Neonates are obligate nasal breathers, and nasal obstruction may have serious implications. We present an extremely rare cause of neonatal nasal obstruction, and its management.

An eight-day-old neonate was referred for upper airway obstruction. Initial investigations had identified no obvious cause. Rigid airway endoscopy revealed a large, cystic lesion appearing to arise from the roof of the posterior nasal space. Computed tomography and magnetic resonance imaging indicated a basal cephalocoele projecting inferiorly into the oropharynx, with an intracranial connection to the pituitary fossa. Histology showed fibrovascular tissue lined on one aspect by respiratory type epithelium, with mucous glands present. The tissue contained multiple cystic spaces lined by choroid plexus epithelium, with glial tissue present in the walls of the mass. A transpalatal excision of the nasopharyngeal cephalocoele, with closure of the intracranial connection, palatal repair and lumbar drain placement was undertaken. Post-operative recovery was uneventful, with no evidence of cerebrospinal fluid leakage or palatal dysfunction.

This surgical approach gave excellent access whilst avoiding the obvious morbidity associated with an intracranial approach. Nasal masses should be considered in the differential diagnosis of neonatal respiratory distress due to nasal obstruction.