Neonatal ENT

Question 1

Difference between choanal atresia and congenital pyriform aperture stenosis (CNPAS)

Answer 1

Choanal atresia: complete obstruction of the nare by a bone or a membrane; no connection posteriorly; can be unilateral or bilateral

CNPAS: narrowing (stenosis) of the anterior nares usually secondary to prominent maxillary nasal processes or other maxillary overgrowth (either bony overgrowth or medial positioning)

Can present very similarly, noising breathing at rest with some respiratory distress +/- cyanosis (esp if bilateral); cyanosis resolves with crying.

Question 2

Choanal atresia can be associated with which syndrome of anomalies?

Answer 2

CHARGE syndrome: Coloboma, Heart defects, Atresia choanae, Retardation of growth, Genitourinary anomalies, Ear anomalies)

Question 3

What condition(s) can be associated with a solitary central incisor?

Answer 3

Choanal atresia AND Congenital Pyriform Aperture Stenosis

Question 4

Congenital Pyriform Aperture Stenosis can be associated with what other congenital anomalies?

Answer 4

Holoprosencephaly, septo-optic dysplasia, pituitary anomalies, mid-face anomalies, etc.

Question 5

Management of choanal atresia?

Answer 5

Unilateral– usually does not require treatment during infancy, as long as no concerns for respiratory distress at rest

Bilateral– establish stable airway (oral vs ETT), transnasal endoscopic surgery +/- stents

Question 6

Management of Congenital Pyriform Aperture Stenosis

Answer 6

Conservative first– stenotic NOT atretic (topical decongestants, nasal steroids, humidification, nasal saline, etc)

If conservative measures fail, then endoscopic surgery via sublabial (upper lip) approach to remove excess bone. Usually will require stents to ensure the opening is patent in the post-operative period.