Synchronous congenital nasal piriform aperture stenosis and atresia : A rare accompaniment of mid-facial dystosis and cause of neonatal upper airway obstruction

Congenital nasal piriform aperture stenosis (CNPAS) is a recently distinguished clinical entity that causes airway compromise in neonate as a result of a narrowing of the nasal piriform aperture. It may be bilateral or unilateral. Often considered as a form of holoprosencaphaly. Being the narrowest part of the nasal airway, any slight reduction in its cross sectional area will profoundly increase the nasal airway resistance with the attendant clinical challenges. Readily making an accurate clinical diagnosis may be very challenging when CNPAS occurs in the setting of a craniofacial dysostosis. This communiqué is aimed at increasing awareness among clinicians involved in paediatric care of the clinical entity congenital nasal piriform aperture stenosis and atresia presenting atypically as synchronous disorder with midfacial dysostosis. Hence, it should be kept in mind as a rare mimicker of bilateral choanal atresia and requires mandatory high resolution computerized tomography to confirm the diagnosis.