A Case of Congenital Nasal Pyriform Aperture Stenosis

ABSTRACT

Congenital nasal pyriform aperture stenosis (CNPAS) is a rare cause of upper airway obstruction in the newborn. This affliction is due to bony overgrowth of the nasal process of the maxilla. CNPAS is diagnosed clinically and confirmed with CT scan. Patients can be managed conservatively or surgically. In this report, a male neonate had respiratory distress, cyclic cyanosis, and apnea after delivery. The patient underwent surgical correction of pyriform stenosis with a transnasal approach. During follow up, the patient showed recurred respiratory distress and cyanosis. Revision operation was necessary to reduce the bony inferior turbinate and pyriform aperture. CNPAS should be suspected in newborns with clinical signs of severe nasal obstruction associated with difficulty passing a small catheter through the anterior nares.