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

Abstract

Congenital nasal pyriform aperture stenosis (CNAPS) is a rare cause of pediatric nasal airway obstruction that clinically mimics choanal atresia in a neonate. CNPAS is suspected clinically and confirmed with CT scanning. Early diagnosis and management is essential for this potentially life-threatening condition. Because of the association this anomaly has with other midline defects, such as holoprosencephaly, it is important to recognize it and pursue a thorough workup. Patients can be managed conservatively or surgically. Surgical treatment is usually reserved for those patients that fail conservative treatment. We present a case of CNPAS, to highlight the importance of recognizing the classic signs of CNPAS on cross-sectional imaging to prevent fatal outcome.

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

“…The main differential diagnose is choanal atresia that is a congenital narrowing of the posterior nasal airway by a bony or membranous septum [2].The symptoms of choanal atresia vary based on the age of the patient and whether the atresia is unilateral or bilateral. Bilateral choanal atresia presents early in newborns, who are obligate nasal breathers, with either respiratory distress noted around the time of birth or breathing difficulties during the first oral feed [8,9].…”

“…Close follow up until growing expansion of the nasal cavity is essentail. Surgical interventions are reserved for cases with severe stenosis after conservative treatment [4,8].…”

“…Timing of surgery and the pyriform aperture width that predicts the need for surgical intervention are still controversial in literature. In fact, many authors reported surgical intervention in cases with sleep apnea, repeat intubation attempts or failure of extubation, feeding problems accompanied by cyanosis, and cases unresponsive to conservative therapy [8,10]. Gonik et al published a review of 16 patients and found that the average size of the pyriform aperture did not correlate with the need for surgery [11].…”