Congenital nasal pyriform aperture stenosis: diagnosis and treatment.

Congenital nasal pyriform aperture stenosis is a rare and an unusual cause of airway obstruction in infants. It has been described as a clinical entity only since 1989. Presenting symptoms in infants include cyclical cyanosis, feeding difficulty or sudden total obstruction. Embryologically, an overgrowth of maxillary ossification at the area of the nasal process of the maxilla may be responsible for this deformity. Definitive surgical management may be accomplished via a sublabial approach with magnification, drilling and post-operative stenting of the airway. Two new cases are presented. The diagnosis, embryology and technique of surgical correction are discussed.