Congenital nasal piriform aperture stenosis.

Congenital nasal piriform aperture stenosis is an unusual cause of upper respiratory obstruction in infants and young children. (1) Clinically, this entity can mimic other congenital anomalies, including choanal atresia. Stenosis of the nasal piriform aperture is believed to occur secondary to overgrowth of the medial portion of the maxilla. Infants may experience respiratory distress at birth or within the first few months of life. (2) Breathing problems can be triggered by an upper respiratory infection, further compromising the already-narrowed nasal passages.

When congenital nasal piriform aperture stenosis is suspected, the diagnosis is easily confirmed with axial computed tomography (CT). Additionally, a single large central upper incisor (megaineisor) can be identified, as in the case illustrated here (figure). This entity is part of the spectrum of midface anomalies in children. This spectrum includes anomalies that are related to the nasal cavity, nasofrontal region, nasolacrimal apparatus, and craniofacial syndromes. (3,4) Magnetic resonance imaging can be useful to rule out other associated congenital midface masses, such as dermoid cysts, epidermoid cysts, nasal gliomas, and encephaloceles.

References

(1.) Belden CJ, Mancuso AA, Schmalfuss IM. CT features of congenital nasal piriform aperture stenosis: Initial experience. Radiology 1999;213:495-501.

(2.) Lowe LH, Booth TN. Joglar JM, Rollins NK. Midface anomalies in children. Radiographics 2000;20:907-22.

(3.) Bignault A, Castillo M. Congenital nasal piriform aperture stenosis. AJNR Am J Neuroradiol 1994;15:877-8.

(4.) Ey EH, Han BK, Towbin RB, Jaun WK. Bony inlet stenosis as a cause of nasal airway obstruction. Radiology 1988;168:477-9.

From the Department of Radiology. Louisiana State University Health Sciences Center, New Orleans.