Obstructive sleep apnea (OSA) is more common in children with Down syndrome, affecting up to 60% of patients, and may persist in up to 50% of patients after adenotonsillectomy. These children with persistent moderate to severe OSA require continuous positive airway pressure, which is often poorly tolerated, or even tracheotomy for severe cases. The hypoglossal nerve stimulator is an implantable device that produces an electrical impulse to the anterior branches of the hypoglossal nerve, resulting in tongue protrusion in response to respiratory variation. It is an effective treatment of sleep apnea in select adult patients because it allows for alleviation of tongue base collapse, improving airway obstruction. Herein we describe the first pediatric hypoglossal nerve stimulator implantation, which was performed in an adolescent with Down syndrome and refractory severe OSA (apnea hypopnea index [AHI]: 48.5 events/hour). The patient would not tolerate continuous positive airway pressure and required a long-standing tracheotomy. Hypoglossal nerve stimulator therapy was well tolerated and effective, resulting in significant improvement in the patient’s OSA (overall AHI: 3.4 events/hour; AHI: 2.5–9.7 events/hour at optimal voltage settings depending on sleep stage and body position). Five months after implantation, the patient’s tracheotomy was successfully removed and he continues to do well with nightly therapy.
Hypoglossal Nerve Stimulator Implantation in an Adolescent With Down Syndrome and Sleep Apnea
POTENTIAL CONFLICT OF INTEREST: Dr Skotko is occasionally asked to serve as an expert witness for legal cases in which Down syndrome is discussed; the other authors have indicated they have no potential conflicts of interest to disclose.
FINANCIAL DISCLOSURE: Inspire Medical Systems provided technical support and provided the nerve stimulators used in this study free of charge. Dr Skotko occasionally consults on the topic of Down syndrome through Gerson Lehrman Group; he has a R40 grant to study obstructive sleep apnea in patients with Down syndrome from the Health Resources and Services Administration’s Maternal and Child Health Bureau and receives remuneration from Down syndrome nonprofit organizations for speaking engagements and associated travel expenses; he also receives annual royalties from Woodbine House, Inc, for the publication of his book, Fasten Your Seatbelt: A Crash Course on Down Syndrome for Brothers and Sisters. The other authors have indicated they have no financial relationships relevant to this article to disclose.
- Views Icon Views
- Share Icon Share
- Search Site
Gillian R. Diercks, Donald Keamy, Thomas Bernard Kinane, Brian Skotko, Allison Schwartz, Ellen Grealish, John Dobrowski, Ryan Soose, Christopher J. Hartnick; Hypoglossal Nerve Stimulator Implantation in an Adolescent With Down Syndrome and Sleep Apnea. Pediatrics May 2016; 137 (5): e20153663. 10.1542/peds.2015-3663
Download citation file: