Congenital nasal pyriform aperture stenosis: Diagnosis and management of 20 cases

The objective of this study was to review the characteristics of congenital nasal pyriform aperture stenosis (CNPAS) in a series of 20 children seen b etween 1993 and 1996. The diagnosis was made by physical examination and co mputed tomography scan. A single central maxillary incisor was detected in 12 cases (60%). Three children had morphological abnormalities of the pitui tary gland shown on magnetic resonance imaging. One child had an antidiuret ic hormone deficiency, and another child had a growth hormone deficiency. T wo children had craniosvnostoses 1 of which was Apert's syndrome. All patie nts underwent operation by a sublabial approach; and 1 was referred for a c olumellar necrosis after nasal stenting. After surgery, all patients showed improvement, and the nasal stenting was usually removed 1 week after surge ry, Follow-up revealed normal breathing. In conclusion, CNPAS was previousl y considered to be an unusual cause of nasal obstruction in neonates and in fants. The number of cases treated recently in our department suggests that this newly recognized entity is more common than expected.