Surgical Management of Congenital Nasal Pyriform Aperture Stenosis: A Case Report

Abstract

Congenital nasal pyriform aperture stenosis (CNPAS) is a rare cause of respiratory distress in newborns. This paper reports a case of severe CNPAS that required endotracheal intubation immediately after birth, and eventually, surgical intervention. At birth, the width of the pyriform aperture was only 4 mm, and the patient was completely unable to breathe through his nose. We performed tracheostomy at 23 days of age and waited for the patient to grow, but at 56 days of age, the width of the pyriform aperture was not sufficient (6 mm) for the patient to breathe through his nose. Therefore, surgical dilation of the pyriform aperture by a sublabial approach was performed on day 79 after birth, and the width was increased to 14 mm. Postoperative stent placement was performed for two weeks. After the removal of the stents, the patient could finally breathe through his nose, and the postoperative course was uneventful, with no restenosis after four months. CNPAS is a rare cause of nasal obstruction, but it can cause respiratory distress in infants because they are dependent on nasal breathing. Conservative treatments are initially recommended for CNPAS; however, in severe cases where conservative treatments are ineffective, surgical treatment is recommended.

Acute Respiratory Distress in the Newborn: Congenital Nasal Pyriform Aperture Stenosis the Underlying Pathology

“…No recurrence has been observed in the literature, even when the first surgery was insufficient [15]. However, the presence of comorbidities such as craniofacial dysmorphisms, neurologic malformations, and airway anomalies have been shown to lower surgical success rates [16]. Several follow-ups should be considered post-operatively especially to look for airway and feeding difficulties, particularly in those requiring hospital readmission.…”

Navigating Congenital Nasal Obstruction: A Contemporary Surgical Paradigm

“…Delaying therapy puts the newborn at risk of respiratory distress and apnea, which can lead to ischemic brain damage and death. Failure of the suction tube to enter through the nostrils may indicate pyriform aperture stenosis [3].…”

“…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]. It manifests as apnea, cyclic cyanosis, difficulty in feeding, and abrupt total airway obstruction immediately after delivery, as the neonate is considered an obligate nasal breather until the eighth week of life, through which crying provides relief [4].…”

Nasal Pyriform Aperture Stenosis in a Newborn: When to Operate

“…It can present with nonspecific symptoms, such as noisy breathing, tachypnoea, apnea, cyanosis, increasing respiratory difficulty on feeding, or severe obstructed breathing relieved by crying or oral airway. Significant respiratory distress and inability to wean off support sometimes require tracheostomy [ 6 ].…”

“…While doing tracheostomy and waiting for the child and nasal aperture to grow, the child still may not reach adequate aperture size to wean off tracheostomy/support, ultimately requiring nasal aperture widening [ 6 ].…”