Hypoglossal Nerve Stimulation in Adolescents With Down Syndrome and Obstructive Sleep Apnea

Abstract

Importance: Obstructive sleep apnea (OSA) affects up to 60% of children with Down syndrome (DS) and may persist in half of patients after adenotonsillectomy. Children with DS who have persistent OSA often do not tolerate treatment with positive pressure airway support devices or tracheotomy for their residual moderate to severe OSA. The hypoglossal nerve stimulator is an implantable device that delivers an electrical impulse to anterior branches of the hypoglossal nerve in response to respiratory variation, resulting in tongue base protrusion that alleviates upper airway obstruction in adults.

Objective: To determine whether hypoglossal nerve stimulation is safe and effective in children with DS.

Design, setting, and participants: Case series of the first 6 adolescents with DS to undergo hypoglossal nerve stimulator implantation. Participants were 6 children and adolescents (12-18 years) with DS and severe OSA (apnea hypopnea index [AHI] > 10 events/h) despite prior adenotonsillectomy.

Intervention: Inspire hypoglossal nerve stimulator placement.

Main outcomes and measures: Patients were monitored for adverse events. Adherence to therapy was measured by hours of use recorded by the device. Efficacy was evaluated by comparing AHI and OSA-18, a validated quality-of-life instrument, scores at baseline and follow-up.

Results: In 6 patients (4 male, 2 female; aged 12-18 years), hypoglossal nerve stimulator therapy was well tolerated (mean use, 5.6-10.0 h/night) and effective, resulting in significant improvement in OSA. At 6- to 12-month follow-up, patients demonstrated a 56% to 85% reduction in AHI, with an overall AHI of less than 5 events/h in 4 children and less than 10 events/h in 2 children. Children also demonstrated a clinically significant improvement (mean [SD] overall change score, 1.5 [0.6]; range, 0.9-2.3) on the OSA-18, a validated quality-of-life instrument.

Conclusions and relevance: Hypoglossal nerve stimulation was well tolerated and effective in the study population, representing a potential therapeutic option for patients with DS and refractory OSA after adenotonsillectomy who are unable to tolerate positive pressure airway devices.

Trial registration: clinicaltrials.gov Identifier: NCT2344108.

Trial registration: ClinicalTrials.gov NCT02344108 NCT02344108.

Conflict of interest statement

Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest. Dr Kinane reports he was a paid consultant for Sarepta Therapeutics for US Food and Drug Administration submission of drug for Duchenne muscular dystrophy. Dr Skotko reports grants from Transition Therapeutics and F. Hoffmann-La Roche, Inc; consults on the topic of Down syndrome (DS) through Gerson Lehrman Group; has an R40 grant to study obstructive sleep apnea in patients with DS from the Health Resources and Services Administration’s Maternal & Child Health Bureau; receives remuneration from DS nonprofit organizations for speaking engagements and associated travel expenses; receives annual royalties from Woodbine House, Inc, for a family-oriented book about DS; and has served as an expert witness for legal cases in which DS is discussed. Dr Skotko also serves in a nonpaid capacity on the Honorary Board of Directors for the Massachusetts Down Syndrome Congress, the Board of Directors for the Band of Angels Foundation, and the Professional Advisory Committee for the National Center for Prenatal and Postnatal Down Syndrome Resources. Dr Skotko has a sister with DS. Dr Soose reports grants and personal fees from Inspire Medical Systems, outside the submitted work. No other disclosures are reported.