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Hypoglossal Nerve Stimulator Implantation in an Adolescent With Down Syndrome and Sleep Apnea
Gillian R Diercks1, Donald Keamy2, Thomas Bernard Kinane3
1Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts; Departments of Otology and Laryngology and.
Insights
Obstructive sleep apnea (OSA) in children with Down syndrome is common and often persists. A hypoglossal nerve stimulator effectively treated severe OSA in a pediatric patient, allowing tracheotomy removal.
Area of Science:
- Neurology
- Otolaryngology
- Pediatric Medicine
Background:
- Obstructive sleep apnea (OSA) affects up to 60% of children with Down syndrome.
- Persistent OSA in this population often requires invasive treatments like tracheotomy due to poor tolerance of continuous positive airway pressure (CPAP).
Observation:
- A hypoglossal nerve stimulator, an implantable device stimulating tongue protrusion, has shown efficacy in adult OSA patients.
- This report details the first pediatric implantation of a hypoglossal nerve stimulator in an adolescent with Down syndrome and severe, refractory OSA.
Findings:
- The adolescent patient had severe OSA (AHI: 48.5 events/hour) and failed CPAP, necessitating a tracheotomy.
- Hypoglossal nerve stimulator therapy was well-tolerated and significantly improved OSA (overall AHI: 3.4 events/hour).
- The patient's tracheotomy was successfully removed five months post-implantation, with continued positive outcomes.
Implications:
- Hypoglossal nerve stimulation presents a viable, effective treatment option for pediatric patients with Down syndrome and severe OSA refractory to other therapies.
- This innovative approach may reduce the need for long-term tracheotomies in this vulnerable patient group.
- Further research into pediatric hypoglossal nerve stimulator use could expand treatment paradigms for complex sleep-disordered breathing.
Abstract:
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.
