Congenital nasal pyriform aperture stenosis (CNPAS) is defined as inadequate formation of the pyriform apertures forming the bony nasal openings resulting in respiratory distress and cyanosis soon after birth. Some clues such as worsening distress during feeding and improvement during crying may indicate a nasal cause of respiratory distress rather than distal airway etiology. Inability or difficulty passing a small tube through the nasal cavities may suggest CNPAS. The presenting clinical features of CNPAS can be similar to other obstructive nasal airway anomalies such as choanal atresia. Diagnosis is confirmed via CT scan with a total nasal aperture less than 11mm.
CNPAS may occur in isolation or it may be a sign of other developmental abnormalities such as holoprosencephaly, anterior pituitary abnormalities, or encephalocele. Some physical features of holoprosencephaly include closely spaced eyes, facial clefts, a single maxillary mega incisor, microcephaly, nasal malformations, and brain abnormalities (i.e. incomplete separation of the cerebral hemispheres, absent corpus callosum, and pituitary hormone deficiencies). It is important to rule out other associated abnormalities to ensure optimal treatment and intervention.
Conservative treatment of CNPAS includes humidification, nasal steroids, nasal decongestants and reflux control. Failure of conservative treatment defined by respiratory or feeding difficulty necessitates more aggressive intervention. The most definitive treatment for CNPAS is surgical intervention to enlarge the pyriform apertures.
Adam Johnson MD, PhD
Abby Nolder MD