Sleep-disordered breathing in children with neurodisabilities

Recent evidence has suggested that low socioeconomic status (SES), race, prematurity, and maternal smoking during pregnancy are associated with childhood sleep disordered breathing (SDB). We investigated (1) the association of SDB with a wide range of risk factors, including prenatal and perinatal complications; (2) the association of these complications with SES and race; and (3) the association of SDB with developmental milestones. Six hundred thirteen school-aged children (105 clinically referred and 508 community control subjects) underwent overnight polysomnography and had a complete history and physical examination. A comprehensive child development questionnaire was completed by a parent. We compared clinically referred children with SDB to population-based control children without SDB from The Penn State Children's Cohort. Maternal smoking during pregnancy; maternal age and weight gain during pregnancy; prenatal complications, such as maternal high blood pressure and gestational diabetes; perinatal complications related to prematurity; delayed motor milestones; race and SES were significantly associated with the presence of childhood SDB. Most of the risk factors became nonsignificant when analyses controlled for race and SES. Delayed motor milestones remained significantly associated with SDB after controlling for race and SES. These data suggest that there is a significant association between children who experience prenatal or perinatal distress and the development of moderate to severe childhood SDB. SES and race may be mediating the impact on SDB through increased prenatal and perinatal risks. The significant delay in motor milestones suggests that prenatal and perinatal distress may result in neurologic insult, which could influence the development of SDB in later childhood.

Chiari malformation Type I (CM-I) has been associated with sleep disordered breathing (SDB). The aim of this study was to evaluate the prevalence of SDB in CM-I and its clinical correlates in a population of children and adolescents. Fifty-three consecutive children and adolescents affected by CM-I were enrolled (27 girls and 26 boys, mean age 10.3 ± 4.3, range: 3-18 years). All patients underwent neurological examination, MRI, and polysomnography (PSG). Otorhinolaryngologic clinical evaluation was performed in patients with polysomnographic evidence of sleep-related upper airway obstruction. Mean size of the herniation was 9.5 ± 5.4 mm. Fourteen patients had syringomyelia, 5 had hydrocephalus, 31 presented neurological signs, 14 had epileptic seizures, and 7 reported poor sleep. PSG revealed SDB in 13 subjects. Patients with SDB, compared to those without SDB, had a higher prevalence hydrocephalus (p = 0.002), syringomyelia (p = 0.001), and neurological symptoms (p = 0.028). No significant difference was observed in age, gender, prevalence of epilepsy, and size of the herniation. Obstructive SDB was associated with syringomyelia (p = 0.004), whereas central SDB was associated with hydrocephalus (p = 0.034). In our population of CM-I patients the prevalence of SDB was 24%, lower than that reported in literature. Moreover, our findings suggest that abnormalities in cerebrospinal fluid dynamics in CM-I, particularly syringomyelia and hydro-cephalus, are associated with SDB.

Individuals with Duchenne muscular dystrophy (DMD) frequently develop sleep-disordered breathing. Noninvasive ventilation is often prescribed for sleep-disordered breathing treatment based on the American Academy of Sleep Medicine (AASM) criteria. In 2018, DMD disease-specific criteria for sleep-disordered breathing were established. Our study aimed to examine the clinical interpretation differences using these different criteria. We performed a multicenter, retrospective chart review of children with DMD followed at The Hospital for Sick Children, Toronto, Canada, and Rady Children's Hospital, San Diego, California, who underwent polysomnography from August 1, 2012, to February 29, 2020. Baseline characteristics and polysomnography data were summarized using descriptive statistics. Agreement for the diagnosis of sleep-disordered breathing evaluated by kappa statistics and sensitivity/specificity analysis was assessed. One hundred five male children with DMD (mean ± SD age: 12.1 ± 3.8 years; body mass index z score: 0.2 ± 2.3) were included. The proportions of children with DMD that met at least 1 AASM criterion and at least 1 DMD criterion were 45.7% and 67.6%, respectively. We found that 32.4% of children met neither AASM nor DMD criteria. Overall agreement between AASM and DMD criteria was moderate (k = 0.57). There was almost perfect agreement in sleep apnea diagnosis (k = 0.90); however, there was only slight agreement in hypoventilation diagnosis (k = 0.12) between AASM and DMD criteria. There were more children with DMD diagnosed with nocturnal hypoventilation and prescribed noninvasive ventilation using DMD criteria compared with AASM criteria. Future studies should address whether the prescription of noninvasive ventilation for children with DMD based on both criteria is associated with different clinical outcomes. Hurvitz MS, Sunkonkit K, Massicotte C, Li R, Bhattacharjee R, Amin R. Characterization of sleep-disordered breathing in children with Duchenne muscular dystrophy by the American Academy of Sleep Medicine criteria vs disease-specific criteria: what are the differences? J Clin Sleep Med. 2022;18(2):609-615.

The aim of this study was to describe sleep-disordered breathing (SDB) and treatment in children with achondroplasia (ACH). A retrospective longitudinal study was conducted at the Sleep Unit, Hospital de Pediatría Garrahan. Children with ACH, aged 0 to 18 years, referred for SDB due to clinical symptoms or foramen magnum stenosis (FMS) with at least one polysomnography (PSG) between 2002 and 2019 were included in the study. The primary outcomes included SDB typification and therapeutic interventions. We included 89 patients; 79.7% had one PSG, 13.4% had two PSGs, and 5.6% had three PSGs. The first PSG confirmed SDB in 65.16% patients. Obstructive sleep apnea (OSA) was present in 59.6% patients, central sleep apnea (CSA) in 1.72% patients, OSA + CSA in 4.49% patients, and normal PSG in 34.83% patients. OSA was mild in 32.07% patients, moderate in 18.86% patients, and severe in 49.05% patients. Clinical and airway evaluation, central imaging, evoked potentials, and PSG were considered before each therapeutic decision. After the first PSG, patients received one or more treatments as needed: watchful waiting; nasal steroids; ear, nose, and throat surgery; foramen magnum decompression; and noninvasive ventilation. A second PSG was available in 18 patients and 72.22% had SDB. A third PSG was available in 10 patients and 77.8% had SDB. We reconfirm an elevated frequency of SDB in ACH children and underline the need for a multidisciplinary and stepwise longitudinal approach.

Novel methods to diagnose obstructive sleep apnea in children

To assess the accuracy of novel algorithms using an oximeter-based finger plethysmographic signal in combination with a nasal cannula for the detection and differentiation of central and obstructive apneas. The validity of single pulse oximetry to detect respiratory disturbance events was also studied. Patients recruited from four sleep laboratories underwent an ambulatory overnight cardiorespiratory polygraphy recording. The nasal flow and photoplethysmographic signals of the recording were analyzed by automated algorithms. The apnea hypopnea index (AHI(auto)) was calculated using both signals, and a respiratory disturbance index (RDI(auto)) was calculated from photoplethysmography alone. Apnea events were classified into obstructive and central types using the oximeter derived pulse wave signal and compared with manual scoring. Sixty-six subjects (42 males, age 54 ± 14 yrs, body mass index 28.5 ± 5.9 kg/m(2)) were included in the analysis. AHI(manual) (19.4 ± 18.5 events/h) correlated highly significantly with AHI(auto) (19.9 ± 16.5 events/h) and RDI(auto) (20.4 ± 17.2 events/h); the correlation coefficients were r = 0.94 and 0.95, respectively (p < 0.001) with a mean difference of -0.5 ± 6.6 and -1.0 ± 6.1 events/h. The automatic analysis of AHI(auto) and RDI(auto) detected sleep apnea (cutoff AHI(manual) ≥ 15 events/h) with a sensitivity/specificity of 0.90/0.97 and 0.86/0.94, respectively. The automated obstructive/central apnea indices correlated closely with manually scoring (r = 0.87 and 0.95, p < 0.001) with mean difference of -4.3 ± 7.9 and 0.3 ± 1.5 events/h, respectively. Automatic analysis based on routine pulse oximetry alone may be used to detect sleep disordered breathing with accuracy. In addition, the combination of photoplethysmographic signals with a nasal flow signal provides an accurate distinction between obstructive and central apneic events during sleep.

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. 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. 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. 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.

In Western societies, sleep apnea (SA) has emerged as a major medical problem with important social ([1][1]) and financial implications. The issue may be even more relevant in nephrology because some of the factors involved in the pathogenesis of renal disease are the same that cause, or are

Sleep problems and sleep disordered breathing in children with cerebral palsy.

Freire C, Sennes LU, Polotsky VY. Opioids and obstructive sleep apnea. J Clin Sleep Med. 2022;18(2):647-652.

Citation:Start J. Portable pediatric sleep diagnostics: 2 measures closer to reliable home sleep apnea tests for all ages. J Clin Sleep Med. 2021;17(4):617–618.

Sleep-disordered breathing is a key childhood complication in children with achondroplasia. This retrospective study aimed to document the prevalence of sleep-disordered breathing in children with achondroplasia assessed by polysomnography. The prevalence of sleep-disordered breathing assessed by polysomnography among children aged 0-18 years with achondroplasia from 2013 to 2024 at The Royal Children's Hospital, Australia, was retrospectively reviewed. The cohort included 80 children with achondroplasia (54% females, 95% confirmed molecular diagnosis) with an average number of 3.6 polysomnographies collected per child (n=288). A total of 85% (68/80) had sleep-disordered breathing and 21% reported no prior symptoms. Sleep-disordered breathing subtypes included obstructive sleep apnoea in 81% (55/68), central sleep apnoea in 3% (2/68), mixed sleep apnoea in 7% (5/68) and primary snoring in 9% (6/68). Among those with obstructive and mixed sleep apnoea, 58% (35/60) had moderate or severe obstructive sleep apnoea. In 44 children, a corresponding MRI was evaluated for foramen magnum stenosis using the Achondroplasia Foramen Magnum Score. No correlation was found with sleep-disordered breathing severity (Spearman's coefficient (ρ)=0.03). Among 27 children who received a precision therapy for achondroplasia (vosoritide, n=18, infigratinib, n=8 and recifercept, n=1), the median respiratory disturbance index/hour improved from 2.7 (25th-75th percentile, (0.9-4.8)) to 1.1 (0.3-2.6) after 1 year of treatment compared with baseline. Sleep-disordered breathing was present in 85% of 80 children with achondroplasia, with 21% being asymptomatic. Respiratory parameters did not correlate with foramen magnum stenosis severity and improved after 1 year of treatment in those treated with a precision therapy.

IntroductionPrader – Willi Syndrome (PWS) is a rare genetic multisystem disorder, with a high prevalence of sleep disordered breathing (SDB). Treatments include; non-invasive ventilation (NIV), oxygen therapy and adenotonsillectomy. Growth...

IntroductionPrader – Willi Syndrome (PWS) is a rare genetic multisystem disorder, with high prevalence of sleep disordered breathing (SDB). Treatments include; non-invasive ventilation (NIV), oxygen therapy and adenotonsillectomy. Growth Hormone...

Sleep is generally considered to be a protected period, when the cardiovascular system benefits from the restorative influences of the sleeping brain. However, the dynamics of cardiovascular control during sleep can tax the capacity of the diseased coronary circulation and myocardium with surges in sleep-state–related autonomic activity and disruptions in airway function and central nervous system regulation. In this regard, sleep may constitute an autonomic stress test for the heart. The scope of sleep-related risk for atrial and ventricular arrhythmias is substantial. The major subgroups susceptible to adverse influences of surges in autonomic activity during sleep are those with ischemic heart disease, heart failure, and channelopathies (Table).1 It is significant that 20% of myocardial infarctions and 15% of sudden deaths occur at night in the United States.2 Most atrial arrhythmias in patients younger than 61 years of age have nocturnal onset.3 The young are not immune to risk, as sudden infant death syndrome (SIDS) claims 2500 lives in the United States annually.4 Cardiovascular risk is compounded by comorbid factors, most notably apnea, which affects an estimated 4% to 9% of the general population5 and is considerably more prevalent among obese individuals.6 The more common form is obstructive sleep apnea (OSA), with partial or complete collapse of the pharynx. Half of heart failure patients experience either OSA or central sleep apnea (CSA) with central nervous system–mediated periodic breathing, commonly referred to as Cheyne-Stokes respiration. Such cardiorespiratory disturbances profoundly alter autonomic nervous system activity and increase risk of arrhythmia, hypertension, and myocardial infarction. View this table: Table. Patient Groups at Potentially Increased Risk for Nocturnal Cardiac Events It is surprising, as recently underscored by Malhotra and Loscalzo,7 that the significance of cardiovascular risk during sleep may not be duly recognized within the cardiology community. The reasons are …