Congenital Airway Anomalies

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Laryngomalacia - Floppy Larynx Fun

  • Most common congenital laryngeal anomaly & cause of stridor.
  • Pathophysiology: Immature, "floppy" supraglottic structures (arytenoids, epiglottis) collapse inward during inspiration.
  • Features:
    • Inspiratory stridor, onset by 4-6 weeks.
    • Worse: Supine, crying, feeding. Better: Prone.
    • Normal cry.
    • Severe cases: FTT, GERD, apnea.
  • Diagnosis: Flexible laryngoscopy (awake) shows dynamic collapse; classic "omega-shaped" epiglottis. Laryngomalacia: Omega-shaped epiglottis
  • Management:
    • Most resolve spontaneously by 18-24 months (conservative).
    • Severe: Supraglottoplasty.

Laryngomalacia is the most common cause of congenital stridor, typically presenting with inspiratory stridor that worsens in the supine position or during agitation.

Tracheo/Bronchomalacia - Weak Windpipe Woes

Tracheomalacia often presents with a characteristic expiratory barking cough and can be associated with tracheo-esophageal fistula.

  • Pathophysiology: Excessive compliance & dynamic collapse of tracheal/bronchial walls during expiration.
  • Etiology:
    • Primary (Intrinsic): Congenital cartilage weakness.
    • Secondary (Extrinsic): Compression (e.g., vascular ring), post-intubation, inflammation.
  • Clinical Features:
    • Expiratory stridor/wheeze, characteristic barking cough (worsens with crying, feeding, supine position).
    • Recurrent respiratory infections; severe cases: cyanotic spells (“dying spells”).
    • Symptoms may improve in prone position.
  • Diagnosis:
    • Gold Standard: Flexible bronchoscopy (shows >50% luminal collapse during expiration).
    • Fluoroscopy, CT/MRI (to identify extrinsic compression).
  • Management:
    • Conservative: Most resolve spontaneously by 1-2 years. Humidification, chest physiotherapy, GERD management.
    • CPAP/BiPAP for moderate symptoms.
    • Surgical (severe/refractory): Aortopexy, tracheopexy, tracheostomy.

Dynamic CT of tracheal collapse in tracheomalacia

Subglottic Stenosis & Webs - Narrow Escape Routes

Subglottic Stenosis (SGS): Airway narrowing below vocal cords.

  • Types:
    • Congenital: Developmental (cricoid anomaly).
    • Acquired: Post-intubation (commonest), trauma, GERD.
  • Symptoms: Biphasic stridor, barking cough, recurrent croup, dyspnea; worsens with URI.
  • Diagnosis: Endoscopy (flex/rigid). X-ray (steeple sign, non-specific).

⭐ Congenital subglottic stenosis is often defined as a subglottic diameter <4mm in a full-term infant; Cotton-Myer grading is used for severity.

  • Cotton-Myer Grading (% obstruction):
    • Grade I: 0-50%
    • Grade II: 51-70%
    • Grade III: 71-99%
    • Grade IV: No lumen

Subglottic Webs: Thin membrane in subglottis; rare.

  • Symptoms: Symptoms like SGS; severity varies.
  • Diagnosis: Endoscopy.

Management Approach:

Endoscopic view of subglottic stenosis

  • SGS Treatment: Observation, dilation, laser, cricoid split, LTR, CTR. Tracheostomy if severe.
  • Web Treatment: Endoscopic division (cold knife/laser).

Vascular Rings & Slings - Artery Squeeze Play

Vascular Rings: Normal vs. Double Aortic Arch

  • Anomalous vessels compress trachea/esophagus.
  • Types & Features:
    • Double Aortic Arch: Complete ring; tracheal & esophageal squeeze.

      ⭐ A double aortic arch is the most common symptomatic vascular ring, causing both tracheal and esophageal compression.

    • Right Aortic Arch with Aberrant Left Subclavian Artery: Lig. arteriosum completes ring. Dysphagia lusoria.
    • Pulmonary Artery Sling: L. PA from R. PA, between trachea & esophagus. Posterior tracheal squeeze. Assoc. tracheal stenosis (ring-sling complex).
    • Innominate Artery Compression: Anterior tracheal squeeze by innominate artery.
  • Symptoms:
    • Respiratory: Stridor (biphasic, expiratory ↑), wheeze, cough, apnea.
    • Esophageal: Dysphagia, feeding issues.
    • Symptoms worsen with feeding, crying, neck flexion.
  • Diagnosis:
    • CXR, Barium swallow (indentations).
    • CT Angiography / MRI: Gold standard.
    • Bronchoscopy: Pulsatile compression, assess tracheomalacia.
  • Management: Surgical division of ring/vessel reimplantation (PA sling).

High‑Yield Points - ⚡ Biggest Takeaways

  • Laryngomalacia: Most common cause of stridor in infants; inspiratory stridor, often self-resolving.
  • Subglottic Stenosis: Biphasic stridor; most common anomaly needing tracheostomy in young children.
  • Vocal Cord Paralysis: Unilateral (weak cry, aspiration); Bilateral (severe stridor, airway compromise).
  • Choanal Atresia: Bilateral is a neonatal emergency (cyanosis with feeding); CHARGE syndrome association.
  • Tracheomalacia: Expiratory stridor/barking cough; dynamic collapse; associated with TEF.
  • Laryngeal Webs: Stridor, weak cry/aphonia; incomplete recanalization; glottic most common.

Practice Questions: Congenital Airway Anomalies

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Most common cause of intermittent stridor in a 10-day-old child shortly after birth is:

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Flashcards: Congenital Airway Anomalies

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_____ classification is used to stage juvenile angiofibromas

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_____ classification is used to stage juvenile angiofibromas

Modified Radkowski's

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