Congenital Nasal Pyriform Aperture Stenosis as a Rare Cause of Cyclical Cyanosis

Congenital nasal pyriform aperture stenosis (CNPAS) is a rare condition affecting the upper airway system. Unlike choanal atresia, the symptom if any, can be insignificant or appear delayed. We report a case of a 3-month-old baby girl who had history of sudden brief episodes of cyanosis and desaturation during sleep and relieved upon awakening and crying. It was associated with intermittent stertor and required oxygen supplementation. The airway symptom worsened with rhinorrhea and nasal blockage, which required re-admission. The inability to introduce a suction catheter size 6 French and pediatric flexible scope size 2.2mm into bilateral nostrils was diagnostic.