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.

The changing face of paediatric airway endoscopic surgery: An 8-year single surgeon review.

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.

Coblation supraglottoplasty: a ten-year experience in a tertiary referral hospital.

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.

Association Between Neonates With Laryngomalacia and Neonatal Abstinence Syndrome.

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.

Synchronous airway lesions in children with severe, progressive and atypical laryngomalacia - Experience of a UK tertiary referral centre.

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.

Infantile tremor syndrome and laryngomalacia: A novel association?

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.

Pharyngomalacia diagnosed by laryngo-tracheo-bronchoscopy in the neonatal intensive care unit.

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.

The prevalence of swallowing dysfunction in children with laryngomalacia: a systematic review.

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.

Prevalence of Laryngomalacia among Young Children Presenting with Stridor in a Tertiary Care Hospital.

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.

Detecting respiratory viruses in children with protracted bacterial bronchitis.

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.

Prevalence of voice disturbances in pediatric airway patients: Are we missing anything?

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.

Common causes of congenital stridor in infants.

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.

Types of laryngomalacia in children: interrelationship between clinical course and comorbid conditions.

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.

Topical Lidocaine Does Not Exaggerate Laryngomalacia in Infants During Flexible Bronchoscopy Under Propofol Anesthesia.

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.

Conocimientos de los pediatras sobre la laringomalacia: ¿siempre es un proceso banal? / Pediatrician knowledge about laryngomalacia: is it always a banal process?

Introducción y objetivo: se diseñó una encuesta nacional para analizar el grado de conocimientos teóricos y la actitud de los pediatras ante la laringomalacia por la percepción de que algunos casos graves son infravalorados. Material y método: estudio transversal, descriptivo y analítico, de ámbito nacional, mediante encuesta online. Incluyó pediatras del área hospitalaria y Atención Primaria, diseñándose un cuestionario de 16 preguntas. Las variables recogidas fueron filiación, conocimientos generales, diagnóstico y manejo de la laringomalacia. Resultados: se contestaron 233 encuestas. La actitud mayoritaria ante un caso de laringomalacia era expectante (54,1%), no existiendo diferencias en función de la experiencia de los pediatras, ni por años trabajados ni por número de casos de laringomalacia atendidos. El grado de conocimientos generales de los encuestados fue superior al 89%, disminuyendo al 57% en conocimientos referidos a las laringomalacias graves. El 67% conocía las pruebas complementarias a aplicar en los casos graves y el 73,3% las posibles comorbilidades. El 85,6% coinciden que la ventilación no invasiva puede ser útil en pacientes con laringomalacia grave. Conclusiones: la encuesta muestra que la laringomalacia es una patología conocida, aunque existe disparidad en la actitud inicial a seguir y en las herramientas que utilizan los pediatras para basar su manejo. A pesar de que el grado de conocimiento general conceptual y diagnóstico es adecuado, este disminuye en conceptos más específicos que engloban a los casos de laringomalacia grave. Sería deseable la elaboración de guías clínicas y protocolos para estandarizar el manejo de la laringomalacia (AU)

Background and aims: a national survey was designed to analyze the knowledge and approach of pediatricians to laryngomalacia, due to the perception that some severe cases were overlooked. Materials and methods: this is a national, transversal, descriptive and analytical study, conducted through an on-line survey. We designed a 16 item questionnaire, and targeted both primary care and specialty pediatricians. The variables included professional data filiation, general knowledge, diagnosis and management of laryngomalacia. Results: 233 surveys were answered. The most common approach in a case of suspected laryngomalacia was expectant (54.1%), no differences were found in terms of pediatricians experience, neither by years of time worked, nor by the number of laryngomalacia cases previously assisted. Level of general knowledge shown by participants was higher than 89%, while it decreased to 57% when analyzing items covering severe laryngomalacia. 67% were aware of the additional tests to perform in severe cases, and 73.3% were alert for possible comorbidities. 85.6% agreed that non-invasive ventilation is useful in patients with severe laryngomalacia. Conclusions: the survey shows that laryngomalacia is a well-known condition, although there is not a clear consensus in the initial approach nor in the tools used by pediatricians to base its management. Though the knowledge in general concepts and diagnostics is suitable, it decreases in more specific concepts related to cases of severe laryngomalacia. It would be desirable that clinical guidelines and protocols were developed, to standardize management of laryngomalacia (AU)

Supraglottoplasty outcomes in neurologically affected and syndromic children.

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.

Primary Presentations of Laryngomalacia.

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.

Prevalence and risk factors for central sleep apnea in infants with laryngomalacia.

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.

Pectus excavatum in children with laryngomalacia.

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.

The otolaryngologic manifestations of Sotos syndrome.

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.