0859 AVAPS to the rescue: A case of severe obstructive sleep apnea with treatment resistant to UPPP and CPAP in a pediatric patient with Prader-Willi syndrome

Abstract

Abstract Introduction Prader-Willi syndrome (PWS) has a prevalence of 1/10,000 to 1/30000 and is the most common syndromic form of obesity. Prader-Willi syndrome is defined by a multitude of features that develop from early childhood to adolescents, but one of the primary developments is obesity in the setting of hyperphagia during early childhood. Due to the development of obesity, there is a high prevalence of obstructive sleep apnea (OSA) among PWS patients. Report of Cases: A 6-year-old boy with PWS with a 2-year history of loud snoring underwent polysomnography (PSG) which showed severe OSA with Apnea-Hypopnea Index (AHI, events/h) 133.7. The patient underwent surgical intervention with a combined uvulopalatopharyngoplasty and adenotonsillectomy and a follow up PSG showed a residual AHI of 37. On CPAP titration the AHI improved to 1.2 with CPAP 14 cm H2O and the patient was discharged on home nasal CPAP. Over the next several years, the patient had suboptimal CPAP compliance. At 11 years of age, the patient was admitted to the intensive care unit (ICU) with volume overload –with a normal cardiac workup- and respiratory failure requiring high flow nasal cannula oxygen. Venous blood gas (VBG) showed pH 7.34 and severe hypercapnia with pCO2 73 mmHg c/w obesity hypoventilation syndrome (OHS). The patient was started on CPAP then changed to bilevel PAP ST with supplemental oxygen. Due to persistent hypercapnia in the high 60s noninvasive ventilation (NIV) was switched to Average Volume-assured Pressure Support with auto titrating EPAP (AVAPS-AE) with significant improvement in hypercapnia and overall tolerance. Discharge VBG showed pH 7.35 and PCO2 56 mm Hg. The patient was discharged home, is doing well and has avoided readmission for at least 6 months. Conclusion This case highlights the potential for newer modes of noninvasive ventilation with autotitrating EPAP to treat severe OSA with OHS and hypercapnic respiratory failure in patients with PWS and other conditions. With the growing obesity epidemic, rates of treatment failures with CPAP and traditional bilevel PAP therapy may increase and consideration of alternative modes of NIV therapy should be considered. Support (If Any)