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Multicenter Study
. 2022 Jun 1;148(6):522-528.
doi: 10.1001/jamaoto.2022.0455.

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

Phoebe K Yu  1 , Matthew Stenerson  1 , Stacey L Ishman  2 , Sally R Shott  2 , Nikhila Raol  3 , Ryan J Soose  4 , Allison Tobey  4 , Cristina Baldassari  5   6 , Raj C Dedhia  7 , Margaret B Pulsifer  1   8 , Julie A Grieco  8 , Leonard J Abbeduto  9 , Thomas B Kinane  10 , Donald G Keamy Jr  1 , Brian G Skotko  5   11 , Christopher J Hartnick  1
Affiliations
Multicenter Study

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

Phoebe K Yu et al. JAMA Otolaryngol Head Neck Surg. .

Abstract

Importance: Patients 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 measures: The 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.

Trial registration: ClinicalTrials.gov Identifier: NCT02344108.

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Conflict of interest statement

Conflict of Interest Disclosures: Dr Yu reported receiving research support from the American Thoracic Society Academic Sleep Pulmonary Integrated Research/Clinical Fellowship. Mr Stenerson reported receiving grants from Massachusetts Eye and Ear during the conduct of the study. Dr Ishman reported receiving grants from Inspire Medical, the National Heart, Lung, and Blood Institute, and the National Institute of Dental and Craniofacial Research outside the submitted work. Dr Soose reported receiving grants from Inspire Medical; and personal fees from Inspire Medical, XII Medical, and Cryosa Inc outside the submitted work. Dr Baldassari reported receiving grants from Inspire Medical outside the submitted work. Dr Dedhia reported receiving grants from Inspire Medical and Nyxoah Medical outside the submitted work. Dr Pulsifer reported serving in a nonpaid capacity on the Board of Directors for the Massachusetts Down Syndrome Congress. Dr Abbeduto reported receiving funding from the National Institutes of Health, US Department of Education, Simons Foundation, National Fragile X Foundation, F. Hoffmann-La Roche Ltd, Roche TCRC Inc, NeurenPharmaceuticals Ltd, and LuMind IDSC Down Syndrome Foundation outside the submitted work. Dr Kinane reported serving as a paid consultant for Sarepta Therapeutics for US Food and Drug Administration submission of drug for Duchenne muscular dystrophy. Dr Skotko reported receiving personal fees from Gerson Lehrman Group, Down syndrome nonprofit organizations, and Woodbine House Inc; grants from F. Hoffmann-La Roche Inc, AC Immune, and LuMind IDSC Down Syndrome Foundation research grant; serving as an expert witness and receiving personal fees for legal cases related to Down syndrome outside the submitted work; serving in a nonpaid capacity on the Honorary Board of Directors for the Massachusetts Down Syndrome Congress and the Professional Advisory Committee for the National Center for Prenatal and Postnatal Down Syndrome Resources; and having a sister with Down syndrome. No other disclosures were reported.

Figures

Figure.
Figure.. Study Design
AHI indicates apnea-hypopnea index; BMI, body mass index (calculated as weight in kilograms divided by height in meters squared); OSA, obstructive sleep apnea; and PAP, positive airway pressure. aAt 12 months, 1 family was unwilling to return for the final polysomnogram because of the COVID-19 pandemic, resulting in a final analysis sample of 41 patients.
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