Sublabial Approach for Repair of Congenital Nasal Pyriform Aperture Stenosis: How I Do It.

Abstract

INTRODUCTION Congenital nasal pyriform aperture stenosis (CNPAS) is a rare congenital narrowing of the anterior aspect of the nose at the pyriform aperture due to bony overgrowth of the frontal process of the maxilla. Clinical suspicion is raised in infants who have noisy, stertorous breathing, desaturations with feeding, or cyclical cyanosis relieved by crying. Infants with CNPAS present similarly to those with choanal atresia; however, a flexible suction catheter (8 French, 2.67 mm diameter) or pediatric flexible fiberoptic laryngoscope (2.5 mm diameter), will not easily pass past 1 cm to 2 cm in the nares. Maxillofacial computed tomography (CT) is used to confirm a suspected diagnosis. Mild cases can be managed with supportive care including a combination of nasal steroids, nasal saline, bulb suctioning, and/or supplemental oxygen. Surgical intervention is pursued when there is persistence of respiratory distress, feeding difficulties, or failure to thrive for at least 2 weeks despite conservative measures. As CNPAS is rare, pediatric otolaryngologists will not have the opportunity to do this surgery routinely. We demonstrate the sublabial drill-out technique with limited postoperative stenting and present lessons learned from doing five cases in a short time frame.