SUBGLOTTIC STENOSIS
 
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SUBGLOTTIC STENOSIS

 

DEFINITION:

A congenital defect of the larynx characterized by soft tissue thickening in the subglottic area resulting in varying degrees of upper airway obstruction.

EPIDEMIOLOGY:

  • incidence: rare
  • age of onset:
    • newborn (stridor) -> childhood (croup-like symptoms)
  • risk factors:
    • sporadic
    • M = F
    • Trisomy 21

PATHOGENESIS:

1. Background

  • enlargement of the subglottic area due to a developmental defect of the conus elasticus or cricoid cartilage
  • minimal laryngeal inflammation may cause upper airway obstruction because the rigid cricoid cartilage prevents outward expansion thus forcing the edema internally
  • occurs in the newborn when the internal diameter of the airway in the region of the cricoid measures less than 3.5 mm in diameter

2. Types

1. Soft Stenosis

  • stenosis due to narrowing of the conus elasticus
  • increased amounts of connective tissue and large dilated mucous glands

2. Hard Stenosis

  • stenosis due to narrowing of the cricoid cartilage
  • increased amounts of cartilage results in a shelf-like cartilaginous plate with a minimal posterior airway

3. Combined Stenosis

  • stenosis due to soft and hard components

CLINICAL FEATURES:

1. Respiratory Manifestations

  • upper airway obstruction
    • inspiratory and expiratory stridor
      • worse with crying & upper respiratory tract infections
    • recurrent croup-like symptoms
    • may be discovered following a failed extubation

2. Complications

  • feeding difficulties +/- failure to thrive

INVESTIGATIONS:

1. Direct Laryngoscopy

  • diagnostic

2. Imaging Studies

1. Lateral Neck X-Ray

  • decreased AP diameter of the subglottic area

MANAGEMENT:

1. Supportive

  • respiratory support with severe life-threatening upper airway obstruction, i.e., intubation, tracheostomy
  • aggressive antibiotic therapy for upper respiratory tract infections

2. Surgery

  • indicated for severe subglottic stenosis
  • anterior cricoid split, excision of stenotic area, laryngeal grafts, internal stenting, larynogotracheoplasty
  • tracheotomy
    • may be needed in up to 40% of cases
    • may be decannulated by 2-3 years of age due to laryngeal growth

3. Prognosis

  • natural history is usually one of spontaneous resolution with laryngeal growth

 

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