Journal of Emergency Medicine
Volume 14 • Number 4 • July 1996
Copyright © 1996 W. B. Saunders Company
Epiglottitis in Adults
MARTIN J. CAREY MB BCh
Veterans Affairs Medical
Acute epiglottitis has become a disease of adults, probably as a result of immunization of children against Haemophilus influenzae. This article is a review of the literature on epiglottitis, including signs and symptoms, investigation, differential diagnosis, and treatment in the emergency department. The microbiology is discussed and the importance of prophylaxis in exposed persons is stressed.
Manuscript received June 23, 1995, returned July 11, 1995; revision received August 2, 1995, accepted August 11, 1995.
Address reprint requests to Dr Carey, Senior Fellow, Ambulatory Care, Seattle VA Medical Center (152), 1660 South Columbian Way, Seattle, WA 98144.
On the morning of December 14, 1799, George Washington awoke with a severe sore throat. He rapidly developed increasing stridor and hoarseness. He could not lie down, and was nursed sitting up. Despite the best treatment of the day, which included the letting of more than 2 liters of blood, he died at 11:30 that night.  His is probably the first recorded death from epiglottitis. Roland of Palma is reported to have described an epiglottic abscess in the 13th century.  Theisen  discussed three cases of epiglottitis in 1900, and reviewed the condition as known at that time. He states that Mainwaring first described the condition in 1791.
Early articles on epiglottitis described it as a disease of adults.  Epiglottitis was considered ``rare in children.''  However, from the 1950s onwards, epiglottitis was described more frequently in children, and from the `60s onwards, acute epiglottitis was thought to be a disease of childhood. The recent widespread use of Haemophilus vaccination has produced a dramatic decline in the number of children with epiglottitis,   and the disease is once again more common in adults. In 1980 the ratio of children to adults was 2.6 to 1; by 1993 it had declined to 0.4 to 1.  The mortality rate for children is now less than 1%, but the mortality in adults is in the range of 6% to 7%,  with series reporting between 0 and 20% mortality rates.    Part of this variation in mortality may result from delays in making the diagnosis; it is estimated that the first physician to see a patient with epiglottitis makes the diagnosis in only 35% to 70% of cases.     This review describes the signs and symptoms of the disease in adults, and discusses predisposing factors, microbiology, investigation, and treatment of epiglottitis in adults in the emergency department (ED).
The incidence of epiglottitis in adults is about 1/100,000 per annum.    The average age is between 42  and 47  years. Kass et al  have noted an increase in cases occurring during the summer months, though others have not been able to confirm this.   Male to female ratios of between 1.8:1 and 4:1 have been reported.  
SIGNS AND SYMPTOMS
Acute epiglottitis is a syndrome produced as a result of inflammation, usually caused by infection, which affects the epiglottis and surrounding structures. It is for this reason that some authors describe this condition as a ``supraglottitis.''  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%. Other symptoms included anterior neck tenderness and hoarseness. These findings have been confirmed in other studies.     The signs include lymphadenopathy, drooling, and respiratory distress.  Gentle palpation of the larynx is frequently extremely painful, which should immediately raise the suspicion of epiglottitis.  A recent study by Frantz et al  found that sore throat was the most common presenting complaint (95% of cases), with pain on swallowing present in 94% of cases. Muffling of the voice occurred in 54% of patients. Diagnosis of the condition in the ED or doctor's office requires a high index of suspicion. The presence of a severe sore throat in an adult, especially when accompanied by anterior neck discomfort or tenderness should immediately raise the possibility of the diagnosis.
In adults, in distinct contrast to the situation in children, the use of indirect laryngoscopy to visualize the epiglottis is considered a safe procedure.  When available, the flexible nasoendoscope has been described as the method of choice for investigation in the ED.  There is little to be gained from radiology of the soft tissues of the neck . Stankiewicz and Bowes  reported a sensitivity of 38% and a specificity of 76% for diagnosing epiglottitis from a radiograph of the lateral soft tissues of the neck.
A definitive diagnosis of epiglottitis can be made as soon as the epiglottis is visualized. The epiglottis and surrounding structures will be edematous and appear inflamed. Epiglottitis should be considered in the differential diagnosis of patients thought to have other infectious processes such as mononucleosis, diphtheria, pertussis, croup, and Ludwig's angina, as well as those with possible retropharyngeal and peritonsillar infections.  Conversely, allergic drug reactions,  angioneurotic edema, foreign bodies, tumors or trauma of the larynx, reflex laryngospasm, and inhalation and aspiration of toxic chemicals such as hydrocarbons  have all been misdiagnosed as epiglottitis. Systemic diseases such as amyloid, sarcoidosis, pemphigus, pemphigoid, and Wegener's granulomatosis should also be considered as possible causes of upper airway obstruction.  Morton and Barr  reported a case of hyperventilation mimicking the signs of acute epiglottitis. 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.
A variety of microorganisms have been implicated in epiglottitis                       (Table 1) . In the majority of cases, however, no definite organism can be identified.    The most common pathogens identified are Haemophilus influenzae and beta-hemolytic streptococci. It is not yet clear if the decrease in Haemophilus infections in children due to vaccination will have an effect on the incidence of Haemophilus infections in adults, too.  A viral etiology has been postulated for some cases of adult epiglottitis, especially the milder cases.  However, of the viruses, only herpes simplex has been positively identified on histology.  Anaerobic organisms are major constituents of the microflora of the upper respiratory tract. However, they have rarely been reported as causing epiglottitis in adults. 
TABLE 1 -- Organisms Responsible for Epiglottitis in Adults
This may be due to properties of the bacteria, for example, their generally poor invasive properties in the presence of normal tissues. However, it may be that infection with these organisms is more common than realized, and the apparent low rates of infection may be due to the difficulties associated with culturing these organisms. If anaerobic infection is suspected, cefoxitin or clindamycin are effective therapies.
The development of epiglottitis secondary to atypical organisms has been related to the presence of significant underlying disease. Acute fatal epiglottitis secondary to Aspergillus and Klebsiella infection has been described in relation to acute lymphocytic leukemia.  Candida epiglottitis was the presenting illness for a patient with acute myelogenous leukemia  and has been described in other patients immunocompromised as a result of chemotherapy.  A study by Farley et al  in Atlanta of invasive Haemophilus influenzae infections, which included two cases of epiglottitis in the series of 47 patients, noted that 92% of the patients had an underlying condition. These conditions included chronic obstructive airway disease, acquired immunodeficiency syndrome (AIDS),  alcoholism, steroid use, use of immunosuppressive drugs, history of splenectomy, and malignancy. Lederman et al  also report two patients with epiglottitis, one of whom had metastatic breast cancer, the other myeloma. Despite these series, the true prevalence of underlying disease in patients presenting with epiglottitis is unknown.
Patients with a diagnosis of epiglottitis who present with tachycardia, an increased white cell count, and a history of rapidly progressive sore throat and respiratory difficulty are at significantly increased risk of respiratory obstruction and need to be treated aggressively.  The presence of a tachycardia out of proportion to other symptoms is considered to be an early indicator of potential airway compromise and hypoxemia.  The treatment of adults without a rapidly progressive history and without significant respiratory compromise is controversial. Friedman et al  have described a staging system to help guide management. They describe patients with a respiratory rate of less than 20 breaths/min and with no respiratory distress as being in Stage I. These patients are managed conservatively with close observation in the intensive care unit. Patients with slight respiratory difficulty and/or a respiratory rate greater than 20 breaths/min (Stage II) are intubated in the operating room. If intubation is not possible, then an immediate formal tracheotomy or cricothyrotomy can be performed in the controlled surroundings of the operating room by experienced personnel. Patients with respiratory rates greater than 30 breaths/min, or with moderate to severe respiratory distress, or with a P co2 of >45 mm Hg, or with cyanosis are considered in Stages III or IV, and require immediate airway intervention. Other authors have reported that no one sign consistently identifies patients who require airway intervention, and that all patients need very careful observation. 
Antibiotics should be administered urgently. A second- or third-generation cephalosporin, active against ampicillin-resistant
Haemophilus influenzae, is the drug of choice.  A combination of ampicillin and chloramphenicol was previously recommended.  The proportion of Haemophilus influenzae resistant to ampicillin varies by institution and may be as high as 36%. 
A number of authors advise the use of steroids, but there are no controlled trials that prove any benefit from this therapy.   Rivron and Murray  describes the use of epinephrine in three cases of severe epiglottitis. Symptoms improved rapidly in two patients with marked stridor who were given intramuscular epinephrine. Epinephrine may ``buy time'' in severe cases while arrangements are being made for the patient to be intubated.
The use of inhalation anesthesia, the standard of care in children with epiglottitis, is more problematical in adults because of a prolonged excitation phase during induction. There is also the risk of aspiration of gastric contents, and of complete respiratory obstruction.   A rapid-sequence induction is recommended by some, with endotracheal intubation initially attempted, followed by cricothyrotomy if unsuccessful.  A proportion of adults with epiglottitis can be managed conservatively with very close observation in the intensive care unit, without intubation.   There are case reports of sudden respiratory compromise occurring, including cases in patients who appeared to be stable or even improving.    Equipment for immediate endotracheal intubation and cricothyrotomy should be available at the bedside of all patients with epiglottitis treated conservatively.
Emergency cricothyrotomy may be a lifesaving procedure in the ED for a patient with complete upper airway obstruction. The procedure can be performed rapidly by trained personnel,  but the emergency procedure is associated with a complication rate five times greater than that of elective surgical airway intervention.  The reported complication rates vary between 6% and 39%.   The immediate complications of the procedure include hemorrhage, mediastinal or subcutaneous emphysema, and tube misplacement.   Later complications of cricothyrotomy include vocal dysfunction and subglottic stenosis. The rate of significant subglottic stenosis is about 4%, while vocal dysfunction may occur in up to 15% of cases.  In view of the high rate of complications from cricothyrotomies, when compared with formal tracheotomies, there is considerable debate in the literature as to whether emergency cricothyrotomies should be converted to formal tracheotomies once the acute situation is under control. Emergency physicians should be guided by the standard practice within their own institutions.
In patients with AIDS, epiglottitis seems to run a more aggressive course than in those without AIDS.  These patients present in a similar fashion to patients who are not immunocompromised, but they need to be treated aggressively.
Occasionally patients who have had an upper airway obstruction such as that produced by epiglottitis and who then have that obstruction relieved by intubation will develop pulmonary edema.   The management of the condition, once recognized, should be to ensure adequate oxygenation with the use of mechanical ventilation and positive end-expiratory pressure (PEEP) if required. The condition resolves spontaneously. If the patient with epiglottitis does not respond rapidly to antibiotic therapy, the possibility of an epiglottic abscess should be considered. Rarely, epiglottitis may be recurrent.   In these cases the patient should definitely be evaluated for underlying disorders, such as collagen vascular disease, sarcoidosis, or occult malignancy.
The incidence of epiglottitis in children has declined markedly, probably as a result of Haemophilus vaccination. It is still unclear whether a similar decrease will occur in adults. The use of rifampin as a prophylaxis against infection in contacts of an index case is very important. There have been cases of transmission of Haemophilus infection from children to adults,  and vice versa,  resulting in epiglottitis and meningitis. In one case transmission of Haemophilus infection from a child with epiglottitis to the mother is thought to have occurred via the medium of a mask used by the child in hospital and then taken home.  The mother developed H influenzae epiglottitis. Rifampin, at a dose of 20 mg/kg/d, to a maximum of 600 mg daily for 4 days eradicates 86% of carrier states. 
Epiglottitis, long feared as a catastrophic disease of children, is now seen more commonly in adults. Prompt recognition of the condition will aid in reducing the mortality from this treatable disease. A high index of suspicion is required in any patient presenting to the ED with symptoms and signs that could be consistent with the diagnosis of epiglottitis.
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