AUTHOR AND EDITOR INFORMATION

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INTRODUCTION

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Background

The epiglottis is a leaf-shaped cartilaginous structure covered with a mucous membrane. It is located at the base of the tongue and functions to cover the larynx during swallowing.

Epiglottitis is an acute inflammation of the epiglottis and the structures surrounding it including the aryepiglottic folds and the arytenoid soft tissue. It can be a severe, life-threatening disease of the upper airway. Though historically a pediatric disease, recent epidemiology suggests that it is now mostly a disease that occurs in adults.

The spectrum of this disease has gone through significant changes since the introduction of the Haemophilus influenzae type b (Hib) vaccine in 1985. This disease had occurred most frequently in children aged 2-7 years and most commonly was caused by Hib.

Many other pathogens can cause epiglottitis including group A, B, and C Streptococcus; Streptococcus pneumoniae; Klebsiella pneumoniae; Candida albicans; Staphylococcus aureus; Haemophilus parainfluenzae; Neisseria meningitidis; varicella zoster; and several other viruses. Direct trauma and thermal injury also can cause inflammation of the epiglottis. The emergency physician is less likely to see this disease in its traditional presentation. Previously, the emergency physician quickly recognized this disease. With decreasing frequency and variable presentation, this may no longer be the case.

Pathophysiology

Epiglottitis is usually caused by infectious agents including H influenzae type b and group A S pneumoniae, H parainfluenzae, S aureus, and beta-hemolytic streptococci (group A, B, or C). Candida species have been found in patients who are immunocompromised. Viral infections including herpes simplex type 1 and parainfluenza have also been rarely documented.

Overwhelming infection leads to inflammation and edema of the epiglottis, aryepiglottic folds, and other adjacent tissues. Bacteria directly invade the mucous membrane of the epiglottis where the submucosa is loosely attached. The airway can be compromised due to the expanding edema, which can lead to respiratory distress and total airway obstruction.

Noninfectious causes including caustic burns and trauma have also been found to rarely cause epiglottitis. Children who ingest hot liquids may develop symptoms of epiglottitis. Children with scald burns to the face should be observed carefully for this complication. Other causes of an epiglottitislike presentation include caustic ingestions, foreign bodies, inhalation injuries, angioneurotic edema, sidestream exposure to crack cocaine, and burns from a crack cocaine pipe screen filter.

Frequency

United States

In 1985, the Hib vaccine was introduced in the United States, which dramatically decreased the incidence of epiglottis. In one study done at the Children's Hospital of Philadelphia, the frequency was 10.9 per 10,000 before 1990. After the vaccine was introduced, the frequency dropped to 1.8 per 10,000 admissions. In 2003, 2,013 cases of H influenzae were reported in all ages in the United States according to the MMWR 2003 survey. Of these cases, only 32 cases of serotype b infection were found in patients younger than 5 years. This includes all etiologies of Hib infections including epiglottitis.

Most cases arise during the years of 1-5, but the average age has increased since the introduction of the vaccine.

International

Prevalence varies widely by geographic location. A Swedish study demonstrated a substantial decrease in the incidence of epiglottitis (20.9 in 1987 to 0.9 in 1996 case per 100,000 per year) in patients younger than 5 years. One study done in Australia showed an increased ratio of adult cases compared with pediatric cases in the postvaccine era (84% vs 17%). In a study done in Israel, the annual incidence of acute epiglottitis per 100,000 adults significantly increased from 0.88 (1986-1990) to 2.1 (from 1991-1995) and to 3.1 (from 1996-2000).

Mortality/Morbidity

Reports of morbidity and mortality rates vary, depending on time to diagnosis, interventions employed, and use of an established protocol. In centers with pediatric expertise and defined protocols, the mortality rate approaches zero and the morbidity rate less than 4%. Delay in diagnosis is associated with a 9-18% mortality rate. Management of patients without intubation is associated with a 6% mortality rate.

Race

No racial predominance has been noted.

Sex

Most studies show a 60% male predominance. This has remained true even with the changing epidemiology of epiglottitis.

Age

Mean age is 36 months in the pediatric population but can range anywhere from 1 to 6 years. A recent study showed an increase in mean age of 5.8 years (1992-1997) to 11.6 (1998-2002).

CLINICAL

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History

Epiglottitis usually presents abruptly and rapidly with fever, sore throat, dysphagia, respiratory distress, drooling, and anxiety. The classic presentation is a young child who develops a fever and may complain of sore throat. The child may refuse to eat. As the disease progresses, patients may not be able to protect their airway and this may lead to airway obstruction.

• The clinical triad of drooling, dysphagia, and distress is the classic presentation. Fever with associated respiratory distress or air hunger occurs in most patients. Drooling occurs in up to 80% of cases.
• Age of patient, prodrome, type of cough, and degree of toxicity can all contribute to differentiation of epiglottitis from severe croup. Usually, croup occurs in younger children and has a viral prodrome. Most importantly, the child with croup has a barking cough and rarely appears toxic.

Physical

Patients tend to appear seriously ill and apprehensive. Characteristically, patients have a "hot potato" muffled voice and may have stridor. Usually children will assume the "sniffing position" with their nose pointed superiorly to maintain an adequate airway.

• Lymphadenopathy may be present.
• If severe hypoxia develops, cyanosis may appear as a late stage in disease progression and is an ominous sign.
• Cough is rare.

Causes

No one organism is predominant in causing epiglottitis.

• Historically, Hib was the predominant organism.
• • The Hib vaccine has decreased the number of cases due to infection with this organism.
  • Recent reports have shown that even vaccinated children can develop epiglottitis from H influenzae.
• S aureus
• S pneumoniae
• C albicans
• Several viruses
• Traumatic epiglottitis can occur from direct trauma and thermal injury.

DIFFERENTIALS

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Other Problems to be Considered

Pediatrics, bacterial tracheitis
Tracheitis

WORKUP

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Lab Studies

• Securing an airway is the overriding priority. All further evaluations should follow.
• Blood cultures and culture of the epiglottis should be performed only after the airway is secured.
• The white blood cell count is usually elevated anywhere from 12,000-45,000 cells/mm³ with bandemia.

Imaging Studies

• Before any imaging is performed, the airway of the patient should be secure. Because patients deteriorate quickly, bedside radiography is advised. Consultation with ear, nose, and throat (ENT) and anesthesia specialists should be done in order to decide on proper airway management.
• • Lateral neck radiographs may show an enlarged epiglottitis protruding from the anterior wall of the hypopharynx called the thumb sign.
  • Negative lateral radiographs do not rule out the diagnosis, especially in the early stages of presentation.

Other Tests

• Blood cultures and cultures of the epiglottis should be obtained after the airway is secured.
• • Blood cultures are positive in more than 80% of cases caused by H influenzae.
  • Epiglottic cultures are positive in 50% of cases caused by H influenzae.

Procedures

• Laryngoscopy is the best way to confirm the diagnosis, but it is not advised to attempt any procedures without securing an airway.
• Simply depressing the child's tongue with a tongue blade may visualize the epiglottis in some situations.

TREATMENT

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Prehospital Care

• Immediate transport to the nearest appropriate facility is necessary (emergency department approved for pediatrics [EDAP] or pediatric critical care center [PCCC]).
• Obtaining vital signs or any other diagnostic procedures are secondary to securing the airway.
• The child should be allowed to assume a position of comfort. The parent should be allowed to hold the child.
• Oxygen may be administered if it does not disturb the child.
• If the child has a respiratory arrest, first attempt ventilation with a bag-valve mask. Long, slow ventilations are best.
• Orotracheal intubation should be attempted if unable to ventilate the child. Needle cricothyroidotomy is used only if unable to secure an airway.
• If the child is to be transported to another facility, the airway should be secured. Only then should an IV line be placed. The child should be sedated and given antibiotics prior to transfer.

Emergency Department Care

The first priority is securing and providing respiratory support before a definitive airway is obtained. Initially humidified oxygen can be given by a nasal cannula or a nonrebreather mask as required. The patient should have respiratory and cardiac monitoring placed, and the patient should be kept in plain view of medical staff at all times.

• If respiratory arrest occurs, the patient should be ventilated using a bag-valve mask device and intubation should be performed. Nonblind, fiberoptic-assisted, nasotracheal intubation under controlled conditions is preferred. In a patient with respiratory distress, "rescue" airway equipment should be prepared prior to rapid sequence intubation. Anesthesia and ENT specialists should be notified of the airway risk, and a collective decision should be made regarding intervention.
• Orotracheal intubation or needle cricothyroidotomy may be necessary in emergent situations.
• Pediatric epiglottis is one of the few instances in which the emergency physician may need to rapidly perform needle cricothyrotomy.
• Most physicians are not aware that normal, volume-controlled, oxygen wall ports are incapable of delivering the pressure needed to adequately oxygenate an adult through a 14-g catheter (50 psi).
• In some trauma centers, needle jet setups specifically are preinstalled to address this concern, with a pressure-controlled port (instead of the volume-controlled port).
• For children, a setting of 1 psi/kg is recommended, although literature is lacking.
• Transtracheal jet insufflation does little for ventilation; however, it may salvage enough time in cases of complete inspiratory airway occlusion to perform tracheostomy or begin extracorporeal bypass maneuvers.
• The child's condition must be periodically evaluated in order to determine need for intubation. Minor procedures, such as intravenous access, may cause respiratory distress and can be performed more safely after intubation.
• Direct visualization of the epiglottis should not be performed unless staff members capable of securing an airway are present.
• Tracheostomy should be reserved for patients in which endotracheal intubation is unsuccessful due to severe laryngeal edema.
• Currently, no controlled studies exist on the use of IV steroids for reduction of airway structure edema due to pediatric epiglottitis. Nonetheless, some clinicians routinely use them in cases with adults.
• Despite evidence of an increased morbidity and mortality in patients treated without intubation, reports of children managed on an observation basis have been reported. Some reports have been made of cases managed on an outpatient basis. Patients managed successfully with observation were generally older and able to tolerate their secretions. This approach should be used with caution.
• Currently, the cause of infection more likely will be S aureus or group A streptococci as opposed to Hib due to vaccine use, and therapy should be directed as such.
• Empiric therapy should cover all organisms mentioned above.

Consultations

• ENT and anesthesia specialists should be consulted regarding airway management, and they should be present during any intervention attempt.
• As the child should be admitted to an intensive care unit, the intensivist must be consulted.

MEDICATION

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Antibiotic therapy is necessary but should be initiated after securing the airway. Prior to culture results, use antibiotics covering the most likely organisms. Following trauma to the epiglottis, S aureus should be suspected. With the presence of white patches, C albicans should be suspected. Sedation for comfort also is required.

Drug Category: Antibiotics

Empiric antimicrobial therapy must cover all likely pathogens in the context of the clinical setting. Treatment should continue for 7-10 d in general.

FOLLOW-UP

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Further Inpatient Care

• The patient should be admitted to the pediatric intensive care unit and if intubated, should be sedated and/or paralyzed. Laryngoscopy should be repeated 24-48 hours after treatment to evaluate the degree of inflammation, and a decision should be made when to consider extubation. Criteria for extubation include air leaks around the endotracheal tube or decreased edema and erythema of the epiglottis. After observation of 24-48 hours postextubation, the patient can be discharged on oral antibiotics.

Transfer

• If the hospital is unable to care for critically ill children, transfer should be arranged to the nearest appropriate facility, which, ideally, would be a hospital with a pediatric intensive care unit.
• Transport of patients with epiglottitis may be a concern, especially for patients who are maintaining an airway in the emergency department but could lose airway protection during transport. A survey done at the 1990 Pediatric Critical Care Transport Leadership Conference showed that 49% of physicians recommend intubation prior to interhospital transfer. The other 49% made decisions on a patient-to-patient basis. Therefore, the physician should use clinical judgment in making this decision.

Deterrence/Prevention

• As mentioned earlier, the Hib vaccine has dramatically reduced the incidence of epiglottitis. This vaccine is recommended for all children. Epiglottitis can still occur in children who are completely vaccinated.
• According to the Red Book 2003 of the American Academy of Pediatrics, rifampin prophylaxis (20 mg/kg per dose over 1 mo of age; maximum of 600 mg) for 4 days should be given to all household contacts if any of the following exist:
• • One or more child in the household younger than 4 years who has not received his or her age-appropriate number of doses of Hib vaccine
  • One or more child younger than 12 months who has not received the primary series of Hib vaccine
  • An immunocompromised child, regardless of the vaccination history
• Children older than 2 years with epiglottitis do not need vaccination because the disease provides immune protection.

Complications

• During the bacteremic phase of the disease, other foci of infection are possible. Pneumonia is the most commonly cited associated illness, with otitis media being the second. Meningitis has been reported in association with epiglottitis.
• As with other causes of upper airway obstruction, pulmonary edema can be observed after the airway has been secured. Accidental extubation and respiratory arrest are the 2 most common complications.
• Accidental extubation can cause additional complications.
• Cervical adenitis, tonsillitis, and otitis media have also been documented.
• Specifically with Hib, meningitis, septic shock, cellulitis, and septic arthritis can be seen.

Prognosis

• Once the airway has been secured, prognosis is excellent with mortality rate falling below 1%.

MISCELLANEOUS

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Medical/Legal Pitfalls

• If epiglottitis is suspected, immediate assembly of a team capable of securing a protected airway is the utmost priority.
• Never leave the child unaccompanied.
• No diagnostic tests are required before taking the child to the operating room.
• Direct visualization of the epiglottis in the ED is unwise, although, in reality, no cases of laryngospasm due to such visualization have ever been reported in the literature.
• As the disease becomes more rare, its existence and its quick progression may be forgotten.
• A written protocol should be available for a child presenting with possible epiglottitis.

Special Concerns

• Laryngoscopy is the best way to confirm the diagnosis, but it is not advised to attempt any procedures without securing the airway. Simply depressing the child's tongue with a tongue blade may visualize the epiglottitis in some situations. Some concern exists regarding the safety of such procedures, which can provoke anxiety and increased respiratory effort during examination leading to airway obstruction.
• A study performed in Germany recommended laryngoscopy to aid in the diagnosis in patients with atypical presentations or with crouplike coughs. It also showed that fiberoptic endoscopy is especially useful in cooperative older children with moderate respiratory distress.

MULTIMEDIA

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Media file 1:  Lateral neck radiograph. Notice the hypopharyngeal dilatation, the swollen epiglottis, and the lack of definable aryepiglottic folds. Image courtesy of Dr Mark Silverberg at Kings County Hospital.
	View Full Size Image
Media type:  X-RAY

REFERENCES

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• Wenger JK. Supraglottitis and group A Streptococcus. Pediatr Infect Dis J. Oct 1997;16(10):1005-7. [Medline].
• Wood N, Menzies R, McIntyre P. Epiglottitis in Sydney before and after the introduction of vaccination against Haemophilus influenzae type b disease. Intern Med J. Sep 2005;35(9):530-5. [Medline].
• Wurtele P. Acute epiglottitis in children and adults: a large-scale incidence study. Otolaryngol Head Neck Surg. Dec 1990;103(6):902-8. [Medline].
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Article Last Updated: Apr 13, 2006