Multi-institutional Assessment of Otitis Media Epidemiology Using Real-world Data.

OBJECTIVES: To determine rates and risk factors of pediatric otitis media (OM) using real-world electronic health record (PEDSnet) data from January 2009 through May 2021. STUDY DESIGN: Retrospective cohort study. SETTING: Seven pediatric academic health systems that participate in PEDSnet. METHODS: Children <6 months-old at time of first outpatient, Emergency Department, or inpatient visit were included and followed longitudinally. A time-to-event analysis was performed using a Cox proportional hazards model to estimate hazard ratios for OM incidence based on sociodemographic factors and specific health conditions. RESULTS: The PEDSnet cohort included 910,265 children, 54.3% male, mean age (months) 1.3 [standard deviation (SD) 1.6] and mean follow up (years) 4.3 (SD 3.2). By age 3 years, 39.6% of children had evidence of one OM episode. OM rates decreased following pneumococcal-13 vaccination (PCV-13) and the COVID-19 pandemic. Along with young age, non-Hispanic Black/African American or Hispanic race/ethnicity, public insurance, higher family income, and male sex had higher incidence rates. Health conditions that increased OM risk included cleft palate [adjusted hazard ratio (aHR) 4.0 [95% confidence interval (CI) 3.9-4.2], primary ciliary dyskinesia [aHR 2.5 (95% CI 1.8-3.5)], trisomy 21 [aHR 2.0 (95% CI 1.9-2.1)], atopic dermatitis [aHR 1.4 (95% CI 1.4-1.4)], and gastroesophageal reflux [aHR1.5 (95% CI 1.5-1.5)]. CONCLUSIONS: Approximately 20% of children by age 1 and 40% of children by age 3 years will have experienced an OM episode. OM rates decreased after PCV-13 and COVID-19. Children with abnormal ciliary function or craniofacial conditions, specifically cleft palate, carry the highest risk of OM.

Recurrent missense variant identified in two unrelated families with MPZL2-related hearing loss, expanding the variant spectrum associated with DFNB111.

MPZL2-related hearing loss is a rare form of autosomal recessive hearing loss characterized by progressive, mild sloping to severe sensorineural hearing loss. Thirty-five previously reported patients had biallelic truncating variants in MPZL2, with the exception of one patient with a missense variant of uncertain significance and a truncating variant. Here, we describe the clinical characteristics and genotypes of five patients from four families with confirmed MPZL2-related hearing loss. A rare missense likely pathogenic variant [NM_005797.4(MPZL2):c.280C>T,p.(Arg94Trp)] located in exon 3 was confirmed to be in trans with a recurrent pathogenic truncating variant that segregated with hearing loss in three of the patients from two unrelated families. This is the first recurrent likely pathogenic missense variant identified in MPZL2. Apparently milder or later-onset hearing loss associated with rare missense variants in MPZL2 indicates that some missense variants in this gene may cause a milder phenotype than that resulting from homozygous or compound heterozygous truncating variants. This study, along with the identification of truncating loss of function and missense MPZL2 variants in several diverse populations, suggests that MPZL2-related hearing loss may be more common than previously appreciated and demonstrates the need for MPZL2 inclusion in hearing loss testing panels.

Clinical Characteristics of Primary Snoring vs Mild Obstructive Sleep Apnea in Children: Analysis of the Pediatric Adenotonsillectomy for Snoring (PATS) Randomized Clinical Trial.

Importance: It is unknown whether children with primary snoring and children with mild obstructive sleep apnea (OSA) represent populations with substantially different clinical characteristics. Nonetheless, an obstructive apnea-hypopnea index (AHI) of 1 or greater is often used to define OSA and plan for adenotonsillectomy (AT). Objective: To assess whether a combination of clinical characteristics differentiates children with primary snoring from children with mild OSA. Design, Setting, and Participants: Baseline data from the Pediatric Adenotonsillectomy Trial for Snoring (PATS) study, a multicenter, single-blind, randomized clinical trial conducted at 6 academic sleep centers from June 2016 to January 2021, were analyzed. Children aged 3.0 to 12.9 years with polysomnography-diagnosed (AHI <3) mild obstructive sleep-disordered breathing who were considered candidates for AT were included. Data analysis was performed from July 2022 to October 2023. Main Outcomes and Measures: Logistic regression models were fitted to identify which demographic, clinical, and caregiver reports distinguished children with primary snoring (AHI <1; 311 patients [67.8%]) from children with mild OSA (AHI 1-3; 148 patients [32.2%]). Results: A total of 459 children were included. The median (IQR) age was 6.0 (4.0-7.5) years, 230 (50.1%) were female, and 88 (19.2%) had obesity. A total of 121 (26.4%) were Black, 75 (16.4%) were Hispanic, 236 (51.5%) were White, and 26 (5.7%) were other race and ethnicity. Black race (odds ratio [OR], 2.08; 95% CI, 1.32-3.30), obesity (OR, 1.80; 95% CI, 1.12-2.91), and high urinary cotinine levels (>5 µg/L) (OR, 1.88; 95% CI, 1.15-3.06) were associated with greater odds of mild OSA rather than primary snoring. Other demographic characteristics, clinical examination findings, and questionnaire reports did not distinguish between primary snoring and mild OSA. A weighted combination of the statistically significant clinical predictors had limited ability to differentiate children with mild OSA from children with primary snoring. Conclusions and Relevance: In this analysis of baseline data from the PATS randomized clinical trial, primary snoring and mild OSA were difficult to distinguish without polysomnography. Mild OSA vs snoring alone did not identify a clinical group of children who may stand to benefit from AT for obstructive sleep-disordered breathing. Trial Registration: ClinicalTrials.gov Identifier: NCT02562040.

Adenotonsillectomy for Snoring and Mild Sleep Apnea in Children: A Randomized Clinical Trial.

Importance: The utility of adenotonsillectomy in children who have habitual snoring without frequent obstructive breathing events (mild sleep-disordered breathing [SDB]) is unknown. Objectives: To evaluate early adenotonsillectomy compared with watchful waiting and supportive care (watchful waiting) on neurodevelopmental, behavioral, health, and polysomnographic outcomes in children with mild SDB. Design, Setting, and Participants: Randomized clinical trial enrolling 459 children aged 3 to 12.9 years with snoring and an obstructive apnea-hypopnea index (AHI) less than 3 enrolled at 7 US academic sleep centers from June 29, 2016, to February 1, 2021, and followed up for 12 months. Intervention: Participants were randomized 1:1 to either early adenotonsillectomy (n = 231) or watchful waiting (n = 228). Main Outcomes and Measures: The 2 primary outcomes were changes from baseline to 12 months for caregiver-reported Behavior Rating Inventory of Executive Function (BRIEF) Global Executive Composite (GEC) T score, a measure of executive function; and a computerized test of attention, the Go/No-go (GNG) test d-prime signal detection score, reflecting the probability of response to target vs nontarget stimuli. Twenty-two secondary outcomes included 12-month changes in neurodevelopmental, behavioral, quality of life, sleep, and health outcomes. Results: Of the 458 participants in the analyzed sample (231 adenotonsillectomy and 237 watchful waiting; mean age, 6.1 years; 230 female [50%]; 123 Black/African American [26.9%]; 75 Hispanic [16.3%]; median AHI, 0.5 [IQR, 0.2-1.1]), 394 children (86%) completed 12-month follow-up visits. There were no statistically significant differences in change from baseline between the 2 groups in executive function (BRIEF GEC T-scores: -3.1 for adenotonsillectomy vs -1.9 for watchful waiting; difference, -0.96 [95% CI, -2.66 to 0.74]) or attention (GNG d-prime scores: 0.2 for adenotonsillectomy vs 0.1 for watchful waiting; difference, 0.05 [95% CI, -0.18 to 0.27]) at 12 months. Behavioral problems, sleepiness, symptoms, and quality of life each improved more with adenotonsillectomy than with watchful waiting. Adenotonsillectomy was associated with a greater 12-month decline in systolic and diastolic blood pressure percentile levels (difference in changes, -9.02 [97% CI, -15.49 to -2.54] and -6.52 [97% CI, -11.59 to -1.45], respectively) and less progression of the AHI to greater than 3 events/h (1.3% of children in the adenotonsillectomy group compared with 13.2% in the watchful waiting group; difference, -11.2% [97% CI, -17.5% to -4.9%]). Six children (2.7%) experienced a serious adverse event associated with adenotonsillectomy. Conclusions: In children with mild SDB, adenotonsillectomy, compared with watchful waiting, did not significantly improve executive function or attention at 12 months. However, children with adenotonsillectomy had improved secondary outcomes, including behavior, symptoms, and quality of life and decreased blood pressure, at 12-month follow-up. Trial Registration: ClinicalTrials.gov Identifier: NCT02562040.

Multiple Independent Gene Disorders Causing Bardet-Biedl Syndrome, Congenital Hypothyroidism, and Hearing Loss in a Single Indian Patient.

We report a 20-year-old, female, adopted Indian patient with over 662 Mb regions of homozy-gosity who presented with intellectual disability, ataxia, schizophrenia, retinal dystrophy, moder-ate-to-severe progressive sensorineural hearing loss (SNHL), congenital hypothyroidism, cleft mi-tral valve with mild mitral valve regurgitation, and dysmorphic features. Exome analysis first on a clinical basis and subsequently on research reanalysis uncovered pathogenic variants in three nu-clear genes following two modes of inheritance that were causal to her complex phenotype. These included (1) compound heterozygous variants in BBS6 potentially causative for Bardet-Biedl syn-drome 6; (2) a homozygous, known pathogenic variant in the stereocilin (STRC) gene associated with nonsyndromic deafness; and (3) a homozygous variant in dual oxidase 2 (DUOX2) gene asso-ciated with congenital hypothyroidism. A variant of uncertain significance was identified in a fourth gene, troponin T2 (TNNT2), associated with cardiomyopathy but not the cleft mitral valve, with mild mitral regurgitation seen in this case. This patient was the product of an apparent first-degree relationship, explaining the multiple independent inherited findings. This case high-lights the need to carefully evaluate multiple independent genetic etiologies for complex pheno-types, particularly in the case of consanguinity, rather than presuming unexplained features are expansions of known gene disorders.

Vinblastine/Methotrexate for Debilitating and Progressive Plexiform Neurofibroma in Children and Young Adults with Neurofibromatosis Type 1: A Phase 2 Study.

Limited therapies exist for neurofibromatosis type 1 (NF1)-associated plexiform neurofibroma (PN). For this reason, the activity of vinblastine (VBL) and methotrexate (MTX) was evaluated in children and young adults with NF1 and PN. Patients ≤ 25 years of age with progressive and/or inoperable NF1-PN received VBL 6 mg/m2 and MTX 30 mg/m2 weekly for 26 weeks, followed by every 2 weeks for 26 weeks. Objective response rate was the primary endpoint. Of 25 participants enrolled, 23 were evaluable. The median age of participants was 6.6 years (range 0.3-20.7). The most frequent toxicities were neutropenia and elevation of transaminases. On two-dimensional (2D) imaging, 20 participants (87%) had stable tumor, with a median time to progression of 41.5 months (95% confidence interval 16.9, 64.9). Two of eight participants (25%) with airway involvement demonstrated functional improvements including decreased positive pressure requirements and apnea-hypopnea index. A post hoc three-dimensional (3D) analysis of PN volumes was completed on 15 participants with amenable imaging; 7 participants (46%) had progressive disease on or by the end of therapy. VBL/MTX was well-tolerated but did not result in objective volumetric response. Furthermore, 3D volumetric analysis highlighted the lack of sensitivity of 2D imaging for PN response evaluation.

Associations of actigraphy measures of sleep duration and continuity with executive function, vigilance, and fine motor control in children with snoring and mild sleep-disordered breathing.

STUDY OBJECTIVES: Children with snoring and mild sleep-disordered breathing may be at increased risk for neurocognitive deficits despite few obstructive events. We hypothesized that actigraphy-based sleep duration and continuity associate with neurobehavioral functioning and explored whether these associations vary by demographic and socioeconomic factors. METHODS: 298 children enrolled in the Pediatric Adenotonsillectomy Trial, ages 3 to 12.9 years, 47.3% from racial or ethnic minority groups, with habitual snoring and an apnea-hypopnea index < 3 were studied with actigraphy (mean 7.5 ± 1.4 days) and completed a computerized vigilance task (Go-No-Go) and a test of fine motor control (9-Hole Pegboard). Caregivers completed the Behavior Rating Inventory of Executive Function. Regression analyses evaluated associations between sleep exposures (24-hour and nocturnal sleep duration, sleep fragmentation index, sleep efficiency) with the Behavior Rating Inventory of Executive Function Global Executive Composite index, pegboard completion time (fine motor control), and vigilance (d prime on the Go-No-Go), adjusting for demographic factors and study design measures. RESULTS: Longer sleep duration, higher sleep efficiency, and lower sleep fragmentation were associated with better executive function; each additional hour of sleep over 24 hours associated with more than a 3-point improvement in executive function (P = .002). Longer nocturnal sleep (P = .02) and less sleep fragmentation (P = .001) were associated with better fine motor control. Stronger associations were observed for boys and children less than 6 years old. CONCLUSIONS: Sleep quantity and continuity are associated with neurocognitive functioning in children with mild sleep-disordered breathing, supporting efforts to target these sleep health parameters as part of interventions for reducing neurobehavioral morbidity. CLINICAL TRIAL REGISTRATION: Registry: ClinicalTrials.gov; Name: Pediatric Adenotonsillectomy for Snoring (PATS); URL: https://clinicaltrials.gov/ct2/show/NCT02562040; Identifier: NCT02562040. CITATION: Robinson KA, Wei Z, Radcliffe J, et al. Associations of actigraphy measures of sleep duration and continuity with executive function, vigilance, and fine motor control in children with snoring and mild sleep-disordered breathing. J Clin Sleep Med. 2023;19(9):1595-1603.

Comparison of Caregiver- and Child-Reported Quality of Life in Children With Sleep-Disordered Breathing.

OBJECTIVE: Caregivers frequently report poor quality of life (QOL) in children with sleep-disordered breathing (SDB). Our objective is to assess the correlation between caregiver- and child-reported QOL in children with mild SDB and identify factors associated with differences between caregiver and child report. STUDY DESIGN: Analysis of baseline data from a multi-institutional randomized trial SETTING: Pediatric Adenotonsillectomy Trial for Snoring, where children with mild SDB (obstructive apnea-hypopnea index <3) were randomized to observation or adenotonsillectomy. METHODS: The Pediatric Quality of Life Inventory (PedsQL) assessed baseline global QOL in participating children 5 to 12 years old and their caregivers. Caregiver and child scores were compared. Multivariable regression assessed whether clinical factors were associated with differences between caregiver and child report. RESULTS: PedsQL scores were available for 309 families (mean child age, 7.0 years). The mean caregiver-reported PedsQL score was higher at 75.2 (indicating better QOL) than the mean child-reported score of 67.9 (P < .001). The agreement between caregiver and child total PedsQL scores was poor, with intraclass correlation coefficients of 0.03 (95% CI, -0.09 to 0.15) for children 5 to 7 years old and 0.21 (95% CI, 0.03-0.38) for children 8 to 12 years old. Higher child age and health literacy were associated with closer agreement between caregiver and child report. CONCLUSION: Caregiver- and child-reported global QOL in children with SDB was weakly correlated, more so for young children. In pediatric SDB, child-perceived QOL may be poorer than that reported by caregivers. Further research is needed to assess whether similar trends exist for disease-specific QOL metrics.

A Trial of Intranasal Corticosteroids to Treat Childhood OSA Syndrome.

BACKGROUND: Intranasal corticosteroids (INCS) are frequently used to treat OSA syndrome (OSAS) in children. However, their efficacy has not been rigorously tested. RESEARCH QUESTION: Do INCS result in improved OSAS symptoms, polysomnography findings, behavior, and quality of life compared with placebo? STUDY DESIGN AND METHODS: In this randomized, double-blind, placebo-controlled trial, children with OSAS aged 5 to 12 years (N = 134) were randomized 2:1 to receive 3 months of INCS or placebo. Children in the INCS arm were then re-randomized to receive 9 months of INCS or placebo. Polysomnography, symptoms, and neurobehavioral findings were measured at baseline, 3 months, and 12 months. The primary outcome was change in obstructive apnea hypopnea index (OAHI) at 3 months, available for 122 children. The secondary outcome was OAHI change at 12 months, available for 70 children. RESULTS: Median (interquartile range) age and OAHI at baseline for the entire group were 7.9 (6.3 to 9.9) years and 5.8 (3.6 to 9.7) events per hour. OAHI changes at 3 months (-1.72 [-3.91 to 1.92] events per hour) and 12 months (-1.2 [-4.22 to 1.71] events per hour) were not different between the two groups (P = not significant). OSAS symptoms and neurobehavioral results did not differ between the INCS and placebo groups at 3 and 12 months. The 38 children who received INCS for 12 months reported a significant OAHI decrease from 7.2 (3.62 to 9.88) events per hour to 3.7 (1.56 to 6.4) events per hour (P = .039). INTERPRETATION: In children with OSAS, treatment with INCS did not result in significant polysomnography, neurobehavioral, or symptom changes at 3 and 12 months of treatment. Twelve months of INCS treatment resulted in a statistically significant but not clinically relevant OAHI reduction. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov; No.: NCT02180672; URL: www. CLINICALTRIALS: gov.

Neurobehavioral morbidity of pediatric mild sleep-disordered breathing and obstructive sleep apnea.

STUDY OBJECTIVES: Obstructive sleep apnea is associated with neurobehavioral dysfunction, but the relationship between disease severity as measured by the apnea-hypopnea index and neurobehavioral morbidity is unclear. The objective of our study is to compare the neurobehavioral morbidity of mild sleep-disordered breathing versus obstructive sleep apnea. METHODS: Children 3-12 years old recruited for mild sleep-disordered breathing (snoring with obstructive apnea-hypopnea index < 3) into the Pediatric Adenotonsillectomy Trial for Snoring were compared to children 5-9 years old recruited for obstructive sleep apnea (obstructive apnea-hypopnea 2-30) into the Childhood Adenotonsillectomy Trial. Baseline demographic, polysomnographic, and neurobehavioral outcomes were compared using univariable and multivariable analysis. RESULTS: The sample included 453 participants with obstructive sleep apnea (median obstructive apnea-hypopnea index 5.7) and 459 participants with mild sleep-disordered breathing (median obstructive apnea-hypopnea index 0.5). By polysomnography, participants with obstructive sleep apnea had poorer sleep efficiency and more arousals. Children with mild sleep-disordered breathing had more abnormal executive function scores (adjusted odds ratio 1.96, 95% CI 1.30-2.94) compared to children with obstructive sleep apnea. There were also elevated Conners scores for inattention (adjusted odds ratio 3.16, CI 1.98-5.02) and hyperactivity (adjusted odds ratio 2.82, CI 1.83-4.34) in children recruited for mild sleep-disordered breathing. CONCLUSIONS: Abnormal executive function, inattention, and hyperactivity were more common in symptomatic children recruited into a trial for mild sleep-disordered breathing compared to children recruited into a trial for obstructive sleep apnea. Young, snoring children with only minimally elevated apnea-hypopnea levels may still be at risk for deficits in executive function and attention. TRIAL REGISTRATION: Pediatric Adenotonsillectomy for Snoring (PATS), NCT02562040; Childhood Adenotonsillectomy Trial (CHAT), NCT00560859.

Endovascular Embolization for Control of Post-Tonsillectomy Hemorrhage.

Post-operative hemorrhage is a potentially life-threatening complication of tonsillectomy. While standard surgical maneuvers including the use of electrocautery, application of topical hemostatic agents, direct pressure, and suturing of the tonsillar pillars have traditionally been used for the treatment of severe bleeding, endovascular approaches are an important adjunct when other techniques are unsuccessful. Here, we describe the case of a 10-year-old female who presented with severe bleeding four days after tonsillectomy and adenoidectomy for chronic tonsillitis. She was taken emergently to the operating room where pulsatile bleeding was noted from the right inferior tonsillar pole. Hemostasis could not be achieved using electrocautery despite multiple attempts. The patient was taken for emergent angiography, which demonstrated an irregularity of the right tonsillar artery consistent with arterial vasospasm, and which corresponded to the intraoral site of bleeding localized by the surgeon. Coil embolization of the tonsillar artery was successfully performed, and the patient experienced no further bleeding. We conclude that endovascular embolization of branches of the external carotid artery is an effective treatment for severe post-tonsillectomy hemorrhage in children and should be considered when attempts at surgical control are ineffective. This procedure requires exceptional collaboration between the surgical, radiology, and anesthesia teams.

Reliability and Accuracy of Remote Fiberoptic Nasopharyngolaryngoscopy in the Pediatric Population.

INTRODUCTION: Telemedicine is an increasingly prevalent component of medical practice. In otolaryngology, there is the potential for telemedicine services to be performed in conjunction with device use, such as with a nasolaryngoscope. This study evaluates the reliability of remote examinations of the upper airway through an iPhone recording using a coupling device attached to a nasopharyngolaryngoscope (NPL). METHODS: A prospective, blinded study was performed for pediatric patients requiring an NPL during an office visit. The NPL was performed using a coupling device attached to a smartphone to record the examination. A second, remote otolaryngologist then evaluated the recorded examination. Both otolaryngologists evaluated findings of anatomic sites including nasopharynx, oropharynx, base of tongue, larynx including subsites of epiglottis, arytenoids, aryepiglottic folds, false vocal cords, true vocal cords, patency of airway, and diagnostic impression, all of which were documented through a survey. Results of the survey were evaluated through inter-rater agreement using the κ statistic. RESULTS: Forty-five patients underwent an NPL, all of which were included in the study. The average age was 4.9 years. The most common complaint requiring NPL was noisy breathing (n = 16). The inter-rater agreement for overall diagnosis was 0.74 with 80% percent agreement, rated as "good." Other anatomic subsites with "good" or better inter-rater agreement were nasopharynx (0.75), oropharynx (0.75), and true vocal cords (0.71), with strong percentage agreement of 89%, 91%, and 87%, respectively. Both users of the adaptor found the recording setup to run smoothly. CONCLUSION: A telemedicine device for NPL use demonstrates strong diagnostic accuracy across providers and good overall evaluation. It holds potential for use in remote settings.

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

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

Magnetic resonance angiography (MRA) in preoperative planning for patients with 22q11.2 deletion syndrome undergoing craniofacial and otorhinolaryngologic procedures.

INTRODUCTION: Patients with 22q11.2 deletion syndrome (22q11.2DS) have a variety of anatomic anomalies. For surgeons operating in proximity to the retropharynx, the most pertinent is medial displacement of the internal carotid arteries. The purpose of this study is to describe the preoperative use of magnetic resonance angiography (MRA) in surgical planning and update the incidence rate of medial carotid displacement in patients with 22q11.2DS. METHODS: This is a retrospective cohort study of patients with a confirmed diagnosis of 22q11.2 deletion and preoperative MRA <18 years old who underwent tonsillectomy, adenoidectomy, Furlow palatoplasty (FPP), posterior pharyngeal flap (PPF), sphincter pharyngoplasty (SPP), or submucosal cleft palate (SMCP) repair between January 1st, 2008 and December 31st, 2019. RESULTS: Ninety patients who met the inclusion criteria underwent 133 procedures. The majority identified as Caucasian (84.4%); 52.2% were female. Cervical MRA was more likely to be ordered before a PPF (80.9%) and tonsillectomy (72.7%) over a FPP (47.6%) or adenoidectomy (11.1%). Carotid medialization was visualized in 23 patients (25.6%) and was mild in 11 cases, moderate in 7 cases, and significant in 5 cases. There was no association between sex, race/ethnicity, or genetic diagnosis with carotid medialization. Flap shortening was necessary in 20% of PPF cases to avoid injuring the medialized vessel. CONCLUSION: Patients with 22q11.2DS may have higher rates of medialization of the carotid arteries than previously thought. Given the risk for complications in these patients during pharyngeal operations, there may be a critical place for MRA in surgical planning for patients with 22q11.2DS.

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

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

Defining Risk of Postoperative Obstructive Sleep Apnea in Patients With 22q11.2DS Undergoing Pharyngeal Flap Surgery for Velopharyngeal Dysfunction Using Polysomnographic Evaluation.

OBJECTIVE: To determine pre- and postoperative prevalence of obstructive sleep apnea (OSA) in patients with 22q11.2 deletion syndrome (DS) undergoing wide posterior pharyngeal flap (PPF) surgery for velopharyngeal dysfunction (VPD). DESIGN: Retrospective study using pre- and postoperative polysomnography (PSG) to determine prevalence of OSA. Medical records were reviewed for patients' medical comorbidities. Parents were surveyed about snoring. SETTING: Academic tertiary care pediatric hospital. PATIENTS: Forty patients with laboratory confirmed 22q11.2DS followed over a 6-year period. INTERVENTIONS: Pre- and postoperative PSG, speech evaluation, and parent surveys. MAIN OUTCOME MEASURE: Severity and prevalence of OSA, defined by obstructive apnea hypopnea index (OAHI), before and after PPF surgery to determine whether PPF is associated with increased risk of OSA. RESULTS: Mean OAHI did not change significantly after PPF surgery (1.1/h vs 2.1/h, P = .330). Prevalence of clinically significant OSA (OAHI ≥ 5) was identical pre- and postoperatively (2 of 40), with both cases having severe-range OSA requiring positive airway pressure therapy. All other patients had mild-range OSA. Nasal resonance was graded as severe preoperatively in 85% of patients. None were graded as severe postoperatively. No single patient factor or parent-reported concern predicted risk of OSA (OAHI ≥ 1.5). CONCLUSIONS: Patients with 22q11.2DS are medically complex and are at increased risk of OSA at baseline. Wide PPF surgery for severe VPD does not significantly increase risk of OSA. Careful perioperative planning is essential to optimize both speech and sleep outcomes.

Lateral Neck Radiography in Preoperative Evaluation of Adenoid Hypertrophy.

OBJECTIVE: To assess the value of lateral neck radiographs in quantifying adenoid hypertrophy to help guide treatment decisions in patients with symptoms of nasal obstruction. STUDY DESIGN: Retrospective review. METHODS: Quantitative radiologic grading of adenoids was correlated with the intraoperative grading to select cases in agreement between the two methods. The percent airway obstruction was calculated as a ratio of adenoid size to the size of the nasopharyngeal airway near the level of the choanae on the lateral neck radiographs for adenoidectomy cases in which radiographic and intraoperative grading of adenoid size were in agreement. RESULTS: A total of 426 adenoidectomy cases with preoperative lateral neck radiographs were reviewed (M:F = 254:172 for age range 9 months to 16 years), and only cases in agreement between radiographic and intraoperative adenoid grading were included in radiographic analysis (N = 234). The percent airway obstruction values were significantly different between "severely obstructive" (N = 137, mean = 94.71, SD = 6.55, range [72.00; 100.00]) and "moderately obstructive" adenoid categories (N = 97, mean = 78.53, SD = 6.91, range [63.67; 98.08]), not only within clinically relevant age groups (1-3 years, 4-7 years, 8-15 years), but also for the entire data set (95% CI [14.41; 17.95], P < .0001). "Mildly obstructive" category was omitted due to small sample size (N = 4). CONCLUSION: Lateral neck radiographs can provide useful supplemental information on the degree of nasopharyngeal airway obstruction when other clinical findings do not clearly point toward adenoid hypertrophy as a primary cause of nasal obstruction. In our data set, a 65% nasopharyngeal airway obstruction represents a value two standard deviations below the mean for "moderately" obstructive adenoid category, and can be viewed as a simplified cut-off to indicate that the degree of adenoid enlargement is clinically relevant. This cut-off value can assist in evaluation of patients with symptoms of nasal obstruction. LEVEL OF EVIDENCE: 4.

New treatments for obstructive sleep apnea in children.

Obstructive sleep apnea (OSA) has a prevalence of up to 5% in children and 50% in obese children. OSA is associated with various comorbidities in the general population and in the perioperative period. In this review, we will provide background of OSA and insights into the available treatment options both surgically and medically. Unlike adults, most pediatric OSA is treated with adenotonsillectomy. Continuous positive airway pressure therapy is the commonly used medical treatment. Management of pediatric OSA is multidisciplinary. We will discuss the surgical options and medical options of managing pediatric OSA.