Spectrum of Disease in Hospitalized Newborns with Congenital Micrognathia: A Cohort of 3,236 Infants at North American Tertiary-Care Intensive Care Units.

OBJECTIVE: To describe the spectrum of disease and burden of care in infants with congenital micrognathia from a multicenter cohort hospitalized at tertiary care centers. STUDY DESIGN: The Children's Hospitals Neonatal Database was queried from 2010 through 2020 for infants diagnosed with micrognathia. Demographics, presence of genetic syndromes, and cleft status were summarized. Outcomes included death, length of hospitalization, neonatal surgery, and feeding and respiratory support at discharge. RESULTS: Analysis included 3,236 infants with congenital micrognathia. Cleft palate was identified in 1266 (39.1%). A genetic syndrome associated with micrognathia was diagnosed during the neonatal hospitalization in 256 (7.9%). Median (IQR) length of hospitalization was 35 (16, 63) days. Death during the hospitalization (n = 228, 6.8%) was associated with absence of cleft palate (4.4%, P < .001) and maternal Black race (11.6%, P < .001). During the neonatal hospitalization, 1289 (39.7%) underwent surgery to correct airway obstruction and 1059 (32.7%) underwent gastrostomy tube placement. At the time of discharge, 1035 (40.3%) were exclusively feeding orally. There was significant variability between centers related to length of stay and presence of a feeding tube at discharge (P < .001 for both). CONCLUSIONS: Infants hospitalized with congenital micrognathia have a significant burden of disease, commonly receive surgical intervention, and most often require tube feedings at hospital discharge. We identified disparities based on race and among centers. Development of evidence-based guidelines could improve neonatal care.

Updated clinical practice recommendations for managing children with 22q11.2 deletion syndrome.

This review aimed to update the clinical practice guidelines for managing children and adolescents with 22q11.2 deletion syndrome (22q11.2DS). The 22q11.2 Society, the international scientific organization studying chromosome 22q11.2 differences and related conditions, recruited expert clinicians worldwide to revise the original 2011 pediatric clinical practice guidelines in a stepwise process: (1) a systematic literature search (1992-2021), (2) study selection and data extraction by clinical experts from 9 different countries, covering 24 subspecialties, and (3) creation of a draft consensus document based on the literature and expert opinion, which was further shaped by survey results from family support organizations regarding perceived needs. Of 2441 22q11.2DS-relevant publications initially identified, 2344 received full-text reviews, including 1545 meeting criteria for potential relevance to clinical care of children and adolescents. Informed by the available literature, recommendations were formulated. Given evidence base limitations, multidisciplinary recommendations represent consensus statements of good practice for this evolving field. These recommendations provide contemporary guidance for evaluation, surveillance, and management of the many 22q11.2DS-associated physical, cognitive, behavioral, and psychiatric morbidities while addressing important genetic counseling and psychosocial issues.

Multimodal Treatment of Robin Sequence Utilizing Mandibular Distraction Osteogenesis and Continuous Positive Airway Pressure.

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.

Comprehensive Long-Term Outcomes Following Mandibular Distraction Osteogenesis.

OBJECTIVE: To describe long-term outcomes and complications following mandibular distraction osteogenesis (MDO) in a diverse patient cohort. DESIGN: Cross-sectional study. SETTING: Single tertiary-care pediatric center. PATIENTS: Forty-eight patients previously undergoing MDO with minimum 4-year follow-up. MAIN OUTCOME MEASURES: Respiratory outcomes, feeding patterns, dental development, motor/sensory nerve function, temporo-mandibular joint function, and postsurgical scarring. RESULTS: Forty-six patients with a median age of 7 years were evaluated. Of 20 nonsyndromic patients, none required additional airway procedures, none required continuous positive airway pressure (CPAP) during sleep, and 19 (95%) fed exclusively by mouth. Among 26 syndromic patients, 7 (27%) required CPAP and 8 (31%) were tube fed. Permanent first molar differences were seen in the majority of subjects; patterns of damage interfering with function were more common in syndromic (13/28, 46%) compared to nonsyndromic (5/24, 21%; P = .014) subjects. MDO prior to age two was associated with more frequent and worse dental damage (P = .001). Inferior alveolar nerve and marginal mandibular nerve function were fully intact in 37 (80%) and 39 (85%) of patients, respectively. Three patients (6%), all with associated genetic syndromes, demonstrated severe nerve impairment. By the Vancouver scar scale, ≥ 80% of surgical scars were rated in the most favorable category for each quality assessed. Temporomandibular joint dysfunction was rare. CONCLUSIONS: MDO shows highly favorable long-term respiratory, feeding, nerve, and scar outcomes in nonsyndromic patients, although permanent molar changes not precluding tooth viability are commonly seen. Patients with associated syndromes demonstrate respiratory and feeding benefits, but higher rates of dental and nerve abnormalities.

Mandibular Distraction Osteogenesis for Tongue-Based Airway Obstruction Without Micrognathia.

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 x̃ = 60.7/h (11.6-109.4) to x̃ = 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.

Cleft palate morphology, genetic etiology, and risk of mortality in infants with Robin sequence.

Robin sequence (RS) has many genetic and nongenetic causes, including isolated Robin sequence (iRS), Stickler syndrome (SS), and other syndromes (SyndRS). The purpose of this study was to determine if the presence and type of cleft palate varies between etiologic groups. A secondary endpoint was to determine the relationship of etiologic group, cleft type, and mortality. Retrospective chart review of patients with RS at two high-volume craniofacial centers. 295 patients with RS identified. CP was identified in 97% with iRS, 95% with SS, and 70% of those with SyndRS (p < .0001). U-shaped CP was seen in 86% of iRS, 82% with SS, but only 27% with SyndRS (p < .0001). At one institution, 12 children (6%) with RS died, all from the SyndRS group (p < .0001). All died due to medical comorbidities related to their syndrome. Only 25% of children who died had a U-shaped CP. The most common palatal morphology among those who died was an intact palate. U-shaped CP was most strongly associated with iRS and SS, and with a lower risk of mortality. RS with submucous CP, cleft lip and palate or intact palate was strongly suggestive of an underlying genetic syndrome and higher risk of mortality.

Defining Risk of Postoperative Obstructive Sleep Apnea in Patients With 22q11.2DS Undergoing Pharyngeal Flap Surgery for Velopharyngeal Dysfunction Using Polysomnographic Evaluation.

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.

Characterization of the Beckwith-Wiedemann spectrum: Diagnosis and management.

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.

Obstructive sleep apnoea and the role of tongue reduction surgery in children with Beckwith-Wiedemann syndrome.

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 obstructive sleep apnoea syndrome in adolescents.

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 CO2 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 CO2 during wakefulness and sleep. RESULTS: ATV, NPAV, activated and hypotonic Pcrit, genioglossal electromyography and ventilatory response to CO2 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.

Obstructive sleep apnoea in children with craniofacial syndromes.

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.

Diagnosis of sleep disorders in child healthcare settings.

OBJECTIVES: Sleep disorders impact at least 10 % of children, pose risks to overall wellbeing, and are key targets of preventive interventions. The objectives of this study were to describe the prevalence of pediatric sleep disorder diagnoses across sociodemographic characteristics and co-occurring conditions, and to explore potential sociodemographic disparities. METHODS: Cross-sectional analysis of 12,394,902 children (0-17 years; 50.9 % Medicaid-insured) in the 2017 MarketScan database. Prevalence was assessed utilizing ICD-10 codes, with multivariate logistic regressions examining disparities (insurance coverage; race and ethnicity in Medicaid-insured) for diagnoses in ≥0.10 % of children. RESULTS: The prevalence of sleep disorder diagnoses was 2.36 %. The most common diagnoses were obstructive sleep disordered breathing (oSDB, 1.17 %), unspecified sleep disorders (0.64 %), insomnia (0.52 %), and other SDB (0.10 %), with <0.10 % for all other diagnoses. Insomnia and parasomnias diagnoses were much lower than diagnostic estimates. Sleep diagnoses were more prevalent in Medicaid versus commercially insured youth, 2-5-year-olds, and in children with co-occurring medical, neurodevelopmental, or behavioral health conditions. Girls and boys were generally equally likely to be diagnosed with any sleep disorder. In Medicaid-insured children, white children were more likely to have any sleep diagnosis compared to all other racial and ethnic groups. Black/African American children were more likely than white children to have oSDB. CONCLUSIONS: Compared to diagnostic estimates, claims data suggest sleep disorders are under-diagnosed, with notable sociodemographic disparities. Findings suggest a need for clinical resources to identify and address sleep disorders and to understand biases potentially driving disparities, given that sleep is a modifiable determinant of child wellbeing.

Incidence of pediatric narcolepsy diagnosis and management: evidence from claims data.

STUDY OBJECTIVES: The purpose of this study was to characterize the incidence of pediatric narcolepsy diagnosis, subsequent care, and potential sociodemographic disparities in a large US claims database. METHODS: Merative MarketScan insurance claims (n = 12,394,902) were used to identify youth (6-17 years of age) newly diagnosed with narcolepsy (International Classification of Diseases, 10th revision codes). Narcolepsy diagnosis and care 1 year postdiagnosis included polysomnography with Multiple Sleep Latency Test, pharmacological care, and clinical visits. Potential disparities were examined by insurance coverage and child race and ethnicity (Medicaid-insured only). RESULTS: The incidence of narcolepsy diagnosis was 10:100,000, primarily type 2 (69.9%). Most diagnoses occurred in adolescents with no sex differences, but higher rates in Black vs White youth with Medicaid. Two thirds had a prior sleep disorder diagnosis and 21-36% had other co-occurring diagnoses. Only half (46.6%) had polysomnography with Multiple Sleep Latency Test (± 1 year postdiagnosis). Specialty care (18.9% pulmonary, 26.9% neurology) and behavioral health visits were rare (34.4%), although half were prescribed stimulant medications (51.0%). Medicaid-insured were 86% less likely than commercially insured youth to have any clinical care and 33% less likely to have polysomnography with Multiple Sleep Latency Test. CONCLUSIONS: Narcolepsy diagnoses occurred in 0.01% of youth, primarily during adolescence, and at higher rates for Black vs White children with Medicaid. Only half overall had evidence of a diagnostically required polysomnography with Multiple Sleep Latency Test, underscoring potential misdiagnosis. Many patients had co-occurring conditions, but specialty and behavioral health care were limited. Results suggest misdiagnosis, underdiagnosis, and limited narcolepsy treatment, as well as possible disparities. Results highlight the need to identify determinants of evidence-based pediatric narcolepsy diagnosis and management. CITATION: Tang SH, Min J, Zhang X, et al. Incidence of pediatric narcolepsy diagnosis and management: evidence from claims data. J Clin Sleep Med. 2024;20(7):1141-1151.

What's New in Pediatric Obstructive Sleep Apnea?

Obstructive sleep apnea (OSA) is common children. There is a demand for more family-focused evaluation and novel diagnostic approaches. Drug-induced sleep endoscopy is increasingly being used clinically in children with Down syndrome and other comorbidities. Several studies have examined the association between OSA and other comorbidities during childhood. Therapeutic options for OSA in children remain limited. Recent studies have examined the utility of hypoglossal nerve stimulation in children with Down syndrome. Positive airway pressure has been a mainstay of OSA treatment. Several recent studies have assessed factors associated with adherence. Infants are challenging to treat for OSA.

Sociodemographic disparities and healthcare utilization in pediatric obstructive sleep apnea management.

OBJECTIVES: We examined (1) disparities in obstructive sleep apnea (OSA) care by insurance coverage, and by child race and ethnicity among Medicaid-insured children (MIC), and (2) healthcare utilization changes after OSA care. METHODS: IBM MarketScan insurance claims were used to index OSA care 1-year before and after initial OSA diagnosis in 2017 among 2-17-year-old children (n = 31,787, MIC: 59%). OSA care and healthcare utilization analyses adjusted for child age, sex, obesity, and complex chronic conditions. RESULTS: We identified 8 OSA care pathways, including no care, which occurred in 34.4% of the overall sample. MIC had 13% higher odds of no OSA care compared to commercially-insured children (CIC). MIC had 32-48% lower odds of any treatment pathway involving specialty care, but a 13-46% higher likelihood of receiving surgical care without polysomnogram (PSG) and PSG only. In MIC, non-Latinx Black/African American (Black) and Hispanic/Latinx children were 1.3-2.2 times more likely than White children to receive treatment involving specialty care and/or PSG, while Black children were 31% less likely than White youth to undergo surgery. In the full sample, surgical care was associated with less outpatient and emergency healthcare utilization compared to those untreated or not surgically treated. CONCLUSIONS: Varied OSA management by insurance coverage suggests disparities in access to and engagement in care and potentially greater disease burden among MIC. Surgical care is associated with reduced healthcare utilization. The lower odds of surgery in Black MIC should be further evaluated in the context of OSA severity, healthcare biases, and family preferences.

Feasibility and performance of home sleep apnea testing in youth with Down syndrome.

STUDY OBJECTIVES: In-laboratory polysomnography is recommended for the evaluation of obstructive sleep apnea (OSA) in youth with Down syndrome. However, insufficient sleep laboratories are available, particularly for youth with neurocognitive disabilities such as Down syndrome. We hypothesized that level II home sleep apnea testing (HSAT) would be feasible, acceptable, and accurate in detecting polysomnography-defined moderate-severe OSA in youth with Down syndrome. METHODS: Youth 6 to 25 years old with Down syndrome were recruited to undergo in-home level II HSAT with electroencephalogram and in-lab polysomnography. Parents completed questionnaires assessing feasibility, acceptability, and test preference. HSAT, scored blinded to polysomnography result, were compared to reference polysomnography. RESULTS: Forty-three youth (23 female) aged [median (range)] 15.5 (6.1, 25.1) years participated in the study. Forty-one participants were able to complete HSAT and 41 completed polysomnography, with 40 who underwent both tests. HSAT was preferred to polysomnography by 73.7% of parents. Total sleep time for HSAT was 437 ± 123 minutes vs 366 ± 90 minutes for polysomnography (P = .003). Obstructive apnea-hypopnea index by polysomnography was 12.7 events/h (0.2, 113.8), and 32 youth (80%) who completed all testing had OSA. Compared to polysomnography, sensitivity of HSAT was: 0.81, specificity was 0.75, accuracy was 0.8 including 2 youth whose HSAT demonstrated OSA when polysomnography did not. CONCLUSIONS: In youth with Down syndrome, level II HSAT was well-tolerated, preferred compared to in-lab polysomnography, and had good accuracy for detecting moderate-severe OSA. Level II HSAT could provide a means for expanding the evaluation of OSA in youth with Down syndrome. CITATION: Cielo CM, Kelly A, Xanthopoulos M, et al. Feasibility and performance of home sleep apnea testing in youth with Down syndrome J Clin Sleep Med. 2023;19(9):1605-1613.

Surgical outcome and treatment trends in 1289 infants with micrognathia: a multicenter cohort.

BACKGROUND: Studies of infants with micrognathia, especially Robin Sequence (RS), are limited by its rarity and both phenotypic and diagnostic variability. Most knowledge of this condition is sourced from small, single-institution samples. METHODS: This is a cross-sectional study including infants with micrognathia admitted to 38 Children's Hospital Neonatal Consortium centers from 2010-2020. Predictor variables included demographic data, birth characteristics, cleft and syndrome status. Outcome variables included length of stay (LOS), death, feeding or respiratory support, and secondary airway operations. RESULTS: 1289 infants with micrognathia had a surgery to correct upper airway obstruction. Mean age and weight at operation were 34.8±1.8 weeks and 3515.4±42 grams, respectively. A syndromic diagnosis was made in 150 (11.6%) patients, with Stickler (5.4%) and Treacher Collins Syndromes (2.2%) most common. Operations included: mandibular distraction osteogenesis (MDO), 66.3%; tracheostomy, 25.4%; and tongue-lip adhesion (TLA), 8.3%. Tracheostomy patients had a lower birth weight, head circumference, gestational age, and APGAR scores. MDO patients were less likely to need a second airway operation compared to TLA patients (3.5%vs17.8%,p<0.001). The proportion of infants feeding exclusively orally at hospital discharge differed significantly, from most to least: MDO, TLA, tracheostomy. Hospital LOS was not statistically different for patients that had MDO and TLA, but was longer for those with primary tracheostomy. Mortality was low for all operations (0.5%). CONCLUSION: In this 1289 surgical patient cohort, MDO was associated with shorter hospital stay, improved oral feeding, and lower rates of secondary airway operations. Prospective multi-center studies are necessary to support these conclusions.

Clinical utility of repeated positive airway pressure titrations in children with obstructive sleep apnea syndrome.

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 SpO2 nadir were 14.8 (8.7-32.7) events/h and 88.5% (85-92%), respectively. The mean differences in obstructive apnea-hypopnea index, SpO2 nadir, and % total sleep time with SpO2 < 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.

Respiratory indices during sleep in healthy infants: A prospective longitudinal study and meta-analysis.

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 < 0.001 for each), and a lower proportion of events were obstructive apneas (8.6%, p = 0.007) and a greater proportion central apneas (52.3%, p = 0.002). Meta-analysis found high AHI in infants with significant heterogeneity. CONCLUSIONS: Central AHI and obstructive AHI are greater in healthy newborns than older children. There is a significant spontaneous reduction in events and change in type of events in the first six months of life in this low-risk population. These findings may serve as a reference for clinicians evaluating for obstructive sleep apnea in infants.