Congenital nasal piriform aperture atresia: A case report and novel finding

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

“…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]. It is caused by narrowing without occlusion in the most anterior opening of the bony nasal airways limited laterally by the nasal process of the maxilla, inferiorly by the horizontal process of the maxilla and the anterior nasal spine, and superiorly by the nasal bones [1][2][3][4][5].…”

“…Stenosis of this anatomic region can produce symptoms, immediately after birth, such as apnea, cyclic cyanosis, difficulty feeding, and respiratory distress [1][2][3]. In less severe cases, when a later diagnosis is made, it can also be responsible for the infant's failure to thrive [1,6].…”

“…In less severe cases, when a later diagnosis is made, it can also be responsible for the infant's failure to thrive [1,6]. It is clinically suspected upon the impossibility to make a nasogastric probe progress through both sides of the nose [1][2][3][4][5]. The frequency of the symptoms and severity is usually proportional to the degree of the stenosis [1][2][3].…”

“…It is clinically suspected upon the impossibility to make a nasogastric probe progress through both sides of the nose [1][2][3][4][5]. The frequency of the symptoms and severity is usually proportional to the degree of the stenosis [1][2][3]. They can worsen in the presence of concomitant infection or during breastfeeding, and they can be relieved by crying [1].…”

“…CNPAS may not be initially diagnosed, and it can be mistaken with other more common causes of neonatal respiratory distress such as choanal atresia [6,7]. The diagnosis of pyriform aperture stenosis can be made with computerized tomography (CT) scan obtaining contiguous axial sections in a plane parallel to the anterior hard palate, showing a whole pyriform aperture width inferior to 11 mm [1,2,3,7]. However, normal whole pyriform aperture width is thought to range from 8.8 to 17.2 mm, and for this reason, some authors consider that measurements smaller than 8.0 mm should be termed as the inferior threshold to define abnormal [4][5][6].…”