Prevalence and Management of Laryngomalacia in Patients With Pierre Robin Sequence.

OBJECTIVE: To characterize the prevalence and presentation of laryngomalacia and efficacy of supraglottoplasty (SGP) in a cohort of patients with Pierre Robin Sequence (PRS). DESIGN: Retrospective cohort study. SETTING: Tertiary-care children's hospital. PATIENTS, PARTICIPANTS: Consecutive patients with PRS born between January 2010 and June 2018. MAIN OUTCOME MEASURES: Chart review included demographics, comorbid airway obstruction including laryngomalacia, timing of surgical interventions, clinical symptoms, sleep study data, and modified barium swallow study data.126 patients with PRS were included; 54% had an associated syndrome, 64% had an overt cleft palate, and 22% had a submucous cleft palate. 64/126 were noted to have laryngomalacia (51%). Patients with concurrent PRS and laryngomalacia were significantly more likely to have submucous cleft palate (P = .005) and present with aspiration with cough (P = .01) compared to patients with PRS without laryngomalacia. Patients with concurrent laryngomalacia and PRS showed a significant decrease in apnea-hypopnea index (AHI) and obstructive AHI (OAHI) after mandibular distraction, with a median AHI and OAHI improvement of 22.3 (P = .001) and 19.8 (P = .002), respectively. Patients who underwent only SGP did not show significant improvement in these parameters (P = .112 for AHI, P = .064 for OAHI).The prevalence of laryngomalacia in our PRS cohort was 51%. Patients with PRS and laryngomalacia are more likely to present with overt aspiration compared to patients with PRS without laryngomalacia. These data support that laryngomalacia does not appear to be a contraindication to pursuing MDO.

Initiation of acid suppression therapy for laryngomalacia.

OBJECTIVE: Evidence supporting the use of acid suppression therapy (AST) for laryngomalacia (LM) is limited. The objective of this study was to determine if outpatient-initiated AST for LM was associated with symptom improvement, weight gain, and/or avoidance of surgery. METHODS: A retrospective cohort was reviewed at a tertiary-care children's hospital. Patients were included if they were diagnosed with LM at ≤6 months of age, seen in an outpatient otolaryngology clinic between 2012 and 2018, and started on AST. Primary outcomes were improvement of airway and dysphagia symptoms, weight gain, and need for surgery. Severity was assessed by symptom severity. RESULTS: Of 2693 patients reviewed, 199 met inclusion criteria. Median age of diagnosis was 4 weeks (range: 0-29 weeks). LM was classified as mild/moderate (71.4%) and severe (28.6%) based on symptom severity. Severity on flexible fiberoptic laryngoscopy (FFL) was not associated with clinical severity. Weight percentile, airway symptoms, and dysphagia symptoms improved within the cohort. In total, 26.1% underwent supraglottoplasty (SGP). In multivariate analysis, only severe LM on FFL was predictive of SGP (OR: 7.28, 95%CI: 1.91-27.67, p = .004). CONCLUSION: Clinical symptom severity did not predict response to AST raising the question of utility of AST in LM. Severity of LM based on FFL, not clinical severity, was associated with decision to pursue SGP. Prospective randomized trials are needed to better understand the role of AST in LM. LEVEL OF EVIDENCE: Level 3.

Socioeconomic Barriers to Care for Pediatric Airways Utilizing Geographic Information Systems.

OBJECTIVE: Geographic information systems (GIS) provide a unique set of tools to spatially analyze health care and identify patterns of health outcomes to help optimize delivery. Our goal is to create maps of pediatric tracheostomy patients using GIS to assess socioeconomic and other factors that impact postoperative care after discharge to home. METHODS: A retrospective study was performed on patients (≤21 years old) who underwent tracheostomy at a tertiary care pediatric hospital from January 1, 2015 to December 31, 2020. Using GIS, we geocoded patient addresses and conducted spatial analyses of the relationship between patients and access to health care providers as well as vulnerable population factors including poverty, educational attainment, and single-parent households. RESULTS: A total of 156 patients were included. Patients initially discharged to transitional care (108/156, 69.2%) had significantly higher likelihood of presenting to the ED regardless of socioeconomic status (OR: 2.28, 95% CI: 1.03-5.05; p = 0.042). There was no relationship between ED visit rate and median household income, poverty level, and percentage of uneducated adults (p = 0.490; p = 0.424; p = 0.752). Median distance to the tertiary care pediatric hospital was significantly longer for patients with no ED visit (median = 61.28 miles; SD = 50.90) compared with those with an ED visit (median = 37.75 miles; SD = 35.92) (p = 0.002). CONCLUSION: The application of GIS could provide geo-localized data to better understand the healthcare barriers to access for children with tracheostomies. This study uniquely integrates medical record data with socioeconomic factors and social determinants of health. LEVEL OF EVIDENCE: 4 Laryngoscope, 134:1919-1925, 2024.

Establishing the Preface for a Unified Otolaryngology Residency Curriculum: Implications and Considerations.

Otolaryngology residency training, along with the world of online medical education, has been continuously evolving and refining methods to educate and produce competent otolaryngologists. Numerous resources have been developed to assist otolaryngology residents in enhancing their clinical training. Although these resources greatly enhance clinical training, the growing volume of material presents a challenge within the constrained schedule of otolaryngology residents. This challenge is compounded by the variability in quality among resources which lack standardization or validation. Recently, the Academy of Otolaryngology-Head and Neck Surgery Foundation has proposed a unified otolaryngology curriculum designed to address these issues. This curriculum aims to incorporate high-quality educational materials, evidence-based adult learning principles, accessible learning sources, and diverse instructional methods within a structured program. Such a curriculum promises a significant positive impact, mirroring successes observed in various other surgical specialties.

Hidden Otolaryngology Malpractice Claims: An Internal Review From 2000 to 2020.

OBJECTIVE: To report key characteristics and patterns of captive insurance claims not publicly reported in otolaryngology across a large tertiary-level academic health system over the previous 2 decades. STUDY DESIGN: Case series. SETTING: The tertiary care health system. METHODS: The internal captive insurance database at a tertiary level healthcare system was queried to identify otolaryngology-related malpractice claims regardless of final disposition (settled or dismissed) filed from 2000 to 2020. The date of the incident, date of claim, error type, patient outcome, provider subspecialty, total expenses, disposition, and final reward amount were recorded. RESULTS: Twenty-eight claims were identified. There were 11 (39.3%) claims from 2000 to 2010 and 17 (60.7%) claims from 2011 to 2020. Head and neck surgery was the most frequently implicated subspecialty (n = 9, 32.1% of all cases), followed by general otolaryngology (n = 7, 25.0%), pediatrics (n = 5, 17.9%), skull base/rhinology (n = 4, 14.3%), and laryngology (n = 1, 3.6%). Improper surgical performance was cited in 35.7% of cases (n = 10), followed by failure to diagnose (n = 8, 28.6%), to treat (n = 4, 14.3%), and to obtain informed consent (n = 3, 10.7%). While 2 cases are ongoing, a total of 17/26 (65.4%) cases were settled and 20/26 (76.9%) dismissed some or all parties. Dismissed claims had significantly higher expenses (p = .022) and duration from incident to disposition (p = .013) compared to settled claims. CONCLUSION: This study expands the malpractice landscape in otolaryngology by including data not readily available through public sources and compares it to national trends. These findings encourage otolaryngologists to better gauge current quality and safety measures that best protect patients from harm.

Validity of the American College of Radiology Thyroid Imaging Reporting and Data System in Children.

OBJECTIVE: To assess the validity of the American College of Radiology Thyroid Imaging Reporting and Data System (ACR TI-RADS) for evaluating thyroid nodules in children. METHODS: Patients aged <19 years with thyroid nodule(s) evaluated by ultrasound (US) from 2007-2018 at a tertiary children's hospital were included. Two radiologists scored de-identified thyroid US images using ACR TI-RADS (from 1, "benign" to 5, "highly suspicious"). The radiologists recorded size and rated vascularity for each nodule. Ultrasound findings were compared to pathology results (operative cases, n = 91) and clinical follow-up without disease progression (non-operative cases, n = 15). RESULTS: Thyroid images from 115 patients were reviewed. Nine patients were excluded due to the absence of an evaluable nodule. Forty-seven benign and 59 malignant nodules were included. Median age at ultrasound was 15 years (range 0.9-18 years). Twenty (18.9%) patients were male. There was moderate agreement between TI-RADS levels assigned by the two raters (kappa = 0.57, p < 0.001). When the raters' levels were averaged, >3 as the threshold for malignancy correctly categorized the greatest percentage of nodules (68.9%). Eleven (18.6%) malignant nodules received a TI-RADS level of 2 (n = 3) or 3 (n = 8). Sensitivity, specificity, and positive and negative predictive values were 81.4%, 53.2%, 68.6%, and 69.4%, respectively. Although not part of TI-RADS, vascularity was similar between benign and malignant nodules (p = 0.56). CONCLUSION: In a pediatric population, TI-RADS can help distinguish between benign and malignant nodules with comparable sensitivity and specificity to adults. However, the positive and negative predictive values suggest TI-RADS alone cannot eliminate the need for FNA. LEVEL OF EVIDENCE: 3 Laryngoscope, 133:2394-2401, 2023.

Novel treatment of endobronchial inflammatory myofibroblastic tumor in a child.

Isolated endobronchial inflammatory myofibroblastic tumors (IMT) are rare, accounting for about 1% of primary endobronchial tumors in children. The mainstay of treatment for this tumor has been surgical resection. Recently, the identification of anaplastic lymphoma kinase (ALK) gene mutations in half of IMTs and promising results of treatment with ALK inhibitors in other ALK-positive tumors have opened the possibility of alternative approaches. We present a 4-year-old child with an ALK-positive endobronchial IMT, treated with endoscopic resection and neoadjuvant therapy with crizotinib, without evidence of tumor recurrence 2 years after the initial resection.

Unique Molecular Signatures Are Associated with Aggressive Histology in Pediatric Differentiated Thyroid Cancer.

Background: Molecular testing (MT) enhances the diagnostic accuracy of thyroid fine-needle aspiration biopsy, reducing the need for diagnostic lobectomy in adult patients with indeterminate nodules (Bethesda class III/IV). However, little is known about genetic alterations in pediatric thyroid carcinoma (TC). Our aim was to analyze MT results of pediatric differentiated TC (DTC) cases to determine associations with histological and clinical features. Methods: A retrospective review identified all patients (aged <19 years) diagnosed with DTC from 2001 to 2017 at the University of Pittsburgh Medical Center. Histology was rereviewed to confirm diagnosis and identify tissue for MT using next-generation sequencing (ThyroSeq, version 3, TSv3). Correlation with histological and clinical features was analyzed using regression analysis. Results: Of 71 patients with MT results, 62 (87%) patients had papillary TC. All patients were alive at a median follow-up of 6 years (range 18 days to 18 years). Genetic alterations were identified in 65 (92%) patients. These alterations were grouped as BRAF-like point mutations or fusions (39, 55%), RAS-like mutations or fusions (21, 30%), or copy number alterations (5, 7%). On multiple regression analysis accounting for patient sex and tumor size in patients with papillary TC, increased tumor stage (ß: 0.234, p < 0.001), multifocal disease (odds ratio [OR]: 3.60, p = 0.042), and lymph node metastases (OR: 6.13, p = 0.044) were associated with BRAF-like gene fusions. When considering individual mutations, ETV6/NTRK3 fusions were associated with increased tumor stage (ß: 2.07, p = 0.023) and BRAF-like point mutations were associated with increased likelihood of surgery for recurrence over time (hazard ratio: 19.5, p = 0.004). Conclusions: Among our cohort of pediatric TC patients who underwent comprehensive MT, >90% had an identifiable genetic alteration. Aggressive features were primarily associated with BRAF-like gene fusions. Preoperative MT results may be useful in guiding the extent of the initial operation in pediatric patients (aged <19 years) with TC.

Chronic Lymphocytic Thyroiditis and Aggressiveness of Pediatric Differentiated Thyroid Cancer.

OBJECTIVES/HYPOTHESIS: Hashimoto's Thyroiditis (HT) is a common cause of hypothyroidism. Among adults with differentiated thyroid cancer (DTC), HT appears to be associated with less severe disease burden. In the absence of information regarding HT and disease burden among children with DTC, we assessed the relationship between pediatric DTC severity and HT. STUDY DESIGN: Retrospective cohort. METHODS: Charts from 90 pediatric patients who underwent surgical removal of DTC from 2002 to 2017 at tertiary-care children's hospital were reviewed. Demographic, clinical, surgical, pathology, and outcome details were compared between patients with and without HT. Consistency among diagnostic modalities of HT was also evaluated. RESULTS: Median age at presentation was 16.0 years (range 4.2-18.9 years). Twenty-two patients were male (24%). Forty-five patients (50%) had HT based on presence of thyroid autoantibodies and/or surgical pathology findings and 45 patients did not have HT. Patients with HT had increased odds of microcalcifications (odds ratio [OR]: 3.01, P = .031) and decreased odds of palpable nodules (OR: 0.212, P = .024) and T2 lesions (vs. T1) (OR: 0.261, P = .015) compared with non-HT. No significant differences in demographics and the incidence of multifocality, extrathyroidal extension, lymphovascular invasion, lymph node or pulmonary metastases, disease recurrence, or radioactive iodine treatment were found between the two groups. Thyroglobulin/thyroid peroxidase autoantibodies and surgical pathology indicative of HT were concordant in 82.4% (κ = 0.635, P < .001). CONCLUSION: HT was present in 50% of children with DTC. Patients with DTC and HT presented with smaller tumors compared to non-HT patients. No significant differences in other markers of disease aggressiveness were found between the two groups. LEVEL OF EVIDENCE: 3 Laryngoscope, 132:1668-1674, 2022.

Dysphagia in Pediatric Patients with Tracheostomy.

OBJECTIVE: Post-tracheotomy swallowing function has not been well described in the pediatric population. This study aims to (1) determine differences in swallowing functioning pre- and post-tracheotomy and (2) examine the association between postoperative dysphagia and indication for tracheotomy, age at the time of tracheotomy, and time between tracheotomy and modified barium swallow (MBS). METHODS: A retrospective chart review was performed on 752 patients who underwent a tracheotomy from 2003 to 2018 and had adequate documentation for review. Patients were included if they received a post-operative MBS. Descriptive statistics, logistic regression, and Fisher's exact test were used to analyze the data. RESULTS: The cohort included 233 patients. The mean age at the time of tracheotomy was 25 months (±50.5). The indications for the tracheotomy were upper airway obstruction (110/233, 47.2%), chronic respiratory failure (104/233, 44.6%), and neurologic disease (19/233, 8.2%). The mean time from tracheotomy to post-operative MBS was 224 days (±297.7). Of the patients who had documented pre- and post-tracheotomy diets, nearly half of patients had improvement in their swallowing function after tracheotomy placement (82/195; 42.1%). Post-tracheotomy MBS recommended thickened liquids in 30.9% of the patients (72/233) and 42.5% (99/233) were recommended thin liquids. The remainder (62/233, 26.6%) remained nothing by mouth (NPO). Patients with neurological disease as the indication for the tracheotomy were more likely to remain NPO (P = .039). CONCLUSION: A tracheotomy can functionally and anatomically affect swallowing in pediatric patients. The majority of our studied cohort was able to resume some form of an oral diet postoperatively based on MBS. This study highlights the need for objective measurements of swallowing in the postoperative tracheotomy patient to allow for safe and timely commencement of an oral diet. LEVEL OF EVIDENCE: Level 3.

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.

Management and Outcomes of Button Batteries in the Aerodigestive Tract: A Multi-institutional Study.

OBJECTIVES/HYPOTHESIS: To describe the clinical presentation, management, and complications associated with button battery impaction in the aerodigestive tract in children. STUDY DESIGN: Retrospective case series. METHODS: This multi-institutional study, endorsed by the American Society of Pediatric Otolaryngology research consortium, is a retrospective medical record review, including all children at five tertiary-care institutions presenting with button batteries impacted in the aerodigestive tract between January 2002 and December 2014. Battery type/size, duration and location of impaction, presenting symptoms, treatment, complications, and outcomes were examined. RESULTS: Eighty-one patients were included (64.2% male), with ingestion witnessed in 20 (24.7%). Median age at presentation was 3 years (range, 1 week-14 years). Median time from diagnosis to removal was 2.5 hours (range, 0.4-72 hours). Locations included the esophagus (n = 48), hypopharynx (n = 1), stomach (n = 6), nasal cavity (n = 22), and ear canal (n = 4). Most common symptoms for esophageal/hypopharyngeal impactions included dysphagia (26.5%), nausea/vomiting (26.5%), drooling (24.5%), cough (18.4%), and fever (18.4%). Most common symptoms for nasal impactions included epistaxis (54.6%), rhinorrhea (40.9%), nasal pain (27.3%), and fever (22.7%). Almost all esophageal impactions were from 3-V (89.5%), 20-mm (81.8%) lithium batteries. Severe esophageal complications included stricture (28.6%), perforation (24.5%), tracheoesophageal fistula formation (8.2%), pneumothorax (4.1%), and bilateral true vocal fold paresis (4.1%). Nasal complications included necrosis (59.1%), septal perforation (27.3%), and saddle nose deformity (4.5%). Duration of impaction correlated with an increased likelihood of persistent symptoms only for nasal batteries (P = .049). CONCLUSIONS: Button batteries in the upper pediatric aerodigestive tract or ear canal should be considered a surgical emergency, requiring urgent removal and careful vigilance for complications. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E298-E306, 2021.

Predictive Utility of the Penetration-Aspiration Scale in Inter-Arytenoid Injection Augmentation.

OBJECTIVES/HYPOTHESIS: To determine the utility of preoperative penetration-aspiration scale (PAS) scores and clinical findings on modified barium swallow (MBS) in predicting advancement of diet after interarytenoid injection augmentation (IAIA). STUDY DESIGN: Retrospective review. METHODS: In this retrospective cohort study, 372 consecutive patients who underwent IAIA for pharyngeal dysphagia between 2009 and 2019 were initially identified. Patients were excluded from the study if they had insufficient preop MBS, no postop MBS within 3 months of injection, supraglottoplasty, or underlying neurological condition. Ninety-three patients were included in the study. Pre- and postoperative PAS scores were recorded, as were pre and postop diets. PAS scores were calculated by a single pediatric speech and language pathologist. RESULTS: Average PAS score on MBS was 5.87 (standard deviation [SD] 2.74); median (range) = 8 (1-8). Postop average was 4.29 (SD 3.02); median (range) = 2 (1-8), P < .001. Those with worse preop PAS scores had increased odds of improvement in diet (odds ratio 1.24, 95% confidence interval [CI] 1.02-1.49, P = .029). An improvement in PAS score of 3.0 or greater predicted an improvement in diet with a sensitivity of 76.7% and a specificity of 85.7%. CONCLUSIONS: PAS score on MBS can be a useful tool when assessing pediatric patients who may be candidates for IAIA. Prospectively comparing PAS score in patients post-IAIA to patients solely undergoing diet modification can help to better objectively assess differences in outcomes and understand the full utility of PAS score. LEVEL OF EVIDENCE: Level III (Individual Cohort Study) Laryngoscope, 131:E1707-E1713, 2021.

Heterotopic gastric mucosa of the proximal esophageal (HGMPE) and its potential role in pediatric dysphonia and dysphagia.

OBJECTIVE: Despite a reported incidence of HGMPE of 10%, proof of acid production, and an increased incidence of respiratory symptoms, the pediatric otolaryngology, swallowing and voice care literature is silent on this entity. This case series describes pediatric patients confirmed to have HGMPE with dysphonia and/or dysphagia. METHODS: Retrospective case series of Pediatric Voice, Resonance, and Swallowing Center patients at a tertiary Children's Hospital in 2019. SETTING: Tertiary academic medical center. SUMMARY OF RESULTS: Three patients who underwent triple endoscopy for dysphonia or dysphagia were histologically diagnosed with HGMPE. Esophageal biopsies were otherwise normal. Two of the three patients resolved their primary aerodigestive symptoms following treatment with acid suppression and a protectant (sucralfate). The third patient reported significant improvement in symptoms by phone. The significance of this case series cannot be understated: 1) A need for increased awareness among pediatric otolaryngologists, voice care and swallowing professionals of this entity given its relatively common incidence of 10% offset by a dearth of presentations & scientific publications in our literature clearly exists. 2) Otolaryngologists have abandoned operative upper aerodigestive tract endoscopy in lieu of office-based less comprehensive videolaryngostroboscopy and fiberoptic endoscopic evaluation of swallowing. HGMPE & other esophageal disorders (i.e. eosinophilic esophagitis) support revisiting triple endoscopy in select patients where office endoscopy has failed to diagnose and successfully treat such patients. 3) Both acid suppression therapy and a protectant (sucralfate) may be useful in these patients. 4) Modification of rigid esophagoscopy technique to carefully assess the introitus and superior esophageal segment is paramount 5) Otolaryngologists over-diagnose & over-treat laryngopharyngeal reflux. The pediatric & adult literature is replete with significant safety warnings associated with acid suppression therapy and guidelines admonish their indiscriminate use, raising the liability bar of empiric treatment. Large scale prospective, randomized and controlled studies are needed to confirm the pathophysiologic role of this entity in pediatric aerodigestive disorders. CONCLUSION: HGMPE is a clinical entity that can be easily missed upon swift entry into the esophagus with rigid endoscopy. Careful scrutiny and visualization of the proximal esophagus is critical in order to identify HGMPE, as there is a higher rate of laryngospasm, stricture, and potentially neoplasm in this population.

Clinical Consensus Statement: Ankyloglossia in Children.

OBJECTIVE: To identify and seek consensus on issues and controversies related to ankyloglossia and upper lip tie in children by using established methodology for American Academy of Otolaryngology-Head and Neck Surgery clinical consensus statements. METHODS: An expert panel of pediatric otolaryngologists was assembled with nominated representatives of otolaryngology organizations. The target population was children aged 0 to 18 years, including breastfeeding infants. A modified Delphi method was used to distill expert opinion into clinical statements that met a standardized definition of consensus, per established methodology published by the American Academy of Otolaryngology-Head and Neck Surgery. RESULTS: After 3 iterative Delphi method surveys of 89 total statements, 41 met the predefined criteria for consensus, 17 were near consensus, and 28 did not reach consensus. The clinical statements were grouped into several categories for the purposes of presentation and discussion: ankyloglossia (general), buccal tie, ankyloglossia and sleep apnea, ankyloglossia and breastfeeding, frenotomy indications and informed consent, frenotomy procedure, ankyloglossia in older children, and maxillary labial frenulum. CONCLUSION: This expert panel reached consensus on several statements that clarify the diagnosis, management, and treatment of ankyloglossia in children 0 to 18 years of age. Lack of consensus on other statements likely reflects knowledge gaps and lack of evidence regarding the diagnosis, management, and treatment of ankyloglossia. Expert panel consensus may provide helpful information for otolaryngologists treating patients with ankyloglossia.

Airway anomalies in patients with craniosynostosis.

OBJECTIVES: 1) Characterize the spectrum of airway anomalies in patients with craniosynostosis, and 2) identify clinical characteristics of these patients that may be associated with the development of airway anomalies. METHODS: This study is a retrospective case series assessing the type and frequency of airway anomalies in all patients with craniosynostosis seen at a tertiary-care children's hospital between 2000 and 2016. Cohort analyses were then performed to identify differences in airway anomalies dependent on syndromic associations, multisutural fusion, and location of suture fusion. Clinical characteristics examined included demographics and additional neurologic and craniofacial abnormalities. RESULTS: Four hundred and ninety-six patients with craniosynostosis (83.5% white, 64.5% male; 33.9% sagittal, 28.8% metopic, 11.5% coronal, 1.2% lambdoid, and 24.6% multisutural) were included. Notable airway anomalies included the following: 13.3% adenotonsillar hypertrophy, 8.9% laryngomalacia, 7.3% tracheomalacia, 7.1% subglottic stenosis, 4.0% bronchomalacia, 3.8% laryngeal cleft, and 1.2% vocal fold paresis. Multisutural craniosynostosis patients (n = 122) were more likely to have obstructive sleep apnea (P = 0.005), adenotonsillar hypertrophy (P = 0.014), tracheomalacia (P = 0.011), subglottic stenosis (P < 0.001), and epiglottic/base of tongue collapse (P = 0.003) and require tracheostomy (P = 0.001) and mechanical ventilation (P = 0.017) compared with single suture craniosynostosis. Syndromic craniosynostosis patients (n = 33) were more likely to have obstructive sleep apnea (P < 0.001), laryngomalacia (P = 0.047), and subglottic stenosis (P = 0.009) compared with nonsyndromic patients. CONCLUSION: Airway anomalies are prevalent in patients with craniosynostosis; patients with multisutural or syndromic types have an increased risk of developing certain abnormalities. There should be a lower threshold for referral for airway evaluation in these populations. LEVEL OF EVIDENCE: 4. Laryngoscope, 129:2594-2602, 2019.

Sensorineural hearing impairment in children with Chiari I malformation.

OBJECTIVES: We describe the prevalance of Chiari I malformation in children presenting to a pediatric tertiary care hearing loss clinic, characterize the phenotype of hearing loss in children with Chiari I malformation, and discuss the potential pathophysiology of sensorineural hearing impairment (SNHI) related to Chiari I malformation. METHODS: This study was a retrospective case series of patients seen in a pediatric tertiary care hearing loss clinic affiliated with an academic medical center. We considered 481 patients with SNHI who had radiographic imaging as part of the evaluation for the cause of their hearing impairment. Hearing impairment was determined by behavioral audiogram and/or auditory brain stem response testing. Radiographic imaging was performed with computed tomography and/or magnetic resonance imaging. RESULTS: Six children with SNHI also had a diagnosis of Chiari I malformation. Four of these 6 children had unilateral hearing impairment. One child had asymmetric bilateral impairment; 1 child had symmetric bilateral impairment. The severity of hearing impairment varied from mild to profound. All children were referred to the neurosurgery department. None underwent surgical decompression. CONCLUSIONS: Chiari I malformation may be associated with SNHI. Central nervous system imaging for the evaluation of children with SNHI may be indicated, particularly in individuals with negative results on temporal bone computed tomography and genetic testing.

Accuracy of chest X-Ray measurements of pediatric esophageal coins.

OBJECTIVE: To determine the accuracy of chest x-ray measurements in children using ingested radiopaque foreign bodies of known size. METHODS: A database of foreign body ingestions at a tertiary care children's hospital was queried from 2013 to 2016 for children who had ingested a US coin, had a pre-operative chest x-ray and documentation of coin type at the time of endoscopic removal. Four blinded research subjects measured the coin diameter on chest x-ray using iSite PACS software and based on the measurement, predicted the coin type. Measurements were compared to the known coin diameters published by the US Mint. RESULTS: A total of 51 patients with sixteen esophageal quarters (diameter 24.26 mm), fourteen nickels (21.21 mm), fourteen pennies (19.05 mm) and seven dimes (17.91 mm) were included in the study. The four subjects had a mean accuracy of 60.3% (range 49.0%-72.5%) in predicting the correct coin type. Across all raters, there was poor agreement for pennies (kappa = 0.161) and dimes (kappa = 0.131), fair agreement for nickels (kappa = 0.259), good agreement for quarters (kappa = 0.687), and fair agreement overall (kappa = 0.371). The study measurements overestimated the coin size in 203 of the 204 measurements by a mean of 1.84 mm (range -0.31-3.85 mm). The mean size discrepancy was larger (2.40 vs. 1.30 mm, p < 0.001) and accuracy of coin type identification was worse (44.6% vs. 74.1%, p = 0.001) in children <4 years old. CONCLUSIONS: Measurement of esophageal coins on chest x-ray is relatively inaccurate and overestimates the size in the majority of cases. Clinicians should use caution when performing fine measurements on chest x-rays, especially in children younger than 4 years old.

Structured care to improve outcomes in primary single stage laryngotracheal reconstruction.

PURPOSE: To examine single stage laryngotracheal reconstruction (SSLTR) care to reduce complication and failure rate. METHODS: Forty-five patients that underwent primary SSLTR were examined retrospectively. All had pre-operative direct laryngoscopy and bronchoscopy, esophagoscopy with biopsy and MRSA screening. Pre-operative subglottic stenosis (SGS) grade and associated comorbidities were recorded. Intraoperative graft location and type was documented. Hospital course and results were evaluated and compared to cited literature. RESULTS: The median age at reconstruction was 2 years (0-15 years). 42.2% were male. 66.7% had gastroesophageal disease and 24.4% a MRSA history. Grade 2 SGS was noted pre-operatively in 37.8% and grade 3 or 4 in 57.7% of patients. Post-surgical hospital course was examined. 77.8% of patients were extubated on planned date. 95.6% of patients had operation specific successful decannulation. Graft type and variations of graft placement as well as MRSA and GERD status didn't affect procedure success rate. Active GERD was related to failure of extubation on planned day (p = 0.02). An abnormal pre-operative swallowing examination was associated with higher complication rates (p = 0.03). CONCLUSION: Utilizing a more structured approach to SSLTR work-up and addressing potential SSLTR pitfalls may result in higher operation specific decannulation rates. Pre-operative GERD and swallowing dysfunction were associated with higher rates of adverse events.

Quality, Readability, and Trends for Websites on Ankyloglossia.

OBJECTIVE: This study evaluates the quality and readability of websites on ankyloglossia, tongue tie, and frenulectomy. METHODS: Google was queried with six search terms: tongue tie, tongue tie and breastfeeding, tongue tie and frenulectomy, ankyloglossia, ankyloglossia and breastfeeding, and ankyloglossia and frenulectomy. Website quality was assessed using the DISCERN instrument. Readability was evaluated using the Flesch-Kincaid Reading Grade Level, Flesch Reading Ease Score, and Fry readability formula. Correlations were calculated. Search terms were analyzed for frequency using Google Trends and the NCBI database. RESULTS: Of the maximum of 80, average DISCERN score for the websites was 65.7 (SD = 9.1, median = 65). Mean score for the Flesch-Kincaid Reading Grade Level was 11.6 (SD = 3.0, median = 10.7). Two websites (10%) were in the optimal range of 6 to 8. Google Trends shows tongue tie searches increasing in frequency, although the NCBI database showed a decreased in tongue tie articles. CONCLUSIONS: Most of the websites on ankyloglossia were of good quality; however, a majority were above the recommended reading level for public health information. Parents increasingly seek information on ankyloglossia online, while fewer investigators are publishing articles on this topic.