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Congenital Nasal Pyriform Aperture Stenosis
Bernadette L. Koch, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Congenital nasal pyriform aperture stenosis (CNPAS)
              • Definitions

                • Pyriform aperture (PA): Single triangular bony opening of anterior skull to nasal passages
                  • Anterior nasal passages separated by septal cartilage
                • CNPAS: Narrowing of single bony opening by characteristic bilateral maxillary bony anomalies

              IMAGING

              • General Features

                • CT Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        CLINICAL ISSUES

                        • Presentation

                          • Demographics

                            • Natural History & Prognosis

                              • Treatment

                                DIAGNOSTIC CHECKLIST

                                • Consider

                                  Selected References

                                  1. Ginat DT et al: CT and MRI of congenital nasal lesions in syndromic conditions. Pediatr Radiol. 45(7):1056-65, 2015
                                  2. Wormald R et al: Congenital nasal pyriform aperture stenosis 5.7 mm or less is associated with surgical intervention: a pooled case series. Int J Pediatr Otorhinolaryngol. 79(11):1802-5, 2015
                                  3. Wine TM et al: Congenital nasal pyriform aperture stenosis: is there a role for nasal dilation? JAMA Otolaryngol Head Neck Surg. 140(4):352-6, 2014
                                  4. Devambez M et al: Congenital nasal pyriform aperture stenosis: diagnosis and management. Cleft Palate Craniofac J. 46(3):262-7, 2009
                                  5. Sultan B et al: Congenital nasal pyriform aperture stenosis: feeding evaluation and management. Int J Pediatr Otorhinolaryngol. 73(8):1080-4, 2009
                                  6. Tate JR et al: Congenital nasal pyriform aperture stenosis. Otolaryngol Clin North Am. 42(3):521-5, 2009
                                  7. Osovsky M et al: Congenital pyriform aperture stenosis. Pediatr Radiol. 37(1):97-9, 2007
                                  8. Guilmin-Crépon S et al: High proportion of pituitary abnormalities and other congenital defects in children with congenital nasal pyriform aperture stenosis. Pediatr Res. 60(4):478-84, 2006
                                  9. Hall RK: Solitary median maxillary central incisor (SMMCI) syndrome. Orphanet J Rare Dis. 1:12, 2006
                                  10. Lee JC et al: The measurement of congenital nasal pyriform aperture stenosis in infant. Int J Pediatr Otorhinolaryngol. 70(7):1263-7, 2006
                                  11. Vercruysse JP et al: Congenital nasal pyriform aperture stenosis: a rare cause of neonatal nasal obstruction. J Pediatr Surg. 41(4):e5-7, 2006
                                  12. Belden CJ et al: CT features of congenital nasal piriform aperture stenosis: initial experience. Radiology. 213(2):495-501, 1999
                                  13. Bignault A et al: Congenital nasal piriform aperture stenosis. AJNR Am J Neuroradiol. 15(5):877-8, 1994
                                  14. Ey EH et al: Bony inlet stenosis as a cause of nasal airway obstruction. Radiology. 168(2):477-9, 1988
                                  Related Anatomy
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                                  Related Differential Diagnoses
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                                  References
                                  Tables

                                  Tables

                                  KEY FACTS

                                  • Terminology

                                    • Imaging

                                      • Top Differential Diagnoses

                                        • Pathology

                                          • Clinical Issues

                                            • Diagnostic Checklist

                                              TERMINOLOGY

                                              • Abbreviations

                                                • Congenital nasal pyriform aperture stenosis (CNPAS)
                                              • Definitions

                                                • Pyriform aperture (PA): Single triangular bony opening of anterior skull to nasal passages
                                                  • Anterior nasal passages separated by septal cartilage
                                                • CNPAS: Narrowing of single bony opening by characteristic bilateral maxillary bony anomalies

                                              IMAGING

                                              • General Features

                                                • CT Findings

                                                  • Imaging Recommendations

                                                    DIFFERENTIAL DIAGNOSIS

                                                      PATHOLOGY

                                                      • General Features

                                                        CLINICAL ISSUES

                                                        • Presentation

                                                          • Demographics

                                                            • Natural History & Prognosis

                                                              • Treatment

                                                                DIAGNOSTIC CHECKLIST

                                                                • Consider

                                                                  Selected References

                                                                  1. Ginat DT et al: CT and MRI of congenital nasal lesions in syndromic conditions. Pediatr Radiol. 45(7):1056-65, 2015
                                                                  2. Wormald R et al: Congenital nasal pyriform aperture stenosis 5.7 mm or less is associated with surgical intervention: a pooled case series. Int J Pediatr Otorhinolaryngol. 79(11):1802-5, 2015
                                                                  3. Wine TM et al: Congenital nasal pyriform aperture stenosis: is there a role for nasal dilation? JAMA Otolaryngol Head Neck Surg. 140(4):352-6, 2014
                                                                  4. Devambez M et al: Congenital nasal pyriform aperture stenosis: diagnosis and management. Cleft Palate Craniofac J. 46(3):262-7, 2009
                                                                  5. Sultan B et al: Congenital nasal pyriform aperture stenosis: feeding evaluation and management. Int J Pediatr Otorhinolaryngol. 73(8):1080-4, 2009
                                                                  6. Tate JR et al: Congenital nasal pyriform aperture stenosis. Otolaryngol Clin North Am. 42(3):521-5, 2009
                                                                  7. Osovsky M et al: Congenital pyriform aperture stenosis. Pediatr Radiol. 37(1):97-9, 2007
                                                                  8. Guilmin-Crépon S et al: High proportion of pituitary abnormalities and other congenital defects in children with congenital nasal pyriform aperture stenosis. Pediatr Res. 60(4):478-84, 2006
                                                                  9. Hall RK: Solitary median maxillary central incisor (SMMCI) syndrome. Orphanet J Rare Dis. 1:12, 2006
                                                                  10. Lee JC et al: The measurement of congenital nasal pyriform aperture stenosis in infant. Int J Pediatr Otorhinolaryngol. 70(7):1263-7, 2006
                                                                  11. Vercruysse JP et al: Congenital nasal pyriform aperture stenosis: a rare cause of neonatal nasal obstruction. J Pediatr Surg. 41(4):e5-7, 2006
                                                                  12. Belden CJ et al: CT features of congenital nasal piriform aperture stenosis: initial experience. Radiology. 213(2):495-501, 1999
                                                                  13. Bignault A et al: Congenital nasal piriform aperture stenosis. AJNR Am J Neuroradiol. 15(5):877-8, 1994
                                                                  14. Ey EH et al: Bony inlet stenosis as a cause of nasal airway obstruction. Radiology. 168(2):477-9, 1988