Congenital Nasal Pyriform Aperture Stenosis (CNPAS) is a rare cause of upper airway obstruction in neonates. Since neonates often breathe through their nose, especially when feeding, any obstruction to airflow can be problematic.
The upper airway is narrow at the pyriform aperture. With congenital stenosis it is smaller but still minimally patent.
Clinical features:
(1) episodes of respiratory distress at birth or within a few months of birth
(2) cyanosis
(3) breathing difficulties while feeding
(4) inability to pass a 5-French catheter
The presence of stenosis can be confirmed on endoscopy or head CT scan.
The stenosis may be isolated or in conjunction with other midface anomalies.
