Epiglottitis is an acute inflammation in the supraglottic region of the oropharynx with inflammation of the epiglottis, vallecula, arytenoids, and aryepiglottic folds (see the image below).[1] Anecdotally, George Washington probably died of epiglottitis in 1799.
View Image | Soft-tissue lateral neck radiograph reveals edema of epiglottis consistent with acute epiglottitis. |
See also Pediatric Epiglottitis and Emergent Management of Pediatric Epiglottitis.
Airway management is the most urgent consideration, and patients should first be assessed for level of distress before any other workup. Adults generally present in a less acute fashion than children.[2] Ensure that an anesthesiologist and an otolaryngologist are available.
Radiographic evaluation for suspected epiglottitis is being replaced by direct visualization of the epiglottis using nasopharyngoscopy/laryngoscopy as the preferred method of diagnosis.
Signs and symptoms associated with a need for intubation include respiratory distress, airway compromise on examination, stridor, inability to swallow, drooling, sitting erect, and deterioration within 8-12 hours. Enlarged epiglottis (thumb sign) on radiographs is associated with airway obstruction. When in doubt, securing the airway is likely the safest approach.
Blood cultures may be taken, particularly if the patient is systemically unwell. The cultures are positive in approximately 25% of adult cases. If the airway is secure, epiglottic cultures may be performed.
Unstable patients
A patient in extremis requires immediate airway management. Patients may deteriorate precipitously, and airway equipment, including that for cricothyrotomy, should be present at the patient's bedside. Needle-jet insufflation (also known as percutaneous transtracheal jet ventilation [PTJV]) may also be considered to ventilate the patient temporarily.[3] Intubation or immediate formal tracheostomy or cricothyrotomy may be performed in the operating room if the case is less severe.
In cases of initial failure to intubate by direct laryngoscopy, PTJV may facilitate success in subsequent attempts at tracheal intubation by direct laryngoscopy.
Stable patients
Patients without signs of airway compromise, respiratory difficulty, stridor, or drooling, and who have only mild swelling on laryngoscopy, may be managed without immediate airway intervention by close monitoring in the intensive care unit (ICU). Because of the rapidity with which airway obstruction can occur in these patients, repeat serial evaluations of airway patency and maintenance of a low clinical threshold for airway placement are indicated.
In adults, the most common organisms that cause acute epiglottitis are Haemophilus influenzae (25%), followed by H parainfluenzae, Streptococcus pneumoniae, and group A streptococci. Less common infectious etiologies include other bacteria (eg, Staphylococcus aureus, mycobacteria, Bacteroides melaninogenicus, Enterobacter cloacae, Escherichia coli, Fusobacterium necrophorum, Klebsiella pneumoniae, Neisseria meningitidis, Pasteurella multocida), herpes simplex virus (HSV), other viruses, infectious mononucleosis, Candida (in immunocompromised patients), and Aspergillus (in immunocompromised patients).
Although community-acquired methicillin-resistant Staphylococcus aureus (CA-MRSA) is becoming an increasingly important pathogen; as of 2007, MRSA rarely causes epiglottitis.[4]
Noninfectious causes of epiglottitis are not uncommon and produce similar disease. Etiologies include thermal causes (including those associated with crack cocaine smoking and marijuana smoking, as well as throat burns affecting the epiglottis of bottle-fed infants), caustic insults (eg, automatic dishwasher soap ingestion), and foreign body ingestion (eg, following ingestion and expulsion of a bottle cap[5] ). Epiglottitis may also occur as a reaction to head and neck chemotherapy.[6]
A retrospective study by Suzuki et al indicated that risk factors for severe epiglottitis in adults includes older age, a body mass index of over 25.0 kg/m2, and the presence of diabetes mellitus, epiglottic cyst, or pneumonia, at admission. The study included 6072 patients with epiglottitis, including 9.4% with a severe form of the condition.[7]
Epiglottitis is classically associated with Haemophilus influenzae type b (Hib) infection and children. However, as has been observed with other infections caused by this agent, the overall incidence of epiglottitis has dramatically dropped in young children globally, as well as older age groups and adults, upon general adoption of Hib vaccine; furthermore, the most typical patient affected by epiglottitis in industrialized areas with vaccination programs is now an urban male in his mid 40s. Groups with higher morbidity include infants younger than 1 year and adults older than 85 years.[8, 9]
In United States, epiglottitis is an uncommon disease with an incidence in adults of about 1 case per 100,000 per year. Adult epiglottitis is most frequently a disease of men (male-to-female ratio, approximately 3:1), occurring during the fifth decade of life (average age, about 45 y). The ratio of incidence in children to adults was 2.6:1 in 1980 and dropped to 0.4:1 in 1993, a dramatic decrease in occurrence since the introduction of the Haemophilus influenzae type b vaccine (Hib). However, keep in mind that vaccine failures are possible.
Globally, epiglottitis is generally more common in nations that do not immunize against H influenzae type b. For example, in Sweden from 1987 to 1989, the incidence was 14.7 per 100,000 people per year in children aged 0-4 years and 3.2 per 100,000 people per year overall.[10] A large-scale Hib vaccination program in 1992-1993 resulted in a substantial decrease in Swedish cases of acute epiglottitis.
A retrospective review of a Danish population demonstrated a mean national incidence of epiglottitis in children of 4.9 cases per 100,000 per year in the decade before Hib vaccination. From 1996 to 2005, with the introduction of widespread Hib vaccination, an incidence of only 0.02 cases of epiglottitis per 100,000 per year was seen. During this period, the incidence of acute epiglottitis in adults remained constant, at 1.9 cases per 100,000 per year.[11]
A retrospective review from the tropical country of Singapore over 8 years, ending in 1999, demonstrated 32 cases of acute epiglottitis, only 1 of which occurred in a child.[12] During this time, Hib immunization was not routine, so Hib immunization cannot be used to explain the increased adult epiglottitis prevalence found in this study.
The prognosis in adults with acute epiglottitis is good with appropriate and timely treatment. Most patients can be extubated within several days. However, unrecognized epiglottitis may rapidly lead to airway compromise and resultant death.
In spite of acute epiglottitis generally having a good prognosis, the risk of death for persons is high due to sudden airway obstruction and difficulty intubating patients with extensive swelling of supraglottic structures. Reported cases do include sudden fatal cardiorespiratory arrest occurring in patients without previous evidence of respiratory obstruction while in an intensive care unit (ICU) setting, emphasizing the importance of providing close monitoring and adequate airway protection in these patients. The adult mortality rate is around 7%.
A retrospective study by Bellis et al of 11 adult cases of fatal acute epiglottitis reported the chief postmortem observations to include hyperemia and edema of the epiglottis and aryepiglottic folds.[13]
A retrospective study by Shapira Galitz et al indicated that in adult acute epiglottitis, patients with an aggressive disease course are more likely to be male, have dyspnea and stridor, present with edema of the epiglottis and aryepiglottic fold, and have an elevated C-reactive protein level, hyperglycemia, and a history of recurrent episodes.[14]
Complications of epiglottitis may include the following:
For patient education information, see the Cold & Flu Center as well as Epiglottitis.
The onset and progression of symptoms of epiglottitis is rapid (George Washington woke up with a sore throat and died the same night), although a less fulminant presentation is frequently demonstrated in adolescents and adults.
Historically, acute epiglottitis was most common in children aged 2-4 years. Since the introduction of the Hib vaccine and the accompanying dramatic reduction in H influenzae type b invasive disease incidence, epiglottitis has become rare in children. A comparison made between a large US children's hospital's chart review from 1995 to 2003 and a previous report from the same hospital completed 27 years earlier, showed a 10-fold decline in acute epiglottitis admissions, with streptococci becoming the major pathogens.[15] Epiglottitis incidence in adults has remained constant.
In a 2005 retrospective review of patients with acute epiglottitis, symptoms of stridor, voice muffling, rapid clinical course, and a history of diabetes mellitus were significantly associated with the need for airway intervention.[16] The following symptoms are also reported:
Physical findings of epiglottitis may include the following[17] :
Airway management is the most urgent consideration, and patients should first be assessed for level of distress before any other workup. Adults generally present in a less acute fashion than children.[2] Ensure that an anesthesiologist and an otolaryngologist are available. Patients may deteriorate precipitously, and airway equipment, including that for cricothyrotomy, should be present at the patient's bedside. Some authors have attempted to grade degrees of epiglottitis severity to guide treatment, and this is a practical approach.
Radiographic evaluation for suspected epiglottitis is being replaced by direct visualization of the epiglottis using nasopharyngoscopy/laryngoscopy as the preferred method of diagnosis. Only 79% of epiglottis cases are diagnosed by neck soft-tissue radiographs, underscoring the importance of direct visualization by fiberoptic endoscopy in obtaining a timely and accurate diagnosis.
A patient in extremis requires immediate airway management. Signs and symptoms associated with a need for intubation include respiratory distress, airway compromise on examination, stridor, inability to swallow, drooling, sitting erect, and deterioration within 8-12 hours. Enlarged epiglottis (thumb sign) on radiographs is associated with airway obstruction. When in doubt, securing the airway is likely the safest approach.
Intubation, or immediate formal tracheostomy or cricothyrotomy, may be performed in the operating room. Needle-jet insufflation (also known as percutaneous transtracheal jet ventilation [PTJV]) may be considered to ventilate the patient temporarily.[3]
In cases of initial failure to intubate by direct laryngoscopy, PTJV may facilitate success in subsequent attempts at tracheal intubation by direct laryngoscopy. PTJV can produce high intratracheal pressures that appear to lift up and open the glottis, with escape of the pressurized gasses causing the glottis edges to flutter, thereby allowing improved identification of the glottic aperture.
Patients without signs of airway compromise, respiratory difficulty, stridor, or drooling, and those who have only mild swelling on laryngoscopy, may be managed without immediate airway intervention by close monitoring in the intensive care unit (ICU). Because of the rapidity with which airway obstruction can occur in these patients, repeat serial evaluations of airway patency and maintenance of a low clinical threshold for airway placement are indicated.
Laryngoscopy is recommended before extubation. An ear, nose, and throat (ENT) specialist and an anesthesiologist should be immediately available at all times.
Direct visualization of the epiglottis using nasopharyngoscopy/laryngoscopy is the preferred method of diagnosis and is replacing radiographic evaluation for suspected epiglottitis. The airway should be secured or should be readily securable if endoscopy is performed.
Avoid radiography for patients who present in extremis until the airway is secure due to the danger of sudden obstruction.
Radiographs are generally unnecessary when the diagnosis can be made by history and physical examination alone or with nasopharyngoscopy. Only 79% of epiglottis cases are diagnosed using neck soft-tissue radiographs. Bedside ultrasonography is rapid, noninvasive, and accurate in the hands of a practitioner experienced in its use.[20]
Most adults are not in extremis and may safely undergo imaging. In evaluating stable patients with suspected epiglottitis, lateral neck soft-tissue radiographs are useful screening tools. Perform radiography with portable equipment, if indicated; this may confirm the diagnosis.
The classic lateral neck radiographic findings are a swollen epiglottis (ie, a thumb sign), thickened aryepiglottic folds, and obliteration of the vallecula (vallecula sign). See the image below.
View Image | Soft-tissue lateral neck radiograph reveals edema of epiglottis consistent with acute epiglottitis. |
The epiglottis is usually 3-5 mm thick; in a small, retrospective study of 30 patients with epiglottitis, using a criterion of 7-mm thickness provided 100% sensitivity and specificity for adult acute epiglottitis.[21] The same small retrospective study yielded 83% sensitivity and 100% specificity for an aryepiglottic fold width greater than 4.5 mm.[21]
Another useful tool in differentiating epiglottitis is to examine the vallecula (pre-epiglottic space). To locate the vallecula, use a soft-tissue lateral neck radiograph taken while the patient's mouth is closed. The vallecula is the air pocket found at the level of the hyoid bone just anterior to the epiglottis. The vallecula is normally well delineated, deep, and roughly perpendicular to the pharyngotracheal air column. The "vallecula sign of epiglottitis" is present when the normal deep linear air space from the tongue base almost to the epiglottis is made shallow or obliterated. Instead of a deep linear space, a V-shaped shallow space is seen.[22]
Avoid radiography until the patient's airway is secure. Obtain a chest radiograph (CXR) to assess endotracheal tube (ET) placement. The chest radiograph may reveal pneumonia.
Initial studies regarding the applicability of using bedside ultrasonography in the evaluation of the normal epiglottis found it to be both easy to perform and accurate.[23] More recently, Prasad et al suggested complementing a sublingual scanning approach with a transcutaneous approach.[24] Further analysis regarding usage of bedside ultrasonography in evaluating epiglottic disease and pathologic epiglottic enlargement may help determine the future clinical role of ultrasonography in the management of acute epiglottitis.
Blood cultures may be taken, particularly if the patient is systemically unwell. The cultures are positive in approximately 25% of adult cases. Given the relatively large number of infectious agents besides H influenzae type b and Streptococcus species that may cause adult epiglottitis, blood culture and sensitivities may be particularly helpful in this population.
In adults with acute epiglottitis, blood cultures for Haemophilus influenzae are positive in about 25% of cases. In remaining adult cases, H parainfluenzae, Streptococcus pneumoniae, and group A streptococci are frequently isolated from pharyngeal cultures.
If the airway is secure, epiglottic cultures may be performed. Some authors have described successful aspiration of epiglottic abscesses via spinal needle.[25]
Avoid agitating the patient with acute epiglottitis. Let the patient take a position in which he or she feels comfortable.
Orotracheal intubation may be required with little warning. Equipment for intubation, cricothyroidotomy, or needle-jet ventilation should be made available at the bedside.
Avoid therapy such as sedation, inhalers, or racemic epinephrine.
Administer supplemental humidified oxygen if possible, but do not force the patient, as the resultant agitation could worsen the condition.
Clinical pitfalls include the following:
In a retrospective study of 216 adult cases of acute epiglottitis, Nonoyama et al found that most cases received conservative treatment, with just 39 patients (18.1%) requiring airway management. The investigators also found that the mean number of days between symptom onset and hospital visit for patients differed between the airway management and conservative treatment groups (1.9 vs 2.9 days, respectively).[26]
Obstruction in acute epiglottitis can be reduced by using dexamethasone therapy or budesonide aerosols to treat pharyngeal edema. In addition, research suggests that length of stay in the intensive care unit (ICU) and in the hospital overall can be reduced with corticosteroid use.[27, 28]
See also Pediatric Epiglottitis and Emergent Management of Pediatric Epiglottitis.
Do not attempt intubation in the field unless acute airway obstruction is present. In the event of respiratory failure or obstruction, if emergency medical services (EMS) is unable to intubate, then cricothyroidotomy or needle-jet insufflation are the next lines of treatment.
Some authors have tried to grade degrees of epiglottitis severity to guide treatment.
A patient in extremis requires immediate airway management. Signs and symptoms associated with a need for intubation include respiratory distress, airway compromise on examination, stridor, inability to swallow, drooling, sitting erect, and deterioration within 8-12 hours. Enlarged epiglottis on radiographs is associated with airway obstruction. When in doubt, securing the airway is likely the safest approach.
Patients may deteriorate precipitously, and airway equipment, including that for cricothyrotomy, should be present at the patient's bedside. Needle-jet insufflation (also known as percutaneous transtracheal jet ventilation [PTJV]) may also be considered to ventilate the patient temporarily.[3] Intubation or immediate formal tracheostomy or cricothyrotomy may be performed in the operating room if the case is less severe.
In cases of initial failure to intubate by direct laryngoscopy, PTJV may facilitate success in subsequent attempts at tracheal intubation by direct laryngoscopy. PTJV can produce high intratracheal pressures that appear to lift up and open the glottis with escape of the pressurized gasses causing the glottis edges to flutter, thereby allowing improved identification of the glottic aperture.
Patients without signs of airway compromise, respiratory difficulty, stridor, or drooling, and who have only mild swelling on laryngoscopy, may be managed without immediate airway intervention by close monitoring in the intensive care unit (ICU). Because of the rapidity with which airway obstruction can occur in these patients, repeat serial evaluations of airway patency and maintenance of a low clinical threshold for airway placement are indicated.
Watch for air leaks around the endotracheal tube.
Laryngoscopy is recommended before extubation. An ear, nose, and throat (ENT) specialist and an anesthesiologist should be immediately available.
Epiglottic abscess may occur, which may or may not benefit from aspiration.[29]
Close contacts of patients in whom Haemophilus influenzae type b is isolated should receive rifampin prophylaxis (20 mg/kg; not to exceed 600 mg/d for 4 d).
Although H influenzae vaccine is available, it is not 100% effective.
Occurrence of recurrent episodes of acute epiglottitis in adults is unusual and, when present, warrants immune system investigation, because a quantitative or specific antibiotic deficiency may be present. Treatment of patients with recurrent acute epiglottitis may involve immunization or antibody replacement.
An anesthesiologist and an ear, nose, and throat (ENT) specialist or a general surgeon should be notified as soon as possible when epiglottitis is suspected. Early anesthesiologist and otolaryngologist consultation facilitates initial safe airway management, which is then followed by appropriate antibiotic treatment. An infectious disease subspecialist should be considered if the patient does not respond to empiric antibiotics.
Antibiotic therapy should begin after blood and epiglottic cultures have been obtained. Antipyretic agents may also be necessary. Racemic epinephrine, corticosteroids, and beta-agonists have not been proven to be helpful in epiglottitis. In addition, corticosteroid usage remains controversial, as anecdotal reports in the past had supported its use.
A study by Lee et al indicated that the treatment of epiglottic abscess with a combination of needle aspiration and antibiotics can reduce hospitalization time for patients below that for patients treated with antibiotics alone. However, the study also found that outcomes of the two treatments did not significantly differ, with significant symptom improvement achieved in both groups.[30]
A study from Beijing that looked at more than 8.5 million outpatient cases of acute upper respiratory tract infection in tertiary hospitals found that the highest antibiotic prescription rate (73.6%) was for acute tonsillitis, sinusitis, and epiglottitis. The overall antibiotic prescription rate was 39.0%.[31]
Clinical Context: Ceftriaxone is the antibiotic of choice (DOC) for epiglottitis. This agent is a third-generation cephalosporin with broad-spectrum activity against gram-negative organisms, lower efficacy against gram-positive organisms, and higher efficacy against resistant organisms. By binding to one or more penicillin-binding proteins, ceftriaxone arrests bacterial cell wall synthesis and bacterial growth.
Clinical Context: Ampicillin with sulbactam is the drug combination of a beta-lactamase inhibitor with ampicillin. This combination interferes with bacterial cell wall synthesis during active replication, causing bactericidal activity against susceptible organisms.
Ampicillin with sulbactam is used as an alternative to amoxicillin when the patient is unable to take medication orally. This combination covers skin, enteric flora, and anaerobes but is not ideal for nosocomial pathogens.
Clinical Context: Chloramphenicol is used if patients are allergic to penicillin and cephalosporins. This agent binds to the 50S bacterial-ribosomal subunits and inhibits bacterial growth by inhibiting protein synthesis. Chloramphenicol is effective against gram-negative and gram-positive bacteria.
Clinical Context: Cefuroxime is a second-generation cephalosporin antibiotic with activity against gram-positive and some gram-negative bacteria, including Haemophilus influenzae. Cefuroxime binds to penicillin-binding proteins and inhibits the final transpeptidation step of peptidoglycan synthesis, resulting in cell wall death.
Clinical Context: Cefotaxime is another third-generation cephalosporin antibiotic with broad-spectrum activity against gram-positive and gram-negative bacteria. This agent binds to penicillin-binding proteins and inhibits the final transpeptidation step of peptidoglycan synthesis, resulting in cell wall death.
Clinical Context: Clindamycin is a semisynthetic antibiotic produced by the 7(S)-chloro-substitution of the 7(R)-hydroxyl group of the parent compound lincomycin. This agent inhibits bacterial growth, possibly by blocking dissociation of peptidyl tRNA from ribosomes, causing RNA-dependent protein synthesis to arrest. Clindamycin widely distributes in the body, without penetration of the central nervous system. This agent is protein bound and excreted by the liver and kidneys.
Empiric coverage for Streptococcus pyogenes, Streptococcus pneumonia, Staphylococcus aureus, and H influenza should be provided (a third-generation cephalosporin or amoxicillin/clavulanic acid) in the management of epiglottitis. Third-generation cephalosporins are preferred as first-line agents because of increasing resistance to ampicillin.
Clinical Context: Aspirin blocks prostaglandin synthetase action, which, in turn, inhibits prostaglandin synthesis and prevents the formation of platelet-aggregating thromboxane A2. This agent acts on the hypothalamus heat-regulating center to reduce fever. The dissipation of heat is enhanced by vasodilation of the peripheral vessels, causing a decrease in body temperature.
Clinical Context: Acetaminophen is the drug of choice (DOC) for treating pain in patients with documented hypersensitivity to aspirin or nonsteroidal anti-inflammatory drugs (NSAIDs), those with upper gastrointestinal disease, or those who take oral anticoagulants. This agent reduces fever by a direct action on the hypothalamic heat-regulating centers, which increases dissipation of body heat via vasodilation and sweating.
Clinical Context: Ibuprofen is usually the drug of choice (DOC) for treating mild to moderate pain, if no contraindications exist. This agent inhibits inflammatory reactions and pain, probably by decreasing the activity of the cyclooxygenase enzyme, which inhibits prostaglandin synthesis. Ibuprofen is one of the few nonsteroidal anti-inflammatory drugs (NSAIDs) that is indicated for reduction of fever.
Analgesic-antipyretic agents are helpful in relieving the lethargy, malaise, and fever associated with epiglottitis.