Congenital Pyriform Aperture Stenosis

Abstract

Neonates rely on the nasal airway for their source of air; thus, any compromise in the ability to inhale will dramatically alter their ability to breathe. Congenital nasal pyriform aperture stenosis is a rare yet serious form of airway obstruction due to overgrowth of the maxilla at the medial nasal process. Infants typically present with difficulty feeding and obvious difficulty breathing. Radiologic imaging aids in confirming the diagnosis and assists in operative planning to open the medial nasal process to reduce airway resistance. Further corrective surgery is often needed and is planned commensurate with facial growth. We present a novel case of a child diagnosed with congenital nasal pyriform aperture stenosis who has performed extraordinarily well intellectually and has achieved an excellent cosmetic and physiological reconstruction of the nose and airway.

Nasal cavity dimensions in congenital pyriform aperture stenosis

“…This deformity results in a distinctive narrowing of the pyriform aperture causing nasal obstruction and even neonatal respiratory distress [1,2]. Infants are obligate nasal breathers, and they rely on a patent nasal airway, particularly during feeding.…”

“…Infants are obligate nasal breathers, and they rely on a patent nasal airway, particularly during feeding. Therefore, severe CPAS typically present with signs and symptoms of neonatal nasal obstruction: stertor, feeding difficulty, inability to pass a nasogastric tube, respiratory distress, and cyanosis [1]. The differential diagnosis in these children is broad and includes choanal atresia, CPAS, encephalocele, and nasolacrimal duct cysts.…”

Novel treatment of neonates with congenital nasal pyriform aperture stenosis

“…[4][5][6] It may be isolated or associated with other abnormalities, especially those related to the holoprosencephaly spectrum, mainly the solitary median maxillary central incisor syndrome (SMMCI) in approximately 50% of cases. 4,[7][8][9][10][11][12] The diagnosis of CNPAS is suspected by history and physical examination and can be confirmed by axial and coronal computed tomography (CT) sections showing a nasal pyriform aperture of <11.0 mm measured at the axial level of the inferior meatus in full-term neonates. 13 However, some authors suggest that the normal measure ranges from 8.8 to 17.2 mm, and that measurements smaller than 8.0 mm should be considered abnormal.…”

“…5 The pyriform aperture is the most anterior and the narrowest part of the airway; therefore, even small changes in its diameter are enough to increase signifi-cantly the airway flow resistance, causing serious respiratory consequences. 4 The severity of the presentation may vary from a mild to life-threatening condition, and the differential diagnosis with other causes of neonatal respiratory distress, such as choanal atresia, encephalocele, and nasal tumors, is very important. 4,6 The initial treatment depends on the severity of presentation, but according to most publications, it should be managed with humidifiers, nasal topic decongestants, and treatment of gastroesophageal reflux.…”

“…4 The severity of the presentation may vary from a mild to life-threatening condition, and the differential diagnosis with other causes of neonatal respiratory distress, such as choanal atresia, encephalocele, and nasal tumors, is very important. 4,6 The initial treatment depends on the severity of presentation, but according to most publications, it should be managed with humidifiers, nasal topic decongestants, and treatment of gastroesophageal reflux. 6,14,15 When there is no improvement in 10 to 15 days or in cases of subtotal nasal obstruction, a surgical approach is considered.…”