ABSTRACT
INTRODUCTION: Hypoglossal nerve stimulation (HGNS) is an effective therapy for carefully selected pediatric patients with Down syndrome (DS) and obstructive sleep apnea (OSA), but optimization of device settings has been focused on stimulation settings. The potential benefits of sensing lead setting optimization have not been investigated. We describe a series of three cases of HGNS in children with DS where optimization of both the stimulation lead and sensing lead settings, as well as utilization of drug-induced sleep endoscopy (DISE), when indicated, was more effective than stimulation lead optimization alone. METHODS: Using our novel titration algorithm, among the three identified pediatric patients with DS in whom HGNS initially failed to reduce the apnea-hypopnea index (AHI), two patients in whom sleep studies were characterized as hypopnea-dominant were assigned to advanced titration (optimization of stimulation settings and sensing lead settings). The third patient, with an obstructive apnea-dominant sleep study, was referred for interrogated DISE. Advanced titration included adjustment of both stimulation settings and sensing lead settings. RESULTS: Application of the advanced titration algorithm, tailored to obstructive apnea- versus hypopnea-dominant sleep patterns, resulted in approximately 50% or greater reduction in AHI in all three patients. "Normal to mild OSA" AHI (AHI <5) was achieved in two patients. CONCLUSIONS: A novel diagnostic and therapeutic titration algorithm for the Inspire HGNS system significantly reduced AHI in three pediatric patients with DS and OSA who had failed to respond to the standard device titration that focused solely on stimulation settings. LEVEL OF EVIDENCE: 4 Laryngoscope, 2024.
ABSTRACT
Obstructive sleep apnea (OSA) is common in children with Down syndrome (DS). Adenoidectomy and/or tonsillectomy are the usual first interventions employed to treat OSA in children with DS but sometimes do not achieve adequate resolution of clinical signs. Positive airway pressure treatment is often used next, but this treatment is poorly tolerated by this population. Persistent OSA can adversely affect a child's health and cognitive development. Hypoglossal nerve stimulation (HGNS), previously shown to be safe and effective in adults with OSA, has been used in children as young as 10 years old with DS and has achieved measurable neurocognitive benefits. The US Food and Drug Administration recently lowered the age for HGNS implantation to 13 years for children with DS. However, questions remain regarding treatment of refractory OSA in younger children. Here, we report the case of a 4-year-old boy with DS and treatment-refractory OSA who underwent successful HGNS implantation. The decision to proceed with HGNS implantation in such a young child involved discussions about anatomic feasibility and potential neurocognitive benefits. The device was implanted without complication and with minimal postoperative bulk. This case suggests a possible treatment option that can be discussed in the course of shared decision-making between clinicians and families of young children with DS and treatment-refractory OSA.