Epiglottitis

Epiglottitis is the rapid progressive inflammation of the epiglottis and surrounding supraglottis that was historically primarily caused by Haemophilus influenzae type b (Hib). Since the introduction of the Hib vaccine in 1985, the incidence of Hib epiglottitis has significantly decreased. Epiglottitis is now more common in adults than in children and is usually caused by other bacteria (e.g., streptococci and staphylococci). Children with epiglottitis typically appear acutely ill 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 clinical presentation. If the diagnosis is unclear and the patient is stable, a lateral cervical x-ray may be considered on which a thumb 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.

• More common in adults than children ^[1]
• Peak incidence in children: 6–12 years ^[2]
• Peak incidence in adults: 40–50 years ^[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
    • Nontypeable H. influenzae
• Risk factors
  • Not immunized against Hib
  • Immunodeficiency

References:^[3]

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) ^[3]

• Respiratory distress (inspiratory retractions, cyanosis)
• Inspiratory stridor
• 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
• Acute onset of high fever (39–40°C; 102–104°F)
• Toxic appearance
• Restlessness and/or anxiety
• Absence of cough
• Tenderness to palpation over larynx/throat ^[4]

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

References:^[3][5]

In young children, airway management is a higher priority than diagnostic evaluation . Advanced airway placement is rarely required in adults. ^[4]

Approach ^[4][6][7]

• Keep the patient sitting upright to prevent further airway compromise.
• Minimize external stressors and stimuli that could worsen respiratory distress and airway edema.
• Consult an airway specialist early.
• Provide supplemental oxygen as needed.
• Assess for:
  • Clinical features of airway obstruction (e.g., stridor, tachypnea, retractions, hypercapnia, cyanosis, loss of air movement, altered mental state)
  • Risk factors for acute deterioration (e.g., immunocompromise or diabetes, epiglottic abscess, rapid onset of symptoms, or rapid progression of severe symptoms) ^[4]
• Patients with clinical features of airway obstruction or risk factors for acute deterioration
  • If there are signs of acute respiratory distress, begin bag-mask ventilation with 100% oxygen.
  • Consider racemic epinephrine in otherwise healthy adults as a temporizing measure until a definitive airway is established. ^[6][8]
  • Secure the airway with emergency endotracheal intubation in a controlled setting by an experienced practitioner.
  • Prepare to perform an emergency surgical airway if endotracheal intubation is unsuccessful.
• Patients without severe airway obstruction or risk factors for acute deterioration: intubation may not be required.
  • Consider careful visualization of the epiglottis or imaging to confirm the diagnosis.
  • Start treatment for epiglottitis.
  • Monitor in an ICU setting for at least 12 hours. ^[5]

Acute epiglottitis is an airway emergency. Urgently consult a physician experienced in difficult airway management (e.g., an emergency physician, anesthesiologist, or otolaryngologist).

Endotracheal intubation ^[5][7][9]

• Indications
  • Respiratory distress
  • Altered mental status
  • Inability to swallow
  • Stridor
  • Drooling
  • Voice changes
• Procedure: Should be performed by an anesthesiologist, emergency physician, or otolaryngologist, ideally in an OR, ICU, or resuscitation area of an emergency department.
  • Ensure difficult airway cart is at the bedside.
  • Prepare for difficult intubation with a backup plan, e.g., emergency surgical airway.
    • Use video-assisted laryngoscopy, if available.
    • Consider flexible fiberoptic intubation or rigid bronchoscopy, if available and trained.
  • 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.

Intubation should be performed under direct visualization; avoid blind nasotracheal intubation as it risks airway obstruction. ^[4]

Emergency surgical airway ^[9][10]

Indicated if intubation is unsuccessful

• Adults and older children: surgical cricothyroidotomy (See “Surgical airway management” for details)
• Children < 8 years old: needle cricothyrotomy

Approach ^{[6][11][12][13][14]}

• Epiglottitis is primarily a clinical diagnosis.
  • See “Clinical features.”
  • See also “Differential diagnoses of stridor” and “Overview of deep neck infections” for the evaluation of alternate diagnoses.
• If the diagnosis is uncertain and there are no signs of impending airway obstruction:
  • Visualize the epiglottis to confirm the diagnosis.
  • Consider imaging to confirm the diagnosis if:
    • Visualization of the epiglottis is unclear or unsuccessful.
    • Alternate diagnoses need to be ruled out, e.g., croup, abscess, foreign body aspiration.

Secure the airway before initiating diagnostic studies or procedures in patients with impending airway compromise, especially in children.

Visualization of the epiglottis ^{[6][11][12][13][14]}

• Indication: There is suspicion for epiglottitis but no clinical features of 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 department.
• 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 structure

Imaging ^{[6][11][12][13][14]}

Soft-tissue lateral neck x-ray ^[15]

• 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
  • Thumb sign (also referred to as thumbprint sign): enlarged epiglottis and supraglottic narrowing
  • Narrowing or complete loss of the normal pre-epiglottic air shadow (vallecula sign)
  • Thick aryepiglottic folds

CT of the neck with IV contrast ^[16]

• 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 ^[11]

• 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 ^[7][11]

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 ). ^{[6][17][18][19]}
• For patients with severe penicillin allergy, consider a fluoroquinolone (e.g., levofloxacin ). ^[7][19]
• Consider the addition of an antibiotic with anti-MRSA activity (e.g., vancomycin , clindamycin ). ^[6][18][19]

Adjunctive therapy ^{[11][14][20][21]}

• Consider empiric steroids. ^[21]
  • Dexamethasone ^[21]
  • OR methylprednisolone ^[21]
• 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 clinical features of airway obstruction.
• Prepare for difficult intubation.
• If emergency intubation is required, ensure the most experienced clinician or specialist performs it.
• 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%) ^[22]

• Hib vaccine (see “Immunization schedule”)
• Postexposure prophylaxis with rifampin ^[11][23][24]
  • 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