Isolated congenital nasal pyriform aperture stenosis

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Synonyms: Isolated apertura pyriformis stenosis | Isolated nasal pyriform aperture hypoplasia

A rare otorhinolaryngological malformation characterized by narrowing of the pyriform aperture (i. e. < 8 to 10 mm in a full-term infant) due to an overgrowth of the nasal process of the maxilla resulting in potentially lethal nasal airway obstruction in the newborn. Depending on the degree of obstruction clinical signs and symptoms include inspiratory stridor respiratory distress cyanosis sternal retraction ribcage asymmetry and feeding difficulties.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview. Reference: Access aggregated data from Orphanet at Orphadata. Orphadata: Free access data from Orphanet. © INSERM 1999. Available on Data version July 2024

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