Upper Airway Obstruction in a Newborn: A Rare Cause of Respiratory Distress

Abstract

Newborns are considered obligate nasal breathers until the eighth week of life. Therefore, upper nasal obstruction in a newborn can present as a potentially life-threatening complication. Congenital nasal pyriform aperture stenosis (CNPAS) is a rare form of upper airway obstruction caused by a narrowing without occlusion in the most anterior opening of the bony nasal airways. According to the severity of this stenosis, early onset of respiratory symptoms can arise in the newborn.In this article, we present the case of a male term newborn with no prior relevant family history and uneventful gestation delivered in a eutocic manner with an adequate transition to the extra-uterine environment. On his first day of life, progressive respiratory distress, inability to breastfeed, and impossibility to make nasogastric probe progress through both sides of the nose were observed, leading the newborn to be admitted to a neonatal intensive care unit.During imagiological assessment with perinasal computerized tomography (CT) scan, an almost total occlusion of the pyriform aperture and a solitary median maxillary central incisor (SMMCI) were identified. Additional evaluation with brain magnetic nuclear resonance imaging (MRI) was unremarkable with no midline defects identified. Endocrine laboratory assessment was also normal.The newborn underwent pyriform aperture permeabilization surgery via a sublabial approach with bilateral nasal stent introduction, enabling total resolution of the initial respiratory symptoms. No incurrences were reported during the post-operatory follow-up period.With the present case report, the authors are trying to raise awareness for CNPAS not only as a rare cause of respiratory distress in the newborn but also as a clinical entity that can be associated with midline defects, which require further additional investigation and intervention.

Navigating Congenital Nasal Obstruction: A Contemporary Surgical Paradigm

“…As in our clinical case, the newborns were born with no craniofacial dysmorphism upon physical examination but immediate respiratory symptoms were observed [2]. When the pediatric team failed to wean the child off from oxygen for more than a month and a 2.5 mm pediatric flexible endoscope with the nasal suction tube could not be advanced through either nasal vestibule, diagnosis of CNPAS was considered.…”

“…It was discovered for the first time by Brown in 1989 [1]. The cause is unknown, but it appears to be due to narrowing without occlusion of the anterior nasal bony aperture bounded superiorly by the nasal bones, the horizontal process of the maxilla and the anterior nasal spine inferiorly, and laterally by the nasal process of the maxilla [2]. CNPAS can exist in isolated form or be associated with other malformations such as holoprosencephaly, pituitary dysfunction, and a solitary median central incisor [3].…”

Comparison of Surgical Techniques for the Treatment of Congenital Nasal Pyriform Aperture Stenosis: A Systematic Review

“…Children may present with symptoms of respiratory distress, apnea, feeding difficulties, and failure to thrive. [4][5][6] This condition is especially important in newborn infants who are obligate nasal breathers until 3 to 6 months of age. 7 It is therefore crucial to make a timely diagnosis of CNPAS in those affected and to develop a management plan.…”