Congenital nasal pyriform aperture stenosis: Elaboration of a management algorithm from 25 years of experience

Introduction: Congenital nasal pyriform aperture stenosis (CNPAS) is a rare disease presenting with neonatal respiratory distress, often associated with other anomalies.

Materials and methods: This study reports the clinical and radiological characteristics of the patients managed in The Department of Pediatric Otolaryngology Head and Neck Surgery of La Timone Children's Hospital in Marseille between 1988 and 2014. Pyriform aperture (PA) widths were measured on CT-scans, obtained by using hand calipers at the largest portion of the PA in a plan parallel to the Francfort plan.

Results: 10 patients were included. Average PA width was 6.6mm, 5/10 patients presented with single central maxillary median incisor, 6/10 patients had associated abnormalities. 8 patients underwent a surgical intervention and 2 patients were medically managed. All the patients had satisfactory nasal airway permeability on late follow-up. A management algorithm was elaborated. CNPAS should be evoked when breathing difficulties are associated with impossibility of passing fiberscope or nasogastric tube at the nasal inlet. Craniofacial CT-scanning is necessary to make the diagnosis and look for associated abnormalities. Medical treatment associating nasal wash and decongestants should be performed. Surgical intervention is necessary when failure of the medical management.

Discussion and conclusions: Our results were close to those found in the literature in terms of clinical characteristics, associated abnormalities and PA width. However, no objective criterion to decide whether a surgical intervention is necessary or not, has been established so far. The algorithm we propose offers guidelines from diagnosis to treatment, but the management should be adapted based on clinical tolerance.