Evaluation of Upper Airway Stimulation for Adolescents With Down Syndrome and Obstructive Sleep Apnea

Abstract

IMPORTANCEPatients with Down syndrome have a high incidence of persistent obstructive sleep apnea (OSA) and limited treatment options. Upper airway hypoglossal stimulation has been shown to be effective for adults with OSA but has not yet been evaluated for pediatric populations.OBJECTIVE To evaluate the safety and effectiveness of upper airway stimulation for adolescent patients with Down syndrome and severe OSA.DESIGN, SETTING, AND PARTICIPANTS This prospective single-group multicenter cohort study with 1-year follow-up was conducted between April 1, 2015, and July 31, 2021, among a referred sample of 42 consecutive adolescent patients with Down syndrome and persistent severe OSA after adenotonsillectomy.INTERVENTION Upper airway stimulation. MAIN OUTCOMES AND MEASURESThe prespecified primary outcomes were safety and the change in apnea-hypopnea index (AHI) from baseline to 12 months postoperatively. Polysomnographic and quality of life outcomes were assessed at 1, 2, 6, and 12 months postoperatively. RESULTS Among the 42 patients (28 male patients [66.7%]; mean [SD] age, 15.1 [3.0] years), there was a mean (SD) decrease in AHI of 12.9 (13.2) events/h (95% CI, -17.0 to -8.7 events/h). With the use of a therapy response definition of a 50% decrease in AHI, the 12-month response rate was 65.9% (27 of 41), and 73.2% of patients (30 of 41) had a 12-month AHI of less than 10 events/h. The most common complication was temporary tongue or oral discomfort, which occurred in 5 patients (11.9%). The reoperation rate was 4.8% (n = 2). The mean (SD) improvement in the OSA-18 total score was 34.8 (20.3) (95% CI, -42.1 to -27.5), and the mean (SD) improvement in the Epworth Sleepiness Scale score was 5.1 (6.9) (95% CI, -7.4 to -2.8). The mean (SD) duration of nightly therapy was 9.0 (1.8) hours, with 40 patients (95.2%) using the device at least 4 hours a night.CONCLUSIONS AND RELEVANCE Upper airway stimulation was able to be safely performed for 42 adolescents who had Down syndrome and persistent severe OSA after adenotonsillectomy with positive airway pressure intolerance. There was an acceptable adverse event profile with high rates of therapy response and quality of life improvement.

Hypoglossal nerve stimulation in adolescents with down syndrome and obstructive sleep apnea: A systematic review and meta-analysis

“…Diercks et al reported the first case of HNS treatment for a 14-year-old boy, whose AHI dropped from 48.5 to 3.4 events/h ( 15 ). Yu et al followed 42 patients for a year; the findings revealed that the average AHI decreased by 12.9 ± 13.2 events/h, 65.9% of the patients experienced a 50% decrease in AHI, and 73.2% of the patients had an AHI of <10 events/h ( 18 ). After excluding 2 case reports, a total of 104 patients were included in the analysis; pooled data revealed significantly lower AHI in patients after HNS (mean AHI reduction 17.43 events/h, 95% CI 13.98–20.88 events/h, P < 0.001); however, there was moderate heterogeneity between the studies (I 2 = 42%, P =0.11).…”

“…Five studies used the OSA-18 (a validated, disease-specific quality of life instrument for OSA) and Epworth Sleepiness Scale (ESS) questionnaires, to examine the improvements in treatment durations ( 18 , 21 , 23 , 25 , 26 ). As a result, it improved their sleep quality and subjective feelings.…”

“…Kay et al found that snoring, daytime sleepiness, behavioral problems, and supine sleeping improved in these patients ( 20 ). According to two studies, HNS can decrease patients' ESS scores in terms of sleep quality ( 18 , 21 ). In addition, Greco et al found that participants' neurocognitive and behavioral outcomes were also amended ( 22 ).…”

“…Four investigations examined the necessity to optimize stimulation voltage for arousal during treatment ( 15 , 18 , 20 , 23 ). According to research, AHI decreases as stimulation voltage increases, with 21.1 events/h at 1.3 V, 3.7 at 1.4 V, and 3.4 at 1.5 V ( 15 ).…”

“…The requirements for studies to be included were as follows: (I) Types of participants – adolescents (the age ranges from 10 to 21 years) ( 18 ) with DS and OSA; (II) type of intervention - HNS; and (III) type of language - English. The exclusion criteria included relevant publications, reporting of only surgeries, cell experiments, comments, no outcomes, not adolescents, and repeat investigations.…”