Congenital nasal pyriform aperture stenosis.

Congenital nasal pyriform aperture stenosis is an unusual and previously undescribed cause of nasal airway obstruction in the newborn. The nasal pyriform aperture is narrowed due to bony overgrowth of the nasal process of the maxilla. This anomaly may produce signs and symptoms of nasal airway obstruction in newborns and infants similar to those seen in bilateral posterior choanal atresia. Computed tomography confirms the diagnosis and delineates the anomaly. A series of six patients with nasal pyriform aperture stenosis is presented. Four patients were treated with surgical enlargement of the nasal pyriform aperture via a sublabial approach. One patient was repaired via a transnasal approach, and one patient did not undergo surgical intervention. Follow-up reveals normal nasal airway and facial growth in all patients. Mildly symptomatic patients with congenital nasal pyriform aperture stenosis may be treated expectantly, while severely symptomatic patients benefit from repair via the sublabial approach.