1145 Stride Along with Stridor in the Setting of CSA: An Unusual Presentation of Pediatric Cervicomedullary Ganglioma

Abstract Introduction We describe an unusual presentation of a cervicomedullary ganglioglioma secondary to findings of stridor despite positive airway pressure (PAP) therapy and the presence of treatment emergent central sleep apnea in a pediatric patient. Report of case(s) Patient is an 8 year old male with a history of congenital subglottic stenosis and no history of neurologic problems who presented to the sleep clinic for stridor despite adequate adherence to CPAP. Split night polysomnogram (PSG) showed moderate OSA (OHI 8.3/h) and treatment emergent central sleep apnea. Patient was unable to tolerate BPAP ST and wanted to go back to CPAP. Drug induced sleep endoscopy findings were significant for mild collapse of upper airway with no snoring. Repeat PSG - 6 months later - was significant for severe obstructive and central sleep apnea (OAHI 10.4, CAI 19, REM CAI 54, AHI of 30). MRI of the brain showed cervicomedullary tumor with marked effacement of the foramen magnum. Further workup showed right vocal cord paralysis. Biopsy confirmed ganglioglioma. Tumor was not amenable for surgical resection hence proton radiation therapy was pursued. Repeat titration study was performed which showed BPAP ST settings of 12/6 with BUR of 12 to be effective with residual AHI of 0.7. Patient developed radiation induced tumor psuedoprogression and tumor necrosis with worsening ataxia and recurrence of central and mixed apneas requiring AVAPS for further respiratory support due to progressive neuromuscular weakness. Stridor resolved after AVAPS support. Conclusion Stidor in a pediatric patient can involve various anatomical abnormalities of the upper airway. However, in the setting of central sleep apnea, abnormalities of the brain stem can lead to stridor. We present a patient with a prolonged period of central sleep apnea of unknown etiology and persistent stridor despite adequate adherence to PAP therapy that ultimately led to a diagnosis of a cervicomedullary ganglioma before neurologic symptoms developed. Support (if any)