Heterotopic Nasopharyngeal Glioneuroma as a Cause of Respiratory Failure in a Newborn.

This case is that of a two-day-old male patient born full term with no pertinent history who presented with excess nasal secretions, feeding difficulty, respiratory distress, and hypoxemia requiring intubation. He was found to have an obstructive, polypoid mass in the nasopharynx on fiberoptic flexible laryngoscopy. Magnetic resonance imaging (MRI) of the brain and neck revealed an ovoid, circumscribed, peripherally enhancing mass in the posterior nasopharynx and oral cavity measuring 2.1 x 1.7 x 2.0 cm. The mass was excised endoscopically, and the patient tolerated extubation with immediate resolution of hypoxemia and feeding difficulty. Pathology of the mass was consistent with benign glioneuronal heterotopia, confirmed via glial fibrillary acidic protein (GFAP) staining. Histology was consistent with multiple cell types, including respiratory mucosa, squamous-type mucosa, seromucous glands, and keratin-filled cysts. Gliomas such as the one found to be the cause of this patient's respiratory failure are locally aggressive, usually congenital lesions. Differential diagnosis for a congenital midline nasal mass should further include dermoid cysts, encephaloceles, neurogenic tumors, and teratomas. Evaluation of such a mass must include imaging to rule out cranial extension prior to further intervention. This case serves to further broaden the understanding of congenital anomalies as an etiology for respiratory distress in the newborn period, with an emphasis on nasopharyngeal masses and, more specifically, heterotopic nasopharyngeal glioneuromas.