Lloyd, Robin M.

BACKGROUND & OBJECTIVE Sleep-disordered breathing (SDB) may be a remediable contributor to abnormal cognitive outcomes for children with myelomeningocele. We investigated the frequency of SDB among neonates with fetal vs postnatal myelomeningocele repair. METHODS In this nine-center, prospective, observational study, neonates with myelomeningocele born at more than 30 weeks gestational age underwent a 10-hour bedside polysomnogram at at least 35 weeks postmenstrual age, before hospital discharge. The primary outcome was SDB severity, reflected by the apnea-hypopnea index (AHI; number of respiratory events per hour of sleep). Decisions regarding surgical timing (fetal vs postnatal), technique (open fetal vs fetoscopic approach), and SDB treatment were made by the clinical teams. RESULTS Among 173 infants, 92/173 (53%) had SDB (median AHI 22.5, IQR 12.2–44.2). In unadjusted analysis, AHI was higher among the 90 neonates with fetal repair vs 83 with postnatal surgery (median 29, IQR 14–49, vs 19, IQR 10–36, P = .008). In propensity-adjusted multivariable models, prematurity was independently associated with AHI, whereas surgical timing (fetal vs postnatal) was not. Hindbrain herniation, myelomeningocele level, and clinical need for ventriculoperitoneal shunt did not predict AHI in adjusted models. Supplemental oxygen was commonly prescribed, for 44% of fetal vs 39% of postnatal myelomeningocele repair infants (P = .5). CONCLUSIONS SDB affects more than half of neonates with myelomeningocele. The higher AHI after fetal surgery could stem from the elevated incidence of prematurity in this group. As SDB is treatable and has a potential impact on neurodevelopment, routine screening could become an integral part of a strategy to optimize long-term medical and neurodevelopmental outcomes.

Abstract Introduction The SleepImage is an HSAT device (sHSAT) that uses photoplethysmography and oxygen saturation data to generate an apnea hypopnea index (AHI). This sHSAT has been FDA cleared as a software-as-a-medical-device for use in children 2 years and older for diagnosing obstructive sleep apnea (OSA). To our knowledge this sHSAT has not been tested on children concurrent with the gold standard polysomnography (PSG). Methods This study evaluated 50 children 2-6 years of age with tonsillar hypertrophy and OSA symptoms with sHSAT during simultaneous in-lab PSG. Aims were to determine the technical feasibility of using sHSAT in this population and to compare sHSAT results to PSG. The primary endpoint was AHI by sHSAT compared to AHI by PSG and presence of OSA. Sensitivity analysis was performed for presence or absence of OSA diagnosis based on sHSAT versus PSG. Bland-Altman and concordance correlation calculations were used for comparisons. Results 50 patients, median age 4 (IQR 3-5), met criteria; 39 completed PSG and HSAT. The sensitivity with sHSAT for OSA prevalence based on proprietary categorization was 94.1%, specificity 22.7%, LR+ 1.22, and LR- 0.26, with PSG OSA prevalence of 43.6%. There was a 9% sHSAT total failure rate, and the device frequently fell off, resulting in TST difference of -226.9 ±182.8 min between sHSAT and PSG. PSG was trimmed to include only segments where HSAT was confirmed to be on before comparisons. Concordance correlation coefficient between sAHI and AHI-PSG was 0.52. The Bland-Altman analysis comparing sAHI and PSG AHI revealed a mean difference of 0.73 (95% confidence limits: -3.47 to 4.93). Considering only obstructive events, the Bland-Altman the sAHI vs the AHI-PSG shows a mean difference of 1.52 (95%CI -5.40 to 8.44). Conclusion This pilot study demonstrated challenges with feasibility in using the sHSAT in this age group given likelihood of technical difficulties leading to absent or truncated data. When compared with PSG, sHSAT demonstrated a high false-positive rate for diagnosis of OSA, particularly concerning if guiding surgical management. This topic is of considerable importance to the practice of pediatric sleep medicine; further investigation of technological advances in this area is imperative. Support (if any)

Abstract Introduction Insomnia is a common sleep disorder in pediatric patients with neurodevelopmental disabilities. There are no FDA approved medications for insomnia in the pediatric population, and treatment can be challenging. We present a case of successful treatment of sleep maintenance insomnia in a 15-year-old male with doxepin with supportive actigraphy data. Report of case(s) A 15-year-old male with a past history pertinent for a genetic neurodevelopmental disorder, autism spectrum disorder (ASD) and attention deficit hyperactivity disorder (ADHD) was evaluated in Pediatric Sleep Medicine Clinic for long standing difficulties with sleep maintenance. He experienced frequent, prolonged nocturnal awakenings and early morning awakenings. Previous polysomnogram was negative for sleep disordered breathing. He previously trialed clonidine, guanfacine, trazodone, melatonin, and mirtazapine without significant benefit. Baseline actigraphy demonstrated frequent and prolonged nocturnal awakenings and early morning awakenings. He was initiated on doxepin 3 mg at bedtime, and it was subsequently increased to 6 mg at bedtime. Parental report and repeat actigraphy 4 months later (on doxepin 6 mg) demonstrated improvement in nocturnal awakenings and early morning awakenings. Conclusion Up to now, there are no FDA approved medications for the treatment of insomnia in the pediatric population. A prior study of patients with ASD and ADHD demonstrated the use of doxepin provided clinical improvement in sleep onset and maintenance insomnia based on subjective evidence with parental report. In this case, doxepin improved subjective report of insomnia and correlated with objective findings on actigraphy. Support (if any) NA

Abstract Introduction A prospective, longitudinal, multicenter study was designed to test the hypothesis that sleep-disordered breathing (SDB) is ubiquitous among newborns with myelomeningocele (MMC). We evaluated variability in polysomnography (PSG) scoring and interpretation. Methods Nine US pediatric centers participated. Neonates with MMC born at >30 weeks gestation underwent bedside PSG at ≥35 weeks postmenstrual age, before hospital discharge. PSGs were scored by registered technologists (RPSGTs) at each site using AASM Scoring Manual infant criteria and interpreted by a board-certified sleep medicine physician (BCSMP). A single, central RPSGT blinded to clinical outcomes then re-scored all PSGs. One of two pediatric BCSMPs reviewed each study; the two BCSMPs jointly determined diagnoses for consensus central reports. Differences in scoring and overall PSG interpretation were evaluated with kappa and intraclass correlation coefficients (ICC). Results For 110 neonates with MMC, the median apnea-hypopnea index (AHI) was 26.5 [11.3-43.3] as reported by sites, and 23.0 [IQR 13.0-44.8] as reported centrally (к 0.81). The central apnea index (CAI) was also scored similarly by sites (3.3 [0.9-7.0]) and centrally (2.6 [0.6-7.3]; к 0.88), as were hypopneas (15.2 [7.5;26.9]) and 14.1 [6.4;27.9]; к 0.82). However, the obstructive apnea index (OAI) was reported to be much higher by sites (10.0 [1.9-29.1]) than centrally (1.5 [0.5 – 4.1]; к 0.29). Moreover, overall PSG interpretation differed between sites and central reviewers. Obstructive sleep apnea (OSA) was reported as a diagnosis by sites for 57.6% of neonates, vs. 10.9% by central review (ICC 0.06); central sleep apnea was reported by sites for 26.6% of neonates, vs. 14.5% by central review (ICC 0.50). When hypopneas were the most prevalent respiratory events, central review favored a less specific diagnosis of sleep-disordered breathing. Conclusion Considerable variability in scoring of obstructive apneas, and in polysomnography-based diagnoses, may exist for neonates. Sites may have designated hypopneas as obstructive, driving the diagnosis of OSA, while central review noted equivocal features of neonatal hypopneas, leading to a less specific diagnosis of sleep-disordered breathing. Further work to develop infant respiratory scoring rules, and diagnostic criteria, and to evaluate their clinical use may be warranted. Support (if any) This study is supported by NIH (R01 HL147261).