Congenital nasal pyriform aperture (bony inlet) stenosis

Congenital nasal pyriform aperture stenosis should be considered in the differential diagnosis of infants with nasal airway obstruction. It can occur as an isolated anomaly or it can be associated with other congenital anomalies. A history of cyclical respiratory distress and cyanosis often associated with feeding and relieved by crying is characteristic. On examination, the nasal airway is narrowed anteriorly. CT is the study of choice to make the diagnosis and rule out other causes of nasal obstruction. Conservative management is recommended, sometimes with the use of a McGovern nipple for feeding. Severe cases or those in which conservative management fails should be considered for surgery.