Navigating Challenges: Case Series of Congenital Nasal Pyriform Aperture Stenosis

Congenital nasal pyriform aperture stenosis (CNPAS) is characterized by a narrowing of the pyriform orifice attributed to excessive growth of the nasal process of the maxilla, resulting in blockage of the nasal passage. Early intervention is crucial as newborns are obligate nasal breathers during the first few months, which may result in respiratory distress in patients with CNPAS. This report reviewed the presentation and management of the CNPAS in a single tertiary paediatric otorhinolaryngology centre. We reported a case series with three term neonates that presented with respiratory distress and noisy breathing at birth. All of them required intubation and were unable to introduce suction catheter size 6Fr via the nostrils. Conservative management, including the use of nasal decongestants and humidification, was tried but failed. The sublabial approach was used for surgical correction in all patients. The pyriform aperture is enlarged with a bone drill and the placement of a nasopharyngeal airway (NPA) into both nostrils, which acts as a stent. The stent is left in place for a total of two to three weeks for all three patients to ensure the patency of the surgical area. All the patients were able to be extubated following surgery and discharged home without oxygen support.