Congenital nasal pyriform aperture stenosis: a rare cause of neonatal nasal airway obstruction

Abstract

Congenital nasal pyriform aperture stenosis (CNPAS) is a rare cause of nasal airway obstruction that clinically mimics choanal atresia in a neonate. The differentiation between the two is very important as the management of the two conditions is different. Timely recognition is important to prevent fatal outcome. CNPAS may present as an isolated condition or with associated craniofacial anomalies. Despite typical findings of CNPAS being present on cross-sectional imaging, this condition is commonly overlooked, probably because of a lack of familiarity with the normal morphological features of the nasal cavity in infants and also owing to a lack of awareness of this rare entity. Here we report a case of CNPAS with pre- and post-surgical CT images and the complication that occurred owing to nasal stenting.

An Unusual Cause of Upper Airway Obstruction in Newborn: Congenital Nasal Pyriform Aperture Stenosis

“…CNPAS can clinically mimic choanal atresia, with infants presenting with respiratory distress, either at birth or within the first few months of life. The neonate can present at birth with cyanosis, breathing difficulty (particularly when feeding) and nasal cavity narrowing to the extent that passage of a nasogastric tube is impossible [6]. Clinical symptoms of CNPAS may be delayed until several weeks, but bilateral choanal atresia is manifested in the first s of life [6].…”

“…A diameter of <5 mm at the inferior meatus and patient not responding to conservative treatment require surgical treatment, which involves pyriform aperture enlargement through an endo-oral sublabial approach to reshape the stenotic area with burs. This is a good and safe method that provides adequate field exposure, preventing damage to nasolabial soft tissues without visible scarring [6]. Surgical results are excellent and prognosis largely depends on co-existing neurological and/or endocrine problems [8].…”

A Rare Cause of Neonatal Respiratory Distress: Congenital Stenosis of Piriform Aperture

“…Congenital airway obstruction is a problem that affects up to 1 in 5000 infants. The majority of these obstructions result from choanalatresia, which affects 1 in 8000 live births [1]. CNPAS is a rare and unsusal cause of nasal obstruction that occurs around 1 in 25,000 births [3].…”

“…Other traumatic causes of neonatal nasal obstruction, Skull base defects such as meningoencephalocele and tumoral processes should be eliminated [1].…”

“…Surgical procedure consists in pyriform aperture enlargement through an endo-oral sublabial approach with diamond burs. This is a safe approach that provides adequate field exposure, preventing damage to nasolabial soft tissues without visible scarring although that some complications such as injuries to lacrimal system and to the tooth buds has been observed [1,2].…”