Basics
Description
- Inflammation of the epiglottis and surrounding supraglottic region, which is potentially life threatening due to progressive airway
obstruction - Children are at greater risk of upper airway obstruction owing to:
- Decreased cross-sectional area of the upper airway (resistance is proportional to the inverse of the radius to the 4th power)
- Loose attachment of mucosal surface and increased vascularity of mucosa allows for edema
- Dynamic collapse of the airway
- A precipitous decline in the incidence of childhood epiglottitis since the introduction of the Haemophilus influenzae vaccination has occurred, although vaccine failure may result in rare cases among children who have been immunized
- In the post-Hib vaccine era, the mean age for this disease has increased, and it is now more commonly seen in adolescents and adults than in toddlers or young school-aged children.
- May occur throughout the year
All patients with suspected epiglottitis require intensive monitoring and intervention. Rapid progression of airway obstruction may occur.
Etiology
- Infection:
- H. influenzae type B
- Streptococcus pneumoniae
- Group A β-hemolytic
- Streptococcus
- Staphylococcus aureus
- Viruses
- Less common infections include Klebsiella, Pseudomonas, Candida
- Caustic
- Thermal
- Traumatic
- Post-transplant lymphoproliferative disorder
- Hereditary angioedema
Diagnosis
Signs and Symptoms
History
- Usually fulminant presentation without prodromal illness
- General:
- Irritability, throat pain (often described as patients worse sore throat), fever, noisy breathing
- Progressive toxicity and respiratory distress
- Adults have often been previously seen by a physician 1 or more times before diagnosis is made. Adults may present with the "worst sore throat of my life."
Physical Exam
- General:
- Toxic appearing
- High fever is typical.
- Rapid onset and progression
- Throat:
- Drooling
- Dysphagia
- Muffled "hot potato" voice
- Older patients often have very painful throat.
- Respiratory:
- Rapidly progressive respiratory distress (dyspnea in only 1/3 of adults)
- Children usually prefer to sit upright, leaning forward with open mouth ("tripod sniffing position") to maximize air entry.
- Subtle stridor that may progress to severe stridor (stridor in only 10% of adults)
- Complications:
- Airway obstruction is the most severe complication.
- Epiglottic abscess
- Associated pneumonia and atelectasis
Essential Workup
- Epiglottitis is a clinical diagnosis.
- Indirect laryngoscopy or any attempts to directly visualize the epiglottis are not indicated in children with suspected epiglottitis unless performed in a controlled environment. (In adolescents or adults, use of fiberoptic nasopharyngoscope may be indicated for patients without impending airway obstruction.)
- If infection is suspected, obtain cultures of the epiglottis during laryngoscopy after airway is secure.
Diagnosis Tests & Interpretation
Lab
- Avoid lab tests until airway is controlled.
- Throat cultures after control of airway
- Blood cultures after airway is secure:
- Often positive if H. influenzae is the pathogen
Imaging
- Radiographs of the soft tissue lateral neck:
- Usually not necessary to make the diagnosis
- Creates additional risk by delaying stabilization of the airway, promoting airway obstruction by agitating the patient, and often removing the child from the ED to an uncontrolled environment. Children should never go unaccompanied to radiology. Personnel and equipment to control airway must always be available.
- Variable findings:
- Normal
- Swelling of the epiglottis ("thumbprint sign") and often supraglottic region
- Ballooned hypopharynx
- Obliteration of vallecula
- EW/C3W (epiglottic width to 3rd cervical vertebral body width) ratio of >0.5
Diagnostic Procedures/Surgery
Laryngoscopy:
- In a controlled environment whenever possible
- Cultures of the epiglottis during laryngoscopy after the airway is secured may help identify pathogens and direct treatment.
- Epiglottis will appear swollen, inflamed, reddened.
Differential Diagnosis
- Other infectious processes:
- Bacterial tracheitis
- Retropharyngeal abscess
- Peritonsillar abscess
- Croup (laryngotracheobronchitis)-primarily in younger children, but there is a significant overlap in the ages of presentation.
- Pertussis
- Mononucleosis
- Ludwig angina
- Diphtheria
- Anaphylactic reaction with angioedema
- Hereditary angioedema
- Foreign body in upper airway
- Laryngeal trauma
- Laryngospasm
- Inhalation or aspiration of toxins (e.g., hydrocarbons)
- Airway burns (have been related to crack cocaine)
- Hyperventilation
- CNS disorders
Treatment
Pre-Hospital
Degree and mode of intervention must reflect degree of obstruction, time and means of transport, capability of care providers, etc. Consult and notify receiving hospital.
Initial Stabilization/Therapy
- Airway management if patient is in extremis
- Bag-valve-mask ventilation with 100% O2 with cricoid pressure often provides adequate ventilation and time to prepare for intubation and move to a controlled setting such as the operating room.
- Oral intubation:
- Use an endotracheal tube (ETT) size that is 1 or 2 sizes smaller than indicated by age or length.
- Direct compression of the anterior neck in the glottic region may help visualize air bubbles at the opening of the swollen glottis.
- Instruments used for difficult airways may be adjunctive devices.
- If oral intubation fails:
- Emergent cricothyrotomy or needle cricothyrotomy if age older than 10-12 yr
- Needle cricothyrotomy if age younger than 10-12 yr
Ed Treatment/Procedures
- 100% O2 as tolerated by patient
- Allow child to remain in position of comfort and do not force child to lie down, which may worsen airway obstruction.
- Although not proven, racemic epinephrine or L-epinephrine by nebulizer may temporize symptoms while plans for a definitive airway are rapidly arranged. It must be done with caution to avoid agitating the child.
- Avoid procedures that agitate the child such as IV access and blood draws.
- Empiric invasive airway management may be indicated:
- Patients with rapidly progressive respiratory difficulty, tachypnea, worsening throat pain, tachycardia, or hypoxemia
- Patients at high risk of acute obstruction (e.g., children with immunodeficiency disorders)
- Intubate in operating room or controlled environment by most skilled person.
- Use inhalational anesthesia before intubation.
- Have appropriate ETTs of various diameters of available to accommodate the inflamed supraglottic region.
- Surgical backup is required in case intubation is not possible; then emergency tracheotomy or cricothyrotomy can be performed.
- Equipment for intubation and for a surgical airway or needle cricothyrotomy must be available at the bedside.
- Administer IV antibiotics: 2nd- or 3rd-generation cephalosporins are active against β-lactamase-producing H. influenzae.
- Steroids are controversial but frequently administered, particularly in patients with chemical or thermal epiglottitis.
Medication
First Line
- Ampicillin/sulbactam: 200-300 mg/kg/24h q6h IV
- Cefotaxime: 150 mg/kg/24h q6-8h IV
- Ceftriaxone: 100 mg/kg/24h q12h IV
Second Line
- Ampicillin: 100-200 mg/kg/24h q6h IV given with chloramphenicol
- Chloramphenicol: 75-100 mg/kg/24h q6h IV
- Meropenem: 120 mg/kg/24h q8h max. dose 6 g/24h
- Decadron: 0.6 mg/kg/d (max. 10 mg) IV.
- Steroid use is controversial
- Epinephrine, racemic: 0.05 mL/kg (max. 0.5 mL) q30min in 2.5 mL normal saline (NS) via nebulizer
- L-epinephrine, 1:1,000: 0.5 mL/kg (max. 5 mL) q30min via nebulizer
- Rifampin for household contact prophylaxis: 20 mg/kg (max. 600 mg) daily for 4 days
- If hereditary angioedema is the suspected cause of epiglottitis, C1 esterase inhibitor concentrate (alternatively, if C1-INH is not available, consider fresh frozen plasma). Expert consultation recommended
Follow-Up
Disposition
Admission Criteria
Patients with suspected or proven epiglottitis should be admitted to ICU after stabilization of airway and administration of antibiotics and fluids.
Discharge Criteria
- Rifampin prophylaxis may be indicated for close contacts of H. influenzae epiglottitis. If the household has children younger than 12 mo of age or children who are unimmunized, incompletely immunized, or immunosuppressed, prophylaxis is indicated for nonpregnant household contacts. Child care center contacts should receive prophylaxis when 2 or more cases of Hib invasive disease have occurred within 60 days.
- All cases of invasive H. influenzae disease should be reported to the local or state public health department.
Issues for Referral
Critical care or pulmonary consult on all patients
Pearls and Pitfalls
True airway emergency. Patient must be monitored and accompanied at all times by someone with airway stabilization capabilities.
Additional Reading
- American Academy of Pediatrics. Red Book: 2012 Report of the Committee on Infectious Diseases. 29th ed. Elk Grove Village, IL: AAP; 2012.
- Faden H. The dramatic change in the epidemiology of pediatric epiglottitis. Pediatr Emerg Care. 2006;22:443-444.
- Grover C. Images in clinical medicine. "Thumb sign" of epiglottitis. N Engl J Med. 2011;365:447.
- Hopkins A, Lahiri
T, Salerno R, et al. Changing
epidemiology of life-threatening upper airway infections: The reemergence of bacterial tracheitis.
Pediatrics.
2006;118:1418-1421. - O'Bier A, Mu ħiz AE, Foster RL. Hereditary angioedema presenting as epiglottitis. Pediatr Emerg Care. 2005;21:27-30.
- Rafei K, Lichenstein R. Airway infectious disease emergencies. Pediatr Clin North Am. 2006;53:215-242.
- Shah RK, Roberson DW, Jones DT. Epiglottitis in the Hemophilus influenzae type B vaccine era: Changing trends. Laryngoscope. 2004;114:557-560.
See Also (Topic, Algorithm, Electronic Media Element)
- Bacterial Tracheitis
- Croup
- Epiglottitis, Adult
Codes
ICD9
- 464.3 Acute epiglottitis
- 464.30 Acute epiglottitis without mention of obstruction
- 464.31 Acute epiglottitis with obstruction
ICD10
- J05.1 Acute epiglottitis
- J05.10 Acute epiglottitis without obstruction
- J05.11 Acute epiglottitis with obstruction
SNOMED
- 232432003 Pediatric acute epiglottitis and supraglottitis (disorder)
- 222008 Acute epiglottitis with obstruction (disorder)
- 80384002 Epiglottitis (disorder)
- 58576005 Haemophilus influenzae epiglottitis
- 29608009 Acute epiglottitis (disorder)