Correlation between the clinical severity of laryngomalacia and endoscopic findings.

OBJECTIVE: To correlate the clinical severity of laryngomalacia (LM) with endoscopic findings, swallowing evaluations and polysomnography in a cohort of patients. Method: We conducted a retrospective analysis between 2017-2018 on a cohort of patients diagnosed with upper airway obstruction (UAO), stridor, noisy breathing or laryngomalacia. This study took place at the Pediatric Pulmonology Department, Riley Children's Hospital, Indianapolis, United States of America. RESULTS:   There were 157 patients with laryngomalacia included in the study. Patients with severe LM were significantly younger than those with mild LM (p=0.0214) and moderate LM (p=0.0220). Subjects with type I of LM were significantly older than type III (p=0.0051). When associations were tested between polysomnogram (PSG) variables and clinical severity, there were significant associations with age at PSG. The overall apnea-hypopnea index (AHI) in mild (p=0.0103) and moderate (p=0.0242) were significantly lower than the severe group. The rapid eye movement (REM) AHI was significantly lower in moderate cases than severe (p=0.0134). The end-tidal carbon dioxide (EtCO2) peak was significantly lower in mild cases than severe (p=0.0141). The total sleep time (TST) peripheral capillary oxygen saturation (SpO2) 90% occurs in both mild (p=0.0197) and moderate (p=0.0498) were significantly lower than the severe group. CONCLUSIONS: The severity of the clinical manifestations of LM did not correlate with the different endoscopic types in our study. The presence of cyanosis was associated with type III LM. Rapid eye movement AHI and EtCO2 in polysomnogram were remained significantly associated with clinical severity.

The Use of Computer Decision Support for Pediatric Obstructive Sleep Apnea Detection in Primary Care.

STUDY OBJECTIVES: To (1) describe outcomes from a computer decision support system (CDSS) for pediatric obstructive sleep apnea (OSA) detection in primary care; and (2) identity the prevalence of children meeting criteria for an OSA referral. METHODS: A CDSS for OSA was implemented in two urban primary care clinics. Parents of children (age 2 to 11 years) presenting to the clinic were asked if their child snored regularly, with a positive response resulting in six additional OSA screening items. Primary care providers (PCPs) received a prompt for all snoring children, listing applicable OSA signs and symptoms and recommending further evaluation and referral for OSA. RESULTS: A total of 2,535 children were screened for snoring, identifying 475 snoring children (18.7%). Among snoring children, PCPs referred 40 (15.4%) for further evaluation. The prevalence of additional OSA signs and symptoms ranged from 3.5% for underweight to 43.7% for overweight. A total of 74.7% of snoring children had at least one additional sign or symptom and thus met American Academy of Pediatrics guidelines criteria for an OSA referral. CONCLUSIONS: A CDSS can be used to support PCPs in identifying children at risk for OSA. Most snoring children met criteria for further evaluation. It will be important to further evaluate this referral threshold as well as the readiness of the sleep medicine field to meet this need. CLINICAL TRIALS REGISTRATION: Registry: ClinicalTrials.gov, Title: Evidence-based Diagnosis and Management of Pediatric Obstructive Sleep Apnea in Primary Care, Identifier: NCT02781376, URL: https://clinicaltrials.gov/ct2/show/NCT02781376.

Polysomnography Reference Values in Healthy Newborns.

STUDY OBJECTIVES: Polysomnography (PSG) is increasingly used in the assessment of infants. Newborn PSG reference values based on recent standardization are not available. This study provides reference values for PSG variables in healthy newborn infants. METHODS: Cross-sectional study of normal term newborn infants using standardized PSG collection and American Academy of Sleep Medicine interpretation criteria. RESULTS: Thirty infants born between 37 and 42 weeks gestation underwent PSG testing before 30 days of age (mean 19.6 days). The infants had a mean sleep efficiency of 71% with average proportions of transitional, NREM and REM sleep estimated at 16.1%, 43.3% and 40.6% respectively. Mean arousal index was 14.7 events/h with respiratory arousal index of 1.2 events/h. Mean apnea-hypopnea index (AHI) was 14.9 events/h. Central, obstructive, and mixed apnea indices were 5.4, 2.3, and 1.2 events/h respectively. Mean oxygen saturation in sleep was 97.9% with a nadir of 84.4%. Mean end tidal CO2 was 35.4 mmHg with an average of 6.2% of sleep time spent above end-tidal CO2 45 mmHg and 0.6% above 50 mmHg. CONCLUSIONS: The sleep efficiency was significantly lower and the AHI was significantly higher compared to healthy children older than 1 year. The AHI was also higher than reported in healthy infants older than 1 month. These findings suggest current severity classifications of sleep apnea may not apply to newborn infants.

Neurodevelopmental Outcomes at Two Years of Age for Premature Infants Diagnosed With Neonatal Obstructive Sleep Apnea.

STUDY OBJECTIVES: Neurocognitive deficits have been shown in school-aged children with sleep apnea. The effect of obstructive sleep apnea (OSA) on the neurodevelopmental outcome of preterm infants is unknown. METHODS: A retrospective chart review was performed for all preterm infants (< 37 weeks) who had neonatal polysomnography (PSG) and completed neurodevelopmental assessment with the Bayley Scales of Infant and Toddler Development, 3rd Edition, between 2006 to 2015 at Riley Hospital. Exclusion criteria included grade IV intraventricular hemorrhage, tracheostomy, cyanotic heart disease, severe retinopathy of prematurity, craniofacial anomalies, or central and mixed apnea on PSG. Sleep apnea was defined as an apnea-hypopnea index (AHI) > 1 event/h. Regression analyses were performed to find a relationship between PSG parameters and cognitive, language, and motor scores. RESULTS: Fifteen patients (males: n = 10) were eligible for the study. Median postmenstrual age at the time of the PSG was 41 weeks (37-46). Median AHI for the cohort was 17.4 events/h (2.2-41.3). Median cognitive, language, and motor scores were 90 (65-125), 89 (65-121), and 91 (61-112), respectively. Mean end-tidal CO2 (median 47 mm Hg [25-60]) negatively correlated with cognitive scores (P = .01) but did not significantly correlate with language or motor scores. AHI was not associated with cognitive, language, or motor scores. CONCLUSIONS: The median score for cognitive, language, and motor scores for preterm infants with neonatal OSA were within one standard deviation of the published norm. Mean end-tidal CO2, independent of AHI, may serve as a biomarker for predicting poor cognitive outcome in preterm infants with neonatal OSA. COMMENTARY: A commentary on this article appears in this issue on page 1233.

Robin sequence: what the multidisciplinary approach can do.

Robin sequence (RS) is a commonly encountered triad of micrognathia, glossoptosis, and airway obstruction, with or without a cleft palate. The management of airway obstruction is of paramount importance, and multiple reviews and retrospective series outline the diagnosis and treatment of RS. This article focuses on the multidisciplinary nature of RS and the specialists' contributions and thought processes regarding the management of the RS child from birth to skeletal maturity. This review demonstrates that the care of these children extends far beyond the acute airway obstruction and that thorough monitoring and appropriate intervention are required to help them achieve optimal outcomes.

Apical Lung Herniation Associated with Continuous Positive Airway Pressure in a 4-Year-Old Girl.

ABSTRACT: We report a case of apical lung herniation through the superior thoracic aperture of an obese child using nocturnal CPAP. Lung herniation has been described in association with congenital thoracic abnormalities and elevated intra-thoracic pressure, such as trauma. This patient was hospitalized with community acquired pneumonia and required nocturnal CPAP for treatment of concurrent obstructive sleep apnea. Her lung hernia was discovered incidentally on routine follow-up chest radiography and resolved with cessation of CPAP treatment. Lung herniation in association with the use of continuous positive airway pressure (CPAP) has not been previously described.

NREM sleep parasomnia associated with Chiari I malformation.

Parasomnias are common sleep disorders in children, and most cases resolve naturally by adolescence.(1) They represent arousal disorders beginning in NREM sleep and are generally non-concerning in children. The diagnosis can usually be made by clinical assessment, and testing with polysomnography is not routinely indicated.(2) However, in certain cases with atypical features, polysomnography and more extensive neurologic evaluation are medically indicated.

Inhalational lung injury associated with humidifier "white dust".

Humidifiers are commonly used in the community to relieve symptoms associated with acute respiratory infections in young children; however, clear benefits of these devices have not been documented. The Environmental Protection Agency has not found any adverse health effects related to humidifier use. We report here the case of a young infant with significant accidental inhalational lung injury related to dispersal of mineral dust from an ultrasonic home-use humidifier. The clinical consequences included prolonged hypoxemia, tachypnea, and failure to thrive. Radiography revealed pneumonitis, and pulmonary-function testing showed a nonreversible mild obstructive ventilatory defect. Because of persistent symptoms, evolution of failure to thrive, and nonresponse to inhaled and short courses of systemic glucocorticoids, an aggressive management approach was successfully pursued with high-dose pulse steroid therapy, which could be a potential therapeutic approach for similar patients. In addition, this case raises important questions about the safety of exposing infants and young children to humidifiers and emphasizes the need for further study.

Treatment of childhood obstructive sleep apnea.

OPINION STATEMENT: An array of surgical, medical and orthodontic treatments is available for treating childhood obstructive sleep apnea. Adenotonsillectomy remains the first choice in treatment, with a need for subsequent clinical and polysomnographic reassessment in selected cases to determine residual sleep-disordered breathing. Residual obstructive sleep apnea is more likely in patients with craniofacial abnormalities or obesity. It may require the use of a positive airway pressure breathing device. Topical corticosteroids, leukotriene antagonists, weight reduction, and positional therapy also play a role in ameliorating childhood obstructive sleep apnea. The published evidence for the efficacy of various treatment modalities consists largely of case-controlled studies and case reports.

Effect of long-term steroids on cough efficiency and respiratory muscle strength in patients with Duchenne muscular dystrophy.

OBJECTIVE: The objective of this study was to determine whether long-term steroid therapy is associated with increased peak cough flow in patients with Duchenne muscular dystrophy and to determine which pulmonary function test variable is most predictive of peak cough flow. METHODS: In this case-control study, the medical charts of patients who had Duchenne muscular dystrophy and had pulmonary function tests at our institution in the previous 2 years were examined. Steroid-treated patients were on therapy for at least 1 year. The measured pulmonary function tests included forced vital capacity, maximum expiratory pressure, maximum inspiratory pressure, maximum voluntary ventilation, and peak cough flow. Multiple linear regression analysis was used to determine which pulmonary function test measure was most predictive of peak cough flow and assess the influence of steroid treatment and patient age on peak cough flow. RESULTS: Ten steroid-treated and 25 untreated patients were analyzed. Peak cough flow and maximum expiratory pressure were significantly higher in the steroid-treated patients. Each of the pulmonary function test variables was significantly associated with peak cough flow. The linear model that had the highest adjusted r2 value included only 2 variables: maximum voluntary ventilation and steroid treatment, demonstrating that steroid-treated patients had peak cough flow values that were 27 L/min higher than the untreated patients. The interaction between maximum voluntary ventilation and steroid was not statistically significant, suggesting that the steroid-associated increase in peak cough flow was approximately constant over the observed range of maximum voluntary ventilation values. The effects of maximum voluntary ventilation and treatment group on peak cough flow were not confounded with the patient age. CONCLUSIONS: Long-term steroid therapy is associated with improved peak cough flow and respiratory muscle strength in patients with Duchenne muscular dystrophy. Maximum voluntary ventilation may be a useful predictor of lung function in Duchenne muscular dystrophy.