• Epidemiology & Risk Factors

    • Most common congenital nasal anomaly.
    • Pathophysiology: Failure of posterior nasal passage (choanae) to canalize (can be bony, membranous, or mixed).
    • Highly associated with CHARGE syndrome: Coloboma, Heart defects, Atresia choanae, Retardation of growth/development, GU anomalies, Ear anomalies/deafness.
  • Clinical Features

    • Bilateral (Neonatal presentation):
      • Neonates are obligate nasal breathers.
      • Paradoxical cyanosis: Cyanosis/resp distress that worsens with sleeping or feeding, and improves with crying (as the mouth opens).
      • Noisy breathing (stertor) and feeding difficulties.
    • Unilateral (Childhood presentation):
      • Often asymptomatic at birth.
      • Presents later with chronic, purulent, or mucoid unilateral nasal discharge.
  • Diagnosis

    • Initial/Screening: Inability to pass a small catheter (e.g., 8-10F) through the nares into the oropharynx.
    • Confirmatory/Gold Standard: CT scan of the facial bones/paranasal sinuses (delineates bony vs. membranous obstruction and narrows surgical approach).
    • Adjunct: Rigid nasal endoscopy (visualizes obstruction).
  • Differential Diagnostics

    • Cyanotic Congenital Heart Disease (e.g., Tetralogy of Fallot): Diff by cyanosis worsening with crying (Tet spells), abnormal cardiac auscultation (murmurs), and abnormal ECG/Echo.
    • Tracheoesophageal Fistula (TEF) / Esophageal Atresia: Diff by choking/coughing with feeding, but no paradoxical improvement with crying. Catheter passes through nose but coils in the esophagus.
    • Nasal Foreign Body: Diff by acute/subacute onset of unilateral foul-smelling purulent discharge in a toddler/older child; visualized directly on rhinoscopy.
  • Management

    1. Stabilize (Bilateral): Immediate airway security is priority. Place an oral airway, McGovern nipple, or perform endotracheal intubation if severe resp distress.
    2. Supportive: Orogastric (OG) tube placement for feeding.
    3. Definitive: Surgical repair (endoscopic transnasal puncture/resection w/ or w/o stenting).
  • Complications

    • Asphyxiation / Respiratory arrest (if bilateral).
    • Aspiration pneumonia.
    • Failure to thrive (FTT) due to feeding difficulties.
    • Post-operative restenosis.