Congenital nasal pyriform aperture stenosis

Congenital nasal pyriform aperture stenosis is an anatomical anomaly which can present as a neonatal airway emergency. The findings of a narrow nasal inlet along with characteristic computerized tomographic findings confirm the diagnosis. This anomaly can occur with other craniofacial syndromes and endocrine disorders which have to be ruled out. The treatment depends on the severity of symptoms with surgery reserved for failure of aggressive medical therapy or severe degrees of nasal narrowing requiring intubation. A sublabial drill-out procedure to widen the pyriform aperture with short duration stenting resolves the problem in a vast majority of patients.