Epiglottitis
Epiglottitis
Since the introduction of the vaccine against H. influenzae, there has been a significant decrease in the number of children presenting with epiglottitis. Although there is little evidence that the disease has increased in adults, clearly the percentage of cases in adults compared to children has increased. Further, there is greater recognition of milder cases with direct visualization and imaging.
Early in my training, I was taught that adults could develop epiglottitis but that the size of their trachea made airway obstruction impossible. One very difficult intubation dealing with a very large epiglottis taught me a respect for this disorder.
Although adults with epiglottitis can develop airway obstruction, there are significant differences in the disease in adults compared to children. To paraphrase, adults are not just large children.
— Sandra M. Schneider, MD, Editor
Executive Summary
- Since the introduction of the H. influenzae vaccine, cases of epiglottitis are decreasing. Those children who get the disease represent vaccine failures (no vaccine offers 100% protection) or infection with other organisms, most often Streptococcus.
- Plain films of the neck are not very accurate in the diagnosis of epiglottitis. CT scans or direct visualization of the epiglottis is recommended.
- The primary concern in a patient with epiglottitis, regardless of age, is airway compromise. Adults with stridor are at particular risk. Those with respiratory distress or who appear toxic are at risk of airway compromise as well.
- As airway compromise can occur rather quickly, the patient should be admitted to an ICU with appropriate airway equipment, including that for a surgical airway, nearby. Treatment with antibiotics, either second- or third-generation cephalosporins or ampicillin with sulbactam, should be given.
Relevancy of the Problem to the Adult Population
Acute epiglottitis has traditionally been considered a pediatric disease. However, with the introduction of Haemophilus influenzae type b vaccination, the pediatric incidence has steadily decreased. Whether the incidence of acute epiglottitis is increasing or stable in adults is unclear. Acute epiglottitis results from edema and inflammation of the epiglottis and supraglottic structures, and requires urgent medical attention. It can rapidly progress to life-threatening airway obstruction.
Epidemiology
The symptoms of acute epiglottitis include fever, sore throat, dysphagia, drooling, stridor, muffled voice, hoarseness, and respiratory distress.1-9 (See Table 1.) The overall incidence of adult acute epiglottitis is 1 to 3/100,000 per year,1,7,10 and reported mortality rates vary widely from just under 1% to as high as 20% in some reviews.11 Acute epiglottitis has traditionally been considered a pediatric disease. However, with the introduction of Haemophilus influenzae type b vaccination, the pediatric incidence has steadily decreased and its incidence is either increasing1,7,10,12 or stable in adults.3,13 The average age of patients with adult epiglottitis is between 4214 and 473 years. Kass et al4 have noted an increase in cases occurring during the summer months, although other studies have not been able to confirm this.6,14 Male-to-female ratios of between 1.8:1 and 4:1 have been reported in the literature.3,15 In adults, epiglottitis has been associated with a number of comorbid conditions, including hypertension, diabetes mellitus, substance abuse, and immune deficiency.2,7,16-18
Etiology
Various microorganisms have been implicated in epiglottitis3,19-39 (See Table 1.) Isolated H. influenzae type b cases still appear in both children and adults, some of which constitute vaccine failure,8,40,41 but other bacteria such as streptococci have become the most common causative agents.10 In the majority of cases, however, no definite organism can be identified.3,42,43 A viral etiology has been postulated for some cases of adult epiglottitis, especially in milder cases.43 However, of the viruses, only herpes simplex has been positively identified by histology.34 Anaerobic organisms are major constituents of the microflora of the upper respiratory tract, but have rarely been reported as causing epiglottitis in adults.27
Table 1: Organisms Responsible for Epiglottitis in Adults
Bacterial Causes
- Bacteroides melaninogenicus
- Beta-hemolytic streptococcus
- Branhamella catarrhalis
- Citrobacter diversus
- Enterobacter cloacae
- Escherichia coli
- Haemophilus influenzae
- Haemophilus parainfluenzae
- Kingella kingae
- Klebsiella pneumoniae
- Moraxella catarrhalis
- Mycobacterium tuberculosis
- Neisseria spp
- Pasteurella multocida
- Pseudomonas aeruginosa
- Serratia marcescens
- Staphylococcus aureus
- Streptococcus milleri
- Streptococcus pneumoniae
- Streptococcus pyogenes
- Streptococcus viridans
- Vibrio vulnificus
Fungal Causes
- Aspergillus
- Candida albicans
- Histoplasma capsulatum
Viral Causes
- Herpes simplex
In immunocompromised hosts, epiglottitis may be caused by Pseudomonas aeruginosa and Candida species.44-46 A case report of epiglottitis caused by Histoplasma capsulatum has also been described in an adult receiving infliximab, prednisone, and azathioprine for Crohn’s disease.47
Noninfectious causes of epiglottitis should also be considered, including thermal injury, foreign body ingestion, and caustic ingestion.48-50
Pathophysiology
Acute epiglottitis in adults is a clinical condition requiring early diagnosis and treatment in order to avoid possible complications, which, although less frequent than that in the pre-antibiotic era or the pediatric population, may be serious and life-threatening.6,9,51 The course of the disease in adults differs in several ways from that in pediatric patients. It has been suggested that both the relative size of the glottic aperture and the difference in anatomic configuration of the epiglottis are protective in adults.52 Others have proposed that the comparative lack of reactive lymphoid tissue in the adult pharynx is another inherent protective factor.6 Epiglottitis takes on two distinct forms. The first is localized cellulitis without bacteremia, and the second is a more serious and fulminant systemic infection with bacteremia and distant seeding.53 Fulminant systemic infection and bacteremia are almost twice as common in pediatric patients (6090%) as in adult patients (2631%).6,54
Clinical Features
Severe cases of adult epiglottis are easily recognized, but a large number of less severe cases are initially misdiagnosed. In up to one-third of adult patients, epiglottitis is present but not diagnosed within 48 hours of admission.55 Adults with epiglottitis typically experience a prodrome resembling that of a benign upper respiratory infection. The duration of the prodrome is usually 12 days, but may be as long as 7 days and as short as several hours. The progression of symptoms is slower in adults than in children. In one case series of 106 patients, 65% presented within two days of symptom onset, and 9% presented more than one week later.16 Patients who have a rapid onset of disease and those with comorbid conditions are more likely to require airway intervention.1,2,56 Sore throat is the most common complaint, with 90100% of patients reporting a sore throat.16,57,58 (See Table 2.) In an analysis of 158 cases from the literature, Khilanani and Khatib described the symptoms of epiglottitis as including a severe sore throat in 100% of cases, painful dysphagia in 76%, fever in 88%, and shortness of breath in 78%.58 Other symptoms included anterior neck tenderness and hoarseness.
Table 2: Presenting Signs and Symptoms of Epiglottitis
in Adults
Signs or Symptoms |
Incidence (%) |
Sore throat |
90 to 100% |
Fever ≥ 37.5°C |
30 to 90% |
Muffled voice |
50 to 80% |
Dysphagia |
61 to 76% |
Drooling |
50 to 80% |
Hoarseness |
20 to 40% |
Pain with palpation of the larynx |
20 to 36% |
The signs of adult epiglottitis include lymphadenopathy, drooling, and respiratory distress.59 Frantz et al found muffling of the voice occurred in 54% of patients.3 Gentle palpation of the larynx is frequently extremely painful, which should immediately raise the suspicion of epiglottitis.60 These findings have been confirmed in other studies.59,61-63 Diagnosis in the emergency department or doctor’s office requires a high index of suspicion. The presence of a severe sore throat with the associated signs and symptoms listed above in an adult should immediately raise the possibility of the diagnosis.
Adult patients with mild epiglottitis can be expected to have a sore throat, absent or mild fever, hoarseness, and no stridor or respiratory distress. In comparison, severe cases present with fever, toxic appearance, drooling, dysphagia, aphonia, stridor, and respiratory distress. The combination of dyspnea and stridor is the most ominous finding, and usually leads to intubation or the requirement for a surgical airway. Airway compromise is less common in adults than in children. Ng et al reported artificial airway support in only 7 of 106 adults (6.6%).16 In a second study conducted by Solomon et al, 9 of 57 (16%) of patients required artificial airway support.57
Examination of the Epiglottis
Visualization of the epiglottis is the accepted standard for clinical diagnosis. Direct examination through fiber-optic nasoendoscopy of the oropharynx as an initial step in examination is generally safer in adults than in children, given the lower frequency of airway compromise when epiglottitis is present.57 Flexible fiber-optic nasoendoscopy is the preferred approach, as it provides direct, minimally invasive examination of the upper airway. Flexible nasoendoscopy makes it possible to examine the supraglottis to confirm the diagnosis, evaluate the extent of mucosal edema, and determine the degree of airway obstruction. Visualization reveals a swollen epiglottis and surrounding structures. The epiglottis may appear "cherry red," but is often pale and edematous.1,56 Repeated nasoendoscopic examinations during treatment allow precise monitoring of the resolution of supraglottic edema.64
In patients with respiratory distress, drooling, aphonia, or stridor, flexible nasoendoscopy is contraindicated. In cases of respiratory distress, direct laryngoscopy should be undertaken as part of a "double setup," with the ability to proceed immediately with a surgical airway as needed.2,56
Imaging
Normal soft-tissue plain films do not exclude mild to moderate adult epiglottitis.16,57 (See Figures 1 and 2.) Lateral neck films have been found to be extremely inaccurate for the diagnosis of epiglottitis in adults. In one retrospective review, the plain films were interpreted as positive in only 31% of patients with epiglottitis.65 James and Holland reported a 33% incidence of false-positive diagnosis for epiglottitis when normal lateral neck radiographs were presented to a group of five radiologists.66 Schumaker et al proposed that an epiglottic width of greater than 8 mm and an aryepiglottic width greater than 7 mm would accurately indicate epiglottitis.67 More recently, Rothrock et al suggested that an epiglottic-width-to-epiglottic-height ratio greater than 0.6, and epiglottic-width-to-C3-vertebral-body-width ratio greater than 0.5, or aryepiglottic-width-to-C3-vertebral-body-width ratio greater than 0.35 are predictors of epiglottitis.68
Figure 1: Normal Soft-tissue Lateral Radiograph
From the collection of Justin L. Weppner, DO
Figure 2: Soft-tissue Lateral Radiograph Demonstrating Epiglottitis
Image courtesy of J. Stephan Stapczynski, MD.
Diagnosis
In the majority of patients, a diagnosis of epiglottitis is based on clinical history and physical examination results, occasionally assisted by lateral neck radiography. In instances in which the patient has mild disease and a stable airway, CT is helpful. (See Figures 3 and 4.) Because the symptoms of epiglottitis can be nonspecific, CT can be useful in excluding conditions with symptoms similar to epiglottitis, such as peritonsillar abscess, abscesses of the deep neck space, lingual tonsillitis, laryngitis, or complications of the epiglottitis such as abscess formation.69,70 CT scanning should be performed in patients who have a stable airway, are not in acute distress, and are able to lie flat without difficulty.
Figure 3: Normal CT Scan of the Neck
From the collection of Justin L. Weppner, DO
Figure 4: CT Scan of the Neck Revealing Swelling Epiglottis and Aryepiglottic Folds with Significant Subglottic Narrowing
From the collection of Justin L. Weppner, DO
Differential Diagnosis
Epiglottitis should be considered in the differential diagnosis of patients thought to have other infectious processes such as mononucleosis, diphtheria, pertussis, lingual tonsillitis, and Ludwig’s angina, as well as those with possible retropharyngeal and peritonsillar infections.71 (See Table 3.) Conversely, noninfectious considerations, including allergic reactions,72 angioedema, foreign bodies, tumors or trauma of the larynx, laryngospasm, and inhalation and aspiration of toxic chemicals such as hydrocarbons,63 have all been misdiagnosed as epiglottitis. Morton and Barr reported a case of hyperventilation mimicking the signs of acute epiglottitis.73 Epiglottitis has been related to the use of crack cocaine; in these cases, it is thought that edema from thermal injury of the epiglottis results from the inhalation of small wads of metal used when smoking cocaine.74,75 (See Table 4.) Systemic diseases such as amyloidosis, sarcoidosis, pemphigus, pemphigoid, and Wegener’s granulomatosis should also be considered as possible causes of upper airway obstruction.76 (See Table 5.)
Table 3: Differential Diagnosis of Epiglottitis — Infectious Causes
Condition |
Characteristic Features |
Diphtheria |
Gradual onset of sore throat, malaise, low-grade fever, and an adherent pseudomembrane on the tonsils, pharynx, and/or nasal cavity |
Lingual tonsillitis |
The lingual tonsil is a collection of lymphoid tissue behind the foramen cecum on the dorsal posterior surface of the tongue. Symptoms may include pain and irritation of the throat, sticky sensation in the throat, dysphagia, cough, and muffled voice. Diagnosis may be made by transnasal fiber-optic visualization or CT scan in a patient with a stable airway. |
Ludwig’s angina |
A bilateral infection of the submandibular space that consists of the sublingual space and the submylohyoid space. The infection begins on the floor of the mouth and is an aggressive, rapidly spreading "woody" or brawny cellulitis involving the submandibular space. In general, there is no lymphatic involvement and no abscess formation. In those patients without respiratory compromise, computed tomography is the imaging modality of choice. |
Mononucleosis |
Characterized by a triad of fever, tonsillar pharyngitis, and lymphadenopathy. Fatigue and atypical lymphocytosis may also be present. Heterophile antibody testing may assist in differentiating mononucleosis from epiglottitis. |
Peritonsillar abscess |
Signs and symptoms include drooling, trismus, muffled voice, and unilateral tonsillar swelling with a deviation of the uvula. |
Pertussis |
Whooping cough is a highly contagious acute respiratory illness that manifests as a prolonged cough with one or more classic symptoms, including inspiratory whoop, paroxysmal cough, and post-tussive emesis. |
Retropharyngeal abscess |
Typical signs and symptoms include neck pain, fever, pain with swallowing, drooling, unwillingness to move neck, trismus, and midline or posterior swelling of the posterior pharyngeal wall. Neck radiographic films may reveal widening of the retropharyngeal space and reversal of the normal cervical spine curvature. |
Table 4: Differential Diagnosis of Epiglottitis — Noninfectious Causes
Condition |
Characteristic Features |
Allergic reaction |
Rapid onset of swelling of the lips, tongue, or airway without a prodromal illness. This may be associated with an urticarial rash. The patient may have a history of a previous attack. |
Angioedema |
Rapid onset of swelling of the lips, tongue, or airway without a prodromal illness. This may be associated with an urticarial rash. Dysphagia without hoarseness. The patient may have a history of a previous attack. |
Foreign body |
History of sudden onset of choking with hoarseness or stridor with laryngeal or upper esophageal foreign body. Neck radiographic films may reveal a radio-opaque foreign body. An upper esophageal foreign body may cause distortion or deviation of the extrathoracic trachea. |
Hyperventilation |
History of sudden onset of transient increase in minute ventilation out of proportion to metabolic needs without a prodromal illness that may result in dyspnea, light-headedness, paresthesias, chest pain, diaphoresis, and carpopedal spasm. |
Inhalation/aspiration of toxic chemicals |
History of an exposure to a toxic chemical. Lack of fever or prodromal illness. |
Laryngospasm |
Laryngospasm is an involuntary muscular contraction of the laryngeal cords. It is characterized by stridor. In some individuals, this can occur spontaneously or as a result of reflux or impaired swallowing. GERD is a common cause of spontaneous laryngospasm. The onset is sudden without a prodromal illness. |
Trauma of the larynx |
History of trauma to the larynx. Lack of fever or prodromal illness. |
Tumor |
Neck tumors can cause hoarseness, stridor, and dysphagia. Symptoms usually progress slowly and may be associated with chest pain, neck pain, fatigue, malaise, unexplained fever, or weight loss. |
Table 5: Differential Diagnosis of Epiglottitis — Systemic Disease Causes
Condition |
Characteristic Features |
Amyloidosis |
Tracheobronchial infiltration can cause hoarseness, stridor, airway obstruction, and dysphagia. The diagnosis of amyloidosis can be confirmed only by tissue biopsy, although the diagnosis may be suggested by history and clinical manifestations such as nephrotic syndrome in a patient with multiple myeloma or long-standing, active rheumatoid arthritis. |
Mucous membrane pemphigoid |
Mucous membrane pemphigoid is characterized by subepithelial blister formation on the mucous membranes. Typically presents as relapsing and remitting mucosal inflammation and erosions. In the oral cavity, the gingival and buccal mucosae are most commonly affected, but progressive laryngeal and tracheal involvement can result in respiratory distress and asphyxiation. |
Pemphigus |
Pemphigus is defined as a group of life-threatening blistering disorders characterized by acantholysis. The oral cavity is the most common site of mucosal lesions and often represents the initial site of disease. Since mucosal blisters erode quickly, oral erosions are often the only clinical findings. Swallowing may be difficult for patients with drooling, and there may be difficulty in controlling oral secretions in severe cases. |
Sarcoidosis |
Sarcoidosis is a multisystem granulomatous disorder of unknown etiology that affects individuals worldwide and is characterized pathologically by the presence of noncaseating granulomas in involved organs. Common presenting respiratory symptoms include cough, dyspnea, and chest pain and are sometimes accompanied by fatigue, malaise, fever, and weight loss. Bilateral hilar adenopathy on chest radiograph should raise suspicion for sarcoidosis. |
Wegener’s granulomatosis |
A form of systemic vasculitis with necrotizing granulomatous inflammation of the upper and lower respiratory tracts, systemic necrotizing vasculitis, and necrotizing glomerulonephritis. Tracheobronchial disease includes subglottic and lower tracheal and bronchial stenosis. Nonspecific complaints of fever, anorexia, weight loss, and malaise are often present. Diagnosis is based on clinical assessment, serologic testing, pulmonary function testing, chest imaging, bronchoscopy, and tissue biopsy. |
Management
Patients with mild signs of epiglottitis such as dysphagia and sore throat, and who are not in respiratory distress or who do not have stridor at rest, may have their airway observed; if they clinically deteriorate, they should immediately undergo intubation.1 A second option, which has lost a degree of support in the literature, is to immediately perform elective intubation in a controlled setting.6,7 Stridor in adults is a distinctive indication of upper airway obstruction, and is regarded as a warning sign for occlusion of the upper airway. In cases of epiglottitis with stridor, toxic appearance, or respiratory distress, the clinician is presented with a difficult airway and there is risk of complete occlusion of the airway. Whenever possible, intubation should be performed under controlled conditions with an anesthetist on standby to assist with intubation. In addition, all the personnel and equipment needed to perform a cricothyroidotomy or tracheostomy should be present. Awake intubation is the technique of choice, and an endotracheal tube with reduced diameter is often required.77
In addition to close monitoring and maintenance of airway patency, epiglottitis is treated with second- or third-generation cephalosporins or ampicillin with sulbactam. In those patients in whom MRSA is suspected, vancomycin or clindamycin may be added to the treatment regimen. The literature has not supported using steroids. Steroids do not reduce the period of intubation or the duration of hospital stay.1,7,78,79 All of the reported studies on steroid use had retrospective designs. Many studies had clinical selection bias due to steroids being administered preferentially to patients with more serious disease. Steroid use for treating epiglottitis will remain controversial until randomized, controlled studies can confirm its efficacy.
Additional Aspects
Potential complications include the following:
• Airway Obstruction.
• Epiglottic Abscess. Epiglottic abscess may result from coalescent epiglottic infection or secondary infection of an epiglottic mucocele.1,80 Epiglottic abscess occurs predominately in adults and may complicate as many as 30% of cases.1 Patients with epiglottic abscess have more severe symptoms and are at increased risk of airway compromised compared to those without epiglottic abscess.1,17,81,82 Epiglottic abscess can be diagnosed by direct visualization or computed tomography. Computed tomography should never be performed on patients with an unstable airway. Treatment of epiglottic abscess requires surgical drainage in addition to airway management and antibiotic therapy.
• Necrotizing Infection. Necrotizing infection is a rare complication of epiglottitis in patients with immunodeficiency.83,84
• Secondary Infection. Secondary infection such as abscess formation, cellulitis, cervical adenitis, meningitis, septic arthritis, and pneumonia may result from bacteremia or direct extension of infection.85,86
• Death. Mortality rates vary widely from just under 1% to as high as 20% in some reviews, and death is almost always due to acute airway obstruction.2,3,5,7,87,88
Potential pitfalls include the following:
• Failure to recognize respiratory compromise and manage the airway in a timely manner.
• In cases of airway compromise and stridor, avoid any unnecessary intervention or airway manipulation until the airway is secured. Do not send a patient with an unstable airway for radiographs.
• When attempting direct laryngoscopy, failure to have a "double setup" with the ability to proceed immediately to a surgical airway.
Disposition
Any patient with a suspected or confirmed diagnosis of epiglottitis should be admitted to an intensive care unit (ICU) setting for intravenous antibiotics and airway management precautions. ENT consultation should be obtained. Patients should not be discharged from the emergency department unless the diagnosis has been excluded by visualization of the supraglottic structures by a physician familiar with the physical appearance of the disease, and other concerning differential diagnoses have been excluded.
Summary
Epiglottitis is inflammation of the epiglottis and adjacent supraglottic structures. In adults, epiglottitis may be caused by a number of bacterial, viral, and fungal pathogens. Acute epiglottitis in adults can be a life-threatening medical condition that requires rapid evaluation and the early involvement of consultants. When available, flexible nasoendoscopy should be used to view the supraglottis and confirm the diagnosis. Soft-tissue radiographs of the lateral neck are not necessary to make a diagnosis of epiglottitis, but represent a reasonable choice in stable patients for whom there is a low clinical suspicion of epiglottitis, and may be indicated when other diagnostic considerations remain on the differential diagnosis. The clinician should be aware of false-negative rates for soft-tissue plain films. Normal soft-tissue plain films do not exclude mild to moderate adult epiglottitis. In addition to lateral neck radiography, CT may be used in diagnosing epiglottitis, and in excluding conditions with similar symptoms. CT scans should be performed in patients who have a stable airway, are not in acute distress, and are able to lie flat without difficulty. Patients with mild signs of epiglottitis may have their airway observed and should undergo immediate intubation if clinical deterioration is observed. If the airway deteriorates, intubation should be performed under controlled conditions with an anesthetist and ENT specialist on standby. Epiglottitis is treated with second- or third-generation cephalosporins or ampicillin with sulbactam. In those patients in whom MRSA is suspected, vancomycin or clindamycin may be added to the treatment regimen. Although steroid use in epiglottitis is common, its use will remain controversial until randomized controlled studies confirm its efficacy.
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