Phoenix Children’s is one of the first health systems in the country to offer pediatric-focused hypoglossal nerve stimulation (HGNS) treatment for sleep apnea. Our experts manage more than 40 pediatric patients with hypoglossal nerve stimulators, making this pediatric cohort one of the largest in the nation. The multidisciplinary team includes specialists from Otolaryngology, Sleep Medicine and Developmental Pediatrics.
Hypoglossal nerve stimulation is an advanced and emerging therapeutic approach designed to manage obstructive sleep apnea (OSA). OSA affects an estimated 3-10% of children in the general population, but the prevalence rises significantly among children with neurologic disorders. In children with epilepsy or cerebral palsy, 20-50% are affected, while up to 60% of children with Down syndrome experience OSA.
Significant OSA in children contributes to cognitive, behavioral and growth/metabolism disruption, among other complications. Most patients with OSA can be managed with traditional measures such as tonsil and adenoid removal and/or medical therapy. For some children, such as those with Down syndrome or those with craniofacial disorders, the etiology of the obstruction is multifactorial and does not respond with these standard treatments. HGNS offers a novel intervention for these cases aimed at enhancing airway patency through targeted neurostimulation, providing an innovative solution for children who require more advanced treatments.
The HGNS Alternative
HGNS works by delivering controlled electrical impulses to the hypoglossal nerve, which activates the genioglossus muscle, helping to maintain airway patency during sleep. These impulses are delivered through a small pulse generator implanted subcutaneously in the patient’s upper chest. Then, a stimulating electrode placed near the hypoglossal nerve in the neck – and a sensing lead, placed in the chest wall below the implanted generator – ensures that stimulation occurs in synchrony with the patient’s inhalation. The system is programmed to activate once the patient falls asleep. The patient and the parent(s) remain in control of the therapy.
Hypoglossal nerve stimulation is primarily utilized for patients who are not suitable candidates for conventional treatments or who are intolerant of traditional therapies such as positive airway pressure. The device is currently approved by the Food and Drug Administration for children aged 13 and older with Down syndrome who have persistent OSA despite prior tonsillectomy and adenoidectomy, have an apnea hypopnea index (AHI) between 10 and 50 and are not candidates for continuous positive airway pressure (CPAP) therapy. Contraindications to HGNS usage at this time include complete concentric collapse of the soft palate, abnormal control of the upper airway, central/mixed apnea > 25% of the total AHI, and patients with an implantable device that may have an unintended interaction with the hypoglossal nerve stimulation.
Ongoing studies are exploring the use of HGNS with other patient populations. Potential future patients include children with craniofacial disorders and mask-fit difficulties, children with sensory disorders such as autism who are unable to tolerate positive airway pressure, and patients for whom positive airway pressure may not be preferred, such as patients with frequent nocturnal seizures.
Pediatric patients referred for HGNS evaluation typically have been diagnosed with severe OSA through an overnight sleep study. They then undergo drug-induced sleep endoscopy to evaluate the specific sites of airway obstruction and determine candidacy for hypoglossal nerve stimulation. Appropriate candidates undergo surgical implantation followed by a series of clinical visits and repeat polysomnograms with technologists trained in HGNS titration who work to identify the most effective settings for each individual patient.
Improving Overall Quality of Life for Patients
Research has shown that treating OSA in children with Down syndrome using HGNS leads to improvements in communication, attention and overall quality of life. These same positive outcomes are echoed in the patients treated at Phoenix Children’s.
OSA can have a significant impact on quality of life and function in pediatric patients. Physicians should refer patients with symptoms suggestive of OSA (snoring, pauses in breathing, restless sleep, daytime hypersomnolence and daytime hyperactivity) to our experienced providers in Sleep Medicine or Otolaryngology for evaluation. Potential candidates for HGNS will be evaluated by the multidisciplinary HGNS team.
Phoenix Children’s multidisciplinary approach, combined with the use of emerging, innovative therapies such as HGNS, helps improve outcomes for those most at risk for negative complications from OSA.
References
- Caloway CL, Diercks GR, Keamy D, de Guzman V, Soose R, Raol N, Shott SR, Ishman SL, Hartnick CJ. Update on hypoglossal nerve stimulation in children with down syndrome and obstructive sleep apnea. Laryngoscope. 2020 Apr;130(4):E263-E267. doi: 10.1002/lary.28138. Epub 2019 Jun 20. PMID: 31219619.
- Grieco JA, Hartnick CJ, Skotko BG, Yu PK, Pulsifer MB. Preliminary Neurocognitive Results Post Hypoglossal Nerve Stimulation in Patients with Down Syndrome. Laryngoscope. 2021 Dec;131(12):2830-2833. doi: 10.1002/lary.29808. Epub 2021 Aug 7. PMID: 34363400.