Basics
Description
Acute life-threatening bacterial infection consisting of cellulitis and edema of the epiglottis, aryepiglottic folds, arytenoids, and hypopharynx, resulting in narrowing of the glottic opening and airway obstruction; also known as supraglottitis
Epidemiology
- Disease due to Haemophilus influenzae type B occurs most often between the ages of 1 and 7 years (overall range: infancy to adulthood).
- Epiglottitis and other invasive disease secondary to H. influenzae have been reduced by 99% since the introduction of the conjugate vaccines in 1987 (approved for use at 15 months) and 1990 (approved for use at 2, 4, and 6 months).
- Nontypeable H. influenzae now appears to be a more common cause of invasive disease than type B.
- Year-round occurrence
- All geographic areas
- Can have secondary cases in households or child care centers
- May be more frequent in children with sickle cell anemia, asplenia, immunoglobulin defects, or hematologic malignancies (e.g., leukemia)
- Increasing ratio of adult to pediatric cases
Incidence
- Incidence of pediatric epiglottitis due to any organism has declined in the postvaccine era (0.3-0.7/100,000 per year from 3.47 to 6.0/100,000 per year).
- Incidence in adults has remained steady (1-4/100,000 per year).
General Prevention
- Universal immunization with H. influenzae type B capsular polysaccharide conjugate vaccines at 2 and 4 months (potential dose at 6 months, depending on the vaccine), with booster at 12-15 months.
- Isolation of hospitalized patient: Droplet precautions should be continued for at least 24 hours from the initiation of effective antimicrobial therapy.
- Control measures: prophylaxis for H. influenzae type B index case and susceptible children in household and child care setting and intimate contacts with the assistance of infection control
- Rifampin: 20 mg/kg/day in single dose for 4 days
Pathophysiology
Edema of the supraglottic structures (uvula, aryepiglottic folds, arytenoids, epiglottis, and vocal cords) that reduces the airway aperture. Respiratory arrest can be caused by airway obstruction, aspiration of oropharyngeal secretions, or mucous plugging.
Etiology
- H. influenzae, nontypeable and type B (type B accounted for up to 90% of cases prior to the introduction of Hib vaccine)
- Streptococcus pneumoniae
- Streptococcus pyogenes (group A β-hemolytic Streptococcus)
- Staphylococcus aureus
- Groups C and G β-hemolytic Streptococcus
- Candida albicans may be an etiologic agent in immunocompromised patients and those receiving prolonged corticosteroid treatment.
- Pasteurella multocida has been implicated in a few cases after exposure to nasopharyngeal secretions from a cat.
- Other rare isolates: Moraxella catarrhalis, Klebsiella pneumoniae, Neisseria meningitidis, Pseudomonas species
- Bacterial superinfection of viral infections including herpes simplex, parainfluenza, varicella, Epstein-Barr
- Varicella can cause primary infection or lead to a secondary infection, often with S. pyogenes.
- Noninfectious etiologies include thermal injuries, trauma, and caustic ingestions.
Diagnosis
History
- Abrupt onset of high fever (39-40 °C), sore throat, and dysphagia
- Drooling or difficulty handling secretions
- Very limited or no prodrome of mild upper respiratory tract infection (URI)
- "Hot potato" voice (muffled)
- Rapid onset of toxicity and respiratory distress
- Cough and hoarseness are late symptoms, if they occur at all.
- Time from onset of symptoms to presentation with progressive respiratory distress is generally <12 hours.
- Immunization against H. influenzae type B
- Child's preferred position or way of sitting (i.e., sitting upright, leaning forward with chin hyperextended)
Physical Exam
- Extremely anxious appearance
- Child prefers to remain sitting up.
- Child often leaning forward with chin hyperextended to maintain airway in a "tripod" position
- Slow and labored respiratory effort
- Drooling is seen as a manifestation of dysphagia.
- Inspiratory stridor, retractions, and late cyanosis
- Diagnosis can be suspected on history and observation of child's appearance alone.
- Do not attempt to examine the throat if epiglottitis is a serious consideration.
Diagnostic Tests & Interpretation
Lab
- Complete blood count: increased white blood cell count with left shift
- Cultures of blood and epiglottis (performed only in the operating room)
Imaging
Lateral neck radiography (should not be performed until airway team is in place): characteristic "thumb sign" of edematous epiglottis, with narrowing of the posterior airway and ballooning of the hypopharynx
Diagnostic Procedures/Other
- Definitive diagnosis requires direct visualization of erythematous and edematous epiglottis.
Alert
- Ensure appropriate airway management prior to any other interventions, including intrusive examination components, radiographs, and blood collection.
- A radiograph is indicated only when the diagnosis is in doubt and should not delay airway management.
Differential Diagnosis
- Viral laryngotracheobronchitis (croup) with or without secondary bacterial tracheitis
- Severe parainfluenza or influenza infection
- Uvulitis
- Peritonsillar, retropharyngeal, or lingual abscess
- Foreign body aspiration in a child with URI
- URI, including croup, in a child with a congenital or acquired airway problem (e.g., premature infant with subglottic stenosis, laryngeal web, vascular ring, tracheal stenosis)
- Hereditary angioedema (deficiency of complement C1 esterase inhibitor) can present with edema of the airway including the epiglottis.
- Diphtheria: rare in the United States
- Laryngeal infections
Treatment
Medication
First Line
- Empiric parenteral antibiotic coverage to include gram-positive cocci and H. influenzae (type B and nontypeable)
- Cephalosporins
- Cefotaxime: 200-225 mg/kg/24 h (max 12 g/24 h) divided q4-6h
- Ceftriaxone: 100 mg/kg/24 h (max 2 g/24 h) divided q12-24h
- Cefuroxime: 100-200 mg/kg/24 h (max 9 g/24 h) divided q6-8h
- Ampicillin/sulbactam: ampicillin 200 mg/kg/24 h (max 8 g/24 h) IV divided q6h
- Serious penicillin/cephalosporin allergies
- Levofloxacin (IV or PO): <5 years: 10 mg/kg/dose twice daily; >5 years: 10 mg/kg/dose once daily; max dose: 750 mg/24 h
- Duration of therapy: 7-10 days for all but staphylococcal disease (14-21 days).
- Switch may be made to oral medication after extubation and resumption of oral intake.
- Steroids are used commonly but without convincing evidence for their efficacy.
Second Line
- Discuss with infectious disease consultant.
- Chloramphenicol: 50-75 mg/kg/24 h (max 4 g/24 h) IV divided q6h; monitor levels
- Ampicillin: 200-400 mg/kg/24 h (max 12 g/24 h) IV divided q6h
- Penicillin: 200,000-300,000 U/kg/24 h (max 24 million U/24 h) IV divided q6h for streptococcal disease
- Oxacillin: 150-200 mg/kg/24 h (max 4 g/24 h) IV divided q4-6h for susceptible staphylococcal disease
Issues for Referral
Airway should be secured by clinician skilled in airway management (e.g., otolaryngologist, anesthesiologist) prior to any attempt to transport a child with suspected epiglottitis.
Inpatient Considerations
Initial Stabilization
- Airway management: Maintain child upright, never supine. Personnel experienced in airway management should accompany the child at all times, including during transport and in radiology.
- Rapid assembly of a team, which should include an anesthesiologist, an otolaryngologist, and a pediatrician, if possible
- Allow the child to assume his or her most comfortable position (usually in the parent's arms/lap).
- Oxygen by mask or blown by face
- Transport to operating room as soon as possible for anesthesia.
- Secure airway via direct laryngoscopy and bronchoscopy with intubation.
- Institute intravenous catheterization and blood collection and culturing of epiglottis only after the airway is secured.
- Perform emergent cricothyrotomy if obstruction occurs prior to controlled airway management.
- Use fluid resuscitation in cases of septic shock.
Admission Criteria
Admit all children with suspicion of epiglottitis for airway management.
Ongoing Care
Follow-up Recommendations
Patient Monitoring
- Extubation is usually possible within 24-48 hours. Criteria include decreased erythema and edema of the epiglottis on direct inspection and development of an air leak around the endotracheal tube.
- Defervescence is usually prompt after initiation of appropriate antimicrobial therapy.
Prognosis
- Mortality is estimated to be 5-10%.
Complications
- Without prompt medical intervention: complete airway obstruction leading to respiratory arrest, hypoxia, and death
- Necrotizing cervical fasciitis (rarely)
- Therapeutic complications
- Aspiration
- Endotracheal tube dislodgment and extubation
- Tracheal erosion or irritation
- Pneumomediastinum
- Pneumothorax
- Pulmonary edema
- Complications of H. influenzae type B bacteremia:
- Septic shock
- Pneumonia
- Cervical lymphadenopathy
- Rarely, arthritis, meningitis, and pericarditis
Additional Reading
- Guardiani E, Bliss M, Harley E. Supraglottitis in the era following widespread immunization against Haemophilus influenzae type B: evolving principles in diagnosis and management. Laryngoscope. 2010;120(11):2183-2188. [View Abstract]
- Rafei K, Lichenstein R. Airway infectious disease emergencies. Pediatr Clin North Am. 2006;53(2):215-242. [View Abstract]
- Shah RK, Roberson DW, Jones DT. Epiglottitis in the Hemophilus influenzae type B vaccine era: changing trends. Laryngoscope. 2004;114(3):557-560. [View Abstract]
- Shah RK, Stocks C. Epiglottitis in the United States: national trends, variances, prognosis, and management. Laryngoscope. 2010;120(6):1256-1262. [View Abstract]
- Stroud RH, Friedman NR. An update on inflammatory disorders of the pediatric airway: epiglottitis, croup, and tracheitis. Am J Otolaryngol. 2001;22(4):268-275. [View Abstract]
Codes
ICD09
- 464.30 Acute epiglottitis without mention of obstruction
- 464.31 Acute epiglottitis with obstruction
- 464.30 Acute epiglottitis without mention of obstruction
ICD10
- J05.10 Acute epiglottitis without obstruction
- J05.11 Acute epiglottitis with obstruction
SNOMED
- 29608009 Acute epiglottitis (disorder)
- 222008 Acute epiglottitis with obstruction (disorder)
- 70976000 Viral epiglottitis
- 49908003 Acute epiglottitis without obstruction (disorder)
FAQ
- Q: What is the incidence of epiglottitis since the introduction of conjugate vaccines against H. influenzae type B?
- A: Because H. influenzae type B caused 90% of epiglottitis and the incidence of all invasive disease due to H. influenzae type B has decreased by 99% in children <5 years of age, it is estimated that the incidence of epiglottitis has been reduced by more than 90%.
- Q: Have there been reports of epiglottitis caused by H. influenzae type B after complete vaccination?
- A: Yes. Several cases due to H. influenzae type B have been reported in the United States and abroad after partial and complete vaccination. Therefore, even a history of having received a full vaccination series does not eliminate the possibility of Hib-associated epiglottitis. In one case series from 2004, 5/6 patients with Hib epiglottitis had completed the Hib vaccination series.
- Q: Should a fully vaccinated child who develops invasive disease due to H. influenzae type B be tested for an underlying immunodeficiency?
- A: Probably. In one study, about 1/3 of children diagnosed with invasive disease due to H. influenzae type B were found to have a previously undiagnosed immunoglobulin deficiency.
- Q: Can epiglottitis recur?
- A: Yes, but rarely.
- Q: Are corticosteroids of any value in the management of epiglottitis?
- A: They are used commonly, but there is no evidence to support their benefit.