Long-term non-invasive ventilation (NIV) is a common modality of breathing support used for a range of sleep and respiratory disorders. The aim of this scoping review was to provide a summary of the literature relevant to long-term NIV use in children. We used systematic methodology to identify 11,581 studies with final inclusion of 289. We identified 76 terms referring to NIV; the most common term was NIV (22%). Study design characteristics were most often single center (84%), observational (63%), and retrospective (54%). NIV use was reported for 73 medical conditions with obstructive sleep apnea and spinal muscular atrophy as the most common conditions. Descriptive data, including NIV incidence (61%) and patient characteristics (51%), were most commonly reported. Outcomes from sleep studies were reported in 27% of studies followed by outcomes on reduction in respiratory morbidity in 19%. Adverse events and adherence were reported in 20% and 26% of articles respectively. Authors reported positive conclusions for 73% of articles. Long-term use of NIV has been documented in a large variety of pediatric patient groups with studies of lower methodological quality. While there are considerable data for the most common conditions, there are fewer data to support NIV use for many additional conditions.

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Continuous positive airway pressure (CPAP) is the standard treatment for moderate-to-severe obstructive sleep apnoea (OSA). However, adherence to CPAP is limited and non-CPAP therapies are frequently explored. Oral appliance (OA) therapy is currently widely used for the treatment of snoring, mild, moderate and severe OSA. The most commonly used and studied OA consists of a maxillary and mandibular splint which hold the lower jaw forward during sleep. The efficacy of OA is inferior to CPAP; however, the effectiveness as measured by sleepiness, quality of life, endothelial function and blood pressure is similar likely due to higher acceptance and subjective adherence. Upper airway stimulation augments neural drive by unilaterally stimulating the hypoglossal nerve. The Stimulation Therapy for Apnea Reduction (STAR) study enrolled 126 patients and demonstrated a 68% reduction in OSA severity. A high upfront cost and variable response are the main limitations. Oropharyngeal exercises consist of a set of isometric and isotonic exercises involving the tongue, soft palate and lateral pharyngeal wall. The collective reported trials (n = 120) showed that oropharyngeal exercises can ameliorate OSA and snoring (~30-40%). Nasal EPAP devices consist of disposable one-way resister valve. A systematic review (n = 345) showed that nasal EPAP reduced OSA severity by 53%. The Winx device consists of a mouthpiece placed inside the oral cavity that is connected by tubing to a console that generates negative pressure. Winx may provide successful therapy for~30-40% of OSA patients. In conclusion, several non-CPAP therapies to treat OSA are currently available.

Scientific inveStigAtionSStudy Objectives: Mandibular repositioning appliance (MRA) therapy is a treatment option for patients with mild to moderate sleep apnea and for patients who do not tolerate continuous positive airway pressure. Titration of MRAs consists of sequential mandibular advancement guided by symptom improvement. The goal of the study was to determine if patients with an elevated apnea hypopnea index (AHI), despite the use of a subjectively optimized MRA, could achieve better results with additional titration during polysomnography (PSG). Methods: Patients were enrolled if they had an AHI ≥ 15/h and were referred for MRA therapy. The MRA was advanced until symptoms improved. During the PSG, the technologist monitored the patient's sleep and increased mandibular protrusion until the AHI was improved. Results: There was a significant improvement in AHI, minimum oxygen saturation, and total sleep time with the MRA before further advance-ment. At the final PSG, 65.2% of patients had an AHI ≤ 10 associated with at least a 50% reduction in AHI. The incomplete responders had their appliance further titrated, and this improved the results of MRA therapy by 30.4% to a total success rate of 95.6%. Conclusions: This study shows that it is possible to improve the results of MRA therapy by further advancing the appliance during a titration PSG in patients with an incomplete response. The titration night improved the results of the usual clinical advancement of the MRA with substantially more patients achieving a successful outcome.

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