Newborn nasal obstruction due to congenital nasal pyriform aperture stenosis

Abstract

Introduction: Nasal obstruction is an important condition that can lead to severe respiratory distress in newborns. There are several differential diagnoses, and one of them is congenital nasal pyriform aperture stenosis (CNPAS). CNPAS is a rare case of respiratory distress caused by excessive growth of the nasal process of the maxilla and leads to narrowing of the anterior third of the nasal cavity. Diagnosis, associated anomalies, and treatment strategies are reviewed by the following presentation of two cases.

Case presentation: We report two cases of infants diagnosed with CNPAS. The patients in the first case had no concomitant comorbidities, and the outcome was successful after surgical correction of stenosis. The patient in the second case had an associated holoprosencephaly, and although surgical correction and nasal cavity patency, the patient remains dependent on tracheostomy due to dysphagia and neurologic impairment.

Discussion: Airway obstruction affects 1 in 5000 children, and CNPAS is a diagnosis frequently forgotten and even unknown to neonatal and pediatric intensivists. Newborns are obligate nasal breathers, and, nasal obstruction, therefore, can lead to severe respiratory distress. CNPAS is not only rare but, many times, is not easily recognized. It is important to bear in mind the diagnostic criteria when evaluating infants with nasal obstruction. Conservative treatment should be prioritized, but surgical treatment is required in severe cases with failure to thrive and persistent respiratory distress. Respiratory distress and dysphagia may persist to some degree despite correction of the stenotic pyriform aperture due to associated narrowing of the entire nasal cavity and association with other anomalies. Final Comments: CNPAS is a rare condition and may be lethal in newborns. Differential diagnosis of nasal obstruction must be remembered to recognize this anomaly, and the otolaryngologist must be familiarized with this condition and its diagnosis. Precise surgical treatment in severe cases have high rates of success in children without other comorbidities.

Figure 1.

Computed tomography of the sinuses. Left, Axial section, showing the region of patent choanae without atresia signals. Right, Coronal section, showing narrowing of pyriform aperture. Right nostril size is 0.32 cm and left nostril size is 0.35 cm.

Figure 2.

Final appearance in the immediate postoperative period, with the tracheal tube no. 3 set in both nostrils to keep airways and allow cleaning.

Figure 3.

Computed tomography of the sinuses. Postoperative aspect with enlargement of pyriform aperture.

Figure 4.

Computed tomography of sinuses. Left, Axial section, showing solitary median maxillary central incisor (arrow). Right, Coronal section, showing 2.8 mm pyriform aperture size.

Figure 5.

Computed tomography 3-D reconstruction, showing solitary median maxillary central incisor (arrow) and pyriform aperture stenosis.

Figure 6.

Sublabial approach.

Figure 7.

Intraoperative, showing postcorrection aspect in the right side.