Choanal atresia

Background

Nasal endoscopic view of posterior choanae, depicting complete atresia bilaterally.

• Obliteration or blockage of the posterior nasal aperture
• Incidence is between 1:5000 and 1:8000 live births
• Approximately 60% of cases are unilateral
• More common in girls than in boys

Clinical Features

• Clinical presentation will depend on if unilateral or bilateral
• Unilateral
  • Typically present later in life
  • Commonly with purulent nasal discharge and obstruction on the affected side and/or a history of chronic sinusitis
  • Rarely will present with respiratory distress

• Bilateral
  • Typically will present in early life with episodes of acute respiratory distress with cyanosis that is relieved with crying and with the return of cyanosis with rest (paradoxical cyanosis)
  • May present with feeding difficulty, as they are unable to breathe and feed simultaneously

• May be an isolated anomaly or associated with a congenital anomaly syndrome (e.g. Treacher-Collins, CHARGE, VACTERL/VATER, Pfeiffer)
• Other congenital anomalies are in present in 50% of individuals with unilateral choanal atresia and 60% of individuals with bilateral choanal atresia

Differential Diagnosis

• Deviated or dislocated nasal septum
• Nasal septal hematoma
• Nasal polyp
• Chordoma
• Isolated piriform aperture stenosis
• Nasal dermoid
• Nasolacrimal duct cyst
• Turbinate hypertrophy

Evaluation

Bilateral choanal atresia on CT scan.

• Examiner can attempt to pass a 5 or 6 fr catheter from the nose to the oropharynx, alternatively the examiner can use a qualitative measure of nasal airflow (i.e. the movement of a wisp of cotton under the nostrils, fogging of a mirror)
• Diagnosis is confirmed with CT imaging with intranasal contrast
• Ideally diagnosis of this condition should be done immediately after birth
• A thorough physical exam should also be performed to detect associated anomalies

Management

• Unilateral
  • Generally does not require emergent surgical repair, but respiratory status should be carefully considered.
  • Nasal saline can be helpful for keeping the nasal route clear
• Bilateral
  • If presenting with respiratory distress, individuals require immediate airway management, either with an oral airway or endotracheal intubation
  • Definitive management is surgical repair