Pyriform Aperture Stenosis Repair in Infants

Congenital nasal pyriform aperture stenosis is a rare developmental anomaly, which is considered a variant of holoprosencephaly. It is characterized by a pyriform aperture width of 11mm or less on maxillofacial computed tomography, and is frequently associated with a solitary median maxillary central incisor. It presents with cyclical cyanosis, feeding difficulty, and respiratory distress. Conservative management includes nasal decongestant, saline and steroid drops, or a McGovern nipple. When conservative management fails, surgical intervention is indicated.