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STUDY OBJECTIVES: Positive airway pressure (PAP) is the second line of treatment for obstructive sleep apnea syndrome in children. It is common practice following initiation of PAP to perform repeat titration polysomnography to re-evaluate the patient’s therapeutic pressure; however, data supporting this practice are lacking. We hypothesized that repeat PAP titration would result in significant setting changes in children with obstructive sleep apnea syndrome. METHODS: We retrospectively analyzed demographic, polysomnographic, and PAP data of children with obstructive sleep apnea syndrome aged 0–18 years who were initiated on PAP and underwent 2 titration studies over a 2-year period. PAP mode and recommended pressure differences between the 2 titrations were compared. RESULTS: 64 children met inclusion criteria. The median (interquartile range) baseline obstructive apnea-hypopnea index and SpO(2) nadir were 14.8 (8.7–32.7) events/h and 88.5% (85–92%), respectively. The mean differences in obstructive apnea-hypopnea index, SpO(2) nadir, and % total sleep time with SpO(2) < 90% between both titrations were negligible, including children with obesity, adenotonsillar hypertrophy, and trisomy 21. Additionally, there was no significant difference in mean PAP pressure between 2 separate titration studies for those on continuous PAP or bilevel PAP. CONCLUSIONS: Overall, repeat PAP titration in children with obstructive sleep apnea syndrome within the timeframe here described did not result in significant changes in PAP mode, continuous PAP pressure, or obstructive apnea-hypopnea index. Based on these data, repeat PAP titration within 2 years of an initial titration does not appear to be necessary. CITATION: Yendur O, Feld L, Miranda-Schaeubinger M, et al. Clinical utility of repeated positive airway pressure titrations in children with obstructive sleep apnea syndrome. J Clin Sleep Med. 2022;18(4):1021–1026.

OBJECTIVES: Acute bronchiolitis commonly causes respiratory failure in children ≤ 2 years, and is particularly severe in those with Down syndrome (DS). Obstructive sleep apnea (OSA), common in DS, is also associated with respiratory complications. However, it is unknown whether OSA is associated with worse outcomes in children with and without DS, hospitalized with bronchiolitis. We hypothesized that in children with bronchiolitis, OSA is associated with worse outcomes in those with DS, independent of DS-related comorbidities. METHODS: Hospital discharge records of children with bronchiolitis aged ≤ 2 years were obtained for 1997–2012 from the Kid’s Inpatient Database. The primary outcome was invasive mechanical ventilation (IMV), and secondary outcomes were non-invasive mechanical ventilation (NIMV), length of hospital stay, and inflation-adjusted cost of hospitalization (IACH). Multivariable regression was conducted to ascertain the associations between OSA and primary and secondary outcomes accounting for DS-associated comorbidities. RESULTS: There were 928,961 hospitalizations for bronchiolitis. The DS group with bronchiolitis (n=8,697) was more likely to have OSA [241 (2.77%) vs 1,293 (0.14%), p

STUDY OBJECTIVES: Healthy infants may have a greater apnea hypopnea index (AHI) than older children during the newborn period, but the trajectory of these sleep-related events beyond the first month of life is poorly understood. In this study, we evaluated the longitudinal changes in respiratory indices during sleep in healthy infants during the first six months of life. METHODS: Single-center prospective cohort study. Thirty healthy infants underwent overnight in-lab polysomnography at one and five months of age and findings were compared between assessments. Systematic review of studies evaluating infant polysomnography and meta-analysis was conducted. RESULTS: At one month of age, total AHI, obstructive AHI, and central AHI model-adjusted means (95% confidence interval) were 16.9 events/hour (12.2, 21.5), 10.2 events/hour (7.4, 13.1), and 6.6 events/hour (4.2, 9.0), respectively. 16.8% of events were obstructive apneas and 36.1% central apneas. By five months of age, there were significant reductions in each index to 4.1 events/hour (3.2, 5.0), 1.9 events/hour (1.4, 2.4), and 2.2 events/hour (1.6, 2.9), respectively (p

OBJECTIVE To determine the impact of obstructive sleep apnea (OSA) on asthma exacerbation severity in children hospitalized for asthma exacerbation. HYPOTHESIS OSA is associated with greater use of invasive mechanical ventilation (IMV) and noninvasive mechanical ventilation (NIMV) in children hospitalized for asthma exacerbation. STUDY DESIGN A retrospective cohort study. PATIENT-SUBJECT SELECTION Hospitalization records of children aged 2-18 years admitted for acute asthma exacerbation were obtained for 2000, 2003, 2006, 2009, and 2012 from the Kids' Inpatient Database. METHODOLOGY The primary exposure was OSA, the primary outcome was IMV, and secondary outcomes were NIMV, length of hospital stay (LOS), and inflation-adjusted cost of hospitalization. Multivariable logistic regression, negative binomial, and linear regression were conducted to ascertain the impact of OSA on primary and secondary outcomes. Exploratory analyses investigated the impact of obesity on primary and secondary outcomes. RESULTS Among 564,467 hospitalizations for acute asthma exacerbation, 4209 (0.75%) had OSA. Multivariable regression indicated that OSA was associated with IMV (adjusted odds ratio [OR], 5.33 [95% confidence interval, CI: 4.35-6.54], p

Mandibular distraction osteogenesis (MDO) and continuous positive airway pressure (CPAP) may each have a role in effectively treating tongue-based airway obstruction (TBAO) in Robin sequence (RS). This study describes longitudinal outcomes after treatment of TBAO with CPAP and/or MDO. Retrospective cohort study. Tertiary Pediatric Hospital. A total of 129 patients with RS treated with CPAP and/or MDO from 2009 to 2019 were reviewed. Subjects receiving baseline and at least one follow-up polysomnogram were included. 55 who underwent MDO ± CPAP and 9 who received CPAP-only treatment were included. Patient characteristics, feeding, and polysomnographic data were compared and generalized linear mixed modeling performed. Baseline obstructive apnea–hypopnea index (OAHI) was greater in the MDO-treated group (median x˜ = 33.7 [interquartile range: 26.5-54.5] than the CPAP-treated group (x˜ = 20.3[13.3-36.7], P ≤ .033). There was significant reduction in OAHI following treatment with CPAP and MDO modalities, P ≤ .001. SpO2 nadir after MDO was lower in syndromic (x˜ = 85.0[81.0-87.9] compared to nonsyndromic patients (x˜ = 88.4[86.8-90.5], P ≤ .005.) CPAP was utilized following MDO in 2/24 (8.3%) of nonsyndromic and 16/31 (51.6%) of syndromic subjects ( P ≤ .001,) for a median duration of 414 days. Three patients (5%) underwent tracheostomy, all had MDO. Nasogastric tube feeding at hospital discharge was more common following MDO (44, 80%) than CPAP-only (4, 44.4%, P ≤ .036), but did not differ at 6-month follow-up ( P ≥ .376). CPAP appears to effectively reduce obstructive apnea in patients with RS and moderate TBAO and be a useful adjunct in syndromic patients following MDO with improved but persistent obstruction.

STUDY OBJECTIVES: In-laboratory titration polysomnography (PSG) is standard to determine optimal therapeutic continuous positive airway pressure (CPAP) in children with obstructive sleep apnea (OSA). The use of auto-titrating CPAP devices (autoCPAP) as an alternative to CPAP titration has not been well studied in children. We hypothesized that autoCPAP-derived pressures (P(MEAN), P(PEAKMEAN), P(90)) would be similar to titration PSG pressure (P(PSG)). METHODS: This is a retrospective study of children with OSAS initiated on autoCPAP between 2007 and 2017, who used autoCPAP for at least 2 h/night and who had adequate titration PSG were included in the analysis. AutoCPAP-derived pressures were obtained from use downloads and compared with P(PSG). P(PSG) predictive factors were analyzed by median regression. Nonparametric methods were used for analysis. RESULTS: Of 110 children initiated on autoCPAP, 44 satisfied the inclusion criteria. Age (median (interquartile range)) was 13.01 (9.98–16.72) years, and 63.6% were obese. P(PSG) median (interquartile range) was 8 (7–11) cmH(2)O, mean autoCPAP-derived pressure (P(MEAN)) was 6.2 (5.6–7.6) cmH(2)O, peak mean pressure (P(PEAKMEAN)) was 9.4 (7.7–11.1) cmH(2)O, and average device pressure ≤ 90% of the time (P(90)) was 8.1 (7.2–9.7) cmH(2)O. AutoCPAP-derived pressures correlated with P(PSG) (P < .05). P(MEAN) was lower than the other 3 pressures (P < .0002). Median regression analysis demonstrated that after adjusting for patient characteristics such as age, sex, and obesity status, autoCPAP-derived pressures remained significant predictors of P(PSG) (P < .05). There were no significant interactions between these patient characteristics and autoCPAP-derived pressures. CONCLUSIONS: This study demonstrates that autoCPAP-derived pressures correlate with the titration PSG-derived pressures. These results indicate that autoCPAP can be used in the pediatric population and can determine pressures that are close to the titration pressures. CITATION: Khaytin I, Tapia IE, Xanthopoulos MS, et al. Auto-titrating CPAP for the treatment of obstructive sleep apnea in children. J Clin Sleep Med. 2020;16(6):871–878.

BACKGROUND: Macroglossia, a cardinal feature of the (epi)genetic disorder Beckwith-Wiedemann syndrome, is associated with obstructive sleep apnea, speech and/or feeding difficulties, and dental or jaw malalignment. These sequelae may be treated and/or prevented with tongue reduction surgery; the authors sought to determine whether certain Beckwith-Wiedemann syndrome patients may benefit from early surgical intervention before age 12 months. METHODS: The authors conducted a retrospective review of patients with Beckwith-Wiedemann syndrome who underwent tongue reduction from 2014 to 2019. The authors assessed primary outcomes of change in obstructive sleep apnea by polysomnography, respiratory support required, and feeding route before and after tongue reduction, and reviewed postoperative complications and the need for repeated tongue reduction. RESULTS: Of the 36 patients included, the median age at tongue reduction was 9.5 months (interquartile range, 3.8 to 22.8 months). For those with severe obstructive sleep apnea, there was a significant reduction in the obstructive apnea hypopnea index from 30.9 ± 21.8 per hour to 10.0 ± 18.3 per hour (p = 0.019) and improvement in nadir oxyhemoglobin saturation from 72 ± 10 percent to 83 ± 6 percent (p = 0.008). Although there was no significant change in overall supplemental feeding tube or respiratory support, there were specific patients who experienced clinically meaningful improvement. Of note, these positive outcomes applied equally to those who underwent surgery at a younger age (

Intranasal corticosteroids (INCS) are frequently used to treat OSA syndrome (OSAS) in children. However, their efficacy has not been rigorously tested.Do INCS result in improved OSAS symptoms, polysomnography findings, behavior, and quality of life compared with placebo?In this randomized, double-blind, placebo-controlled trial, children with OSAS aged 5 to 12 years (N = 134) were randomized 2:1 to receive 3 months of INCS or placebo. Children in the INCS arm were then re-randomized to receive 9 months of INCS or placebo. Polysomnography, symptoms, and neurobehavioral findings were measured at baseline, 3 months, and 12 months. The primary outcome was change in obstructive apnea hypopnea index (OAHI) at 3 months, available for 122 children. The secondary outcome was OAHI change at 12 months, available for 70 children.Median (interquartile range) age and OAHI at baseline for the entire group were 7.9 (6.3 to 9.9) years and 5.8 (3.6 to 9.7) events per hour. OAHI changes at 3 months (-1.72 [-3.91 to 1.92] events per hour) and 12 months (-1.2 [-4.22 to 1.71] events per hour) were not different between the two groups (P = not significant). OSAS symptoms and neurobehavioral results did not differ between the INCS and placebo groups at 3 and 12 months. The 38 children who received INCS for 12 months reported a significant OAHI decrease from 7.2 (3.62 to 9.88) events per hour to 3.7 (1.56 to 6.4) events per hour (P = .039).In children with OSAS, treatment with INCS did not result in significant polysomnography, neurobehavioral, or symptom changes at 3 and 12 months of treatment. Twelve months of INCS treatment resulted in a statistically significant but not clinically relevant OAHI reduction.ClinicalTrials.gov; No.: NCT02180672; URL: www.gov.

Purpose Mandibular distraction osteogenesis (MDO) effectively treats tongue-based airway obstruction (TBAO) in micrognathic patients with Robin Sequence. Mandibular distraction osteogenesis may also address TBAO in certain nonmicrognathic patients who have severe obstructive apnea, although there is no current literature to guide MDO use in these atypical patients. This study describes outcomes of MDO in a series of patients with TBAO without micrognathia. Methods Patients who underwent MDO for TBAO from 2013-20 were reviewed, and patients with micrognathia were excluded. Study subjects received baseline/follow up polysomnography. Polysomnography variables, including Obstructive Apnea Hypopnea Index, oxyhemoglobin saturation nadir (SpO2 nadir), percent sleep time end tidal CO2 greater than 50 mm Hg (%ETCO2 > 50), and respiratory-related arousals were compared before and after MDO. Demographics, syndromic/cleft palate status, airway anomalies, respiratory support, and feeding outcomes were collected. Results One hundred and twenty-four patients underwent MDO during this study period; 5 were nonmicrognathic and included in analysis. Sixty percent (n = 3) of the cohort was syndromic: 1 patient each had Trisomy 9, Beckwith Wiedemann syndrome, and duplicated pituitary gland plus syndrome. Forty percent (n = 2) of patients had a cleft palate, 60% (n = 3) had laryngomalacia, and 40% had tracheomalacia. Median (range) age at MDO was 53 days (47-167 days), and median length of distraction was 16 mm (14-20 mm). After MDO, median Obstructive Apnea Hypopnea Index decreased from x[Combining Tilde] = 60.7/h (11.6-109.4) to x[Combining Tilde] = 5.3/h (3.5-19.3) (P = 0.034). SpO2 nadir increased (69% [58-74] to 85% [80-88], P = 0.011), and median %ETCO2 > 50 mm Hg decreased (5.8% [5.2-30.1] to 0.0% [0.0-1.3], P ≤ 0.043). Continuous positive airway pressure was used by all patients immediately after MDO, and at 6 months postoperatively, 1 patient remained on continuous positive airway pressure and 1 patient required supplemental oxygen. At last follow up, no patients had significant residual airway obstruction or required a tracheostomy. Conclusions Mandibular distraction osteogenesis can effectively treat severe TBAO in some patients without micrognathia that would otherwise be candidates for tracheostomy. When used in select patients, MDO significantly improves obstructive sleep apnea and reduces need for ventilatory support, although feeding support is still needed in most patients at 6 months. Further study in a larger cohort will help identify appropriate candidates for MDO and characterize outcomes of unique patient populations.

Study Objectives Increased neck circumference, a surrogate for the neck fat that can narrow the upper airway in obese individuals, is a risk factor for obstructive sleep apnea syndrome (OSAS) in adults, but the association between neck fat and OSAS in adolescent males and females is unknown. We hypothesized that obese adolescents with OSAS have more neck fat than controls, females more neck fat than males, and that neck fat correlates with obesity and OSAS severity. Methods Obese adolescents with OSAS and obese and normal-weight controls underwent upper airway magnetic resonance imaging, polysomnography, and anthropometrics, including neck circumference measurement. Intra-neck and subcutaneous neck fat measurements were manually segmented and compared among the three groups using ANOVA and between males and females using t-tests. The relationship between polysomnographic parameters and neck fat measurements was assessed in adolescents with OSAS using Pearson correlations. Results One-hundred nineteen adolescents (38 females) were studied: 39 obese with OSAS, 34 obese controls, and 46 normal-weight controls. Neck fat was not greater in adolescents with OSAS compared to obese controls (p=0.35), and neck fat volume was not related to OSAS severity (p = 0.36). However, obese adolescents had more neck fat than normal-weight controls (p < 0.001), and neck fat volume correlated with neck circumference (r = 0.53, p < 0.001). Females had significantly greater cross-sectional neck fat than males (p < 0.001). Conclusions While neck fat is associated with obesity and neck circumference in adolescents and is greater in females versus males, it does not appear to correlate with presence and severity of OSAS.

Beckwith-Wiedemann syndrome (BWS) is the most common epigenetic overgrowth and cancer predisposition disorder. Due to both varying molecular defects involving chromosome 11p15 and tissue mosaicism, patients can present with a variety of clinical features, leading to the newly defined Beckwith-Wiedemann spectrum (BWSp). The BWSp can be further divided into three subsets of patients: those presenting with classic features, those presenting with isolated lateralized overgrowth (ILO) and those not fitting into the previous two categories, termed atypical BWSp. Previous reports of patients with BWS have focused on those with the more recognizable, classic features, and limited information is available on those who fit into the atypical and ILO categories. Here, we present the first cohort of patients recruited across the entire BWSp, describe clinical features and molecular diagnostic characteristics, and provide insight into practical diagnosis and management recommendations that we have gained from this cohort.

Study ObjectivesObstructive sleep apnea (OSA) is associated with cardiovascular and cerebrovascular morbidity. Patients with sickle cell disease (SCD) are at increased risk for both neurologic complications (NC) and OSA. However, the relationship between OSA and SCD complications is unclear. We hypothesized that there would be an association between OSA diagnosis and SCD complications.MethodsHospital discharge records of patients with SCD aged < 19 years were obtained for the years 1997, 2000, 2003, 2006, 2009, and 2012 from the Kid’s Inpatient Database. The primary outcome, NC, a composite of stroke, transient ischemic attack, and seizures. Secondary outcomes included acute chest syndrome (ACS), vaso-occlusive crisis, length of hospital stay, and inflation-adjusted cost of hospitalization. Multivariable regression was conducted to ascertain the association of OSA with primary and secondary outcomes. Analyses were adjusted for the use of noninvasive mechanical ventilation (NIMV) to determine its role as NC risk modifier.ResultsThere were 203,705 SCD discharges included in the analysis, of which 2,820 (1.4%) and 4,447 (2.2%) also included OSA and NC diagnoses. Multivariable logistic regression indicated that OSA was associated with NC (adjusted odds ratio [OR], 1.50 [95% CI 1.02–2.21], p = 0.039) and ACS (OR, 1.34 [95% CI 1.08–1.67], p = 0.009) in children with SCD. In the multivariable analysis adjusted for NIMV, the significant association between OSA and NC was no longer observed (OR, 1.39 [95% CI 0.94–2.05], p = 0.100).ConclusionsOSA is associated with a 50% increase of odds of NC in children with SCD in this nationwide dataset. The use of NIMV to treat OSA may modify the risk of OSA-associated NC.

BACKGROUND: Positive airway pressure (PAP) is a standard therapy for the treatment of OSA in children, but objective data on the effectiveness of PAP in infants are sparse. The aim of this study was to compare the effectiveness of PAP in infants younger than 6 months of age with that in school-aged children. RESEARCH QUESTION: Compared with PAP in school-aged children, can PAP be titrated as successfully in infants, and is adherence to PAP similar in both age groups? STUDY DESIGN AND METHODS: Single-center retrospective study. For consecutive infants younger than 6 months of age and school-aged children 5 to 10 years of age with OSA treated with PAP, baseline and titration polysomnography data, PAP adherence data, and parent-reported barriers to adherence were compared between groups. RESULTS: Forty-one infants and 109 school-aged children were included. Median obstructive apnea hypopnea index (OAHI) in infants was 25.7/h (interquartile range [IQR], 17.8-35.9/h) and was greater than that in school-aged children (12.1/hr; IQR, 7.6-21.5/h; P < .0001). After PAP titration, OAHI was reduced by a median of 92.1% in infants, similar to the median 93.4% reduction in school-aged children (P = .67). PAP was used in infants on 94.7% of nights, which was more than the 83% in school-aged children (P = .003). No differences were found in barriers to adherence between infants and school-aged children, with behavioral barriers being most common in both groups. INTERPRETATION: Objective data demonstrate that PAP is both highly effective at treating OSA and well-tolerated in infants. Like older patients, PAP should be considered along with other therapies for the treatment of OSA in even the youngest children.

Objective: To determine pre- and postoperative prevalence of obstructive sleep apnea (OSA) in patients with 22q11.2 deletion syndrome (DS) undergoing wide posterior pharyngeal flap (PPF) surgery for velopharyngeal dysfunction (VPD). Design: Retrospective study using pre- and postoperative polysomnography (PSG) to determine prevalence of OSA. Medical records were reviewed for patients’ medical comorbidities. Parents were surveyed about snoring. Setting: Academic tertiary care pediatric hospital. Patients: Forty patients with laboratory confirmed 22q11.2DS followed over a 6-year period. Interventions: Pre- and postoperative PSG, speech evaluation, and parent surveys. Main Outcome Measure: Severity and prevalence of OSA, defined by obstructive apnea hypopnea index (OAHI), before and after PPF surgery to determine whether PPF is associated with increased risk of OSA. Results: Mean OAHI did not change significantly after PPF surgery (1.1/h vs 2.1/h, P = .330). Prevalence of clinically significant OSA (OAHI ≥ 5) was identical pre- and postoperatively (2 of 40), with both cases having severe-range OSA requiring positive airway pressure therapy. All other patients had mild-range OSA. Nasal resonance was graded as severe preoperatively in 85% of patients. None were graded as severe postoperatively. No single patient factor or parent-reported concern predicted risk of OSA (OAHI ≥ 1.5). Conclusions: Patients with 22q11.2DS are medically complex and are at increased risk of OSA at baseline. Wide PPF surgery for severe VPD does not significantly increase risk of OSA. Careful perioperative planning is essential to optimize both speech and sleep outcomes.

STUDY OBJECTIVES: Beckwith-Wiedemann syndrome (BWS) is a rare pediatric overgrowth disorder that includes a spectrum of clinical findings including macroglossia, especially in those with loss of methylation at the imprinting control region (IC2 LOM) on chromosome 11. Children with BWS can have very severe obstructive sleep apnea (OSA), but the prevalence of OSA in this population is poorly understood, as is the relationship between OSA and the BWS genotype/phenotype. We hypothesized that there would be a high prevalence of OSA in children with BWS, and that OSA would be more severe in children with IC2 LOM. METHODS: Medical records from children evaluated from March 2015 through January 2018 were reviewed for results from polysomnography, genetic testing, and clinical assessment. RESULTS: A total of 26 children with BWS not previously treated for OSA underwent polysomnography, genetic testing, and clinical assessment. Median (range) age was 5 months (3 days to 33 months). Most children, 20 (76.9%), had an obstructive apnea-hypopnea index (OAHI) > 2 events/h. Median (range) OAHI was 4.4 events/h (0 to 56.1 events/h). OAHI was significantly greater in participants younger than 6 months compared with those older than 6 months (P = .008). Those with IC2 LOM did not have a greater OAHI (P = .61) than those with other genetic causes of BWS, but OAHI had a strong positive correlation with BWS clinical score (Spearman rho = .54, P = .004). CONCLUSIONS: There is a high prevalence of OSA in children with BWS with macroglossia. Younger children with BWS and those with more phenotypic features may be at greatest risk of OSA. CITATION: Cielo CM, Duffy KA, Taylor JA, Marcus CL, Kalish JM. Obstructive sleep apnea in children with Beckwith-Wiedemann syndrome. J Clin Sleep Med. 2019;15(3):375–381.

Sleep-related movement disorders (SRMD) are a group of conditions that involve simple, non-purposeful, and stereotyped movements that occur during sleep or sleep-wake transitions. In pediatric patients, these events may be concerning to parents and may be brought up during the screening for sleep disorders of the allergic child. Fortunately, the majority of these disorders are benign, require no treatment, and resolve spontaneously. Some sleep-related movement disorders, like restless legs syndrome and rhythmic movement disorder, are seen at sleep onset, while others, such as periodic limb movement disorder and benign sleep myoclonus of infancy, occur in the sleeping child. In some cases, like with bruxism, a condition may occur in either during sleep or while the child is awake. Normal variants, including hypnic jerks, hypnogogic foot tremor, and catathrenia, which may be confused with more concerning conditions, are also discussed. For each condition, diagnostic criteria and pediatric literature regarding the evaluation and treatment are reviewed.

Introduction The contribution of nasal cavity narrowing to obstructive sleep apnea (OSA) in children has not been well-defined. Acoustic rhinometry is a non-invasive technique that uses acoustic pulses to measure nasal cavity cross-sectional area (CSA) at defined distances from the tip of the naris. This study evaluated the relationship between nasal cavity CSA and OSA in children. We hypothesized that OSA severity would correlate with reduced nasal CSA. Methods Children 5–12 years of age with OSA underwent polysomnography and acoustic rhinometry at baseline and after 3 months and 12 months of growth as part of a randomized controlled trial. Statistical analysis of the nasal cavity CSA up to 6 cm from the tip of the naris was performed using mixed-effects linear regression model with visit month, age at visit, trial number, OSA severity, and side (left or right naris), and statistical interaction between OAHI category and distance from the tip of the nose as a fixed effect and the random effects set on the level of individual subject. Least significant differences were used to account for multiple comparisons. An unbiased approach using latent class analysis was used to determine OSA severity categories based on obstructive apnea hypopnea index (OAHI). Post-hoc analysis was used assess the model adjusted (marginal) means and pairwise effects. Results 112 participants completed testing (50% male, aged 7.9±2.1 years). Median (IQR) OAHI for subjects with mild OSA (n=69) was 4.4/hr (3.2), moderate OSA (n=35) was 9.7/hr (7.1), and severe OSA (n=8) was 21.3/hr (17.2). There was a significant difference in linear trend for nasal cavity narrowing of the CSA between patients with mild vs. moderate OSA, p=0.023. There was no difference in nasal cavity narrowing between the severe group and other groups, likely due to the small sample size of this group. Conclusion There is a difference in the anterior nasal cavity narrowing between children with mild OSA and moderate OSA. In addition to structural narrowing from adenotonsillar hypertrophy, this may be another contributor to pediatric OSA. Support (if any) R01 HL120909 K23 HL135346 K01 HL130719

Background The obstructive sleep apnoea syndrome (OSAS) results from a combination of structural and neuromotor factors; however, the relative contributions of these factors have not been studied during the important developmental phase of adolescence. We hypothesised that adenotonsillar volume (ATV), nasopharyngeal airway volume (NPAV), upper airway critical closing pressure (Pcrit) in the hypotonic and activated neuromotor states, upper airway electromyographic response to subatmospheric pressure and the ventilatory response to CO 2 during sleep would be major predictors of OSAS risk. Methods 42 obese adolescents with OSAS and 37 weight-matched controls underwent upper airway MRI, measurements of Pcrit, genioglossal electromyography and ventilatory response to CO 2 during wakefulness and sleep. Results ATV, NPAV, activated and hypotonic Pcrit, genioglossal electromyography and ventilatory response to CO 2 during sleep were all associated with OSAS risk. Multivariate models adjusted for age, gender, body mass index and race indicated that ATV, NPAV and activated Pcrit each independently affected apnoea risk in adolescents; genioglossal electromyography was independently associated in a reduced sample. There was significant interaction between NPAV and activated Pcrit (p=0.021), with activated Pcrit more strongly associated with OSAS in adolescents with larger NPAVs and NPAV more strongly associated with OSAS in adolescents with more negative activated closing pressure. Conclusions OSAS in adolescents is mediated by a combination of anatomic (ATV, NPAV) and neuromotor factors (activated Pcrit). This may have important implications for the management of OSAS in adolescents.

In infants with craniofacial disorders, upper airway obstruction is one of the primary causes for morbidity and mortality in the neonatal period. Infants with craniofacial disorders, including Pierre Robin sequence, are at high risk for obstructive sleep apnea syndrome. Because of the complexity of their care, these neonates are usually followed by a multidisciplinary team to ensure timely evaluation and optimal treatment. In addition to history and physical examination, clinical evaluation may include genetic testing, imaging, endoscopy, and polysomnography. There are various treatment options, both surgical and non-surgical, that may be used depending on clinical assessment, underlying condition, and severity of disease. Recent advances have led to better assessment and treatment of these patients, but many questions remain. This review outlines the available literature pertaining to the evaluation and management of upper airway obstruction in the neonate with craniofacial conditions, with a particular focus on Pierre Robin sequence.

Children with craniofacial anomalies are a heterogeneous group at high risk for obstructive sleep apnea (OSA). However, the prevalence and structural predictors of OSA in this population are unknown. We hypothesized that infants with micrognathia would have more significant OSA than those with isolated cleft palate ± cleft lip (ICP), and those with ICP would have more significant OSA than controls. We postulated that OSA severity would correlate with reduced mandibular size, neurodevelopmental scores, and growth.Prospective cohort study. 15 infants with ICP, 19 with micrognathia, and 9 controls were recruited for polysomnograms, neurodevelopmental testing, cephalometrics (ICP and micrognathia groups) at baseline and a follow-up at 6 mo.Baseline apnea-hypopnea index (AHI) [median (range)] of the micrognathia group [20.1 events/h (0.8, 54.7)] was greater than ICP [3.2 (0.3, 30.7)] or controls [3.1 (0.5, 23.3)] (p = 0.001). Polysomnographic findings were similar between ICP and controls. Controls had a greater AHI than previously reported in the literature. Cephalometric measures of both midface hypoplasia and micrognathia correlated with OSA severity. Neurodevelopment was similar among groups. OSA improved with growth in participants with ICP and postoperatively in infants with micrognathia.Micrognathia, but not ICP, was associated with more significant OSA compared to controls. Both midface and mandibular hypoplasia contribute to OSA in these populations. OSA improved after surgical correction in most infants with micrognathia, and improved without intervention before palate repair in infants with ICP.

There are a variety of therapies available for the treatment of pediatric obstructive sleep apnea syndrome (OSAS). In children with enlarged adenoids or tonsils, adenotonsillectomy (AT) is the preferred treatment, but other surgical options include partial tonsillectomy and lingual tonsillectomy. In specific populations, craniofacial or bariatric surgery may be indicated, and tracheostomy should be reserved for cases where there is no other therapeutic option. Positive airway pressure (PAP) is the most effective non-surgical therapy for OSAS as it can be successfully used in even cases of severe OSAS. Nasal steroids and leukotriene receptor antagonists may be used in the treatment of mild or moderate OSAS. Rapid maxillary expansion and dental appliances may be effective in select populations with dental problems. Other non-surgical therapies, such as positional therapy, supplemental oxygen, and weight loss have not been shown to be effective in most pediatric populations.

h T ere are a variety of pediatric patient groups that are at higher risk of obstructive sleep apnea due to a combination of factors that include craniofacial anatomy, muscular weakness, dysfunction in central control of breathing, medication use, and weight gain. In the next sections, we will discuss the pathophysiologic characteristics of specific pediatric populations that place these patients at risk of OSAS.

Introduction Positive Airway Pressure (PAP) is an efficacious treatment of pediatric obstructive sleep apnea syndrome (OSAS). However, it is unknown whether PAP initiation is associated with reduced healthcare utilization, an important metric of care management. We hypothesized that healthcare utilization would be reduced after initiation of PAP in a cohort of pediatric patients prescribed PAP for OSAS. Methods Data were extracted from electronic medical records of 475 patients (Mean±SD age at PAP initiation=7.7±5.7 years; 58.7% male; 40.6% White; 38.3% Black; 18.1% multiracial/other; 12.1% Hispanic/Latinx) prescribed PAP for OSAS and followed in our Sleep Center quality improvement program. We extracted the total number of emergency department (ED) visits and hospitalizations and computed the related average length of stay (LOS) in hours for these visits in the 18 months prior to and 18 months following PAP initiation. Results Paired samples t-tests showed that number of ED visits and hospitalizations, and the related visit LOS, were significantly reduced following PAP initiation. The average number of visits reduced from 2.20 pre-PAP to 1.77 post-PAP initiation [t(474) = 3.48, p Conclusion PAP initiation was associated with fewer and shorter ED visits and hospitalizations in a large sample of pediatric patients. We speculate that PAP initiation could help reduce morbidity associated with pediatric OSAS, as well as improve healthcare utilization, capacity management and care in this population. Support K23HD094905 and Sleep Research Foundation (AAW)

CITATIONCielo CM, Tapia IE. Diving deeper: rethinking AHI as the primary measure of OSA severity. J Clin Sleep Med. 2019;15(8):1075–1076.

Early postnatal tracheostomy for airway compromise is associated with high morbidity and cost. In certain patients with tongue-base airway obstruction (TBAO), mandibular distraction osteogenesis may be preferred. We present a comprehensive analysis of surgical, airway, and cephalometric outcomes in a large series of neonatal patients with TBAO. A retrospective review was performed of patients with laryngoscopically proven TBAO who underwent mandibular distraction osteogenesis before 1 year of age at our institution. Demographic, operative, postoperative, polysomnographic, and radiographic data were analyzed with the appropriate statistical test. Between 2010 and 2013, 28 patients younger than 1 year underwent mandibular distraction for TBAO. Distraction was performed for documented TBAO and failure to thrive at an average age of 58 days (range, 11-312) days with distractor removal after an average of 90 days. Preoperative polysomnograms were obtained on 20 patients with an average apnea-hypopnea index of 39.3 ± 22.0/h; the apnea-hypopnea index on postoperative polysomnograms obtained after distraction completion was significantly reduced in all 14 patients in whom it was measured (mean, 3.0 ± 1.5/h; P < 0.0001). Twenty patients transitioned to oral feeding, and cephalometric and airway diameters were improved (P < 0.0001). Distraction was successful in all but 4 patients including all patients with GILLS scores of 2 or less and 66% of patients with GILLS scores of 3 or greater. Neonatal mandibular distraction is a powerful tool to treat critical obstructive apnea in patients with TBAO. Appropriate patient selection remains a challenge; however, mandibular distraction represents a compelling treatment modality.

Background Children with craniofacial disorders are at increased risk for obstructive sleep apnea syndrome. Methods for diagnosing obstructive sleep apnea syndrome in this population remain controversial. Sleep studies are the criterion standard but are impractical for all patients. The utility of obstructive sleep apnea syndrome questionnaires such as the Pediatric Sleep Questionnaire is unknown in children with craniofacial disorders. The authors hypothesized that the Pediatric Sleep Questionnaire would be a sensitive tool for detecting obstructive sleep apnea syndrome in children with craniofacial abnormalities. Methods A retrospective review of consecutive children with diagnosed craniofacial disorders who both completed the Pediatric Sleep Questionnaire and underwent polysomnography was performed. Demographics, Pediatric Sleep Questionnaire score, and polysomnographic data were recorded. Statistical analysis included calculation of sensitivity, specificity, positive predictive value, and negative predictive value for the Pediatric Sleep Questionnaire. Results Eighty-three children aged 2 to 18 years were included in the study. Of these, 44 (53.0 percent) screened positive on the Pediatric Sleep Questionnaire and 23 (27.7 percent) had polysomnographic evidence of obstructive sleep apnea syndrome, but the sensitivity of the Pediatric Sleep Questionnaire for detecting obstructive sleep apnea syndrome in this sample was only 0.57 and the specificity was 0.48. Positive predictive value and negative predictive value were 0.30 and 0.74, respectively. The correlation between the apnea hypopnea index and Pediatric Sleep Questionnaire score was 0.152 (p = 0.17). Conclusions A substantial portion of craniofacial patients referred for polysomnography was found to have obstructive sleep apnea syndrome. However, the Pediatric Sleep Questionnaire is not a good screening tool for obstructive sleep apnea syndrome in children with craniofacial conditions. More research is needed to determine which patients with craniofacial disorders should be evaluated for obstructive sleep apnea syndrome by polysomnography or other means. Clinical question/level of evidence Diagnostic, II.

Beckwith-Wiedemann syndrome (BWS) is a rare paediatric overgrowth disorder. Associated macroglossia is a feature of many children with BWS and is felt to be a risk factor for obstructive sleep apnoea (OSA). Sleep-disordered breathing is highly variable in this population. The relationship between degree of macroglossia or other genotypic or phenotypic factors and OSA severity has not been established. The natural history of OSA in this population is unknown; a variety of conservative and surgical therapies have been used to treat OSA in children with BWS but none have been studied systematically. Tongue reduction is the mainstay of surgical therapy for macroglossia associated with BWS, but limited data are available regarding its efficacy in treating OSA or its effect on speech and swallowing. More research is needed to better identify which children with BWS are at risk for OSA and the most effective treatment for these patients.

The mainstays of therapy for obstructive sleep apnea in children are adenotonsillectomy and positive airway pressure. Nonsurgical therapies, including anti-inflammatory medications, dental devices, and weight loss, may be useful in specific circumstances. Airway surgery has been shown to be effective in children with craniofacial abnormalities, but not in the general pediatric population. Children with Down syndrome, craniofacial abnormalities, obesity, or Prader-Willi syndrome are at increased risk for obstructive sleep apnea syndrome and may require more frequent polysomnography and specific therapies.

STUDY OBJECTIVES The obstructive sleep apnea syndrome (OSAS) is more prevalent in ex-preterm children compared to the general pediatric population. However, it is unknown whether OSAS in ex-preterm children is associated with specific perinatal risk factors. This multicenter cohort study aimed to determine perinatal factors associated with OSAS at school age. METHODS 197 ex-preterm (500-1,250 g) children aged 5-12 y who participated as neonates in a double-blind, randomized clinical trial of caffeine versus placebo (Caffeine for Apnea of Prematurity) underwent comprehensive ambulatory polysomnography. A negative binomial regression model was used to identify perinatal risk factors associated with OSAS. RESULTS 19 children had OSAS (9.6%). Chorioamnionitis and multiple gestation were positively associated with OSAS with P values of 0.014 and 0.03, respectively. Maternal white race (P = 0.047) and maternal age (P = 0.002) were negatively associated with OSAS. Other risk factors, such as birth weight, Apgar score at 5 min, antenatal corticosteroids, delivery route, and sex were not significant. CONCLUSIONS OSAS is very frequent, and is associated with chorioamnionitis and multiple gestation in ex-preterm children. Those born to older white mothers appear to be protected. We speculate that the former may be due to systemic inflammation and the latter to a higher socio-economic status. COMMENTARY A commentary on this article appears in this issue on page 721.

Little is known about the pediatric population at an increased risk of restless legs syndrome (RLS) and periodic limb movement disorder (PLMD). Polysomnographic data from the Caffeine for Apnea of Prematurity-Sleep (CAPS) study showed a high prevalence of elevated periodic limb movement index (PLMI) in a cohort of ex-preterm children, but the clinical importance of this finding, such as association with RLS, is unknown. We hypothesized that ex-preterm children would have a high prevalence of RLS and PLMD.Ex-preterm children enrolled in CAPS, now aged 5-12 years, completed home polysomnography (PSG) and standardized questionnaires. A diagnosis of RLS or PLMD was established by participants meeting the International Classification of Sleep Disorders, 3rd edition, criteria based on questionnaires and polysomnograms. The clinically available serum ferritin levels were assessed.In total, 167 participants underwent polysomnography and completed all questionnaires. The overall prevalence of RLS was 14/167 (8.4%). An additional 13 subjects (7.8%) were found to have PLMD. Of the 26 participants who had PLMI 5/h, seven (26.9%) had RLS and 13 (50%) had PLMD. The serum ferritin levels were50 mcg/L (range -38.4) for all eight participants referred for testing.Children with a history of prematurity have a high prevalence of RLS, particularly those with elevated periodic limb movements. Iron deficiency likely contributes to RLS and PLMD symptoms in this population. Clinicians evaluating ex-preterm children with sleep disturbances should evaluate for RLS and PLMD. Further studies including serum ferritin evaluation are required to confirm these findings.

Introduction The Children’s Hospital of Philadelphia (CHOP) Global Health Allies program in the Dominican Republic trains nine health promoters (HPs) in various health subjects with the goal to educate the community. This time we evaluated our teaching session on sleep and sleep disorders. Methods Questionnaires were randomly administered to HPs, and community members in the Dominican Republic before and after the HP educational intervention. Nine HPs already enrolled in the CHOP program, received a 4-hour training session in sleep and sleep disorders. HPs were then instructed to educate the community as they usually do during their supervised weekly home visits. Results The nine HP demonstrated knowledge of sleep disorders in their post-training assessment. In the community, 93 adults responded to the initial questionnaire. Although 92 (99%) stated that sleep is important, very few knew about obstructive sleep apnea (n = 17; 18%) and narcolepsy (n = 11; 12%). The post-education questionnaire revealed no statistically significant change in the knowledge about sleep and sleep disorders in the community. Conclusion The HP knowledge about sleep disorders improved after a 4-hour training session. The community knowledge did not change after education by HPs. Barriers to community education must be explored and the need for other forms of community education about sleep and sleep disorders should be addressed.

Obstructive sleep apnoea syndrome (OSAS) is common in children. Craniofacial anomalies such as cleft palate are among the most common congenital conditions. Children with a variety of craniofacial conditions, including cleft palate, micrognathia, craniosynostosis, and midface hypoplasia are at increased risk for OSAS. Available evidence, which is largely limited to surgical case series and retrospective studies, suggests that OSAS can be successfully managed in these children through both surgical and non-surgical techniques. Prospective studies using larger cohorts of patients and including polysomnograms are needed to better understand the risk factors for this patient population and the efficacy of treatment options for OSAS and their underlying conditions.