Congenital Pyriform Aperture Stenosis

Neonates rely on the nasal airway for their source of air; thus, any compromise in the ability to inhale will dramatically alter their ability to breathe. Congenital nasal pyriform aperture stenosis is a rare yet serious form of airway obstruction due to overgrowth of the maxilla at the medial nasal process. Infants typically present with difficulty feeding and obvious difficulty breathing. Radiologic imaging aids in confirming the diagnosis and assists in operative planning to open the medial nasal process to reduce airway resistance. Further corrective surgery is often needed and is planned commensurate with facial growth. We present a novel case of a child diagnosed with congenital nasal pyriform aperture stenosis who has performed extraordinarily well intellectually and has achieved an excellent cosmetic and physiological reconstruction of the nose and airway.