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Objective: Studies have shown a high prevalence of sleep-disordered breathing (SDB) among children with myelomeningocele (MMC), but there are few published data on the longitudinal care of these patients. The objective of this study was to determine the effectiveness of standard treatments for SDB in children with MMC., Methods: The authors analyzed records from three multidisciplinary spina bifida clinics to identify all patients with both MMC and SDB diagnosed by polysomnography (PSG). The primary outcome of this study was a change in apnea-hypopnea index (AHI; the number of apneic or hypopneic events per hour of sleep) before and after clinically recommended SDB treatments. Clinical and demographic variables were recorded and evaluated for possible association with posttreatment improvement of AHI. Analysis included change in AHI (a continuous variable) and whether SDB improved (defined as an AHI < 2.5 or decrease of AHI by ≥ 50% from baseline)., Results: Seventy-one eligible patients (aged 2 days-21 years, 52% male) had an initial AHI > 2.5 and had follow-up PSG after treatment for SDB. The mean AHI decreased from 20.5 (SD 21.6) at baseline to 11.6 (SD 15.7) after treatment (p = 0.0006). Children treated with supplemental oxygen and with continuous positive airway pressure had improvement on PSG (18 of 25 and 12 of 18, respectively). Children treated with adenotonsillectomy were less likely to improve (7 of 19). Forty-one patients (58%) improved from a baseline AHI > 2.5 to an AHI < 2.5 after treatment., Conclusions: Children with MMC and SDB who undergo standard SDB treatments guided by pediatric sleep medicine physicians show improvement in PSG parameters after treatment.

Objective: The objective was to identify clinical and radiological factors associated with sleep-disordered breathing (SDB) in children with Chiari type I malformation (CIM) and to evaluate the efficacy of foramen magnum decompression (FMD) in resolving SDB., Methods: A retrospective chart review was conducted for all children evaluated for CIM at a single institution from 2002 to 2022, identifying all children who had undergone nocturnal polysomnography (PSG). Apnea-hypopnea index (AHI) score, sleep apnea type (obstructive, central, mixed, and unspecified), clinical manifestations, and radiological measurements were recorded. SDB was considered present when officially diagnosed in the PSG report. Logistic regression was performed to identify factors correlating with the presence of SDB. For children with SDB who underwent FMD, the Wilcoxon signed-rank test was used to assess AHI improvement., Results: Of the 997 children referred for CIM, 310 completed PSG. SDB was diagnosed in 147 patients (overall prevalence 14.7%, 95% CI 12.7%-17.1%; prevalence among children with PSG 47.4%, 95% CI 41.9%-53%). Specific SDB diagnosis consisted of 33% of patients with central sleep apnea, 27% with obstructive sleep apnea, 9% mixed, and 31% unspecified. Lower cranial nerve (CN) dysfunction (OR 3.891, p = 0.009), tonsillar position (OR 1.049, p = 0.017), Chiari type 1.5 malformation (OR 1.862, p = 0.044), and BMI (OR 1.039, p = 0.036) were significantly associated with presence of SDB. Of the 310 patients who underwent PSG, 47 were originally categorized as asymptomatic: 27 (57%) of these asymptomatic patients were diagnosed with SDB on PSG. Of children diagnosed with SDB, 34 completed PSG before and after FMD. Median AHI score decreased from 6.5 preoperatively to 1.8 postoperatively, with a median (IQR) difference of -2.3 (-11.9 to 0.1) (p = 0.001). Twelve (35%) had resolution of SDB., Conclusions: The authors' findings suggest that the prevalence of SDB in children with CIM is high (15%-47%). Furthermore, lower CN dysfunction, Chiari type 1.5, lower tonsillar position, and higher BMI may be risk factors. Notably, SDB can be present even in the absence of clinical symptoms. This study also demonstrates that surgical intervention has the potential to reduce the severity of SDB. These results could help clinicians identify CIM patients at risk for SDB and those who may benefit from surgical decompression.

Background: Pierre Robin sequence (PRS) is a triad of congenital facial abnormalities that can present as a syndrome (syndromic PRS [sPRS]) or an isolated entity (isolated PRS [iPRS]). Patients with PRS can develop airway and feeding problems that may result in failure to thrive. Mandibular distraction osteogenesis (MDO) is a method for improving the functional issues associated with breathing and feeding. There is a Paucity of literature evaluating the outcomes of MDO between sPRS and iPRS patients., Methods: An institutional review board-approved retrospective review of PRS patients managed by a single surgeon and treated with MDO between January 2015 and December 2019 at a tertiary referral hospital was performed. The patients were stratified into iPRS or sPRS based on gene testing. Airway outcome measures included avoidance of tracheostomy, relief of sleep apnea, and oxygen saturation improvement. Primary feeding measures included achievement of full oral feeds and growth/weight gain. Statistical analysis included t tests and χ2 tests where appropriate using SPSS., Results: Over the study period, of the 29 infants with PRS, 55% identified as iPRS and 45% as sPRS. There were no significant differences in the patient characteristics, apnea-hypoxia index (22.27 ± 12.27) and laryngeal view (3 ± 0.79) pre-MDO. After MDO, 83% of the subjects achieved a positive feeding outcome and 86% achieved a positive airway outcome with no statistical significance between sPRS and iPRS (P = 0.4369). There was a statistically significant change post-MDO in apnea-hypoxia index (5.24 ± 4.50, P = 0.02) and laryngeal view (1.59 ± 1.00, P = 0.01)., Conclusions: Our recent experience would lead us to believe that sPRS patients have greater morbidities and challenging clinical developments that, when properly evaluated, can be managed by MDO. There is a potential role for MDO in reducing the need for traditional surgical interventions for respiratory and feeding problems in both iPRS and sPRS patients., Competing Interests: Conflicts of interest and sources of funding: This article was funded in part by the National Center for Advancing Translational Sciences of the National Institutes of Health under award number UL1TR003096., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Retrospective studies have shown high rates of sleep disordered breathing in children with myelomeningocele. However, most patients included in those studies underwent polysomnography because of symptoms, so the prevalence of sleep disordered breathing in this population is unknown., Objective: To determine the prevalence of sleep disordered breathing in children with myelomeningocele using screening polysomnography., Methods: In this cross-sectional study, all children with myelomeningocele seen in a multi-disciplinary spina bifida clinic between 2016 and 2020 were referred for polysomnography regardless of clinical symptoms. Included children had not previously undergone polysomnography. The primary outcome for this study was presence of sleep disordered breathing, defined as Apnea-Hypopnea Index (AHI, number of apnea or hypopnea events per hour of sleep) greater than 2.5. Clinical and demographic variables relevant to myelomeningocele were also prospectively collected and tested for association with presence of sleep apnea., Results: A total of 117 participants underwent polysomnography (age 1 mo to 21 yr, 49% male). The majority were white, non-Hispanic. Median AHI was 1.9 (interquartile range 0.6-4.2). A total of 49 children had AHI 2.5 or greater, yielding a sleep disordered breathing prevalence of 42% (95% CI 33%-51%). In multivariable logistic regression analysis, children with more rostral neurological lesion levels had higher odds of sleep disordered breathing (OR for thoracic, mid-lumbar, and low-lumbar: 7.34, 3.70, 4.04, respectively, compared to sacral level, P = .043)., Conclusion: Over 40% of a sample of children with myelomeningocele, who underwent screening polysomnography, had significant sleep disordered breathing. Routine screening polysomnography may be indicated in this population., (© Congress of Neurological Surgeons 2020.)

Study Objectives: Nocturnal hypoxemia is associated with increased risk of sickle cell disease (SCD) complications. The association of nighttime hypoxemia and acute chest syndrome (ACS) in children with SCD has yet to be determined., Methods: This is a retrospective study of children with SCD who underwent polysomnography at a SCD center. Univariate logistic regression was used to assess the association between nocturnal hypoxemia and ACS admissions. Multivariate logistic regression was performed to verify the effects of different clinical covariates on ACS. Secondary analysis comparing patients with one vs multiple ACS admissions was performed., Results: One hundred ten individuals with SCD who completed their polysomnogram (mean age of 9.4 years) were identified. Fifty-nine (54%) had a history of at least one episode of ACS admission (mean age of 4.1 years), including 40 with multiple episodes. The percentage of total sleep time with O₂ saturation < 90% was greater in the ACS group (P < .05). Similarly, mean nocturnal O₂ saturation was lower in the ACS group (P < .0005). Mean nocturnal O₂ saturation of < 97.3% and the percentage of total sleep time with O₂ saturation < 90% higher than 2.7% were associated with ACS. There was no difference in nocturnal hypoxemia between patients with single and multiple ACS admissions., Conclusions: Nocturnal hypoxemia later in life is associated with previous ACS admissions in children with SCD. This can increase the yield of interpreting polysomnograms in this vulnerable population. Prospective studies are needed to determine the temporal relations of nocturnal hypoxemia and ACS, which may identify a modifiable risk for ACS., (© 2021 American Academy of Sleep Medicine.)