1289 Late Onset Laryngomalacia Manifesting as Severe Obstructive Sleep Apnea in a 14-Year-Old Male

Abstract Introduction Laryngomalacia is a common occurrence in infants but a very rare phenomenon in adolescents and adults. It can present as stridor, snoring, exercise intolerance, or dysphagia. Diagnosis is confirmed with bronchoscopy. We present a case of a 14-year-old male with late onset laryngomalacia manifesting as severe obstructive sleep apnea (OSA). Report of case(s) Fourteen year-old male with history of presumed perinatal left MCA stroke, Lennox-Gastaut syndrome, hemiplegic cerebral palsy, and intellectual disability. He presented to Pediatric Pulmonology with a two-month history of new-onset snoring and worsening oral aversion/dysphagia. His mother disclosed videos illustrating his severe snoring, apneic pauses, and gasping during sleep. Prior to this two-month period, the patient had no snoring nor signs/symptoms of sleep disordered breathing for at least ten years. Patient history included tonsillectomy and adenoidectomy at 4-years-old for suspicion of OSA. Polysomnography (PSG) was performed and revealed severe OSA with apnea hypopnea index (AHI) of 31.8 and oxygen desaturation nadir of 70%, with minimal central apneas. No seizure activity was appreciated. Due to concern that patient’s vagal nerve stimulator (VNS) was negatively contributing to OSA by causing airway collapse, a drug-induced sleep endoscopy (DISE) with microlaryngoscopy, rigid bronchoscopy, and flexible bronchoscopy was performed. Airway evaluation revealed severe laryngomalacia with complete arytenoid prolapse, shortened epiglottic folds, and epiglottic retroflexion. No evidence of tracheomalacia or bronchomalacia was seen. No difference in the severity of laryngomalacia or upper airway collapse was noted between VNS being on or off during DISE. Thus, VNS was continued, and airway obstruction was treated with positive airway pressure (PAP) therapy. Patient underwent a PAP titration PSG, which resulted in a pressure at 7 centimeters of water. At higher pressures, treatment emergent central sleep apnea developed. Home PAP therapy was initiated successfully with good compliance and resolution of observed apneas and snoring at 2 month follow-up, with corrected AHI of 2.2. Supraglottoplasty for surgical correction of severe laryngomalacia is planned. Post-operatively, patient will undergo follow-up PSG and PAP titration, as indicated. Conclusion Late onset laryngomalacia is a rare phenomenon in adults and adolescents. Diagnosis should be considered in patients with snoring, stridor, OSA, or dysphagia. Support (if any)