Validity of laryngomalacia classification systems: A multi-institutional agreement study.

OBJECTIVES: To determine the agreement of paediatric otolaryngologists on classifying laryngomalacia (LM). DESIGN: Intra- and interobserver agreement study of two classification systems. SETTING: Three tertiary referral paediatric centres. PARTICIPANTS: Three paediatric otolaryngologists, who were blinded to any clinical details, interpreted the videos of children diagnosed with LM using the Holinger and Olney classifications independently. They rated the videos twice with a washout period of at least 2 weeks. THE MAIN OUTCOME MEASURES: Inter- and intra-observer agreement measured by overall Fleiss kappa and unweighted Cohen's kappa coefficients. The secondary outcome measures were inter- and intra-observer agreement on the individual anatomical subunits of the supraglottis affected by LM, characterised by the subcategories of the classifications. RESULTS: Video records of infants and children <18 years who had an endoscopic diagnosis of LM from 2012 to 2017 were retrospectively chosen for inclusion (n = 73). The overall Fleiss kappa coefficient was 0.25 (95% CI 0.18-0.32) amongst the raters using the Holinger classification and 0.31 (95% CI 0.21-0.42) for the Olney classification. Intra-observer agreement using the Holinger classification was 0.30 (95% CI 0.18-0.42), 0.62 (95% CI 0.23-0.85) and 0.84 (95% CI 0.75-0.94], whilst the Olney classification yielded values of 0.41 (95% CI 0.26-0.56), 0.51 (95% CI 0.29-0.63) and 0.63 (95% CI 0.48-0.78). CONCLUSIONS: The agreement on types of LM between expert observers is modest using the Holinger and Olney classifications. This has significant implications for accurately diagnosing LM and exposes potential obstacles against credible pooling of data and extrapolation of information.

Management Outcome of Severe Laryngomalacia at Queen Sirikit National Institute Child Health.

Background: Outcomes of the different management in severe laryngomalacia (LM) have not been evaluated. Objective: To identify the management practices and to evaluate the outcomes in patient with severe LM. Material and Method: The medical records of LM at Queen Sirikit National Institute Child Health between January2007 and December 2012 were retrospectively reviewed. Results: Severe LM 69.8% (30/43) were found in patients diagnosed with LM. Type B (complete collapse) at 46.67% were the most common finding. Decision of management were made individually based on consideration of disease severity and comorbidity. The outcomes after management were evaluated by pre- and post-symptoms score. Post-symptoms scores were statistically significant better than pre-symptom score in all management (observation p<0.001, laser supraglottoplasty p = 0.003, and tracheotomy p = 0.001). Conclusion: Our management in severe LM include: observation, laser supraglottoplasty, and tracheostomy. The overall post-management outcome were satisfactory but the present study was limited to relatively small number of patients.

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.

Clinical relevance of quality of life in laryngomalacia.

OBJECTIVES/HYPOTHESIS: To examine aspects of laryngomalacia and correlate findings with quality of life (QOL). STUDY DESIGN: Prospective cross-sectional study. METHODS: Seventy-two patients with laryngomalacia were examined; the mean age was 8.8 weeks. Parents answered questions from the Infant and Toddler Quality of Life Questionnaire-47 (ITQOL-SF47). Fiberoptic laryngoscopy and endoscopic examination of swallowing (FEES) were performed. The presence of laryngomalacia-associated characteristics and swallowing status were recorded. Patient age, sex, presence of reflux, clinical severity, anatomical findings, and swallowing results were evaluated through logistic regression. Independent sample t tests were used to compare responses on the ITQOL-SF47. Overall laryngomalacia ITQOL-SF47 scores were compared to the scores of a large healthy sample population. RESULTS: Forty-three (60%) patients had mild laryngomalacia, and 61 (85%) patients had findings suggesting gastroesophageal reflux disease. The most common abnormality was shortened aryepiglottic folds. Ten patients failed FEES. Patients with moderate laryngomalacia (χ = 7.62; P = .006) or prolapsing cuneiforms (χ = 4.79; P = .029) were more likely to fail FEES. Laryngomalacia severity impacted parental perception of their child's health (P < .05). Parents of children who demonstrated aspiration or penetration reported significant emotional impact (mean = 56.9; t = 2.74; P = .008). The mean ITQOL-SF47 scores of patients were significantly lower in certain sections than the reported general sample population. CONCLUSIONS: Epiglottal prolapse correlated with severity of laryngomalacia and cuneiform prolapse with swallowing dysfunction. Perceptions of worsening health and physical ability were related to severity of disease. Swallowing dysfunction had a significant emotional impact on parental daily life. Infants with laryngomalacia have a lower QOL LEVEL OF EVIDENCE: 3 Laryngoscope, 126:1232-1235, 2016.

The groningen laryngomalacia classification system--based on systematic review and dynamic airway changes.

OBJECTIVE: Laryngomalacia is the most common cause of dyspnea and stridor in newborn infants. Laryngomalacia is a dynamic change of the upper airway based on abnormally pliable supraglottic structures, which causes upper airway obstruction. In the past, different classification systems have been introduced. Until now no classification system is widely accepted and applied. Our goal is to provide a simple and complete classification system based on systematic literature search and our experiences. STUDY DESIGN: Retrospective cohort study with literature review. METHODS: All patients with laryngomalacia under the age of 5 at time of diagnosis were included. Photo and video documentation was used to confirm diagnosis and characteristics of dynamic airway change. Outcome was compared with available classification systems in literature. RESULTS: Eighty-five patients were included. In contrast to other classification systems, only three typical different dynamic changes have been identified in our series. Two existing classification systems covered 100% of our findings, but there was an unnecessary overlap between different types in most of the systems. Based on our finding, we propose a new a classification system for laryngomalacia, which is purely based on dynamic airway changes. CONCLUSION: The groningen laryngomalacia classification is a new, simplified classification system with three types, based on purely dynamic laryngeal changes, tested in a tertiary referral center: Type 1: inward collapse of arytenoids cartilages, Type 2: medial displacement of aryepiglottic folds, and Type 3: posterocaudal displacement of epiglottis against the posterior pharyngeal wall.

[Laryngomalacia: principal cause of stridor in infants and small children]. / Laryngomalacie: cause principale de stridor chez le nourrisson et le petit enfant.

Laryngomalacia (LM) is the most common cause of congenital stridor. It is caused by obstruction of the upper airway by collapse of redundant supraglottic tissues during inspiration. In the management of a child with congenital stridor, it is important to rule out other malformations of the upper airway that could mimic or be synchronous with LM. Symptoms of LM are usually mild and disappear spontaneously by 2 years. About 20% of patients with LM may have extreme symptoms (severe stridor, feeding difficulties and growth retardation) requiring treatment by endoscopic surgery (supraglottoplasty), which has an excellent success rate with little risk of recurrence and complications.

Factors associated with the morphological type of laryngomalacia and prognostic value for surgical outcomes.

IMPORTANCE: The classic presentation of laryngomalacia (LM) is stridor, but alternate presentations include snoring and/or sleep-disordered breathing (S-SDB) and swallowing dysfunction (SWD). Several classification schemes have been developed for LM, but to our knowledge, none have been successfully investigated as to the ability to predict parameters of patients with LM or surgical outcomes. OBJECTIVE: To compare parameters of patients with different types of LM and determine whether the type has prognostic value for surgical outcomes and to explore if any variable predicts or correlates with the type of LM. DESIGN, SETTING, AND PARTICIPANTS: Retrospective case series from a single tertiary pediatric otolaryngology practice. Patients with LM treated with supraglottoplasty (SGP) were eligible. We included patients with confirmed diagnosis of LM who underwent a cold steel SGP and had complete resolution of symptoms or at least 3 months of follow-up, with complete data. INTERVENTIONS: Investigations and treatment of the patients were followed as per routine practice for the senior author (H.E.). MAIN OUTCOMES AND MEASURES: Demographics, type of LM, secondary airway lesions, secondary diagnosis, primary presentation (stridor, S-SDB, SWD), and outcome of SGP were collected. Correlation and multiple regression analysis were performed. RESULTS: A total of 125 children with LM who underwent SGP for LM were identified. Of these procedures, 119 were cold steel technique, and 8 were repeated procedures. Ninety patients met criteria and were included (mean [SD] age, 1.46 [2.34] years [range, <6 months to 15 years]; male to female ratio, 1.9:1). The primary presentation was stridor in 66 children, S-SDB in 14, and SWD in 10. The type of LM correlated significantly with age (-0.9), and presentation (0.49). Sex and presence of neurological diagnosis (correlation coefficient [SE], -0.317 [0.136], P = .02; and -0.968 [0.361], P <.01, respectively) were associated with outcome. Presentation and obesity were associated with type of LM (-0.251 [0.071], P <.001; and 0.593 [0.296], P = .048, respectively). CONCLUSIONS AND RELEVANCE: Type of LM varies by age and primary presentation. Outcome of management is poorer for males and in the presence of a neurological diagnosis. The findings of the present study may help in counseling parents on the risks and benefits of SGP surgery as well as on expected outcomes postoperatively. Further work is required in validating an existing classification scheme for LM or developing a new, validated classification system with may be used for future outcomes research.

Congenital airway lesions and lung disease.

Structural upper and lower airway disorders and parenchymal disorders are uncommon in pediatric practice, but many pediatricians will encounter them and be responsible for the ongoing care of these patients. Pediatricians need to be cognizant of these diagnoses because, even though management of these disorders generally lacks an evidence base, existing principles of good care surrounding accurate diagnosis, classifications of severity, judicious use of investigations, medication, and surgical approaches are essential to good outcomes.