in the NP of DS children contributes to their increased susceptibility to OM compared with controls. These findings suggest that DS influences regulation of the mucosal microbiota and contributes to OM pathology.

Posterior tracheopexy for preterm infants with severe bronchopulmonary dysplasia and severe tracheobronchomalacia: A case series.

We report a series of four patients with severe bronchopulmonary dysplasia (BPD) who underwent posterior tracheopexy for severe tracheomalacia (TM). While posterior tracheopexy is an established surgical treatment for TM associated with tracheoesophageal fistula, it has not been previously described in TM associated with BPD. There were no significant intraoperative or postoperative complications from the surgeries. Three of the four patients required tracheostomy and mechanical ventilation, which may reflect the degree of lung disease and other multisystem comorbidities in these patients. More investigation is needed to determine whether posterior tracheopexy is an effective surgical option for TM related to BPD.

Outcomes in Pediatric Thyroidectomy: Results From a Multinational, Multi-institutional Database.

OBJECTIVE: Traditionally, data regarding thyroidectomy were extracted from billing databases, but information may be missed. In this study, a multi-institutional pediatric thyroidectomy database was used to evaluate recurrent laryngeal nerve (RLN) injury and hypoparathyroidism. STUDY DESIGN: Retrospective multi-institutional cohort study. SETTING: Tertiary care pediatric hospital systems throughout North America. METHODS: Data were individually collected for thyroidectomies, then entered into a centralized database and analyzed using univariate and multivariable regression models. RESULTS: In total, 1025 thyroidectomies from 10 institutions were included. Average age was 13.9 years, and 77.8% were female. Average hospital stay was 1.9 nights and 13.5% of patients spent at least 1 night in the pediatric intensive care unit. The most frequent pathology was papillary thyroid carcinoma (42%), followed by Graves' disease (20.1%) and follicular adenoma (18.2%). Overall, 1.1% of patients experienced RLN injury (0.8% permanent), and 7.2% experienced hypoparathyroidism (3.3% permanent). Lower institutional volume (odds ratio [OR], 3.57; 95% CI, 1.72-7.14) and concurrent hypoparathyroidism (OR, 3.51; 95% CI, 1.64-7.53) correlated with RLN injury on multivariable analysis. Graves' disease (OR, 2.27; 95% CI, 1.35-3.80), Hashimoto's thyroiditis (OR, 4.67; 95% CI, 2.39-9.09), central neck dissection (OR, 3.60; 95% CI, 2.36-5.49), and total vs partial thyroidectomy (OR, 7.14; 95% CI, 4.55-11.11) correlated with hypoparathyroidism. CONCLUSION: These data present thyroidectomy information and complications pertinent to surgeons, along with preoperative risk factor assessment. Multivariable analysis showed institutional volume and hypoparathyroidism associated with RLN injury, while hypoparathyroidism associated with surgical indication, central neck dissection, and extent of surgery. Low complication rates support the safety of thyroidectomy in pediatric tertiary care centers.

International Pediatric Otolaryngology Group (IPOG) consensus recommendations: Evaluation and management of congenital tracheal stenosis.

OBJECTIVES: To outline an expert-based consensus of recommendations for the diagnosis and management of pediatric patients with congenital tracheal stenosis. METHODS: Expert opinions were sought from members of the International Pediatric Otolaryngology Group (IPOG) via completion of an 18-item survey utilizing an iterative Delphi method and review of the literature. RESULTS: Forty-three members completed the survey providing recommendations regarding the initial history, clinical evaluation, diagnostic evaluation, temporizing measures, definitive repair, and post-repair care of children with congenital tracheal stenosis. CONCLUSION: These recommendations are intended to be used to support clinical decision-making regarding the evaluation and management of children with congenital tracheal stenosis. Responses highlight the diverse management strategies and the importance of a multidisciplinary approach to care of these patients.

Tonsillectomy and hematologic malignancy: Should routine pediatric tonsillectomy specimens be sent to pathology?

OBJECTIVES: Routine pediatric tonsillectomy ± adenoidectomy (T ± A) is one of the most common procedures for children worldwide, accounting for approximately 2000 procedures per year at our institution. To determine the utility of pathologic analysis of routine, nonsuspicious pediatric tonsil specimens, we investigated the incidence of hematologic and lymphoid malignancy diagnosed at the time of or following T ± A. METHODS: Retrospective review of patients 0-18 years undergoing T ± A between 2012 and 2020 with or without pathologic analysis. RESULTS: Included were 14,141 patients who underwent routine T ± A (mean age 11 ± 4.6 years, 48% female). Of these, tonsils of 2464 patients were sent to pathology, where zero were found to harbor malignancy. Seven patients (0.050%) developed malignancy after T ± A. Of these, 4 had unremarkable tonsils per pathology, and 3 did not have tonsils analyzed. There were 5 cases of Acute Lymphocytic Leukemia (ALL, 0.035%), 1 case of Acute Myeloid Leukemia (0.007%), and 1 case of Lymphoma (0.007%). The average length of time from T ± A to diagnosis was 2.4 ± 1.8 years. CONCLUSION: No cases of occult malignancy were identified in specimens from routine T ± A with pathologic analysis, even among patients who later developed malignancy. This study suggests that sending routine pediatric T ± A specimens for formal pathologic analysis is an inefficient use of resources without appreciably improving the quality and safety of patient care.

Slide Tracheoplasty for Tracheal Cartilaginous Sleeve in a Patient With Apert Syndrome.

Apert syndrome is a form of acrocephalosyndactyly involving craniosynostosis, syndactyly, and less commonly, tracheal cartilaginous sleeve (TCS), a potential cause of tracheal stenosis. Slide tracheoplasty is performed in children with tracheal stenosis. No reports exist for its application in stenosis related to TCS. We present a case in which slide tracheoplasty was used for the expansion of long segment tracheal stenosis owing to TCS in a newborn with Apert syndrome. Using this technique, a safe and durable airway was achieved without tracheostomy.

The risks of polytetrafluoroethylene pledget use in tracheopexy.

Posterior tracheopexy, performed most often for intrinsic tracheomalacia, is a procedure that is gaining in popularity and application. In this procedure, pledgeted sutures are placed between the trachea and a more fixed structure, in order to provide the airway with greater patency. Polytetrafluoroethylene pledgets are readily available but risk migration into adjacent structures over time. We present a case of a pledget migrating in to the airway and presenting as an obstructing lesion that has required multiple repeat interventions. Given that bioabsorbable materials are available, surgeons should consider alternative pledgets for these patients.