1328 The Temporary Role of Negative Pressure Ventilation in an Adolescent with Congenital Central Hypoventilation Syndrome

Abstract Introduction The aim of this case report is to describe the role of negative pressure ventilation as temporary ventilatory support while an adolescent is undergoing nasal surgery. Report of case(s) A 17-year-old male with genetically confirmed heterozygous PHOX2B mutation with congenital central hypoventilation syndrome (CCHS). Hypoventilation was initially managed with tracheostomy and continuous ventilation from birth to age nine, followed by noninvasive AVAPS therapy (IPAP 10–20 cm H₂O, EPAP 6, backup rate 16) during sleep alone. Most recent baseline polysomnography at age 10 showed AHI 11.3, OHI 10.3, SpO₂ nadir 83%, mean SpO₂ 93%, peak CO₂ 71 mmHg, mean CO₂ 63 mmHg, 100% of total sleep time with CO₂ > 50 mmHg. During adolescence, he developed nasal obstruction and deformity from sports trauma along in conjunction with long-term nasal-mask use. Otolaryngologic evaluation revealed a C-shaped external nasal and septal deformities obstructing the entire left nostril, and septorhinoplasty was recommended. Because interfaces could not be safely used perioperatively, ventilatory strategies were reviewed by a multidisciplinary team, and negative-pressure ventilation (NPV) with Hayek biphasic cuirass was chosen as the optimal therapy. A titration study in February 2024 demonstrated effective ventilatory control with NPV (settings of -22/8 with back up rate of 16 breathe per minute with 1:1 sync and trigger of 8). NPV was initiated one week before surgery and continued for three months postoperatively. He was monitored in the PICU during the immediate perioperative period given his complex respiratory history and initiation of NPV. After three months of stable use and full recovery from septorhinoplasty, he transitioned back to AVAPS therapy. This case illustrates successful perioperative use of biphasic cuirass ventilation in a CCHS patient unable to use nasal interfaces with non-invasive ventilation (NIV), highlighting its utility as a safe and effective temporary ventilatory option during facial surgery or situations where conventional noninvasive support is not feasible. Conclusion Despite multiple modes of therapy available, interfaces can markedly affect utilization during septorhinoplasty. Our case shows that NPV can be considered for temporary ventilation management during upper airway surgery in adolescents with CCHS when normal interfaces are contraindicated. Support (if any)