Management of Obstructive Sleep Apnea in the Infant and Newborn.

Obstructive sleep apnea in newborns and infants presents a unique challenge with distinct differences in sleep physiology, etiologies, and management compared to older children. The indications for and interpretation of polysomnography are less well defined in infants. There are also no broadly accepted clinical practice guidelines for treating sleep apnea in this age group. Etiologies include general causes of upper airway obstruction in infants such as laryngomalacia, micrognathia, and nasal obstruction in addition to adenotonsillar hypertrophy. Treatment strategies must be tailored to the specific anatomic features and comorbidities of the specific patients and often require a multidisciplinary approach.

Role of upper airway evaluation in the multidisciplinary management of obstructive sleep apnea in children below two years of age.

BACKGROUND: Diagnosis and treatment of obstructive sleep apnea (OSA) in infants and young children is challenging because of its clinical heterogeneity and lack of age-specific guidelines. AIM: We report the management and treatment outcome of OSA in children below 2 years of age. Treatment decisions were based upon the pattern of upper airway (UA) obstruction, clinical presentation and OSA severity. METHODS: Retrospective, non-randomized observational cohort study at a tertiary center. Children with OSA who underwent an UA evaluation (drug-induced sleep endoscopy or direct laryngoscopy) were included. RESULTS: We studied 100 patients, 57 boys and 43 girls, age 0.72 years (0.0-2.0) and OSA confirmed by polysomnography. Multilevel UA collapse was present in 26%, (adeno)tonsillar hypertrophy in 31% and 21% had laryngomalacia. Laryngomalacia was more common in children below 6 months of age and adenotonsillar hypertrophy was observed mainly in children >1.5 year of age. Surgical and nonsurgical treatment guided by UA findings, improved OSA severity at group level with a significant reduction (p < 0.001) in obstructive apnea/hypopnea index from 10.8/h (2.1-99.1) to 1.7/h (0.0-73.0), an improvement in mean oxygen saturation from 96.9% (88.9-98.4) to 97.4% (92.3-99.0), in minimal oxygen saturation from 85.4% (37.0-96.0) to 88.8% (51.0-95.5) and oxygen desaturation index from 5.1/h (0.2-52.0) to 1.3/h (0.0-47.8). CONCLUSION: Multidisciplinary management of young children with OSA guided by the pattern of UA obstruction and OSA severity, reduces OSA severity. The pattern of UA obstruction changes in the first 2 years of life from a dynamic collapse to structural abnormalities.

[New progress in diagnosis and treatment of congenital laryngomalacia in infants].

Congenital laryngomalacia is the most common disease causing laryngeal stridor in infants. The pathogenesis has not yet been clearly concluded. It may be related to abnormal development of laryngeal cartilage anatomical structure, neuromuscular dysfunction, gastroesophageal and laryngeal reflux disease, etc. The typical manifestations of the disease are inspiratory laryngeal stridor and feeding difficulties, which can be divided into mild, moderate and severe according to the severity of symptoms. The diagnosis is mainly based on clinical symptoms, signs and endoscopy, among which endoscopy is an important diagnostic basis. The treatment of laryngomalacia depends on the severity of symptoms. Mild and some moderate congenital laryngomalacia children can be relieved by conservative treatment, and severe and some moderate congenital laryngomalacia children should be treated by surgery. Supraglottic plasty is the main surgical method, which can effectively improve the symptoms of laryngeal stridor, dyspnea, feeding difficulties and growth retardation in most children, and the surgical effect is good.

Childhood Respiratory Conditions: Stridor.

The most important issue in diagnosing stridor in infants and children is determining whether it is acute or chronic. The most common cause of acute stridor is croup, a viral infection with a straightforward diagnosis when patients have typical signs and symptoms (ie, inspiratory or biphasic stridor, barking cough, hoarse voice or cry, chest wall retractions). Management of mild and severe cases includes steroids. Patients with severe croup should undergo evaluation in an urgent care center or emergency department; hospital admission may be required. When symptoms are atypical for croup, physicians should consider other causes of acute stridor, including foreign body aspiration, bacterial tracheitis, and epiglottitis. The most common cause of chronic stridor is laryngomalacia, an abnormality in the laryngeal structures that causes the collapse of supraglottic structures over the airway. Symptoms typically develop in the first 1 to 2 months of life, are positional, and do not interfere with growth and development. However, if the diagnosis is in doubt or if the laryngomalacia is severe and the patient has cyanosis, worsening stridor after feeding, or inadequate weight gain, consultation with an otolaryngology subspecialist can be helpful. Most infants outgrow symptoms as the airway enlarges, but some may need pharmacotherapy for gastroesophageal reflux disease and careful feeding until this occurs. Others may need supraglottoplasty. Other causes of chronic stridor include vocal fold paralysis, subglottic hemangiomas, and glottic webs.

Noninvasive Respiratory Support as an Alternative to Tracheostomy in Severe Laryngomalacia.

OBJECTIVES/HYPOTHESIS: To analyze the role of noninvasive respiratory support (NRS) as an alternative to tracheostomy in the management of severe laryngomalacia. STUDY DESIGN: We conducted a monocentric retrospective study in a tertiary pediatric care center. METHODS: All children under the age of 3 years with severe laryngomalacia, treated between January 2014 and December 2019, were included. Patient demographics, medical history, nutrition, surgery, NRS, and outcome were reviewed. Predictors for NRS were analyzed. RESULTS: One hundred and eighty-eight patients were included. Mean age was 4 ± 5 months and mean weight was 4,925 ± 1,933 g. An endoscopic bilateral supraglottoplasty was performed in 183 (97%) patients and successful in 159 (87%). NRS was initiated in 29 (15%) patients at a mean age of 3 ± 2 months (1-11 months): 15 (52%) patients were treated with NRS after surgical failure, 9 (31%) were treated with NRS initiated prior to surgery because of abnormal overnight gas exchange, and 5 (17%) were treated exclusively with NRS due to comorbidities contraindicating an endoscopic procedure. NRS was successfully performed in all patients with a mean duration of 6 ± 11 months. No patient required a tracheostomy. Univariate analysis identified the following predictors of NRS: neonatal respiratory distress (P = .003), neurological comorbidity (P < .001), associated laryngeal abnormality (P < .001), cardiac surgery (P = .039), surgical endoscopic revision (P = .007), and nutritional support (P < .001). CONCLUSION: NRS is a safe procedure, which may avoid a tracheostomy in severe laryngomalacia, in particular, in case of endoscopic surgery failure, respiratory failure before surgery, and/or severe co-morbidity. LEVEL OF EVIDENCE: 3 Laryngoscope, 132:1861-1868, 2022.

High-flow nasal cannula application in an infant patient with laryngomalacia during general anesthesia: A case report.

RATIONALE: Laryngomalacia is defined as the collapse of supraglottic structures and can cause not only strider but also trigger difficulties with ventilation and endotracheal intubation during anesthesia management. High-flow nasal cannula (HFNC) has been used to manage patients at high risk of hypoxemia in the intensive care unit; however, limited literature information is available for the application of HFNC to infant patients with laryngomalacia during anesthesia practice. PATIENT CONCERNS: A 2-month-old male infant was scheduled to undergo surgery for inguinal hernia and undescended testis with general anesthesia. DIAGNOSIS: The patient had subcostal retraction while breathing and frequent oxygen desaturation events and was diagnosed laryngomalacia. INTERVENTIONS: After the patient was supplied oxygen via HFNC and then given general anesthesia, the initial 2 attempts of endotracheal intubation with a rigid laryngoscope were unsuccessful because the vocal cords were obscured by the epiglottis. A third intubation attempt was performed and successful with a 3.0-sized, uncuffed endotracheal tube within 20 minutes of the initial attempt. OUTCOMES: No airway complications emerged and oxygen saturation remained at greater than 98% during general anesthesia. The patient was discharged 5 days after surgery without any adverse side effects. LESSONS: Continuous oxygenation via HFNC is a good choice to prevent desaturation during difficult tracheal intubations in infant patients with laryngomalacia. This device is expected to be useful for intubation not only in patients with laryngomalacia, but also in infant patients with a predicted high risk of oxygen desaturation events during general anesthesia.

Laryngomalacia in the Premature Neonate.

Laryngomalacia is the most common cause of stridor in newborns. Affected patients may present with noisy breathing, a classic high-pitched inspiratory stridor that worsens with feeding. While the exact etiology remains unclear, the condition is characterized by softening of the supraglottic structures, including the epiglottis, aryepiglottic folds, and arytenoid cartilages. The condition is most often self-limited and requires expectant management. However, in some infants, severe disease, including failure to thrive or respiratory distress, may require medical or even surgical intervention. When caring for premature neonates, special care is required to evaluate for synchronous airway lesions.

Acid Suppression Does Not Improve Laryngomalacia Outcomes but Treatment for Oropharyngeal Dysphagia Might Be Protective.

OBJECTIVE: To determine whether the use of acid suppression and thickened feeds impact laryngomalacia outcomes in infants, including supraglottoplasty risk, time to supraglottoplasty, and hospitalization risk. STUDY DESIGN: We performed a retrospective cohort study to compare risk and time with supraglottoplasty and frequency and duration of hospitalizations for infants diagnosed with laryngomalacia at Boston Children's Hospital between January 1 and December 31, 2017. The primary outcomes were supraglottoplasty requirement, time to supraglottoplasty, and hospitalization risk. Multivariate analyses were performed to determine predictors of supraglottoplasty and hospitalization risk after adjusting for laryngomalacia severity and comorbidities in addition to propensity score adjustment. Kaplan-Meier curves were created to determine the impact of acid suppression use on time to supraglottoplasty. RESULTS: In total, 236 subjects with mean age 62.6 ± 4 days were included in the analysis; 55% were treated with acid suppression. Subjects treated with acid suppression had a greater risk of supraglottoplasty (hazard ratio 3.36, 95% CI 1.36-8.29, P = .009), shorter time to supraglottoplasty (5.64 ± 0.92 vs 7.98 ± 1.92 months, P = .006), and increased respiratory hospitalization risk (relative risk 1.97, 95% CI 1.01-3.85, 0.047), even after adjustment for covariates. Subjects receiving thickening had fewer respiratory hospitalization nights and longer time to supraglottoplasty (9.3 ± 1.7 vs 4.56 ± 0.73 months, P = .004), even after adjustment. CONCLUSIONS: Acid suppression use does not reduce the frequency of supraglottoplasty and related hospitalizations compared with untreated subjects. However, patients treated with thickening have decreased hospitalization and longer time to supraglottoplasty, suggesting that thickening of feeds may be a preferred intervention over acid suppression.

The burden of laryngomalacia and its effects on caregivers: A support group survey evaluation.

OBJECTIVE: To evaluate which factors may affect anxiety and child's health perception of parents with children that have laryngomalacia. STUDY DESIGN: Survey Study. SETTING: "Coping with Laryngomalacia" - largest online laryngomalacia support group for parents with children who have laryngomalacia. SUBJECTS: and Methods: A survey was sent to support group member that gathered information regarding the patient's diagnosis and associated course of treatment and evaluated for parental anxiety and perceived health of their child. Analysis was performed to evaluate which variables are associated with increased parental anxiety and worsened perceived child's health. RESULTS: Data was gathered on 434 patients. All caregivers reported some level of anxiety due to the diagnosis and 64% felt completely anxious. Parents who saw an increased number of physicians prior to a pediatric ENT rated their child with poorer health (p < .05). Those that felt their concerns were brushed off initially reported increased anxiety (p < .05). If the child was admitted to the hospital, ICU, required intubation, had feeding troubles, required a feeding tube, or underwent pH monitoring, the patient was rated to have poorer health per the parents (p < .05). CONCLUSION: Laryngomalacia causes a significant burden on patients and their families due to the eating problems, feeding problems, hospital stay, and other various conditions associated with the disease. Caregivers should take a more modern and compassionate approach to management and diagnosis.

Prevalence of obstructive sleep apnea in children with laryngomalacia and value of polysomnography in treatment decisions.

OBJECTIVES: Children with laryngomalacia may present with obstructive sleep apnea (OSA). The role of polysomnography (PSG) in treatment decision making for laryngomalacia is not well defined. We aimed to investigate the prevalence of OSA in children with laryngomalacia and the role of PSG in treatment decision. METHODS: Retrospective medical record review of children with laryngomalacia, confirmed by direct laryngoscopy, during a period of 3 years. Demographic data, presenting symptoms, severity classification, comorbidities and pre- and postoperative PSG data were retrieved and analyzed. Data are expressed as a median (25th - 75th percentile). RESULTS: Forty-six patients were with diagnosed laryngomalacia between March 2016 and April 2019. A complete data set was available for 44 patients, 24 males and 20 females. The median age at the time of PSG was 12 weeks (6.3-29.8). Thirty-four children (77.4%) were diagnosed with concomitant OSA. A diagnosis of OSA changed the severity classification and treatment decision in 24 cases (54.5%). Twenty-three patients underwent supraglottoplasty, five patients were treated with continuous positive airway pressure (CPAP) and nine patients had both treatments. Seven patients received conservative treatment. The obstructive apnea/hypopnea index decreased from 8.9 events/hour (4.4-12.1) to 2.4 events/hour (1.5-4.4) after supraglottoplasty (p = 0.009). CONCLUSIONS: A diagnosis of OSA was established in 77.4% of patients with larygomalacia The presence of OSA may increase the severity of symptoms in laryngomalacia, leading to a transition from watchful-waiting to active intervention with CPAP therapy or supraglottoplasty. Supraglottoplasty is a safe and effective surgical procedure for laryngomalacia. When performed in the setting of laryngomalacia with concomitant OSA, it also significantly improves OSA symptomatology.

CHARGE syndrome patient with novel CHD7 mutation presenting with severe laryngomalacia and feeding difficulty.

We report a case of CHARGE syndrome with atypical phenotype and a novel mutation in the CHD7 gene. Laryngomalacia and swallowing difficulties are prominent features in this case. These are commonly found in patients with CHARGE syndrome and are well described in previous studies. However, with the traditional diagnostic criteria, diagnosis is difficult without the presence of coloboma or choanal atresia. Early diagnosis is possible with the aid of clinical genetics. The current diagnostic criteria would need to be broadened with the inclusion of pathogenic CHD7 variant status as a major criterion. Further research on the function of CHD7 gene may also give us more insight on the pathogenic mechanism of various clinical features of CHARGE syndrome.

Vallecular cyst with coexisting laryngomalacia: Successful diagnosis and laser therapy by flexible endoscopy with a novel noninvasive ventilation support in infants.

OBJECTIVE: Vallecular cyst coexisting with laryngomalacia (VC-LM) can cause significant pharyngolaryngeal obstruction. Traditionally, it is diagnosed with flexible endoscopy (FE) and treated by rigid endoscopy. This study evaluates the effectiveness of solely using FE with novel noninvasive ventilation (NIV) of sustained pharyngeal inflation (SPI) support for both diagnosis and treatment in such infants. METHODS: A retrospective review of consecutive infants who were diagnosed and treated for VC-LM in the 12-year period, 2007 to 2018, was conducted. Clinical variables, techniques, and outcomes were analyzed and reported. RESULTS: Eighteen infants (10 males) were included. The mean age was 3.0 ± 0.6 months and the mean body weight was 4.6 ± 1.3 kg. Before FE, 14 infants were supported with bi-nasal prongs NIV (BN-NIV) and four infants with tracheal intubation. During diagnostic and therapeutic FE, all infants supported with a nasopharyngeal NIV (NP-NIV) only. All diagnoses were made in the first FE inspection of 3.5 ± 1.2 minutes. Thirteen lesions were immediately treated with FE laser therapy in 18.1 ± 1.7 minutes in the same FE course. Total FE time was 24.6 ± 2.8 minutes. Three infants needed revision laser therapy 4 days later. There was no desaturation (<90%), bradycardia (<100/min), or pneumothorax. After FE therapy, all infants were supported with BN-NIV only with significantly (<0.01) lower pressure and completely weaned off before being discharged 8.4 ± 1.5 days later. All infants, followed up for a 6-month period, showed many clinical improvements. CONCLUSIONS: FE, with this NP-NIV and SPI supports, could offer accurate diagnosis and successful laser therapy of the VC-LM with procedural sedation in the same session in infants.

Laryngomalacia in Neonates Versus Older Infants: HCUP-KID Perspective.

This study evaluated the hospital course for neonates and older infants with a diagnosis of laryngomalacia (LM). Data came from the 2016 Kids' Inpatient Database of the Healthcare Cost Utilization Project. A total of 6537 children aged <1 year with a diagnosis of LM (International Classification of Diseases, 10th Revision, code Q31.5) were identified: 2212 neonates and 4325 non-neonates. Neonates had a higher mortality rate, 1.31% versus 0.72% in older infants, had more diagnoses (median 9 vs 7) and procedures (mean 85.24 vs 21.83), longer length of stay (median 10 vs 4 days), and higher total charges (median US$65 722 vs US$25 582). A total of 23.3% of neonates born during the admission and diagnosed with LM had undergone laryngoscopy. Second airway lesions were present in 12.33% of neonates and 15.77% of older infants. It appears that neonates are being discharged with a diagnosis of LM without laryngoscopy. Neonatal intensive care unit and newborn nursery policies should require visualization of the larynx prior to diagnosis of LM.

What parents are reading about laryngomalacia: Quality and readability of internet resources on laryngomalacia.

OBJECTIVE: The goal of this study is to measure the quality and readability of websites related to laryngomalacia, and to compare the quality and readability scores for the sites accessed through the most popular search engines. INTRODUCTION: Laryngomalacia is a common diagnosis in children but is often difficult for parents to comprehend. As information available on the internet is unregulated, the quality and readability of this information may vary. METHODS: An advanced search on Google, Yahoo, and Bing was conducted using the terms "laryngomalacia" OR "soft larynx" OR "floppy voice box." The first ten websites meeting inclusion and exclusion criteria were evaluated, for each search engine. Quality and readability were assessed using the DISCERN criteria and the Flesch reading ease scoring (FRES) and Flesch-Kincaid grade level (FKGL) tests, respectively. RESULTS: The top 10 hits on each search engine yielded 15 unique web pages. The median DISCERN score (out of a possible high-score of 80) was 48.5 (SD 12.6). The median USA grade-level estimated by the FKGL was 11.3 (SD 1.4). Only one website (6.7%), had a readability score in the optimal range of 6th to 8th grade reading level. DISCERN scores did not correlate with FKGL scores (r = 0.10). CONCLUSION: Online information discussing laryngomalacia often varies in quality and may not be easily comprehensible to the public. It is important for healthcare professionals to understand the quality of health information accessible to patients as it may influence medical decision-making by patient families.

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.

Hold your horses: A comparison of human laryngomalacia with analogous equine airway pathology.

OBJECTIVES: Laryngomalacia is the most common cause of stridor in infants. Dynamic airway collapse is also a well-recognised entity in horses and an important cause of surgical veterinary intervention. We compare the aetiology, clinical features and management of human laryngomalacia with equine dynamic airway collapse. METHODS: A structured review of the PubMed, the Ovid Medline and the Cochrane Collaboration databases (Cochrane Central Register of Controlled Trials, Cochrane Database of Systemic Reviews). RESULTS: There are numerous equine conditions that cause dynamic airway collapse defined specifically by the anatomical structures involved. Axial Deviation of the Aryepiglottic Folds (ADAF) is the condition most clinically analogous to laryngomalacia in humans, and is likewise most prevalent in the immature equine airway. Both conditions are managed either conservatively, or if symptoms require it, with surgical intervention. The operative procedures performed for ADAF and laryngomalacia are technically comparable. CONCLUSION: Dynamic collapse of the equine larynx, especially ADAF, is clinically similar to human laryngomalacia, and both are treated in a similar fashion.

Predictors for routine admission to paediatric intensive care for post-supraglottoplasty laryngomalacia patients.

OBJECTIVES: Supraglottoplasty for the treatment of laryngomalacia has little current evidence regarding post-operative care. Our study aimed to: (1) retrospectively assess what proportion of patients required paediatric intensive care unit level of care; (2) identify pre-operative predictive factors common to these cases; and (3) report patient outcomes at six weeks' follow up. METHODS: A 10-year retrospective case series analysis was conducted of all patients diagnosed with laryngomalacia and subsequently treated with supraglottoplasty. Paediatric intensive care unit level of care was defined as the need for intubation or tracheostomy, positive pressure ventilation, multiple doses of nebulised adrenaline, and oxygen dependency beyond 12 hours. RESULTS: Forty-two patients (19 males, 23 females) were identified; 28.5 per cent of cases met our criteria for paediatric intensive care unit level of care. A low pre-operative oxygen saturation was the only significant risk factor that predicted a future need for paediatric intensive care unit level of care (p = 0.0008). CONCLUSION: This is the first study published in the UK to suggest the importance of pre-operative oxygen saturation as a predictor of a future need for paediatric intensive care unit level of care.

Fifteen-minute consultation: investigation and management of an infant with stridor.

Stridor is an abnormal sound caused by turbulent airflow through the airway. In an infant under 3 months of age presenting with stridor, there are many different underlying aetiologies. Some key assessment points help to differentiate those patients who can be reassured and monitored versus those requiring referral to an ear, nose and throat (ENT) surgeon for further assessment or management. We present a structured approach to guide the clinician through initial assessment, examination and management, including referral to ENT surgery.

[Research progress of laryngomalacia in children].

Laryngomalacia is defined as a supraglottic collapse of the glottis, resulting in intermittent airflow obstruction and associated wheezing. This disease is the most common cause of stridor in neonates and infants. Associated feeding difficulties are present in approximately half of the children. A definitive diagnosis can generally be made with flexible fiberoptic laryngoscopy. The disorder is most often self-limited with resolution of symptoms within the first 24 months of life, and the majority of children can thus be managed conservatively. The approximately 5%-20% of children with severe or refractory disease may require more aggressive intervention, most commonly in the form of transoral supraglottoplasty. High success rates and a low rate of complications have been reported for this procedure in otherwise healthy children. Children with syndromes or medical comorbidities are more likely to have complications or persistent symptoms after supraglottoplasty and may require additional interventions.