[Congenital nasal pyriform aperture stenosis]

Congenital nasal pyriform aperture stenosis (CNPAS) is a rare and an unusual cause of airway obstruction in newborns.

We report the case of a female neonate delivered by C-section at 39 weeks of amenorrhea for hydramnios and macrosomia. She presented with mandibular retrognathia, nasal saddling, submucous cleft palate, and loud mouth respiration. She presented with cyanosis every time feeding was attempted. CT revealed permeable choans with a single central incisor and nasal pyriform aperture stenosis. Nasal respiration returned to normal progressively after 56 days of hospitalization. The status was unchanged at 5 months.

CNPAS is a rare cause of congenital nasal obstruction. It is sometimes associated to a median incisor syndrome. The diagnosis should be made as early as possible for an optimal management. Cyanosis and swallowing disorders may be lethal consequences. Associated abnormalities should be screened for with TDM or MRI. The treatment depends on the severity and may be surgical for a severe stenosis. The prognosis is good if no severe malformation or mental retardation is associated.