Introduction
Background
Epiglottitis, also termed supraglottitis, is an inflammation of the epiglottis and/or the supraglottic tissues surrounding the epiglottis, including the aryepiglottic folds, arytenoid soft tissue, and, occasionally, the uvula.
As with many other aspects of the pediatric airway, the epiglottis is significantly different in the child than in the adult. In the infant, the epiglottis is located more anteriorly and superiorly than in the adult, and it is at a greater angle with the trachea. It is also more omega shaped and floppy than the more rigid, U-shaped epiglottis in the adult. Numerous causes of the inflammation exist, but the common problem is airway occlusion caused by the tissue swelling, and, when untreated, can lead to respiratory failure and death. In the young child, this can take place in hours.
Comparison of a normal pediatric airway and airway from a child who died from epiglottitis.
Pathophysiology
Epiglottitis was historically caused by infection of the supraglottic structures by Haemophilus influenza B (see Haemophilus Influenzae Infection). Since the widespread use of the HiB vaccine, the incidence and causative agents of epiglottitis have changed. Both H influenza type B (HiB) and Streptococcus pneumonia (see Causes) can colonize the pharynges of healthy children through respiratory transmission from intimate contact. These bacteria may penetrate the mucosa invading the bloodstream, causing bacteremia and seeding of the epiglottis and surrounding tissues. Bacteremia can also lead to infection of the meninges, skin, lungs, tears, and joints.
Bacterial infection of the epiglottis leads to acute onset of inflammatory edema, beginning on the lingual surface of the epiglottis where the submucosa is loosely attached. Swelling significantly reduces the airway aperture. Edema rapidly progresses to involve the aryepiglottic folds, the arytenoids, and the entire supraglottic larynx. The tightly bound epithelium on the vocal cords halts edema spread at this level. Aspiration of oropharyngeal secretions or mucus plugging can cause respiratory arrest.
Inflammation of any of the structures around the epiglottis may also become inflamed from trauma, mechanical, thermal, or chemical. Reports have been made of epiglottitis caused by blunt injury to the neck.1
Frequency
United States
The use of the HiB vaccine has reduced the incidence of epiglottitis. Introduction of the polysaccharide vaccine in 1985, followed by the highly effective conjugate vaccine, has dramatically reduced the incidence of epiglottitis, with consequent decline in hospital admissions. Studies show an annual incidence rate of 0.3 cases per 100,000 persons. Studies in children of all ages with epiglottitis report no seasonal variation in incidence.
International
International incidence varies widely and is significantly more prevalent in countries without universal immunization. In countries with mandatory immunization, the reported incidences are 0.9 cases per 100,000 persons in Sweden and 0.6 cases per 100,000 in the United Kingdom. Recent discussion exists that epiglottitis is increasing in frequency in the United Kingdom.2 The reason for this is unclear and may be due to giving 3 vaccines rather than 4. Recent studies suggest that bacterial tracheitis is now the most common serious airway infection in children.3,4
Mortality/Morbidity
Mortality rates as high as 10% can occur in children whose airways are not protected by endotracheal incubation. With endotracheal intubation, mortality is less than 1%.
Race
Most studies showed no racial predominance, although a recent study showed higher incidence among African Americans and Hispanics.
Sex
Most studies show a 60% male predominance. This has remained true even with the changing epidemiology of epiglottitis.
Age
Historically, epiglottitis occurred most commonly in children aged 2-7 years. However, it may occur at any age. Once believed to occur exclusively in children, adult cases have been reported for years and some evidence suggests the incidence in adults is increasing.
Clinical
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 a sore throat. The child may refuse to eat. As the disease progresses, the patient may not be able to maintain his or her 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. Bacterial tracheitis can mimic severe croup or epiglottitis.
- If the cause of epiglottitis is not infectious, the presentation may vary. A child presenting with upper airway respiratory distress without an obvious source or fever should be questioned regarding the possibility of ingestion of a toxic or hot liquid, or a traumatic event such as falling on an object with an open mouth or swallowing or having a foreign body removed.5
Physical
- In classic epiglottitis, the patient appears acutely ill, anxious, and usually assumes a characteristic tripod position if old enough to do so.
- Because of the pain in the supraglottic area, even secretions are not tolerated and the child is often drooling.
- Early on, the child may have stridorous respirations, but as the disease progresses, airway sounds may diminish. Additional signs of upper airway obstruction are also evident including suprasternal, subcostal, and intercostal retractions.
- In the older child, pain may be noted on movement of the hyoid bone.6
- Although not recommended in the child suspected to have epiglottitis, visualization of the epiglottis may show the classic swollen, cherry red epiglottis.
- In more advanced cases, the child is in respiratory failure and shock.
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
- Varicella can cause a primary or secondary infection often with group A beta-hemolytic streptococci.
- C albicans, especially in immunocompromised patients7
- Several viruses, including herpes species and parainfluenza
- Lymphoproliferative diseases may causes epiglottic swelling.
- Traumatic epiglottitis can occur from direct trauma and thermal injury.
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References
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Ng HL, Sin LM, Li MF, Que TL, Anandaciva S. Acute epiglottitis in adults: a retrospective review of 106 patients in Hong Kong. Emerg Med J. May 2008;25(5):253-5. [Medline].
Shah S, Sharieff GQ. Pediatric respiratory infections. Emerg Med Clin North Am. Nov 2007;25(4):961-79, vi. [Medline].
Sivakumar S, Latifi SQ. Varicella with stridor: think group A streptococcal epiglottitis. J Paediatr Child Health. Mar 2008;44(3):149-51. [Medline].
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Further Reading
Keywords
epiglottitis, epiglottis, Hib, supraglottitis, epiglottitis in children, Haemophilus influenzae type b, Hib vaccine, H influenzae, Streptococcus, Streptococcus pneumoniae, S pneumoniae, Klebsiella pneumoniae, K pneumoniae, Candida albicans, C albicans, Staphylococcus aureus, S aureus, Neisseria meningitidis, N meningitidis, Haemophilus parainfluenzae, H parainfluenzae, varicella zoster, herpes simplex type 1, parainfluenza
