Epiglottitis

Epiglottitis is the rapid progressive inflammation of the epiglottis and surrounding supraglottis that classically was primarily caused by Haemophilus influenzae type b (Hib). Acute epiglottitis has become rare following the implementation of the Hib vaccine and most cases now involve other bacteria. Although acute epiglottitis can occur at any age, especially when unimmunized, young children are most commonly affected. Children suffering from epiglottitis typically appear toxic and position themselves in a tripod stance (sitting and leaning forward) in an attempt to improve their airway diameter. The disease is characterized by the acute onset of fever, drooling, sore throat, dysphagia, and, in severe cases, respiratory distress accompanied by inspiratory retractions and cyanosis. Impending airway obstruction is also accompanied by a muffled voice and restlessness. Epiglottitis is diagnosed based on the clinical presentation. If the diagnosis is unclear and the patient is stable, a lateral cervical x-ray may be considered on which a thumbprint sign may be seen. If the patient is unstable, their airway should first be secured, after which direct laryngeal examination may be performed. Patients should be closely monitored in a hospital and receive IV antibiotics. Most patients make a full recovery after prompt and adequate treatment.

Peak incidence: 6–12 years; (but can occur at any age, including adults, especially when unimmunized) ^[1]

Epidemiological data refers to the US, unless otherwise specified.

• Pathogens
  • Traditionally: Haemophilus influenzae type b (Hib)
  • Most cases now involve:
    • Streptococcus pyogenes
    • Streptococcus pneumoniae
    • Staphylococcus aureus
    • Nontypable H. influenzae
• Risk factors
  • Not immunized against Hib
  • Immunodeficiency

References:^[2]

Bacteria invades tissue (directly or through hematogenous spreading) of the epiglottis and/or surrounding supraglottic structures (i.e., arytenoids, aryepiglottic folds, and vallecula) → supraglottic inflammation and edema → narrowing of the airway → airway obstruction (partial or complete) ^[2]

• Acute onset of high fever (39–40°C; 102–104°F)
• Toxic appearance
• Tripod position: eases respiration as the airway diameter is increased by leaning forward and extending the neck in a seated position
• Sore throat
• Dysphagia and odynophagia
• Drooling
• Muffled voice (i.e., resembling a “hot-potato” voice) with painful speech
• Respiratory distress (inspiratory retractions, cyanosis) and inspiratory stridor
• Restlessness and/or anxiety

The hallmarks of epiglottitis are the three Ds: Dysphagia, Drooling, and Distress.

References:^[2]

Approach ^{[3][4][5][6][7][8]}

• Apply supplemental oxygen as needed.
• Evaluate for signs of severe airway obstruction (e.g., stridor, tachypnea, retractions, hypercapnia, altered mental state).
• If there is suspicion for severe airway obstruction, secure the airway with emergency endotracheal intubation.
• If there are no signs of severe airway obstruction, consider careful visualization of the epiglottis or imaging to confirm the diagnosis.

Acute epiglottitis is an airway emergency and requires airway management by an experienced physician (e.g., an emergency physician or otolaryngologist).

Endotracheal intubation ^[5][9]

• Indications
  • Respiratory distress
  • Inability to swallow
  • Stridor
  • Drooling
• Procedure
  • Perform in an OR, ICU, or emergency room.
  • Should be performed by an anesthesiologist or otolaryngologist, if available
  • Ensure difficult airway cart is at the bedside.
  • Prepare for difficult intubation.
    • Use video-assisted laryngoscopy, if available.
    • Prepare for rigid bronchoscopy, if available .
    • Prepare for a surgical airway .
  • Maintaining spontaneous ventilation under general anesthesia is preferable.
  • Consider rapid sequence induction if there is rapid clinical deterioration.
  • Intubation tubes
    • In adults: small-sized endotracheal tubes
    • In children: nasotracheal tubes with a small diameter
  • Confirm and check the adequacy of ventilation.
  • Extubation should be performed 2–3 days (at the earliest) after starting antibiotic treatment.

Surgical airway ^[9][10]

• Indication: if intubation is unsuccessful
  • In adults and older children: surgical cricothyroidotomy. See “Surgical airway management”.
  • In children < 8 years of age: needle cricothyrotomy

Epiglottitis is primarily a clinical diagnosis. In patients without signs of impending airway obstruction, visualization of the epiglottitis can confirm the diagnosis. Imaging may not be necessary. ^{[3][4][6][11][12]}

Emergency airway management is indicated when airway obstruction is imminent and should not be delayed by diagnostic evaluation.

Visualization of the epiglottis ^{[3][4][6][11][12]}

• Indication: There is suspicion for epiglottis but no signs of impending airway obstruction.
• Procedure
  • Direct pharyngoscopy: oropharyngeal examination with a tongue blade
  • Direct laryngoscopy: can be performed during or after intubation
  • Indirect laryngoscopy (mirror examination) or flexible fiberoptic laryngoscopy
  • Perform in an OR, ICU, or emergency room.
• Additional considerations
  • Avoid increasing anxiety (especially in children).
    • Keep the patient comfortable and in a calm setting.
    • Keep the patient in a sitting position at all times (do not force the patient to lie supine).
    • If the patient is a child, let the parent/guardian hold the mask, and use distractions and humor to help keep the child relaxed.
  • In children, this procedure should only be performed by a skilled otolaryngologist.
• Characteristic findings
  • Direct pharyngoscopy: often normal; epiglottis is often not seen.
  • Indirect laryngoscopy or flexible fiberoptic laryngoscopy
    • Cherry-red epiglottis
    • Pooled secretions
    • Inflammation and edema of the supraglottic structures

In epiglottitis with impending airway compromise, it is imperative to secure the airway before attempting diagnostic laryngoscopy, especially in children.

Imaging ^{[3][4][6][11][12]}

If pharyngoscopy findings are unclear (e.g., the epiglottis cannot be visualized) in stable patients with no signs of impending airway obstruction and laryngoscopy cannot be performed, imaging can confirm the diagnosis and exclude other diagnoses (e.g., croup, abscess, or a foreign body).

Soft-tissue lateral neck x-ray ^[13]

• Indication: mainly performed in children if the clinical presentation in early cases is inconclusive
• Procedure: should be carried out under the supervision of an experienced physician
• Characteristic findings
  • Thumbprint sign: enlarged epiglottis and supraglottic narrowing
  • Loss of vallecular air space (vallecula sign)
  • Thick aryepiglottic folds

CT of the neck with IV contrast ^[14]

• Indication: only performed in adults, mainly to exclude other diagnoses
• Procedure: requires the supine position, which can compromise the airway
• Characteristic findings
  • Thickening of any of the following may be present:
    • Epiglottis
    • Aryepiglottic folds
    • False vocal cords and true vocal cords
    • Platysma muscle and prevertebral fascia
  • Loss of vallecular air space
  • Obliteration of preepiglottic fat

Additional diagnostic studies ^[3]

• Blood cultures (2 sets)
• Swab of the epiglottis and epiglottic culture : to guide antibiotic therapy
• Hib immunization status of the patient (and close contacts, if applicable)

Empiric IV antibiotics ^[3][5]

There are no guidelines on specific empiric antibiotic recommendations. All patients should receive IV antibiotics that are active against Hib, S. aureus, S. pyogenes, and S. pneumonia. Following cultures, antibiotics can be narrowed according to identified organisms.

• Most sources recommend monotherapy with a third-generation cephalosporin (e.g., cefotaxime; , ceftriaxone ); or a beta-lactam with a beta-lactamase inhibitor (e.g., ampicillin/sulbactam , amoxicillin/clavulanate , piperacillin/tazobactam ). ^{[4][15][16][17]}
• For patients with severe penicillin allergy, consider a fluoroquinolone (e.g., levofloxacin ). ^[5][17]
• Consider the addition of an antibiotic with anti-MRSA activity (e.g., vancomycin , clindamycin ). ^[4][16][17]

Adjunctive therapy ^{[3][12][18][19]}

• Consider empiric steroids. ^[19]
  • Dexamethasone ^[19]
  • OR methylprednisolone ^[19]
• IV fluid resuscitation: 20–30 mL/kg of isotonic fluids in children.

• Administer supplemental oxygen as needed.
• Establish IV access.
• Immediate otolaryngology and anesthesiology consultation
• Assess for indications for emergency airway intervention.
• Airway management (prepare for difficult intubation)
• Continuous monitoring: pulse oximetry, serial pulmonary examination
• Keep the patient calm and in the sitting position.
• Obtain blood cultures and epiglottis cultures prior to starting antibiotic therapy.
• Start empiric antibiotics.
• Consider steroids.
• Admit to the ICU.

• See “Differential diagnoses of pediatric inspiratory stridor” and “Differential diagnosis of dyspnea”
• Foreign body aspiration
• Anaphylactic reaction
• Chemical injury or thermal injury (burns)
• Laryngitis
• Peritonsillar abscess or retropharyngeal abscess

The differential diagnoses listed here are not exhaustive.

• Mortality rate < 1% (in patients without endotracheal intubation ∼ 10%) ^[20]

• Hib vaccine (see “Immunization schedule”)
• Postexposure prophylaxis with rifampin ^[3][21][22]
  • Indications
    • All index patients that are < 2 years of age and did not receive ceftriaxone or cefotaxime to treat Hib infections should receive postexposure prophylaxis.
    • All household contacts: if any member of the household is < 4 years of age and unimmunized and/or < 18 years of age and immunocompromised
    • All daycare attendees: if ≥ 2 cases of invasive Hib disease occurred within 60 days in this setting and unimmunized children attend the daycare facility