Epiglottitis is the inflammation of the epiglottis and surrounding structures. The majority of cases are caused by bacterial infection; however, several viral and fungal pathogens have been identified depending on the patient’s immune status and age. Symptoms are rapid in onset and severe. Without treatment, epiglottitis can cause life-threatening airway obstruction that presents with difficulty breathing, stridor, and cyanosis. Diagnosis is mainly clinical but can be confirmed by pharyngoscopy. The focus of treatment is airway management and administration of antibiotics.

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Doctor with child

Image : “Doctor with child” by
skeeze. License: CC0


Epidemiology

  • Changes after the introduction of the Haemophilus influenzae type b conjugate vaccine to routine infant immunization schedule in the US include:
    • Increased age of children affected post-vaccination:
      • Median age of children with epiglottitis is 6-12 years of age  
      • Can still occur at any age
    • Decreased incidence post-vaccination:
      • Affects 1.6 in 100,000 adults per year
      • Affects 0.5 in 100,000 children per year
  • Worldwide:
    • More common in adult men, with a male to female ratio of 3:1
    • No seasonal predilection in incidence
    • More common in nations with no immunization for Haemophilus influenzae type b
  • Risk factors:
    • Children:
      • Incomplete or lack of immunization for Hib
      • Immune deficiency
    • Adults:
      • Immune deficiency
      • Substance abuse
      • Hypertension
      • Diabetes mellitus
      • BMI >25
      • Concurrent pneumonia

Video

Infections of the Upper Pediatric Airway: Epiglottitis by Brian Alverson, MD

Etiology

  • Definition: rapidly progressing inflammation of the epiglottis and adjacent supraglottic structures (area above the vocal cords).
  • Disease can be bacterial, viral, fungal, or noninfectious. In previously healthy children, most causes are bacterial. 
  • Disease is mainly caused by Haemophilus influenzae type b (especially in nonimmunized children), Haemophilus parainfluenzae, Streptococcus pneumonia, and group A Streptococci 
    • Most common organism causing the disease is Haemophilus influenzae, accounting for approximately 25% of cases
    • Infection is now less prevalent due to Hib vaccination.
  • The disease is less commonly caused in immunocompromised patients by the following:
    • Staphylococcus aureus
    • Mycobacteria
    • Bacteroides
    • Escherichia coli 
    • Candida
    • Pseudomonas aeruginosa
    • Serratia
    • Enterobacter
  • Non-infectious causes: trauma from thermal injury, foreign body ingestion, and caustic ingestion, crack cocaine smoking, chemotherapy to head and neck region
  • Other causes: lymphoproliferative disorders (e.g., graft-versus-host disease)

Signs and Symptoms

  • Hallmark in children is ‘the 3 D’s’: dysphagia, drooling, and distress
  • Difficulty in swallowing
  • Painful swallowing (odynophagia)
  • Sudden onset of high fever
  • Inspiratory stridor: abnormal high pitched sound while breathing
  • Excessive drooling
  • Hoarseness or muffled speech, often described as a ‘hot potato’ voice
  • Difficulty breathing → respiratory distress → cyanosis
  • Restlessness, anxiety, irritability
  • Pharyngitis
  • Severe sore throat
    • Most common complaint in adults
  • Refusal to lie flat
  • Insisting on sitting up and leaning forward (tripod position) with neck hyperextended and chin thrust forward (sniffing posture)
    • Maximizes the diameter of the narrowed airway

Diagnosis

  • Upon clinical suspicion, proceed to airway management before further testing. 
    • Suspected epiglottitis is a medical emergency!
  • Diagnosis is made on visualization of the epiglottis via indirect or direct fiberoptic laryngoscopy. Suggested to happen under general anesthesia in the operating room.
    • Epiglottis appears to be cherry red in color and swollen.
    • Inflammation and edema of the supraglottic structures.
    • Tenderness to palpation to the hyoid bone and region.
  • Inspection by a tongue depressor is discouraged as it may provoke airway spasm or distress in children!
  • Laboratory: CBC with differential, blood culture, epiglottal culture if intubated
    • Not required for diagnosis
    • Most patients will have elevated WBC, but this is a nonspecific finding
    • Do not perform needle sticks in young children with suspected epiglottitis unless already intubated
  • Lateral neck X-ray: 
    • Disease can be confirmed by a ‘thumb sign’ (enlarged, swollen epiglottis and supraglottic region and dilated hypopharynx)
    • Radiographs are not necessary to make the diagnosis
  • CT scan imaging: 
    • Halloween sign describes normal thickness epiglottis but excludes other causes of swelling (e.g., retropharyngeal abscess or peritonsillar abscess)
    • Radiographs are not necessary to make the diagnosis

Image: Swollen epiglottis in epiglottitis observed on laryngoscopy. License: CC BY-SA 3.0

epiglottitis x-ray

Image: Acute epiglottitis presenting with the ‘thumb sign’ in a lateral neck X-ray. By Med Chaos – Own work. License: CC0 1.0

Image: Halloween sign on CT showing a normal thickness epiglottis and excluding epiglottitis. By Chaustanley. License: CC BY-SA 3.0

Treatment and Prevention

  • Airway management
    • Tracheal intubation should be performed in case of airway compromise.
      • Extubation usually takes place 2-3 days after starting antibiotics.
    • If intubation fails, tracheostomy should be performed.
      • Intubation of a child should be performed in the operating room since unsuccessful intubation makes tracheostomy necessary.
    • Monitor in an intensive care unit
  • Antibiotic therapy
    • Third-generation cephalosporin (ceftriaxone or cefotaxime) and an antistaphylococcal agent (vancomycin) for 7-10 days
    • Severe hypersensitivity reaction to penicillin or cephalosporin antibiotics: vancomycin + quinolone or carbapenem
      • Consult infectious disease for recommendation and follow up.
    • Adjust accordingly to blood culture and epiglottis culture if epiglottis culture was possible to obtain.
  • Prevention
    • Hib conjugate vaccine and pneumococcal vaccine
    • In case of exposure to infected individuals, rifampicin can be used prophylactically (e.g., all close contacts).

Complications

  • Prognosis is good if diagnosed and treated immediately but the disease can lead to death if there is acute and untreated airway obstruction.
  • Complications of epiglottitis
    • Airway obstruction → asphyxia → death
    • Epiglottic abscess 
    • Secondary infection
      • Pneumonia
      • Cervical adenitis
      • Cellulitis
      • Septic arthritis
      • Empyema
      • Meningitis
      • Bacteremia/septic shock
    • Vocal granuloma
    • Cartilaginous metaplasia of epiglottis
    • Necrotizing epiglottitis with immunodeficiencies

Clinical Relevance

The following is a list of differential diagnoses for epiglottitis: 

  • Tuberculosis: a disease caused by Mycobacterium tuberculosis which is a very acid-fast bacteria that grows slowly and can survive in macrophages.
  • Pertussis: a bacterial infection caused by Bordetella pertussis where infected patients present with paroxysmal whooping cough, which is persistent for 2 weeks or more.
  • Pharyngitis: inflammation of the back of the throat or pharynx, which is usually caused by an upper respiratory tract infection. It typically results in a sore throat and fever. Other symptoms may include a runny nose, cough, headache, and hoarseness.
  • Pneumonia: acute or chronic inflammation of lung tissue caused by infection with bacteria, viruses, or fungi. It can be also due to toxic triggers through inhalation of toxic substances, immunological processes, or in the course of radiotherapy.
  • Peritonsillar abscess: a bacterial infection that has an onset of an untreated strep throat or tonsillitis, which involves a pus-filled pocket that forms near a tonsil.
  • Retropharyngeal abscess: a collection of pus in the back of the throat caused by a bacterial infection, which clinically manifests as pain when swallowing, fever, stiff neck, and noisy breathing.
  • Croup: croup, or laryngotracheobronchitis, is caused by a viral infection or rarely by a bacterial infection that results in swelling inside the trachea and interferes with normal breathing. Patients develop a characteristic barking cough and stridor.
  • Laryngomalacia: laryngomalacia and tracheomalacia are the most common upper airway conditions that produce stridor in newborns and have characteristic stridor to respiratory distress.
  • Measles: measles morbillivirus is a virus of the genus Morbillivirus within the family Paramyxoviridae. Humans are the natural hosts of this virus and no other animal reservoirs are known to exist.
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