Infant Pulmonary Function Tests in Children with Airway Anomalies and Correlation with Bronchoscopy Findings.

OBJECTIVES: To evaluate the role infant pulmonary function tests (Tidal Breathing Flow Volume Loops, TBFVL) in children with airway anomalies and to correlate the TBFVL so obtained with bronchoscopy findings. METHODS: In this prospective cohort study, we enrolled children aged 0-2 years with airway anomalies and performed TBFVL and bronchoscopy. The primary outcome measure was graphic pattern of TBFVL in laryngomalacia. Secondary outcome measures were types of TBFVL results in various airway anomalies and controls. RESULTS: Out of 53 children enrolled, 28 (52.3%) had laryngomalacia. Pattern 3 (fluttering of inspiratory limb) was commonest TBFVL pattern in laryngomalacia. Among TBFVL parameters, the ratio of inspiratory time to expiratory time (Ti/Te) and tPTEF/tE was significantly high in children with isolated laryngomalacia compared to controls. At six months of follow-up, TBFVL pattern 1 (normal) became the commonest pattern. CONCLUSION: A particular type of airway anomaly may have a characteristic graphic pattern in TBFVL and TBFVL pattern may indicate improvement in airway anomalies in follow-up.

Flexible Endoscopic Evaluation of Swallowing in Breastfeeding Infants With Laryngomalacia: Observed Clinical and Endoscopic Changes With Alteration of Infant Positioning at the Breast.

OBJECTIVES: This retrospective cohort study uses endoscopic assessment of the pharyngeal phase of swallowing in infants with laryngomalacia, to ascertain the impact of infant positioning on airway compromise and fluid dynamics during breastfeeding. The study aims to identify whether modification of infant positioning at the breast may improve the possibility of safe, successful breastfeeding in infants with laryngomalacia and concurrent breastfeeding difficulty. METHODS: Twenty-three infants referred for noisy breathing and difficulty feeding were assessed with flexible endoscopic evaluation of swallowing (FEES) during breastfeeding. All had endoscopically confirmed laryngomalacia. During FEES, observations were made of clinical signs of airway compromise as well as endoscopically observable anatomical features and swallowing dynamics during breastfeeding, including tongue base position, view of laryngeal inlet and vocal folds, dynamic supraglottic soft tissue collapse, timing of milk flow into pyriform fossae/hypopharynx relative to sucking, and presence of penetration and/or aspiration. If airway and/or swallowing compromise was present, the infant's initial position at the breast was altered from supine or semi lateral decubitus position to semi-prone, with a description of the clinical and endoscopically observable changes that subsequently occurred. RESULTS: Signs of dynamic airway obstruction and/or compromised airway protection with swallowing were present in 20 of the 23 infants (87%) in their initial supine or semi lateral decubitus position. These 20 infants were repositioned to semi-prone, with improvement and/or resolution of stridor and an improved ability to maintain latch in all infants. Continued endoscopic evaluation following positional change was possible in 16 infants, identifying anterior positioning of the tongue base, reduced dynamic supraglottic tissue collapse, reduced volume of milk flow into pyriform fossae during pauses in sucking and resolution of penetration and aspiration. CONCLUSION: This study has shown how alteration of breastfeeding position to semi-prone may improve dynamic airway obstruction and reduce aspiration risk in infants with laryngomalacia.

Laryngomalacia and Swallow Dysfunction.

OBJECTIVES: Laryngomalacia is an established cause of stridor and sleep-disordered breathing in children. However, the relationship between laryngomalacia and dysphagia has not been well characterized. The objectives of this study were to (1) describe the patient characteristics, symptoms, and prevalence of dysphagia in children with laryngomalacia and (2) examine the effectiveness of supraglottoplasty in improving feeding. METHODS: This was a retrospective study of patients with laryngomalacia who underwent a modified barium swallow study (MBSS) at a tertiary academic pediatric medical center between March 1, 2014, and March 1, 2018. Patients were excluded if they did not undergo a MBSS. Comorbidities, airway and feedings symptoms, MBSS results, and surgical history were recorded from each patient's electronic medical record. RESULTS: Forty-four children met inclusion/exclusion criteria. The median age at presentation was 96 days. There was a male predominance (66%). About one-third had a genetic or neuromuscular comorbidity. Most children had stridor (93%) and feeding difficulty (86%), while 50% had parent-reported poor weight gain. Fifty-seven percent of patients had evidence of penetration or aspiration on MBSS. All patients with a positive MBSS had dysphagia symptoms. Fifty-seven percent of patients underwent supraglottoplasty. Postoperatively, 92% reported improvement in dysphagia symptoms. CONCLUSION: Dysphagia is prevalent among a subset of children with laryngomalacia. Symptomatic children may benefit from a swallow evaluation to help determine the need for further intervention. Children who undergo supraglottoplasty for laryngomalacia have improved dysphagia at follow-up, though the amount of improvement directly attributable to surgery is unclear and warrants further investigation.

Laryngomalacia, Tracheomalacia and Bronchomalacia.

Airway malacia can occur in the larynx (larygomalacia), trachea (tracheomalacia), or bronchi (bronchomalacia). As a group these are the most common congenital abnormalities of the pediatric airway and are characterized by increased airway compliance, resulting in excessive dynamic collapse during the respiratory cycle. While a diagnosis can be suspected based on clinical history and physical examination, definitive evaluation is based of nasopharyngolaryngoscopy and/or bronchoscopy. Observation and conservative management are typically all that are required. However, surgical intervention can be necessary in the most severe cases, and can result in significant improvement in symptoms.

Accuracy of clinical swallowing evaluation for diagnosis of dysphagia in children with laryngomalacia or glossoptosis.

OBJECTIVE: To investigate the accuracy of clinical evaluation of swallowing in a sample of children with laryngomalacia or glossoptosis and describe the prevalence of dysphagia in each of these diseases, as well as characterize the swallow response to speech and language therapy interventions. STUDY DESIGN: Children aged 1 month to 11 years receiving care at the Department of Otolaryngology, Hospital de Clínicas de Porto Alegre, Brazil, were evaluated in a cross-sectional design. Evaluation of swallowing was performed at two time points by two blinded speech-language pathologists, one responsible for clinical evaluation and the other for videofluoroscopic study. The protocols employed were based on the instruments proposed by DeMatteo et al. (DeMatteo C, Matovich D, Hjartarson A. Comparison of clinical and videofluoroscopic evaluation of children with feeding and swallowing difficulties. Dev Med Child Neurol 2005;47:149-157.). RESULTS: The study sample consisted of 29 patients: 10 patients with laryngomalacia and 19 patients with glossoptosis. The sensitivity of clinical evaluation did not exceed 50% in any of the evaluations, but specificity reached 100% in some cases, using thickened liquids. The prevalence of dysphagia was 100%, and the use of thickened liquids significantly reduced tracheal aspiration. CONCLUSIONS: Dysphagia was highly prevalent in this sample. The sensitivity of clinical evaluation to detect laryngeal penetration and tracheal aspiration was low, as the majority of aspiration events were silent. The videofluoroscopic study is important in order to determine a safest method to feed the patient. Pediatr Pulmonol. 2017;52:41-47. © 2016 Wiley Periodicals, Inc.

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.

A systematic review of the evidence on spontaneous resolution of laryngomalacia and its symptoms.

INTRODUCTION AND OBJECTIVES: Laryngomalacia (LM) is the most common cause of congenital stridor. Tradition holds that the majority of patients resolve spontaneously by 12-18 months of age. The objective of this study was to systematically review the literature on the spontaneous resolution of LM and/or its presenting symptoms, in otherwise healthy infants. METHODS: Data sources included Medline/PubMed, EMBASE, Scopus, CINAHL, Proquest, Cochrane database, Cochrane Methodology Register, Web of Science Conference Proceedings Citation Index, and ACP Journal Club. Study inclusion criteria included participants with an endoscopic diagnosis of LM, and symptoms of stridor, swallowing dysfunction, and/or snoring/sleep-disordered breathing, that documented subjective or objective resolution endpoints. Surgical series, case reports, and narrative reviews were excluded. Studies with insufficient follow-up (<3 months), and patients with comorbidities without subgroup analysis were excluded. Two independent reviewers extracted follow-up duration, rate of retention, time to resolution of LM, and method of documentation of resolution. RESULTS: Of the 1146 articles identified, three met inclusion/exclusion criteria (n=411 patients). All were retrospective and used stridor resolution as the only endpoint. Resolution rate was 89%, with time to resolution ranging from 4 to 42 months. Level of evidence was low, and studies suffered from lack of follow-up, and no objective endpoints. Meta-analysis was not possible. CONCLUSIONS: Endoscopic evidence of the natural history of laryngomalacia and its resolution is lacking. Low level of evidence supports that stridor and respiratory distress resolve, but the range of the time to resolution and rate of resolution is wide. Other clinical manifestations have not been studied. Prospective longitudinal trials are required to better understand the natural history.

Laryngomalacia and swallowing function in children.

OBJECTIVES/HYPOTHESIS: 1) To determine the prevalence of dysphagia in children with laryngomalacia, 2) To ascertain whether severity of laryngomalacia influences the presence of swallowing dysfunction, and 3) To examine whether patients with medical comorbidities and laryngomalacia have a higher prevalence of swallowing dysfunction. STUDY DESIGN: Retrospective cohort study. METHODS: All patients seen in the aerodigestive center at our institution between January 2007 and December 2012 with the diagnosis of laryngomalacia were included. Swallowing function was assessed by symptoms, clinical swallowing evaluations (CSE) performed by speech pathologists, modified barium swallow (MBS) studies, and fiberoptic endoscopic evaluations of swallowing (FEES). RESULTS: There were 324 patients with laryngomalacia identified (41.4% female, 58.6% male). Severity of laryngomalacia was categorized in 279 patients, with 62.7% mild, 28.7% moderate, and 8.6% severe. Gastroesophageal reflux disease (GERD) was diagnosed in 69.8% of patients. Other medical comorbidities included Down syndrome (3.1%), neurological impairment (6.5%), and congenital heart disease (0.9%). Symptoms of dysphagia or feeding difficulty were present in 163/324 (50.3%), and failure to thrive was present in 31/324 patients (9.6%). At least one abnormal swallowing assessment was present in 97/120 (80.8%) patients presenting with subjective dysphagia and 43/65 (66.2%) patients without subjective dysphagia. A total of 140/185 (75.7%) patients had at least one abnormal baseline swallowing assessment. There was no significant relationship between severity of laryngomalacia and presence of abnormal swallowing function based on symptoms, CSE, MBS, or FEES. However, patients with greater severity were more likely to have failure to thrive. There was not a significant association between the presence of swallowing dysfunction or disease severity and medical comorbidities such as Down syndrome, neurological impairment, or congenital heart disease. However, GERD was more likely to be present in patients with moderate and severe laryngomalacia than in patients with mild disease. CONCLUSIONS: Swallowing dysfunction is common in children with laryngomalacia regardless of disease severity or other medical comorbidities. Swallowing studies are frequently abnormal in laryngomalacia patients presenting both with and without subjective symptoms of dysphagia. Dysphagia assessment should be considered as part of the evaluation of infants with laryngomalacia. LEVEL OF EVIDENCE: 4.

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.

Supraglottoplasty in Infants: A Staged Approach.

OBJECTIVE: While generally well tolerated for the treatment of severe laryngomalacia, bilateral supraglottoplasty has potential complications including supraglottic stenosis and aspiration. We report a more conservative staged supraglottoplasty in infants with severe laryngomalacia. METHODS: A retrospective review was performed of our patients who underwent staged supraglottoplasty from June 2007 to June 2012. Fifteen infants were identified and scored based on stridor, retractions, oxygen saturation, and feeding quality. Outcomes were compared with those reported in the literature for conventional bilateral supraglottoplasty. RESULTS: Seventy-three percent had significant improvement or resolution of stridor following the first stage of surgery and 100% in those undergoing a second stage. Twelve patients (80%) had mild to no retractions following one procedure and 100% had resolution after a second surgery. All 6 patients with recurrent preoperative desaturations had resolution after the first stage of surgery. Of the 11 infants who had preoperative moderate-severe feeding problems, 9 of them (82%) had resolution after one surgery and the remaining 2 had resolution after a second surgery. There were no complications in any of the patients. CONCLUSIONS: Staged supraglottoplasty appears to be an effective, low-risk method to treat severe laryngomalacia. A second procedure was only required in 40% of patients.

Adult-onset laryngomalacia: case reports and review of management.

Laryngomalacia is a dynamic airway condition characterised by inward collapse of flaccid supraglottic structures during inspiration. Although the most common cause of stridor in the paediatric population, adult-onset laryngomalacia remains a rare entity and its management, challenging. Two cases of adult-onset laryngomalacia are reported. A review of the English literature is performed and additional publications identified by hand-searching relevant papers; 13 case reports/series comprising 28 cases of adult-onset laryngomalacia were identified, divided into two main groups: idiopathic (6/28) and acquired (22/28). The aetiology of the acquired form includes neurological, traumatic and iatrogenic. Reported therapeutic measures used are laser supraglottoplasty, epiglottopexy, partial epiglottidectomy, defunctioning tracheostomy and intubation whilst correcting the underlying cause. The majority of patients only required one therapeutic procedure (follow-up of 2-24 months). A strong index of suspicion is required to diagnose adult-onset laryngomalacia aided by in-office laryngoscopy. The rarity of this condition prevents management-based randomised controlled trials.

Tetrasomy 13q32.2qter due to an apparent inverted duplicated neocentric marker chromosome in an infant with hemangiomas, failure to thrive, laryngomalacia, and tethered cord.

BACKGROUND: Approximately 100 small supernumerary marker chromosomes (sSMCs) with a non-α-satellite neocentromere structure have been reported in the literature. Of the few derived from chromosome 13, five have consisted of inverted duplicated segment 13q32qter. CASE REPORT: We herein describe the sixth case, characterized by genome wide SNP array, conventional cytogenetics and FISH studies. The de novo occurrence of the marker, the poor prognosis and the presence of hemangiomas are consistent with previous cases. CONCLUSION: We hereby expand the clinical spectrum of this rare cytogenetic disorder and suggest a possible mechanism for the pathogenesis of associated congenital vascular malformations.

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.

Laryngomalacia.

Laryngomalacia is the most common cause of stridor in infants. Stridor results from upper airway obstruction caused by collapse of supraglottic tissue into the airway. Most cases of laryngomalacia are mild and self-resolve, but severe symptoms require investigation and intervention. There is a strong association with gastroesophageal reflux disease in patients with laryngomalacia, and thus medical treatment with antireflux medications may be indicated. Supraglottoplasty is the preferred surgical treatment of laryngomalacia, reserved only for severe cases. Proper identification of those patients who require medical and surgical intervention is key to providing treatment with successful outcomes.

Pathophysiology and diagnostic approach to laryngomalacia in infants.

Laryngomalacia is defined as collapse of supraglottic structures during inspiration. It is the most common laryngeal disease of infancy. Laryngomalacia presents in the form of stridor, a high-pitched, musical, vibrating, multiphase inspiratory noise appearing within the first 10 days of life. Signs of severity are present in 10% of cases: poor weight gain (probably the most contributive element), dyspnoea with permanent and severe intercostal or xyphoid retraction, episodes of respiratory distress, obstructive sleep apnoea, and/or episodes of suffocation while feeding or feeding difficulties. The diagnosis is based on systematic office flexible laryngoscopy to confirm laryngomalacia and exclude other causes of supraglottic obstruction. Rigid endoscopy under general anaesthesia is only performed in the following cases: absence of laryngomalacia on flexible laryngoscopy, presence of laryngomalacia with signs of severity, search for any associated lesions prior to surgery, discrepancy between the severity of symptoms and the appearance on flexible laryngoscopy, and/or atypical symptoms (mostly aspirations). The work-up must be adapted to each child; however, guidelines recommend objective respiratory investigations in infants presenting signs of severity.

Diagnosing aerodynamic supraglottic collapse with rest and exercise flexible laryngoscopy.

OBJECTIVE: Laryngomalacia is best known as a self-resolving infantile disorder characterized by inspiratory stridor with occlusion of the larynx by collapse of arytenoid tissues due to Bernoulli forces. Adult laryngomalacia has been sporadically described in the literature. We identified a series of patients with aerodynamic supraglottic collapse mimicking laryngomalacia in our Otolaryngology clinic. STUDY DESIGN: Case series. METHODS/PATIENTS: A series of five patients from our Otolaryngology clinic with aerodynamic supraglottic collapse presented with complaints ranging from noisy breathing to dyspnea with exertion. Diagnosis was made using rest and exercise flexible laryngoscopy. RESULTS: Symptoms resolved in all patients who underwent traditional or modified supraglottoplasty. CONCLUSIONS: These patients, all with abnormal corniculate/cuneiform motion occluding the airway during forceful inspiration, reinforce the diagnostic role of rest and exercise flexible laryngoscopy in patients with dyspnea and stridor. These results may suggest that aerodynamic supraglottic collapse is an underdiagnosed clinical entity.

Vocal cord dysfunction, paradoxic vocal fold motion, or laryngomalacia? Our understanding requires an interdisciplinary approach.

This article presents disorders of periodic occurrence of laryngeal obstruction (POLO) resulting in noisy breathing and dyspnea and a variety of secondary symptoms. Included in this classification are glottic disorders, such as paradoxic vocal fold movement and vocal cord dysfunction. The supraglottic disorder, termed, intermittent arytenoid region prolapse or laryngomalacia, is also reviewed. Three categories of POLO are defined as irritant, exertional, and psychological.