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.
Highlights
Upper nasal obstruction in the newborn can present as a potentially lethal complication as a newborn is considered an obligate nasal breather until the eighth week of life [1,2]
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
A whole pyriform aperture’s width with less than 11 mm obtained by computerized tomography (CT) scan with thin contiguous axial sections in a plane parallel to the anterior hard palate in a term infant is considered confirmative of CNPAS diagnosis [1,2,3,7]
Summary
Upper nasal obstruction in the newborn can present as a potentially lethal complication as a newborn is considered an obligate nasal breather until the eighth week of life [1,2]. Congenital nasal pyriform aperture stenosis (CNPAS) was first, clinically, described by Brown et al in 1989 It is a rare form of upper airway obstruction with an estimated incidence of 1:25,000 cases [2]. While mild obstruction can be treated conservatively with intranasal corticoids or nasal decongestants for a few days, a moderate or severe obstruction will require surgical intervention [13] This case report aims to highlight the importance of a timely diagnosis to prevent severe respiratory complications and the deleterious clinical evolution of the patient while raising awareness for other possible findings that should prompt additional investigation. The newborn was, immediately, transferred to the neonatal intensive care unit (NICU) of a central hospital with pediatrics and otorhinolaryngology support He was breathing spontaneously upon admission, but during his first 24 hours of life, a sudden clinical deterioration with progressive respiratory distress and hypoxemia needing orotracheal intubation ventilatory support was soon noticed. The infant registers a completely normal development without any relevant clinical incurrences to be reported
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