title: Hypoglossal Nerve Stimulator Implantation in an Adolescent With Down Syndrome and Sleep Apnea.
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.
authors.affiliation: Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts; Departments of Otology and Laryngology and.
authors.forename: Gillian R
authors.initials: GR
authors.lastname: Diercks
authors.affiliation: Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts; Departments of Otology and Laryngology and Pediatric Sleep Associates and.
authors.forename: Donald
authors.initials: D
authors.lastname: Keamy
authors.affiliation: Pediatric Sleep Associates and Pediatrics, Harvard Medical School, Boston, Massachusetts;
authors.forename: Thomas Bernard
authors.initials: TB
authors.lastname: Kinane
authors.affiliation: Pediatrics, Harvard Medical School, Boston, Massachusetts; Down Syndrome Program, Division of Medical Genetics, Department of Pediatrics, Massachusetts General Hospital, Boston, Massachusetts; and.
authors.forename: Brian
authors.initials: B
authors.lastname: Skotko
authors.affiliation: Pediatrics, Harvard Medical School, Boston, Massachusetts; Down Syndrome Program, Division of Medical Genetics, Department of Pediatrics, Massachusetts General Hospital, Boston, Massachusetts; and.
authors.forename: Allison
authors.initials: A
authors.lastname: Schwartz
authors.affiliation: Pediatric Sleep Associates and.
authors.forename: Ellen
authors.initials: E
authors.lastname: Grealish
authors.affiliation: Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts; Departments of Otology and Laryngology and.
authors.forename: John
authors.initials: J
authors.lastname: Dobrowski
authors.affiliation: Department of Otolaryngology, University of Pittsburgh, Pittsburgh, Pennsylvania.
authors.forename: Ryan
authors.initials: R
authors.lastname: Soose
authors.affiliation: Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts; Departments of Otology and Laryngology and christopher_hartnick@meei.harvard.edu.
authors.forename: Christopher J
authors.initials: CJ
authors.lastname: Hartnick
mesh_terms: D000293:Adolescent; D004314:Down Syndrome / Q000150:complications*; D004599:Electric Stimulation Therapy / Q000295:instrumentation* / Q000379:methods*; D004567:Electrodes, Implanted; D006801:Humans; D007002:Hypoglossal Nerve / Q000502:physiology; D008297:Male; D020181:Sleep Apnea, Obstructive / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*