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OBJECTIVES No previous study has examined the management of hospitalized children with orbital cellulitis at both children’s and community hospitals across multiple sites in Canada. We describe variation and trends over time in diagnostic testing and imaging, adjunctive agents, empiric antibiotics, and surgical intervention in children hospitalized with orbital cellulitis. PATIENTS AND METHODS Multicenter cohort study of 1579 children aged 2 months to 18 years with orbital cellulitis infections admitted to 10 hospitals from 2009 to 2018. We assessed hospital-level variation in the use of diagnostic tests, imaging, antibiotics, adjunctive agents, surgical intervention, and clinical outcomes using X2, Mann-Whitney U, and Kruskal-Wallis tests. The association between clinical management and length of stay was evaluated with median regression analysis with hospital as a fixed effect. RESULTS There were significant differences between children’s hospitals in usage of C-reactive protein tests (P < .001), computed tomography scans (P = .004), MRI scans (P = .003), intranasal decongestants (P < .001), intranasal corticosteroids (P < .001), intranasal saline spray (P < .001), and systemic corticosteroids (P < .001). Children’s hospital patients had significantly longer length of hospital stay compared with community hospitals (P = .001). After adjustment, diagnostic testing, imaging, and subspecialty consults were associated with longer median length of hospital stay at children’s hospitals. From 2009 to 2018, C-reactive protein test usage increased from 28.8% to 73.5% (P < .001), whereas erythrocyte sedimentation rate decreased from 31.5% to 14.1% (P < .001). CONCLUSIONS There was significant variation in diagnostic test usage and treatments, and increases in test usage and medical intervention rates over time despite minimal changes in surgical interventions and length of stay.

Demographics and Oncogenic Variants for Tumors evaluated by WES/RNASeq. NA-Not applicable (infiltrative tumors without defined borders)

Gene Expression Clusters, Gene Ontology and KEGG Gene Set Enrichment by Cluster

Gene Set Variation Analysis

Chromosomes, Genes, Breakpoints, and Fusion Validator Scores for Oncogenic Fusions. In the event of multiple splicing confirmations, the fusion with highest validator score is displayed.

BRAF-RAS Score (BRS) Designation by Oncogenic Variant

Distribution of Histologic Variants by Oncogenic Variant Class or Gene Expression Cluster

Pediatric papillary thyroid carcinoma (PPTC) is clinically distinct from adult-onset disease. Although there are higher rates of metastasis and recurrence in PPTC, prognosis remains highly favorable. Molecular characterization of PPTC has been lacking. Historically, only 40% to 50% of childhood papillary thyroid carcinoma (PTC) were known to be driven by genomic variants common to adult PTC; oncogenic drivers in the remainder were unknown. This contrasts with approximately 90% of adult PTC driven by a discrete number of variants. In this study, 52 PPTCs underwent candidate gene testing, followed in a subset by whole-exome and transcriptome sequencing. Within these samples, candidate gene testing identified variants in 31 (60%) tumors, while exome and transcriptome sequencing identified oncogenic variants in 19 of 21 (90%) remaining tumors. The latter were enriched for oncogenic fusions, with 11 nonrecurrent fusion transcripts, including two previously undescribed fusions, STRN-RET and TG-PBF. Most fusions were associated with 3′ receptor tyrosine kinase (RTK) moieties: RET, MET, ALK, and NTRK3. For advanced (distally metastatic) tumors, a driver variant was described in 91%. Gene expression analysis defined three clusters that demonstrated distinct expression of genes involved in thyroid differentiation and MAPK signaling. Among RET-CCDC6–driven tumors, gene expression in pediatric tumors was distinguishable from that in adults. Collectively, these results show that the genomic landscape of pediatric PTC is different from adult PTC. Moreover, they identify genomic drivers in 98% of PPTCs, predominantly oncogenic fusion transcripts involving RTKs, with a pronounced impact on gene expression. Notably, most advanced tumors were driven by a variant for which targeted systemic therapy exists.Significance:This study highlights important distinctions between the genomes and transcriptomes of pediatric and adult papillary thyroid carcinoma, with implications for understanding the biology, diagnosis, and treatment of advanced disease in children.

Determination of cluster numbers by Elbow and Gap Statistic Modeling

Most button battery (BB) ingestions in children are unwitnessed leading to prolonged exposures and severe complications. One third of ingestions occur from free BB, that are stored or awaiting disposal. Recommendations have been made to cover the terminals of discarded BB with adhesive tape; however, it is unclear if this practice prevents injury. Our aim was to determine if tape could prevent oesophageal injury in a cadaveric porcine model.Electrical, masking, packing and duct tape were compared. One BB was left untaped. Taped BBs were placed in a cadaveric porcine oesophagus controlled for temperature and humidification. Specimens were assessed at 0, 0.5, and hourly for 6 h by visual inspection, temperature and pH. BB voltage was measured before and after testing. All tests were repeated in triplicate.Oesophageal specimens demonstrated burn prevention in the packing and duct tape trials. Burns were seen in 2/3 trials with electrical tape and 3/3 trials with masking tape. pH remained neutral throughout the study for all packing and duct tape specimens. pH remained neutral initially for masking tape but increased rapidly to 12 by 2 h. There was no change in battery voltage for the packing tape and duct tape trials. There was a 16.3% reduction in voltage for masking tape which was similar to controls.Taping BB with packing tape and duct tape prevented oesophageal burns. This may provide a novel method of burn prevention for loose BB intended for disposal.

Pediatric papillary thyroid carcinoma (PPTC) is clinically distinct from adult-onset disease. Although there are higher rates of metastasis and recurrence in PPTC, prognosis remains highly favorable. Molecular characterization of PPTC has been lacking. Historically, only 40% to 50% of childhood papillary thyroid carcinoma (PTC) were known to be driven by genomic variants common to adult PTC; oncogenic drivers in the remainder were unknown. This contrasts with approximately 90% of adult PTC driven by a discrete number of variants. In this study, 52 PPTCs underwent candidate gene testing, followed in a subset by whole-exome and transcriptome sequencing. Within these samples, candidate gene testing identified variants in 31 (60%) tumors, while exome and transcriptome sequencing identified oncogenic variants in 19 of 21 (90%) remaining tumors. The latter were enriched for oncogenic fusions, with 11 nonrecurrent fusion transcripts, including two previously undescribed fusions, STRN-RET and TG-PBF. Most fusions were associated with 3′ receptor tyrosine kinase (RTK) moieties: RET, MET, ALK, and NTRK3. For advanced (distally metastatic) tumors, a driver variant was described in 91%. Gene expression analysis defined three clusters that demonstrated distinct expression of genes involved in thyroid differentiation and MAPK signaling. Among RET-CCDC6–driven tumors, gene expression in pediatric tumors was distinguishable from that in adults. Collectively, these results show that the genomic landscape of pediatric PTC is different from adult PTC. Moreover, they identify genomic drivers in 98% of PPTCs, predominantly oncogenic fusion transcripts involving RTKs, with a pronounced impact on gene expression. Notably, most advanced tumors were driven by a variant for which targeted systemic therapy exists. Significance: This study highlights important distinctions between the genomes and transcriptomes of pediatric and adult papillary thyroid carcinoma, with implications for understanding the biology, diagnosis, and treatment of advanced disease in children.

Background There is a critical gap in identifying effective interventions for children with obstructive sleep apnea (OSA) who do not tolerate continuous positive airway pressure therapy. Positional OSA (POSA) is a common clinical phenotype whereby OSA occurs predominantly while sleeping in supine position. POSA may be amenable to treatment with a positional device, a belt worn around the chest with cushions on the back to prevent supine positioning, but no data exists in children. The primary aim of this study was to evaluate the efficacy of positional device therapy for the treatment of POSA in children. Methods This observational study included children aged 4–18 years with POSA and an obstructive apnea-hypopnea index (OAHI) ≥ 5 events/hour on baseline polysomnogram (PSG) who underwent a second PSG to evaluate the efficacy of a positional device. The primary outcome was the change in OAHI. Results Ten children were included (8 male, median age 11.2 years, median body mass index z-score 1.6). Compared to the baseline PSG, PSG data obtained while using a positional device showed a reduced median (interquartile range) OAHI (15.2 [8.3–25.6] versus 6.7 [1.0–13.7] events/hour respectively; p = 0.004) and percentage of total sleep time in supine position (54.4 [35.0–80.6]% versus 4.2 [1.1–25.2]% respectively; p = 0.04). Despite observed improvements in the oxygen desaturation index, these results were not statistically significant. Significance and conclusions In this novel pilot study, positional device therapy was effective for the treatment of POSA. Positional device therapy may potentially change clinical practice as a cost-efficient and non-invasive treatment option for POSA.

Objective To provide a systematic review of the existing pediatric decannulation protocols, including the role of polysomnography, and their clinical outcomes. Methods Five online databases were searched from database inception to May 29, 2020. Study inclusion was limited to publications that evaluated tracheostomy decannulation in children 18 years of age and younger. Data extracted included patient demographics and primary indication for tracheostomy. Methods used to assess readiness for decannulation were noted including the use of bronchoscopy, tracheostomy tube modifications, and gas exchange measurements. After decannulation, details regarding mode of ventilation, location, and length of observation period, and clinical outcomes were also collected. Descriptive statistical analyses were performed. Results A total of 24 studies including 1395 children were reviewed. Tracheostomy indications included upper airway obstruction at a well-defined anatomic site (35%), upper airway obstruction not at a well-defined site (12%) and need for long-term ventilation and pulmonary care (53%). Bronchoscopy was routinely used in 23 of 24 (96%) protocols. Tracheostomy tube modifications in the protocols included capping (n = 20, 83%), downsizing (n = 14, 58%), and fenestrations (n = 2, 8%). Measurements of gas exchange included polysomnography (n = 13/18, 72%), oximetry (n = 10/18, 56%), blood gases (n = 3,17%), and capnography (n = 3, 17%). After decannulation, children in 92% of protocols were transitioned to room air. Observation period of 48 h or less was used in 76% of children. Conclusions There exists large variability in pediatric decannulation protocols. Polysomnography plays an integral role in assessing most children for tracheostomy removal. Evidence-based guidelines to standardize pediatric tracheostomy care remain an urgent priority.

OBJECTIVE. This project aims to prospectively and objectively assess otolaryngological manifestations and quality of life of children with primary ciliary dyskinesia (PCD) and compare these findings with healthy pediatric controls. STUDY DESIGN. Cross-sectional. SETTING. Two high-volume pediatric PCD specialty centers. METHODS. Standardized clinical assessment; Sino-Nasal Outcome Test 22 (SNOT-22); Hearing Environment and Reflection Quality of Life (HEAR-QL); Reflux Symptom Index (RSI); standardized physical examination of the sinonasal, laryngeal, and otological systems; and investigations including pure-tone audiograms (PTAs) and sinonasal cultures were collected. RESULTS. Forty-seven children with PCD and 25 control participants were recruited. Children with PCD had more upper airway symptoms than healthy children. They had significantly higher scores in both SNOT-22 and RSI, indicating worse sinonasal and reflux symptoms, with worse quality of life on the HEAR-QL index compared to healthy children (P < .05). Fifty-two percent of children with PCD-related hearing loss were not aware of their hearing deficit that was present on audiological assessment, and only 23% of children who had ventilation tubes had chronic otorrhea, most of which was easily controlled with ototopic drops. Furthermore, although all children with PCD had chronic rhinosinusitis, only 36% of them were using topical nasal treatment. The most common bacteria cultured from the middle meatus were Staphylococcus aureus in 11 of 47 (23%), followed by Streptococcus pneumoniae in 10 of 47 (21%). CONCLUSION. This multisite cohort highlights the importance of otolaryngology involvement in the management of children with PCD. More rigorous otolaryngological management may lead to reductions in overall morbidity and improve quality of life for children with PCD.

Neonates and infants are obligatory nasal breathers. Nasal obstruction can therefore be life-threatening and prompt recognition of its cause is critical. Salivary gland anlage tumor (SGAT) is a rare, benign condition that can present with nasal obstruction and respiratory distress within the first weeks of life. Our objective was to outline the clinical presentation, evaluation, and management of SGAT and provide a review of existing literature.A retrospective chart review was performed at a tertiary children's hospital. A comprehensive literature search for SGAT was conducted using Medline, Embase, PubMed, and EBSCO. We present a case from our institution to describe the clinical approach to SGAT.Forty-one cases of previously reported SGAT, plus our new patient, were included. Patients presented with respiratory distress (30/42, 71.4%), feeding difficulty (14/42, 33.3%) and/or upper airway obstruction (13/42, 31.0%). The majority of tumors were in the nasopharynx (25/42, 59.5%). Surgical excision (36/42, 85.7%) was most commonly performed. Surgical approaches have evolved to include transoral, transpalatal, transnasal endoscopic or a combination of the above. Our patient had a suspected residual/recurrent tumor, later found to be negative on pathology. To date, there have been no reported recurrences.SGAT is a rare pediatric condition that is often excluded from the differential diagnosis of nasal obstruction. Clinical evaluation includes flexible nasopharyngoscopy and neuroimaging. Management involves surgical resection, except in cases of spontaneous expulsion. Tissue pathology is required for definitive diagnosis. Tissue biopsy should be performed on follow-up if there is a suspicion for recurrence.

Button batteries (BBs) are commonly found in many household items and present a risk of severe injury to children if ingested. The direct apposition of the trachea and recurrent laryngeal nerves with the esophagus puts children at risk of airway injury secondary to the liquefactive necrotic effects of BB impactions.To review airway injuries, including long-term sequelae, after BB ingestion in children.For this systematic review, a comprehensive strategy was designed to search MEDLINE, Embase, Cochrane Database of Systematic Reviews, Web of Science, and CINAHL (Cumulative Index of Nursing and Allied Health Literature) from inception to July 31, 2021, in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) reporting guideline. Additional cases were identified from the National Capital Poison Center BB registry. Individual authors were contacted for additional information. Studies with pediatric patients (18 years) who developed airway injuries after BB ingestion were included. A total of 195 patients were included in the analysis; 95 were male. The mean (SD) age at BB ingestion was 17.8 (10.2) months. The mean (SD) time from BB ingestion to removal was 5.8 (9.0) days. The 2 most common airway sequelae observed in our series were 155 tracheoesophageal fistulae and 16 unilateral vocal cord paralyses. Twenty-three children had bilateral vocal cord paralysis. The mean (SD) duration of ingestion leading to vocal cord paralysis was shorter than that of the general cohort (17.8 [22.5] hours vs 138.7 [216.7] hours, respectively). Children presenting with airway symptoms were likely to have a subsequent tracheoesophageal fistula or vocal cord paralysis.Airway injuries are a severe consequence of BB ingestion, occurring more often in younger children. This systematic review found that tracheoesophageal fistulae and vocal cord paralyses were the 2 most common airway injuries, often requiring tracheostomy. Vocal cord injury occurred after a shorter BB exposure time than other airway injuries. Continued efforts should be directed toward prevention strategies to avoid the devastating sequelae of BB-associated airway injury.

Complete and accurate documentation of surgical procedures is essential for optimizing patient care, yet significant variation in operative notes persists within and across institutions. We sought to reach consensus on the most important components of an operative note for pediatric microlaryngoscopy and bronchoscopy.A modified Delphi consensus process was used. A checklist for operative documentation, created by fellowship-trained pediatric otolaryngologists-head and neck surgeons, was sent to surgeons identified as experts in pediatric laryngoscopy and bronchoscopy. In the first round, items were rated as "keep" or "remove". In the second round, each item was rated on a 7-point Likert scale for importance. The mean score of each item was calculated to determine if consensus was reached.Overall, 43/74 (58.1%) surgeons responded to our survey. After two rounds of editing, 28 components reached consensus, 24 were near consensus, and 26 did not reach consensus. Items that reached final consensus had mean (SD) ratings of 6.12 (0.94) (range, 5.31-6.72).Pediatric otolaryngologists identified as bronchoscopy experts were able to create a checklist of essential components of an operative note for pediatric laryngoscopy and bronchoscopy using a Delphi method. Items reaching consensus included procedure name, description of breathing, grade of airway view, description of normal anatomic structures, grade of subglottic stenosis if present, presence and description of tracheobronchomalacia, presence of fistulae, cleft and rings, and several special cases including foreign body and tracheostomy management, as well as end of procedure disposition and complications.5 Laryngoscope, 2022.

Caustic ingestions are rare, life-threatening injuries with life-long consequences. Injury can occur anywhere from the lips to stomach but tends to be most severe in the esophagus. Aspiration of caustic substances can lead to laryngeal or tracheobronchial injury and airway compromise. Caustic injuries come in two main varieties: acidic and alkaline-induced injuries. The type and state of agent determines in part the location of injury and depth of injury. Management consists of comprehensive evaluation and stabilization and must consider airway safety. Endoscopic evaluation and staging of the esophagus should be done within 24 hours of ingestion but not before 6 hours. Nasogastric tube placement should be considered based on the stage of the injury. Antibiotics, acid-suppressants, and corticosteroids may have a roll for certain patients but must be determined on an individual basis. Long-term follow up is required to monitor for strictures and patients with caustic esophageal injury are at increased risk of esophageal carcinoma. This review will help both junior and senior Otolaryngology residents recognize, evaluate and manage causing ingestions in pediatric patients. This review contains figures, tables, and references Keywords: Caustic ingestion, acid, alkali, endoscopic grading, strictures, mitomycin C, balloon dilatation

Create a competency-based assessment tool for pediatric esophagoscopy with foreign body removal.Blinded modified Delphi consensus process.Tertiary care center.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.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.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.5. Laryngoscope, 131:1168-1174, 2021.

Fc receptor–like (FCRL) 4 is an immunoregulatory receptor expressed on a subpopulation of human memory B cells of mucosa-associated lymphoid tissue. Fc receptor function of FCRL4 was demonstrated by binding of IgA to FCRL4 following heat aggregation of the Ig. In this study, we demonstrate that FCRL4 recognizes J chain–linked systemic IgA in the absence of heat aggregation. We further demonstrate that mucosal secretory IgA is not recognized by FCRL4 and that systemic IgA binding can be competitively inhibited by recombinant secretory component protein. Finally, we provide evidence that primary FCRL4-bearing human memory B cells are constitutively bound to IgA. Our study provides a mechanism for the negative regulatory activity of FCRL4 on AgR-mediated B cell activation.

To evaluate risk factors associated with surgical intervention and subperiosteal/orbital abscess in hospitalized children with severe orbital infections.We conducted a multicenter cohort study of children 2 months to 18 years hospitalized with periorbital or orbital cellulitis from 2009 to 2018 at 10 hospitals in Canada. Clinical details were extracted, and patients were categorized as undergoing surgical or medical-only management. Primary outcome was surgical intervention and the main secondary outcome was clinically important imaging. Logistic regression was used to identify predictors.Of 1579 patients entered, median age was 5.4 years, 409 (25.9%) had an orbital/subperiosteal abscess, and 189 (12.0%) underwent surgery. In the adjusted analysis, the risk of surgical intervention was associated with older age (age 9 to14: aOR 3.9, 95% CI 2.3-6.6; and age 14 to ≤18 years: aOR 7.0, 95% CI 3.4-14.1), elevated C-reactive protein120 mg/L (aOR 2.8, 95% CI 1.3-5.9), elevated white blood cell count of 12-20 000/μL (aOR 1.7, 95% CI 1.1-2.6), proptosis (aOR 2.6, 95% CI 1.7-4.0), and subperiosteal/orbital abscess (aOR 5.3, 95% CI 3.6-7.9). There was no association with antibiotic use before hospital admission, sex, presence of a chronic disease, temperature greater than 38.0°C, and eye swollen shut. Complications were identified in 4.7% of patients, including vision loss (0.6%), intracranial extension (1.6%), and meningitis (0.8%).In children hospitalized with severe orbital infections, older age, elevated C-reactive protein, elevated white blood cell count, proptosis, and subperiosteal/orbital abscess were predictors of surgical intervention.

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.

Objective: The objectives of this study are to present a case series of immunodeficient children who underwent a transcervical thymic biopsy and to describe the transcervical approach to the thymus gland. Design: Case series. Setting: Pediatric otolaryngology practice in an academic setting. Patients: Consecutive sample of immunodeficient children (≤18 years old) who underwent thymic biopsies from 1996 to 2019 for the purpose of confirming or excluding profound T cell immunodeficiency. Intervention: Diagnostic transcervical thymic biopsy. Results: A total of 14 patients with atypical combined immunodeficiency underwent the procedure during the study period, with minimal post-operative complication. The thymus was found to be abnormal histologically in 9 children and normal in another 5 patients. In all cases, thymus morphology helped define the extent of the immunodeficiency, resulting in either supporting a decision to perform a bone marrow transplant (8 patients) or avoid this high risk procedure (3 patients). Conclusion: Thymus biopsy is helpful in the characterization of childhood immunodeficiency and provides critical information that affects the medical management. The transcervical approach to the thymus is feasible in children and can be accomplished with minimal morbidity. Statement of novelty: Biopsies of the thymus have assisted in the characterization of new entities of primary immunodeficiency.

OBJECTIVESTo examine the association between systemic corticosteroid use and outcomes for children hospitalized with orbital cellulitis at US children’s hospitals.METHODSWe conducted a multicenter observational study using administrative data from the Pediatric Health Information System database from 2007 to 2019. Children between the ages of 2 months and 18 years with International Classification of Diseases, Ninth Revision, Clinical Modification or 10th Revision, Clinical Modification discharge diagnostic codes of orbital cellulitis were included. The primary exposure was receipt of systemic corticosteroids on the day of hospital admission. The primary outcome was hospital length of stay, and secondary outcomes included surgical intervention, ICU admissions, revisits, and health care costs. We used generalized logit model with inverse probability weighting logistic regression to adjust for demographic factors and assess for differences in clinical outcomes reported.RESULTSOf the 5832 patients hospitalized with orbital cellulitis, 330 (5.7%) were in the corticosteroid group and 5502 (94.3%) were in the noncorticosteroid group. Patients in the corticosteroid group were older, had more severe illness, and received broad spectrum antibiotics. In adjusted analyses, corticosteroid exposure was not associated with differences in length of hospital stay, need for surgical intervention, ICU admissions, emergency department revisits, 30-day hospital readmissions, or hospital costs. Restricting the analysis to only those patients who received broad spectrum antibiotics did not change the findings.CONCLUSIONSEarly use of systemic corticosteroids in hospitalized children with orbital cellulitis is not associated with improved clinical outcomes. Use of corticosteroids in hospitalized children with orbital cellulitis should be discouraged outside of clinical trials.

Primary Subject area Respirology Background Despite the large morbidity and potential mortality associated with tracheostomy tube decannulation failure, there are currently no consensus guidelines on pediatric tracheostomy decannulation. This has led to wide practice variation that is largely based on expert option. This is the largest review of pediatric decannulation protocols. Objectives To systematically review the literature on existing pediatric decannulation protocols, including the role of polysomnography, and their clinical outcomes. Design/Methods Five online databases were searched for relevant studies from database inception to May 29, 2020. Study inclusion was limited to publications that evaluated tracheostomy decannulation in children 18 years of age and younger. Independent reviewers extracted data, including patient demographics and primary indication for tracheostomy. Methods used to assess readiness for decannulation were noted, including the use of bronchoscopy, tracheostomy tube modifications, and gas exchange measurements. After decannulation, details regarding mode of ventilation, location and length of observation period, and clinical outcomes were also collected. Quality assessment of included studies was performed using the Newcastle-Ottawa Scale (NOS) tool. Descriptive statistical analyses were performed. Results Twenty-three studies with 1328 children were included (Figure 1). Tracheostomy indications included upper airway obstruction at a well-defined anatomic site (37%), upper airway obstruction not at a well-defined site (13%), and need for long-term ventilation (50%). Bronchoscopy was routinely used in 96% of protocols. Tracheostomy tube modifications in the protocols included capping (83%), downsizing (57%), and fenestrations (9%). Measurements of gas exchange in the protocols included polysomnography (72%), oximetry (61%), blood gases (17%), and capnography with end-tidal CO2 (17%). After tracheostomy decannulation, children in 92% of protocols were transitioned to room air, and 38% of protocols used non-invasive ventilation. Most children (76%) were observed in hospital for 48 hours or less. Of all decannulation attempts, 79% were successful. Overall risk of bias in included studies was low. Conclusion The absence of clear evidence-based guidelines in pediatric tracheostomy decannulation has led to large variability in clinical practice. Most protocols include bronchoscopy, tube modifications, gas exchange measurements, and brief hospital admission. Polysomnography plays an integral role in assessing the majority of children for tracheostomy removal. Evidence-based guidelines to standardize pediatric tracheostomy care remain an urgent priority.

Mammalian target of rapamycin (mTOR) inhibitors are clinically effective at treating some complex lymphatic malformations (LM). The mTOR inhibitor rapamycin blocks the phosphoinositide 3-kinase (PI3K) pathway, which is commonly mutated in this condition. Although rapamycin is effective at controlling symptoms of LM, treatment courses are long, not all LMs respond to treatment, and many patients relapse after treatment has stopped. Concurrent rat sarcoma virus (RAS) pathway abnormalities have been identified in LM, which may limit the effectiveness of rapamycin. Protein tyrosine phosphatase-2 (SHP2) controls the RAS pathway upstream, and SHP2 inhibitors are being investigated for treatment of various tumors. The objective of this study was to determine the impact of SHP2 inhibition in combination with rapamycin on LM growth in vitro. Using primary patient cells isolated from a surgically resected LM, we found that combination treatment with rapamycin and the SHP2 inhibitor SHP099 caused a synergistic reduction in cell growth, migration and lymphangiogenesis. These results suggest that combination treatment targeting the PI3K and RAS signaling pathways may result in effective treatment of LMs of the head and neck.

OBJECTIVE To prospectively evaluate 1) use of endotracheal tube (ETT) surface electrodes for recurrent laryngeal nerve (RLN) monitoring in thyroid surgery in children, and 2) effects of thyroid surgery on the RLN in children. METHODS Patients

ObjectiveUndiagnosed and untreated obstructive sleep apnea (OSA) can predispose children to neurobehavioural consequences. However, there is a lack of data identifying rate of, and risk factors for, OSA in very young healthy children. The objective of this study was to determine the rate of OSA and identify risk factors associated with the presence and severity of OSA in children aged 3 years and younger.MethodsThis was a retrospective chart review of healthy children between 1 and 3 years old who had a baseline polysomnogram (PSG) between January 2012 and June 2017. Patient demographics, referral history, and PSG data were recorded.ResultsOne hundred and thirteen children were referred for a PSG, of which 66 (58%) were diagnosed with OSA and 47 (42%) did not have OSA. In the OSA group, 13 (20%) were mild and 53 (80%) were moderate-severe. Nasal congestion (P=0.001), adenoid hypertrophy (P=ConclusionA high rate of OSA was found among children aged 3 years and younger. Children referred by an otolaryngologist are more likely to be diagnosed with moderate-severe OSA. Children aged 3 years and younger with symptoms of OSA should be considered high-risk for OSA and be prioritized for early PSG and management.

Introduction Mucoepidermoid carcinoma is a rare malignant salivary gland neoplasm in the pediatric population. Few studies have discussed best practice with respect to diagnosis and treatment in children. Objective To present our institution's experience with the evaluation and management of pediatric mucoepidermoid carcinoma of the head and neck. Methods Retrospective chart review of patients under 20 years of age diagnosed with mucoepidermoid carcinoma of the head and neck between 1998 and 2017. Data assessed includes demographics, clinical presentation, imaging examinations, histopathology, treatment, complications, local recurrence, distant metastasis, and follow-up. Results Sixteen patients (10 female, 6 male) were identified with a median age of 12.9 (IQR: 10.9–15.0) years. Tumors were located within the parotid gland (n = 11, 68.8%), accessory lobe of the parotid gland (n = 2, 12.5%), palate (n = 2, 12.5%), and submandibular region (n = 1, 6.3%). In 9 patients (56.3%) a neoplastic etiology was suspected based on the clinical and/or radiographic findings and confirmed pathologically on biopsy or excision. All patients were treated surgically and five patients required adjuvant radiotherapy. One patient had recurrence requiring re-excision. Seven patients (43.8%) had transient facial paresis post-operatively and one had Frey syndrome. Median follow-up time was 59.7 months (IQR: 18.9–99.3). Conclusion The malignant nature of mucoepidermoid carcinoma requires comprehensive, multidisciplinary management. Imaging and tissue sampling by fine needle aspiration give clinicians the best insight into location and nature of the mass. Complete surgical excision with attention to preservation of facial nerve and achieving negative margins is desired.

Surgical intervention for obstructive sleep apnea (OSA) in overweight and obese children may not be as effective as it is in normal-weight children. The purpose of this study was to systematically review the effects of various surgical interventions for OSA in obese children and to meta-analyze the current data.PubMed, OVID, and Cochrane databases.Databases were searched for studies examining adenotonsillectomy, uvulopalatopharyngoplasty, supraglottoplasty, or tongue base surgeries and combinations in obese children with OSA. Adenotonsillectomy was the only procedure with enough data for meta-analysis; polysomnographic data were extracted and analyzed using a random-effects model.For adenotonsillectomy, 11 studies were included in the meta-analysis. Despite significant improvement in the apnea-hypopnea index (22.9 to 8.1 events/h, P.001), respiratory disturbance index (24.8 to 10.4 events/h, P.001), and oxygen saturation nadir (78.4% to 87.0%, P.001), rates of persistent OSA ranged from 51% to 66%, depending on the outcome criterion used. There was evidence of limited effectiveness for surgical interventions to treat OSA in obese children using uvulopalatoplasty (12.5%) and tongue base surgery (74%-88%).Surgical interventions for OSA in overweight and obese children are effective at reducing OSA but with higher rates of persistent OSA than reported for normal-weight children. However, the amount of reduction appears to vary by surgical procedure. More attention should be paid toward preoperative weight loss and patient selection, and parents should be provided with realistic postoperative expectations in this difficult-to-treat population.

Objective Tracheo-innominate fistula (TIF) is a rare but fatal complication of tracheotomy. To date, there is a paucity of literature regarding pediatric TIFs. The objectives of this study were to conduct a systematic review of literature on pediatric TIF following tracheotomy and describe three demonstrative cases from our institutional experience. Methods We conducted a systematic review using MEDLINE, Embase, Cochrane Database of Systematic Reviews, Web of Science, and CINAHL. All studies with pediatric patients (under 18 years of age) who developed TIF following tracheotomy were included. Results Fifty-four publications met inclusion criteria, reporting on 77 cases. The most common indication for tracheotomy was prolonged intubation and the need for ventilatory support (38.6%), with neurological comorbidities being the most common indication (72.7%). The mean time to TIF was 395.7 days (95% confidence interval, 225.9-565.5). Fifty-four patients (70.1%) presented with massive hemorrhage, whereas 18 patients (23.3%) presented with a sentinel bleeding event. The most common diagnostic interventions were computed tomography scan with or without contrast and bronchoscopy (55.8%). A substantial number of patients did not have any investigations (41.6%). Surgical management occurred in 70.1% of patients. Mortality was 38.6% in reported cases with variable follow-up periods. Conclusion TIF may occur in long-term tracheostomy-dependent children, contrary to the conventionally described 3-week postoperative period. The mortality may not be as high as previously reported with timely intervention. Our results are limited by inherent risks of bias. Further research including well-designed cohort studies are needed to guide an evidence-based approach to TIF. Level of evidence NA Laryngoscope, 130:217-224, 2020.

OBJECTIVE Define the length of the subglottis and trachea in children to predict a safe intubation depth. METHODS Patients

Objectives/hypothesis Assess the risks and benefits of adenotonsillectomy (AT) for obstructive sleep apnea (OSA) in children with cerebral palsy (CP). Study design Systematic review. Methods We conducted a systematic review of Medline, Embase, and Cochrane Central Registry from 1946 to 2021. Broad search concepts included cerebral palsy, pediatric, tonsillectomy/adenoidectomy, and sleep. Additional articles were identified by searching reference lists. Studies on the safety and efficacy of AT for OSA management in children with CP were included. Results Fifteen articles met inclusion criteria. Articles were classified into one or more of four themes: intraoperative risk (n = 1), postoperative risk (n = 3), postoperative care requirements (n = 6), and surgical outcomes (n = 7). No intraoperative anesthetic complications were reported. Postoperatively, respiratory complications including pneumonia were common and necessitated additional airway management. Following AT, children with CP required close postoperative observation, experienced increased lengths of stay, and had increased odds of unplanned intensive care unit (ICU) admission. Benefits following AT were improvement in OSA as measured by a reduction in obstructive apnea-hypopnea index (OAHI) as well as improved quality of life in some; however, many patients went on to require tracheostomy due to persistent OSA. Conclusions Children with CP who undergo AT have a significant risk of developing a postoperative respiratory complication. Realistic counseling of families around increased perioperative risks in this population is imperative and close postoperative monitoring is critical. Many children will obtain a reduction in OAHI, but additional surgical management is often required, including tracheostomy. Further research is needed to determine the best management strategy for OSA in children with CP. Laryngoscope, 2021.

Introduction Ultrasonic bone removal devices (UBD) are capable of cutting through bony tissue without injury to adjacent soft tissue. The feasibility and safety of using this technology for removal of bone from an intact ossicular chain (as might be required for otosclerosis or congenital fixation) was investigated in an animal model. Methods This was a prospective animal study conducted on seven anesthetised adult chinchillas. An UBD was used to remove bone from the malleus head in situ. Pre and post-operative distortion product otoacoustic emission (DPOAE) levels and auditory brainstem response (ABR) thresholds were recorded. Scanning electron microscopy (SEM) was used to assess cochlear haircell integrity. Results Precise removal of a small quantity of bone from the malleus head was achieved by a 30s application of UBD without disruption of the ossicular chain or tympanic membrane. DPOAEs became undetectable after the intervention with signal-to-noise ratios (SNR) 85 dB SPL in 13 ears. SEM showed significant disruption of structural integrity of the organ of Corti, specifically loss and damage of outer haircells. Conclusions Although UBD can be used to reshape an ossicle without middle ear injury, prolonged contact with the ossicular chain can cause structural and functional injury to the cochlea. Extensive cochlea pathology was found, but we did not investigate for recovery from any temporary threshold shift. In the authors’ opinion, further study should be undertaken before consideration is given to use of the device for release of ossicular fixation. Graphical abstract

OBJECTIVE(S) To create and validate a synthetic simulator for teaching tracheostomy and laryngotracheal reconstruction (LTR) using anterior costal cartilage and thyroid ala cartilage grafts. METHODS A late adolescent/adult neck and airway simulator was constructed based on CT scans from a cadaver and a live patient. Images were segmented to create three-dimensional printed molds from which anatomical parts were casted. To evaluate the simulator, expert otolaryngologists - head and neck surgeons performed tracheostomy and LTR using anterior costal cartilage and thyroid ala cartilage grafts on a live anesthetized porcine model (gold standard) followed by the synthetic simulator. They evaluated each model for face validity (realism and anatomical accuracy) and content validity (perceived effectiveness as a training tool) using a five-point Likert scale. For each expert, differences for each item on each simulator were compared using Wilcoxon Signed-Rank tests with Sidak correction. RESULTS Nine expert faculty surgeons completed the study. Experts rated face and content validity of the synthetic simulator an overall median of 4 and 5, respectively. There was no difference in scores between the synthetic model and the live porcine model for any of the steps of any of the surgical procedures. CONCLUSION The synthetic simulator created for this study has high face and content validity for tracheostomy and LTR with anterior costal cartilage and thyroid ala cartilage grafts and was not found to be different than the live porcine model for these procedures. LEVEL OF EVIDENCE 5 Laryngoscope, 131:E2378-E2386, 2021.

Purpose The beta-blocker propranolol is the standard medical therapy for subglottic hemangioma (SGH), but side effects and incomplete response rates require close monitoring. Nadolol has been identified as a potential alternative but its use has not been examined for SGH. Methods Single institution retrospective cohort study of pediatric SGH treated with propranolol or nadolol. Results Thirteen children (1 male, 12 female) with SGH were included: 6 were treated with propranolol (2.0–3.5 mg/kg/d) and 7 with nadolol (2.0–4.0 mg/kg/d). The most common presenting symptom was stridor (85%) and mean (SD) symptom duration prior to diagnosis was 4.6 (3.8) weeks. Cutaneous vascular lesions were present in 54%. There were 7 right-sided, 5 left-sided and 1 bilateral SGH. The mean (SD) percentage of airway obstruction was 60.6% (27.4). The response rate was 100% (6/6) for propranolol and 85.7% (6/7) for nadolol (p = 0.36). Mean (SD) time to symptomatic improvement was 2.6 (2.2) days with no difference across groups (p = 0.71). There was no hypotension, hypoglycemia, weight loss, or sleep disturbances in either group. One patient in the propranolol group experienced vomiting. Two patients in the nadolol group required dosage reduction due to asymptomatic bradycardia. The mean (SD) duration of admission was 14.4 (12.6) days and duration of treatment was 13.8 (11.2) days with no difference across groups (p = 0.23; p = 0.31, respectively). All patients had treatment initiated as inpatients and completed as outpatients. Conclusion Children with SGH treated with propranolol or nadolol had similar response rates and side effect profiles.

OBJECTIVE Evaluate patterns and predictors of spread to the neck in pediatric metastatic differentiated thyroid carcinoma (DTC). METHODS Patients

A one-day-old girl was brought to the OR for the repair of a type C esophageal atresia (EA) [EA with tracheoesophageal fistula (TEF)]. Rigid bronchoscopy was performed to locate the fistula, and it revealed a severe long-segment tracheal stenosis. Therefore, the airway could not have been secured past the fistula using normal-sized endotracheal tubes (ETTs). A nontraditional airway using an aortic cannula was used to intubate the stenotic tracheal segment, and the patient received ventilation during the TEF/EA repair.

Aerosolization procedures during the COVID-19 pandemic place all operating room personnel at risk for exposure. We offer detailed perioperative management strategies and present a specific protocol designed to improve safety during pediatric laryngoscopy and bronchoscopy. Several methods of using disposable drapes for various procedures are described, with the goal of constructing a tent around the patient to decrease widespread contamination of dispersed droplets and generated aerosol. The concepts presented herein are translatable to future situations where aerosol generating procedures increase risk for any pathogenic exposure. This protocol is a collaborative effort based on knowledge gleaned from clinical and simulation experience from Children's Hospital Colorado, Children's Hospital of Philadelphia, The Hospital for Sick Children in Toronto, and Boston Children's Hospital.