Congenital nasal pyriform aperture stenosis

Abstract

Congenital pyriform aperture stenosis represents an unusual cause of nasal obstruction in neonates and is caused by overgrowth of the nasal process of the maxilla. A pyriform aperture width of less than 11 mm (Fig. 1) is diagnostic [1]. Its clinical presentation is similar to that of bilateral choanal atresia and may range from mild nasal obstruction to lifethreatening asphyxia. Up to 63% of the patients have a solitary median incisor (Fig. 2), thought to represent a mild form of the holoprosencephaly spectrum [2], in which case MRI of the brain and chromosomal analysis are also indicated.