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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., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objective: Laryngomalacia (LM) is the most common congenital anomaly of the larynx. The cause of LM is still largely unknown, but a neurological mechanism has gained the most acceptance. There have not been any studies examining the prevalence of LM in infants with Neonatal Abstinence Syndrome (NAS). The aim of our study is to determine if infants with NAS are more likely to be diagnosed with LM., Methods: This study was a population-based inpatient registry analysis. We examined nationwide neonatal discharges in 2016 using the Kids' Inpatient Database (KID). Only patients listed as neonates were included. The International Classification of Diseases, 10th revision, Clinical Modification (ICD-10-CM) codes for neonatal withdrawal symptoms from maternal use of drugs of addiction (P96.1) and diagnoses denoting LM were used. To quantify associations between the LM and NAS groups, prevalence rates and odds ratios (ORs) were used., Results: There were 3 970 065 weighted neonatal discharges in the 2016 KID. Among patients included in our dataset, 0.809% (32 128) had NAS and 0.075% (2974) had LM. There was an increased odds ratio for neonates with NAS and LM (OR of 2.85, 95% CI = 2.24-3.63) compared to infants without NAS. Multiple logistic regression accounting for possible confounders produced an adjusted OR of 1.68 (95% CI = 1.29-2.19)., Conclusion: Our study found an association between NAS and LM. This suggests that prenatal exposure to opioids or possibly the sequelae of withdrawal symptoms may be risk factors for the development of LM.

Introduction: As a recently established division, we sought to reflect on the development of our paediatric airway surgery service, and prospectively examine the diagnoses that underwent microlaryngobronchoscopy (MLB) to help quantify the evolving population demographics of paediatric airway disorders., Material and Methods: This was a prospective longitudinal study conducted of all paediatric MLBs performed by a single surgeon in a tertiary paediatric ENT centre between 2012 and 2019., Results: A total of 1040 MLBs were performed in 498 patients at the paediatric ENT centre of the Royal London Hospital. Median age at first procedure was 19 months. Median follow-up was 48 months. Primary diagnoses were laryngomalacia (21%), subglottic stenosis (SGS - 18%), laryngeal cleft (13%), and normal anatomy (28.3%). Repeat procedures were needed in 39.1% patients, who underwent a median of 2 repeat procedures. SGS (57.7%) constituted majority of the repeat category, followed by laryngeal cleft (12.36%), laryngomalacia (10.15%), unilateral/bilateral vocal cord palsy(4.24%) and laryngeal papilloma(4.24%). Laryngeal papilloma constituted largest number of procedures per patient (Median = 4, IQR = 5.75), followed by subglottic web and SGS. Mean length of stay(LOS) was 0.67 ± 0.96 days(d), with laryngeal cleft cases recording longest mean LOS. There was a steady increase in proportion of day-surgeries across study period [6.9% (2012) vs 59%(2019)]., Conclusion: SGS constitutes the major bulk of paediatric airway surgery, reflective of increasing number of premature births and prolonged intubation among neonates. Day-case MLB is a safe and feasible option in selected patients. This long-term data provides useful information to accurately prognosticate patients regarding potential number of repeat procedures for each diagnosis., (Copyright © 2022 Elsevier B.V. All rights reserved.)

Purpose: The present study aims to review the outcomes of coblation supraglottoplasty performed for children with different types of laryngomalacia, and we discuss the factors affecting these outcomes., Methods: We retrospectively reviewed the medical records of laryngomalacia patients admitted to the Otorhinolaryngology Department, Mansoura University, from 2010 to 2020. We examined the patient's demography, symptoms, comorbidities, type of laryngomalacia, oxygen saturation, and final outcomes., Results: Our study included 235 patients; 122 patients responded to medical therapy, while 113 underwent surgical management. There is a significant relation between the types and therapy they underwent (p ≤ 0.001). Larger percentage within type I underwent medical therapy. There is a statistically significant difference between the studied groups regarding age at surgery. On pairwise comparison, patients with type II had the lowest age significantly at the surgery when compared with each other individual group (p ≤ 0.001). On multivariate regression analysis, the presence of comorbid congenital heart disease, neurological comorbidities significantly increased the risk of failure of surgical intervention by 17.32 and 5.803 folds, respectively., Conclusions: Coblation supraglottoplasty is effective and safe to treat severe laryngomalacia. Different morphological types of laryngomalacia require slight surgical variations of coblation supraglottoplasty. The presence of comorbid congenital heart disease, neurological comorbidities significantly increased the risk of failure of surgical intervention., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objectives: Laryngomalacia is the commonest cause of stridor in the paediatric population. Whilst usually self-limiting, studies have suggested that the presence of synchronous airway lesions (SALs) might be associated with more severe clinical presentation and additional morbidity. However, evidence in the literature is scarce, contributing to controversy regarding the appropriate investigation and management of children with laryngomalacia. The aim of this study was to explore the prevalence, clinical significance and risk factors of SALs in children with severe, progressive and atypical laryngomalacia symptoms., Methods: Retrospective analysis of the records of all paediatric patients diagnosed with laryngomalacia in a UK tertiary referral centre, over a 2-year period, was undertaken. Data on demographics, comorbidities, management and presence of SALs were collected., Results: The study included 130 patients with laryngomalacia. Seventy-eight patients required investigation with microlaryngobronchoscopy (MLB), based on clinical presentation. SALs were identified in 22 patients (28.2%), 6 of which (7.7%) required surgical intervention. The commonest airway lesions included tracheomalacia, bronchomalacia and subglottic stenosis. A correlation between the presence of neurodevelopmental conditions in children and SALs was noted (p value < 0.05). The presence of SALs was not associated with sex, gestational age at birth or other medical comorbidities., Conclusion: The results of this study allow professionals to counsel families about the utility of MLB in children with severe, progressive and atypical laryngomalacia symptoms and inform management pathways for clinicians not working in airway centres. We support the clinical practice that progressive, atypical or severe laryngomalacia symptoms should prompt consideration of an MLB, to identify potential synchronous airway lesions. Furthermore, we propose that a higher index of suspicion and more thorough investigation might be indicated in children with neurodevelopmental disorders., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Background: Flexible fiber-optic laryngo-tracheo-bronchoscopy has become widely performed in infants and neonates since the introduction of thin flexible fiberscopes. Laryngomalacia is the most common airway disease in infants causing stridor. Pharyngomalacia, termed pharyngeal occlusion during inspiration, was the second most common airway disease found in our hospital in patients that underwent laryngo-tracheo-bronchoscopy, but the incidence, patient characteristics, and natural course have not been reported in large numbers in Japan., Methods: A retrospective review was performed of medical records on patients admitted to our neonatal intensive care unit during the neonatal period diagnosed with pharyngomalacia between April 2009 and November 2018. Patient characteristics, concurrent airway diseases, comorbidities, and treatment were reviewed., Results: Forty-eight patients were diagnosed with pharyngomalacia. The median gestational age was 37.1 weeks, and the median birthweight was 2,552 g. Patients were diagnosed at a median age of 29 days, and cure was achieved at a median age of 4 months. Twenty-eight patients had concurrent airway diseases, laryngomalacia being the most common. Continuous positive airway pressure or high flow nasal cannula was used in 34 patients., Conclusions: In patients with pharyngomalacia, half were born preterm, and more than half had concurrent airway diseases. The onset and diagnosis were made within the first month of life in more than half of the patients, and resolution was seen mostly within the first 6 months of life. Whenever a patient is suspected of having an airway disease, the pharyngeal space should be carefully observed to diagnose pharyngomalacia., (© 2021 Japan Pediatric Society.)

Background: Infantile tremor syndrome (ITS) is a rare clinical entity, predominantly characterized by coarse tremors, regression of motor and/or cognitive, and language milestones. Although the exact pathogenesis yet remains to be unknown, various micronutrient deficiencies like vitamin B12, zinc, magnesium, and vitamin C have been shown to be associated with ITS. Vitamin B12 deficiency is the most accepted etiology of this entity. Here we are describing a seemingly novel association of laryngomalacia with infantile tremor syndrome., Methods: Clinical details, laboratory investigations, demographic and socioeconomic parameters of all children<2 years of age, diagnosed with ITS between August 2019 and November 2020 was obtained by a retrospective chart review. The study population was divided into two subgroups based on the presence/absence of laryngomalacia and different variables were compared between the two subgroups., Results: During the study period, 22 ITS and 13 pre-ITS cases were identified, out of which 5 ITS cases had laryngomalacia, the prevalence is higher as compared to estimated prevalence in reported literature (p < 0.0001). All these 5 cases had associated gastro-esophageal reflux and swallowing dysfunction and all of them were managed conservatively, apart from standard care treatment for ITS. On follow-up, all 5 of them showed improvement in symptoms of laryngomalacia, apart from clinical and hematological improvement. Differences between various clinical and biochemical parameters in the subgroup with and without laryngomalacia were not significantly different. Seventeen out of twenty-two children with ITS and 10/13 children with pre-ITS had macrocytic anemia and the rest had dimorphic anemia on peripheral smear. There was moderate vitamin B12 deficiency in 13/22 and 8/13 children with ITS and pre-ITS respectively, and the rest had a severe vitamin B12 deficiency., Conclusion: Clinicians need to screen cases with ITS for probable symptoms of laryngomalacia and accordingly advise management measures. Although our study showed a seemingly apparent association between ITS and laryngomalacia, large prospective controlled studies with long-term follow-up are necessary to prove this association., Competing Interests: Declaration of competing interest All the authors declare that they have no conflict of interest., (Copyright © 2021 European Society for Clinical Nutrition and Metabolism. Published by Elsevier Ltd. All rights reserved.)

Objectives: Laryngomalacia (LM) is commonly diagnosed in infants and children with upper aerodigestive symptoms. In the literature, the focus has been on the respiratory impairment, with fewer studies addressing swallowing dysfunction (SwD). The objective of this study is to systematically review the literature for evidence on the prevalence of SwD in children diagnosed with LM., Methods: A search was conducted on the following databases: OVID Medline, Ovid EMBASE, EBSCO CINAHL, PROSPERO, and Cochrane Library. We included all the studies that reported on children with LM and documented objective swallowing assessment using fiberoptic endoscopic evaluation of swallowing (FEES) or videofluoroscopic swallowing study (VFSS). Two authors independently screened all the studies, assessed the level of evidence in the included studies, and extracted data. Risk of bias assessment and pooled data analysis were performed., Results: The search yielded 512 abstracts. Four studies met the selection criteria representing 425 children. Three studies were retrospective uncontrolled case series and one was a prospective cohort study. In all studies but one, an instrumental assessment of swallowing was selectively performed in patients with clinical indicators of SwD. The pooled estimate (range) of prevalence of SwD was 49% (13.9-90.6%)., Conclusion: The literature suggests a high prevalence of SwD in children with LM, however the level of evidence is low and generalizability is poor. The wide range of prevalence figures suggests a significant variability in the threshold and indications to assess swallowing in children with LM., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Introduction: Laryngomalacia is one of the most common causes of stridor in young children. It can be a serious concern to both parents and caregivers. The main objective of this study is to find the prevalence of laryngomalacia among young children presenting with stridor in a tertiary care hospital in central Nepal., Methods: A descriptive cross-sectional study was carried out form 1st December 2017 to 1st May 2020 in children less than two years of age in a tertiary care hospital. Ethical approval was taken from the Institutional Review Committee of the hospital (reference number: 2020/23). Convenient sampling was done. Detailed demography, clinical examination, and video laryngoscopy findings were evaluated to find the prevalence of laryngomalacia among all children with stridor. Data were analyzed by using Statistical Package for the Social Sciences version 20. Point estimate at 95% confidence interval was calculated along with frequency and proportion for binary data., Results: Out of 430 participants who presented with stridor, the laryngomalacia was found in 234 (66%) (58.7-74.07) cases at a 95% confidence interval. The male: female ratio was 1.7:1. Most children, 192 (67.6%), presented with a milder form of laryngomalacia. The most common type was a mixed type of laryngomalacia in 159 (56%). Sleep-disordered breathing was seen in 113 (39.79%) of children diagnosed with laryngomalacia., Conclusions: Our study concluded that laryngomalacia was the most common cause of stridor in children less than two years of age. However, in most cases, the problem is not serious and a regular follow-up with weight monitoring is warranted.

Objective: This study is to investigate the status and clinical significance of respiratory viruses in bronchoalveolar lavage fluid (BALF) of children with PBB., Methods: Sixty-eight children with PBB aged from 3 months to 5 years were enrolled and retrospectively reviewed from January 2014 to December 2017. Thirty-five children with persistent pneumonia or chronic pneumonia were matched as controls. Bronchoalveolar lavage fluid samples were collected for respiratory virus detection and bacterial culture., Results: The detection rate of bacteria in BALF of children with PBB was 61.8%, which was significantly higher than that of control group (20%) (P < 0.001). The detection rate of virus in BALF of children with PBB was 23.5%, including 6 (8.8%) of rhinovirus, 4 (5.9%) of parainfluenza virus type 3, 2(2.9%) of bocavirus, 2 (2.9%) of respiratory syncytial virus 1 (1.5%) of human metapneumonia virus and 1 (1.5%) of influenza virus A. 10 cases (28.6%) of virus were detected in the control group, including 3 (8.6%) respiratory syncytial virus, 3 (8.6%) rhinovirus and 2 (5.7%) bocavirus. There was no significant difference of viral detection rate between the two groups (P = 0.577)., Conclusion: Respiratory viruses can be detected in BALF of children with PBB, However, there is no evidence that PBB is virus-induced., (Copyright © 2019. Published by Elsevier Ltd.)

Objective: To report on the prevalence of voice disturbances in pediatric airway patients., Methods: Consecutive patients seen in a specialized Center for Airway Disorders at a tertiary children's hospital from February 2017 to September 2017 were included. Patients' families were invited to complete a pediatric voice health handicap index (pVHI) questionnaire. Patients underwent evaluation including flexible laryngoscopy and/or direct laryngoscopy and bronchoscopy., Results: 146 patients were included. Of these children, 73 patients (50.3%) presented with swallowing difficulty and 44 patients (30.3%) presented with respiratory complaints. Only 9 patients (6.2%) reported hoarseness initially. The median age at referral was 2.7 years of age (interquartile range: 1.4-4.3). The mean total pVHI score was 9.5 (± 12.9). Sixty-seven patients (45.9%) had abnormal pVHI findings of score > 4. Six patients (4.1%) had pVHI > 40. The mean pVHI score was 26.0 (± 21.1) among 12 patients with a history of tracheostomy, 12.0 (± 14.3) among 30 patients with laryngeal cleft, and 9.0 (± 9.9) among 19 patients with laryngomalacia., Conclusion: Voice disturbances are not uncommon in pediatric patients evaluated for airway disorders. Although patients may present with primary concerns for breathing or swallowing difficulties, many of these patients may need further work-up and treatment for dysphonia., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Stridor is a high-pitched respiratory sound that signals upper airway obstruction. It can be encountered by clinicians in a variety of clinical settings and requires a team-based, interdisciplinary approach. Early recognition is crucial, as the differential diagnosis can be broad, and causes range from benign to life-threatening. This article reviews the most commonly encountered causes of chronic congenital stridor in infants, focusing on the diagnostic approach, pathophysiology, clinical presentation, and management strategies.

The aim of this study was to: (1) find out whether laryngomalacia (LM) types are related to clinical course; (2) which patients with LM are at higher risk of other airway malacia [tracheomalacia (TM) and/or bronchomalacia (BM)]; and (3) evaluate the prevalence of LM in our region. Patients with established LM diagnosis and complete clinical and endoscopy records were enrolled. They were classified into different LM types according to classification based on the side of supraglottic obstruction. One hundred ten children were included. The most common LM appearance was type I-58 children, followed by combine types (I + II and I + III)-38. The other airway malacia were found in 47 patients: TM in 31, BM in 10, and TM with BM in 6. Other comorbidities (cardiac, neurological, and genetic disorders) were identified in 30 children. Patients with combine types of LM differ from those with single type of LM in terms of prematurity (13 vs 31 %, p = 0.04) and higher weight on the examination day (p = 0.006). Patients with other airway malacia differ from children with isolated LM in terms of prematurity (40 vs 13 %, p = 0.008), comorbidities (38 vs 19 %, p = 0.024), and lower weight on the examination day (p = 0.014). The prevalence of clinically relevant LM was one in 2600-3100 newborns. Clinical course of LM cannot be anticipated on the basis of solely endoscopic evaluation of the larynx. Comorbidities and prematurity increase the risk of other airway malacia. The prevalence of LM is relatively high in the middle-south part of Poland., Competing Interests: The authors declare that they have no conflict of interest. Funding This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. Ethical standards All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. Informed consent This study was retrospective, and formal consent is not required.

Background: Topical lidocaine has been found to result in overestimation of the severity of laryngomalacia in infants undergoing flexible bronchoscopy under conscious sedation with midazolam and nalbuphine. This effect has never been confirmed and may depend on the level of sedation and the drugs used. We assessed the effect of topical lidocaine on laryngomalacia in infants undergoing flexible bronchoscopy under general anesthesia with propofol., Methods: Thirteen infants with congenital stridor referred to diagnostic flexible video-bronchoscopy were studied under propofol anesthesia before and 3 minutes after topical lidocaine administration to the larynx at a dose of 3 mg/kg body weight. Laryngomalacia was scored using 60 seconds video recordings of the larynx before and after lidocaine administration in random order by 2 independent blinded observers using the previously described arytenoid score (AS), epiglottis score (ES), and the total score (TS=AS+ES)., Results: No significant differences in AS, ES, and laryngomalacia score were found between the prelidocaine and postlidocaine assessments by the 2 raters. The intraclass correlation coefficients were 0.995 (95% confidence interval, 0.986-0.998) and 0.975 (0.930-0.991) and 0.989 (0.971-996) for AS, ES, and TS, respectively., Conclusions: The assessment of laryngomalacia is not affected by topical lidocaine under propofol anesthesia. The lidocaine effect on laryngomalacia may vary depending on the medication regime used and the depth of procedural sedation.

Importance: Supraglottoplasty (SGP) failure is frequently attributed to coexistent medical comorbidities, but studies specifically evaluating outcomes in these populations are lacking., Objective: To assess SGP outcomes in patients with neurologic and syndromic comorbidities and severe laryngomalacia (LM)., Design, Setting, and Participants: Case series with retrospective review of medical records of 54 patients with neurologic and/or syndromic comorbidity and severe LM who underwent SGP between 2004 and 2012 at a tertiary care pediatric institution., Interventions: Patients presented with severe LM that required SGP. Supraglottoplasty failure necessitated revision SGP, tracheostomy, or gastrostomy tube insertion, or LM and obstructive sleep apnea that required assisted ventilation (continuous positive airway pressure and bilevel positive airway pressure)., Main Outcomes and Measures: Medical records were reviewed with a focus on patient factors, surgical timing, complications, and surgical and dysphagia outcomes. Patients were grouped based on their age at the time of SGP as infants (aged ≤12 months) and children (aged >12 months). Statistical comparisons were performed with SGP outcomes of infants with LM and no comorbidities., Results: Fifty-four patients met the inclusion criteria. Thirty-one (13 infants, 18 children) had a neurologic condition and 23 (15 infants, 8 children) had syndromes. The overall success rate of SGP was 67% (36 of 54) in these populations. Neurologic (P = .003) and syndromic (P < .001) comorbidities were associated with significant reduction in SGP success rates vs no comorbidities. Among SGP failures (18 of 54 [33%]), 13% (7 of 54) required tracheostomy, 9% (5 of 54) needed assisted ventilation, 7% (4 of 54) required a postoperative gastrostomy tube, and 4% (2 of 54) required revision SGP. In the neurologic comorbidities group, patients with cerebral palsy had significantly higher tracheostomy rates compared with those who had other neurologic pathologies constituting comorbidities (2 of 11 [18%] vs 0 of 20; P = .049). In infants, acute airway obstruction was the most common indication for SGP in the neurologically comorbidity and syndrome populations (success rates, 69% and 67%, respectively). In children, obstructive sleep apnea was the most common indication for SGP in the neurologic comorbidity and syndrome populations (success rates, 78% and 50%, respectively)., Conclusions and Relevance: Supraglottoplasty remains useful and outcomes were better in patients with neurologic comorbidity than in patients with syndromic comorbidity.

Importance: Laryngomalacia (LM) classically presents with stridor in early infancy but can present atypically with snoring and/or sleep-disordered breathing (S-SDB) or swallowing dysfunction (SwD). The epidemiology of these atypical presentations has not been established in the literature., Objective: To document the primary modes of presentation for LM in a consecutive series of children and to compare the characteristics of each subgroup., Design, Setting, and Participants: Retrospective case series in a single tertiary pediatric otolaryngology practice. Outpatient and surgical records were searched for patients diagnosed as having LM from 2002 to 2009. We included all children with endoscopically confirmed LM without prior documentation of the diagnosis (n = 88)., Interventions: Patients were investigated and managed according to the routine practice pattern of the senior author., Main Outcomes and Measures: The primary outcome measure was the proportion of the various primary presentations of LM. Age, sex, type of LM, management, and secondary diagnoses were also collected. Subgroup analysis was performed., Results: Of 117 potentially eligible patients identified, 88 children had a confirmed diagnosis of LM and were thus included (1.9:1 male to female sex ratio; mean [SD] age, 14.5 [23.0] months; age range, 0.2-96.0 months). Fifty-six children (64%) presented primarily with awake stridor and variable respiratory distress; 22 (25%) with S-SDB; and 10 (11%) with SWD. The difference in mean (SD) age for each group was statistically significant by analysis of variance: stridor, 3.5 (2.8) months; S-SDB, 46.0 (27.2) months; and SwD, 4.8 (4.6) months (P < .001). By χ2 analysis, sex distribution was not significantly different between subgroups (P = .29), nor was the proportion of children who underwent supraglottoplasty (P = .07). The difference in proportion of types of LM between the stridor and atypical presentations was statistically significant (χ2P < .05), with type 1 LM predominating in children presenting with S-SDB., Conclusions and Relevance: Because LM may present primarily with S-SDB and SwD in a significant proportion of children, the diagnosis must be considered in patients presenting with these upper airway complaints. The morphologic type of LM may vary by presentation.

Objective: To identify the prevalence of and risk factors for central sleep apnea (CSA) in infants who are diagnosed with laryngomalacia., Study Design: Case series with chart review., Setting: Quaternary care pediatric hospital., Subjects and Methods: We performed a chart review in infants with laryngomalacia. All infants had diagnostic polysomnography (PSG) performed from 2003 to 2012. Infants who underwent supraglottoplasty or other upper airway surgery prior to PSG were excluded. CSA was defined as central apnea index ≥ 5. Demographic data, underlying diseases, and PSG data were reviewed and analyzed., Results: Fifty-four patients met the inclusion criteria. The mean age at the date PSG was performed was 3.4 ± 2.7 months. The prevalence of CSA in infants with laryngomalacia was 46.3%. Odds ratio (OR) of CSA was above 2.0 in patients with the following risk factors: underlying neurologic disease, hypotonia, or syndrome (OR = 2.5, P = .13), history of apparent life-threatening events (OR = 2.7, P = .19), premature infants (OR = 2.2, P = .33), and age less than 3 months (OR = 2.3, P = .15). However, none of the risk factors were statistically significant. Analysis of sleep architecture revealed a decrease in total sleep time (345.4 ± 70.6 minutes vs 393.5 ± 68.3 minutes, P = .02) and sleep efficiency (67.7 ± 8.9% vs 75.2 ± 9.3%, P = .004) in the CSA group., Conclusion: CSA is relatively common in infants with laryngomalacia. There seems to be a higher prevalence of CSA in infants with certain risk factors, but none of the risk factors are statistically significant. The presence of CSA can lead to alteration in sleep architecture. In addition to clinical evaluation, polysomnography may be warranted for the evaluation of infants with laryngomalacia and associated complex medical conditions.

Objective: Soto's syndrome is a genetic disorder caused by mutations in the NSD1 gene. It is characterized by excessive growth in early life. It features craniofacial abnormalities, developmental delay, hypotonia and advanced bone age. A review of the current literature reveals only chronic otitis media and conductive hearing loss as otolaryngologic manifestations of Soto's syndrome. Our objective was to determine if there are additional manifestations relevant to the otolaryngologist., Methods: We performed a retrospective case series in which the Department of Defense electronic medical record was searched for ICD 9 code 253.0 (acromegaly/gigantism). Records were reviewed for genetic testing indicative of Soto's syndrome. These records were further analyzed for evidence of otolaryngologic problems., Results: Seventeen patients were identified with five having confirmed NSD1 mutations consistent with Soto's syndrome. Of these, 4/5 had otolaryngologic problems such as conductive hearing loss, aspiration, laryngomalacia, obstructive sleep apnea and sensorineural hearing loss., Conclusions: Currently there is no description in the literature of these additional manifestations of Soto's syndrome. We present this case series to support the idea that an otolaryngologist should be involved in the multidisciplinary care required for these patients., (Published by Elsevier Ireland Ltd.)

Background: Laryngomalacia is the most common congenital laryngeal anomaly and is associated with several disorders including gastric reflux, sleep apnea, hypotonia and failure to thrive. Pectus excavatum (PE) is the most common chest wall deformity affecting 1-300/1000 individuals. Though many authors presume a relationship between PE and laryngomalacia, there is no published data to establish this association., Goal: To test the hypothesis that patients referred to our pediatric otolaryngology clinic for evaluation of laryngomalacia exhibit higher rates of PE than the general population., Methods: Retrospective review of prospectively enrolled children who presented with laryngomalacia (January 2008-June 2012) to a tertiary care, hospital based, pediatric otolaryngology practice. Each chart was examined for a concurrent diagnosis of pectus deformity., Results: Of the 137 laryngomalacia patients, 9 (6.6%) had documented PE. This represents a significantly increased rate of PE when compared to children without laryngomalacia (p = 0.001). Four of the 9 children with PE underwent supraglottoplasty for laryngomalacia, a significantly greater proportion than the 9/128 of the children with isolated laryngomalacia who underwent supraglottoplasty (p = 0.004)., Conclusions: This study suggests an association between laryngomalacia and PE. Pediatric otolaryngologists should be cognizant of this relationship, though further studies are needed to elucidate the nature of this association., (Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.)

Objective: To identify and appraise the evidence for an association between laryngomalacia (LM) and acid reflux through a systematic review of the existing literature., Data Sources: MEDLINE, EMBASE, the Cochrane Library, Google Scholar, and collected additional publications cited in bibliographies., Review Methods: Literature search by both authors with structured criteria to select studies evaluated for systematic review. The Oxford Centre for Evidence-Based Medicine (CEBM) guidelines were applied to assess study quality of evidence., Results: Twenty-seven studies, representing 1295 neonates with LM, were included. Levels of evidence varied from CEBM level 2a (n = 1) to 4 (n = 23). Although reflux definitions were diverse, overall reflux prevalence in this group was 59% (pooled odds ratio [OR] of 4 controlled studies = 1.15, P = .67). Further evidence supporting an association between reflux and LM included the ubiquity of acid reflux using dual-probe pH monitoring in children with LM (2 studies; n = 84), the increased prevalence of reflux in severe as compared with mild LM (3 studies; n = 237; pooled OR = 9.86, P < .0001), case series and reports of LM improvement with antireflux therapy (6 studies; n = 275), and histological evidence of reflux-related laryngeal inflammation in children with LM (2 studies; n = 18)., Conclusion: The literature shows a coexistence between acid reflux and LM, but the evidence for a causal association is limited. In view of the widespread use of antireflux treatment in LM, a randomized controlled trial of antireflux medication vs placebo appears justified.

Objectives: Protracted bacterial bronchitis is defined as the presence of more than 4 weeks of chronic wet cough that resolves with appropriate antibiotic therapy, in the absence of alternative diagnoses. The diagnosis of protracted bacterial bronchitis is not readily accepted within the pediatric community, however, and data on the incidence of bacterial bronchitis in children are deficient. The objective of this study was to determine the frequency of bacterial bronchitis in children with chronic wet cough and to analyze their bronchoscopic findings., Methods: We performed a retrospective review of charts of children who presented with chronic wet cough, unresponsive to therapy, before referral to the pediatric pulmonary clinic., Results: A total of 197 charts and bronchoscopy reports were analyzed. Of 109 children who were 0 to 3 years of age, 33 (30.3%) had laryngomalacia and/or tracheomalacia. The bronchoscopy showed purulent bronchitis in 56% (110) cases and nonpurulent bronchitis in 44% (87). The bronchoalveolar lavage bacterial cultures were positive in 46% (91) of the children and showed nontypable Haemophilus influenzae (49%), Streptococcus pneumoniae (20%), Moraxella catarrhalis (17%), Staphylococcus aureus (12%), and Klebsiella pneumoniae in 1 patient. The χ(2) analysis demonstrated that positive bacterial cultures occurred more frequently in children with purulent bronchitis (74, 69.8%) than in children with nonpurulent bronchitis (19, 19.8%) (P < .001)., Conclusions: Children who present with chronic wet cough are often found to have evidence of purulent bronchitis on bronchoscopy. This finding is often indicative of a bacterial lower airway infection in these children.

In children with persistent respiratory symptoms despite regular anti-asthma inhalation treatment, diagnostic investigations to exclude underlying disease are warranted. 124 children were prospectively enrolled, and 24-h oesophageal pH measurement and fibreoptic bronchoscopy with bronchoalveolar lavage (BAL) were performed. BAL fluid (BALF) was processed for neutrophil counting and bacterial culture. Inflammation of the respiratory mucosa was assessed. A structural abnormality of the central airways was found in 47% of subjects (40% females). In 19% of subjects, neither anatomical anomalies nor inflamed respiratory mucosa were observed, whereas in 64%, definite macroscopic mucosal inflammation was observed. Inflammation of the respiratory mucosa was associated with a significantly higher percentage of neutrophils in the BALF: median (interquartile range) 48 (14-82)% compared with 7 (0-16)% (p<0.025). A positive BALF culture was found in 62% of the infants with mucosal inflammation compared with 25% in the group without inflammation (p<0.016). 56% of the BALF samples were positive for bacterial culture. In children with persistent respiratory symptoms, nearly half have anatomical anomalies of the central airways. In 62% of the children with mucosal inflammation, a positive BAL culture and a significantly higher percentage of BALF neutrophils were detected.

The objective of the present work was to study epidemiology of congenital stridor as a leading symptom of laryngeal malformation. The continuous sampling method was employed to perform the retrospective analysis of the growth charts of the patients attending three children's polyclinics in Moscow (9.625 patients born between 2005 and 2009). In addition, the medical histories of 4.623 newborn and breast-fed babies under the age of 1 year admitted to the Department of Newborn and Neonatal Pathology, Saint Vladimir City Children's Clinical Hospital, and 347 patients of the Department of Reconstructive Laryngeal Surgery were analysed. The children with the history of tracheal intubation in the preceding period were excluded from the study. The frequency of congenital stridor annually diagnosed in the aforementioned polyclinics varied from 0.17 to 5.8% compared with 1.5% in the general population. It was 2.21 to 3.14% (mean 2.47%) among the children treated at the Clinical Hospital. In the children under the age of 1 year, congenital malformations accounted for 90.8% of all laryngeal diseases. The principal cause of stridor was shown to be laryngomalacia. This pathology was diagnosed in 91.9% of the cases included in this study. In 11.2% of the patients, this condition occurred in combination with other congenital pathologies. It is concluded that the diagnosis of congenital stridor is an indication for laryngeal endoscopy regardless of the children's age starting from the first day of life. Meeting this recommendation allows the cause of stridor to be established and the treatment strategy to be developed on an individual basis.

Introduction: The vast majority of infants in published studies regarding laryngomalacia are Caucasian. These studies suggest affected infants are likely to be male, of term pregnancies, and not of low birth weight. Our study seeks to identify possible associations among different genders and ethnicities with laryngomalacia as well as differences between our diverse population and published Caucasian-predominant studies., Methods: Data was collected by chart review for seventy-eight children diagnosed with laryngomalacia before the age of 5, prospectively entered into a database at our academic, tertiary-care hospital from 1/16/2008 to 10/15/2010. Data was compared to expected values from the 2009 census data for the Bronx as well as published data in the literature for multiple factors, including gender, ethnicity, low birth weight, and prematurity., Results: There were 45 male and 33 female infants in our study. Twenty-five children were African-American, 17 Hispanic, 12 multi-racial, and 4 Caucasian. Eighteen infants were premature, and 21 were of low birth weight. Twenty-eight percent of our infants had neurologic comorbidity at diagnosis and 30% received surgical treatment., Discussion: Although larger studies are required, we have preliminary evidence that information learned from Caucasian-predominant studies may not apply to all patients of laryngomalacia. When ethnically diverse infants are represented, a strong association between male gender and laryngomalacia does not appear to exist. Our data suggests that premature African-American and Hispanic infants are at greater risk for laryngomalacia. In addition, African-American infants of all gestational ages may be at greater risk. Low birth weight may be a strong predictor of laryngomalacia regardless of the patient's gender or ethnicity., (Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.)

Objective: To document the prevalence of secondary airway lesions (SALs) among children with laryngomalacia (LM)., Study Design: A cross-sectional observational study., Setting: Single practice in a tertiary care pediatric facility., Subjects and Methods: Patients diagnosed with LM were initially identified from a prospectively kept surgical database. Patients with cardiac/large-vessel malformations, prematurity, repeated or prolonged intubations, acute infections, or prior airway surgery were excluded. Only children who had a full examination of the airway were included. Other associated diagnoses along with demographics, surgical intervention, and type of LM were included., Results: Over an 8-year period, 1112 patients underwent laryngoscopy and bronchoscopy. A total of 108 consecutive cases were diagnosed with LM (78 primary diagnosis and 30 secondary diagnosis). After excluding 17 with disorders associated with large airway disease, 91 LM patients were included. The mean age was 553.23 days (ranging between 5 days and 15 years); 60 were boys and 31 were girls. The prevalence of SALs was 7.7% (95% confidence interval, 7.65-7.76; 7 children; 4 subglottic stenosis, 4 tracheomalacia, 1 bronchomalacia, and 1 choanal atresia). Fifty-nine patients underwent supraglottoplasties, while 1 patient had a tracheostomy., Conclusions: After accounting for conditions known to be associated with large airway lesions, the prevalence of SALs associated with laryngomalacia was much less than previously reported. The authors hypothesize that the upper-end figures are a reflection of highly select cohorts and the inclusion of patients with other pathology known to be associated with subglottic stenosis, tracheomalacia, and bronchomalacia and low thresholds for diagnosing mild subglottic stenosis.

Objective: To describe the presentation, diagnosis, and treatment of late-onset laryngomalacia in children with obstructive sleep apnea syndrome (OSAS)., Design: Retrospective study., Setting: Tertiary care children's hospital., Patients: Seventy-seven children were identified who had OSAS diagnosed by polysomnography and underwent airway endoscopy to evaluate for laryngomalacia between July 2006 and December 2008. Children with significant neurologic disease or craniofacial malformations were excluded. Seven children under 3 years of age had laryngomalacia and OSAS (Group A), 19 children 3-18 years of age had laryngomalacia and OSAS (Group B), and 51 children 3-18 years of age had OSAS but not laryngomalacia (Group C)., Main Outcome Measures: Comparison of pre-operative findings, intra-operative findings, interventions, and outcomes between the 3 groups., Results: Group A was consistent with previous reports of congenital laryngomalacia with respect to presentation, diagnosis, and treatment. Groups B and C had similar pre-operative findings, including a high incidence of adenotonsillar hypertrophy, and the only significant difference was the intra-operative finding of laryngomalacia in Group B. Treatments were individualized to include supraglottoplasty, adenoidectomy, tonsillectomy, adenotonsillectomy, or a combination of the above. Of the 52 patients who returned in follow-up, 44 noted improvement, but this was rarely confirmed by polysomnogram., Conclusions: Late-onset laryngomalacia may act alone or in concert with additional dynamic or fixed lesions to cause pediatric OSAS. Although there is no specific pre-operative indicator to diagnose late-onset laryngomalacia, it can be readily identified intra-operatively and effectively treated with supraglottoplasty, with or without concurrent adenotonsillectomy., (Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.)

Purpose of Review: Laryngomalacia is a well described cause of newborn stridor. The spectrum of disease presentation, progression, and outcomes is varied. Some infants will have inconsequential stridor where others will develop feeding symptoms or even severe or life-threatening complications of airway obstruction. The purpose of this review is to identify factors that influence disease severity and outcomes., Recent Findings: Review of the current literature and a prospectively collected database shows that patient factors that influence disease severity include APGAR scores, resting SAO2 level at time of presentation, and the presence of a secondary airway lesion. Gastroesophageal reflux disease (GERD) and laryngopharyngeal reflux (LPR) contribute to feeding symptoms. Medical co-morbidities of GERD/LPR, neurologic disease, congenital heart disease, a genetic syndrome, or anomaly also contribute to disease severity and additively affect surgical outcomes. Those with severe disease requiring surgery who have GERD/LPR and one additional co-morbidity are more likely to require revision supraglottoplasty. Those with three medical co-morbidities are more likely to require tracheostomy., Summary: Most with laryngomalacia will have mild to moderate symptoms and not require surgical intervention. Those with GERD/LPR have symptoms improvement from acid suppression therapy. Those with severe enough disease to require supraglottoplasty will have minimal complications and good outcomes if multiple medical co-morbidities are not present. Those with multiple medical co-morbidities could be offered supraglottoplasty as the first intervention but counseled that tracheostomy may be required.

Objective: To determine the prevalence of laryngomalacia among children presenting with symptoms of sleep-disordered breathing (SDB)., Method: A retrospective observational study was conducted at a tertiary care paediatric hospital. All children presenting with SDB during a 55-month period were investigated using sleep nasopharyngoscopy (SNP). Patients who had laryngomalacia were identified. Patients who did not present primarily with SDB, or were not examined with SNP were excluded. Data for analysis was collected from a prospectively kept surgical database and medical records. This included patients' demographics, symptoms (including symptoms in infancy), diagnoses, SNP findings, overnight pulse oximetry findings, and treatment., Results: We identified 358 patients with documented primary diagnosis of SDB and who had undergone SNP. Fourteen of these also had a documented diagnosis of laryngomalacia, giving a prevalence rate of 3.9%. Three children were syndromic, and one had cerebral palsy in addition to SDB and laryngomalacia. Three children were obese, and three children had gastroesophageal reflux disease. Seven cases (50%) had symptoms of snoring and/or swallowing dysfunction and/or stridor in infancy. Twelve patients had adenotonsillar surgery. In eight cases symptoms resolved completely with adenotonsillar surgery only. In total, six patients had a supraglottoplasty. There were three failures to supraglottoplasty., Conclusion: The prevalence of laryngomalacia within children presenting with SDB is 3.9%. Our findings support full evaluation of the airway to identify the site of pathology mediating SDB symptoms.

Objective: To determine if age and comorbid conditions effect outcomes in children undergoing supraglottoplasty for severe laryngomalacia., Design: Retrospective study., Setting: Urban tertiary-care children's hospital., Patients: Children undergoing supraglottoplasty for severe laryngomalacia between February 2004 and July 2008. 56 patients were identified., Outcome Measures: Persistence of upper airway obstruction, revision surgery (supraglottoplasty), and additional surgery (tracheostomy)., Results: 33/56 (58.9%) patients had no comorbid conditions and 23/56 (41.1%) patients had comorbid conditions. In noncomorbid patients, 36.4% of those less than 2 months of age at the time of surgery required revision supraglottoplasty, compared to 5.3% of patients between 2 and 10 months (p<0.05). Compared to the 2-10-month age group, there was a significantly higher percentage of patients with comorbid conditions in the >10-month group (32.1% vs. 79%, p<0.01). Patients with comorbid conditions were diagnosed at a significantly later age than those without (6 mo vs. 2 mo, respectively), and had significantly higher rates of revision supraglottoplasty (47.8% vs. 18.2%) and tracheostomy (39.1% vs. 0.0%). 70% of children with neurological conditions required revision surgery, with 60% requiring tracheostomy. The revision surgery and tracheostomy rates were significantly higher compared to the noncomorbid group (p<0.01 and p<0.0001). Children with cardiac conditions had a higher rate of tracheostomy than noncomorbid children (30% vs. 0%, p<0.01). 16.7% of children with genetic conditions required supraglottoplasty, and none required tracheostomy., Conclusions: In noncomorbid patients, those undergoing supraglottoplasty less than 2 months of age had a significantly higher rate of revision supraglottoplasty. Patients with neurologic and cardiac comorbidities require tracheostomy at a significantly higher rate than noncomorbid patients., (Copyright 2009 Elsevier Ireland Ltd. All rights reserved.)

Objective: To quantify the prevalence and the impact of synchronous airway lesions identified by endoscopy in infants undergoing supraglottoplasty for severe laryngomalacia (LM)., Design: Retrospective study., Setting: Tertiary care pediatric hospital., Patients: Sixty patients who underwent supraglottoplasty for severe LM from 2002 to 2006. Patients who underwent preoperative tracheotomy, had previous airway surgery, or did not have 6 months of follow-up were excluded. Fifty-two patients met inclusion criteria., Intervention: Supraglottoplasty (with carbon dioxide laser)., Main Outcome Measures: Presence of synchronous airway lesions and their contribution to upper airway obstruction (UAO) and their effect on the postoperative course after supraglottoplasty., Results: Fifty-eight percent of patients had synchronous airway lesions (SALs), of whom 77% had subglottic stenosis (SGS) and 47% had tracheomalacia, bronchomalacia, or both. Sixty-three percent of all patients required postoperative nonsurgical airway support. Eight patients had residual UAO requiring additional surgical intervention, with 3 revision supraglottoplasties and 7 tracheotomies performed. Infants with neurological conditions had a high rate of surgical intervention (55%; P = .001). Patients with SGS exceeding 35% but without any neurological condition had a prolonged hospital stay (>3.6 days; P = .02) and an 83% incidence (P = .04) of postoperative UAO requiring intubation. Infants with LM with laryngeal edema (LE) alone had increased frequency of postoperative nonsurgical airway support (P = .02) and a prolonged hospital stay of 1 day (P = .01) compared with infants without edema., Conclusions: There is a high incidence of SALs in patients undergoing supraglottoplasty. Neurological conditions, hypoplastic mandible, SGS greater than 35%, and preexisting LE independently adversely affected the postoperative course.

Objective: To compare bleeding after partial intracapsular tonsillectomy (PIT) and bipolar diathermy tonsillectomy (BDT)., Design: Retrospective chart review., Setting: Stollery Children's Hospital, tertiary pediatric referral centre., Patients: All children (