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Adult epiglottitis
TheJournalof EmergencyMedune, Vol. 7. pp. 223-231, 1989
Printed in the USA ??Copyright 0 1989 Pergamon Press plc
ADULT EPIGLOTTITIS Phil B. Fontanarosa,
MD, FACEP,
S. Scott Polsky,
MD, FACEP,
George E. Goldman,
MD, FACEP
Akron City Hospital, Northeastern Ohio Universities College of Medicine, Akron, Ohio Reprin,ntaddress: Phil B. Fontanarosa, MD, FACEP, Department of Emergency Medicine, Akron City Hospital, 525 East Market Street, Akron, Ohio 44309
?? Abstract-Adult epiglottitis (or “supraglottitis”) is an uncommon but increasingly recognized entity. Though prior studies emphasized the fulminant nature of the disease, recent evidence suggests that epiglottitis in adults may follow a relatively less severe clinical course, especially if Hemophilus influenza is not isolated. The records of 28 patients with adult epiglottitis were retrospectively analyzed to characterize the presenting features and clinical course of the disease. The diagnosis was established by laryngoscopy, lateral cervical radiographs, or both. Laryngoscopy did not precipitate airway obstruction in any patient. The majority of patients experienced a relatively benign clinical course and improved with medical management that consisted of ICU admission, intravenous antibiotics, hydration, inhaled mist, and corticosteriods. Only two patients (7%) required airway support with orotracheal intubation because of respiratory difficulty. There were no instances of respiratory arrest or airway obstruction. No tracheostomies were performed, and there were no deaths. It was concluded that adult epiglottitis can follow a less severe course than classically described. 0 Keywords -epiglottitis,
more benign clinical course than pediatric epiglottitis (13,14), and the majority of cases have no definable pathogen (13,14,15,16,17). It was the presentation of six cases of adult epiglottitis at our institution over a recent 8-month period that prompted this review. A retrospective study was undertaken to evaluate the clinical presentation and to characterize the clinical course of adults with epiglottitis. METHODS Akron City Hospital is a 540-bed acute care hospital with an annual emergency department (ED) census of 60,000 adult patients. The medical records of all adult patients treated at Akron City Hospital between January, 1982, and July, 1988, with a primary or secondary final diagnosis of acute epiglottitis (or supraglottitis) were obtained. The emergency department chart and hospital records were retrospectively reviewed and analyzed for presenting symptoms, physical findings, diagnostic procedures, radiologic findings, laboratory data, treatment, clinical course, complications, and outcome. The diagnosis of epiglottitis was established in all cases by direct laryngoscopic findings or lateral cervical soft tissue radiographs that demonstrated an edematous epiglottis. The original prospective interpretation of the lateral neck films was performed by an attending radiologist. The radiographs were then reviewed by the investigators without knowledge of the initial radiologic impression. The films were retrospectively interpreted as
in adults; supraglottitis
INTRODUCTION Acute epiglottitis is a potentially life-threatening infectious disease that can cause sudden, unpredictable, upper airway obstruction (l-4). While most commonly seen in children, acute epiglottitis or “supraglottitis” in adults is now recognized with increasing frequency (2,3,5,6,7,8). Although previous studies have emphasized the fulminant nature of the disorder (9,10,11,12), recent evidence suggests that adult epiglottitis may follow a =
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Phil B. Fontanarosa, S. Scott Polsky, George E. Goldman
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positive or negative for epiglottitis, and measurements of the width of the epiglottis at its widest point were made. Films demonstrating an epiglottic width greater than 8 mm (8) were considered positive.
RESULTS Our review identified 28 cases of adult epiglottitis that had presented during the study period. There were 18 females and 10 males. Patients ranged in age from 18 to 70 years, with a mean age of 39.5 years. No medical diseases predisposing to epiglottitis were found. Historical factors and presenting physical findings are summarized in Table 1. The mean length of time from onset of symptoms to emergency department presentation was 2.6 days, range 0 to 5 days. Thirtynine percent (1 l/28) of patients presented within 24 hours of symptoms, while 32% (9128) patients were not seen until 4 days after initial symptoms occurred. Twelve patients (43 %) had been seen by a physician prior to ED evaluation and admission and had been placed on antibiotics for treatment of pharyngitis (11 cases) and bronchitis (1 case). Of the nine patients with delayed ED presentation- over 4 days after symptom onset -7 were on a prescribed antibiotic regimen. All patients complained of sore throat and dysphagia. Other common symptoms included voice change (64%) and shortness of breath (21%). The most common physical findings were anterior cervical adenopathy (79%), excess salivation and secretions or some degree of drooling (29%), and “mild” respiratory distress (21%). The absence of stridor was noted in all cases. Other specific indicators of respiratory distress, such as retractions and use of accessory muscles, were not documented. The mean temperature was 38.O”C. (range 37.039.5). Sixteen patients (57%) had temperatures in excess of 37.8”C. The mean respiratory rate was 20.8/ min with a range of 12 to 32/min. Tachypnea (respiratory rate > 20) was noted in 11 patients. The pulse rate ranged from 72 to 140 beats/min, mean 102 beats/ min, with tachycardia (> 100 beats/min) present in 15 patients (54%). Leukocyte counts ranged from 6,300 to 31,000 cells/mm3 with a mean of 14,700 cells/mm’. Seventyfive percent of patients (21/28) had white cell counts over 13,000 cells/mms, and 5 patients (18%) had counts under 10,000 cells/mm3. Blood cultures were obtained in 24 patients with 2 positive results (8%): one for Hemophilus influenza type b and one for Hemophilus parainfluenza. Throat cultures were performed in 13 patients with
two positive for non-group-A beta streptococci. Both patients with bacteremia had negative throat cultures. No viral studies were obtained. Laryngoscopic visualization of the epiglottis was performed in 18 patients (64%): 14 by indirect laryngoscopy, 2 by direct laryngoscopy with a fiberoptic laryngoscope, and 2 by direct laryngoscopy during endotracheal intubation. Laryngoscopy was performed by an otolaryngologist (8 cases), emergency physician (7 cases), anesthesiologist (2 cases), and intensivist (1 case). Laryngoscopy did not precipitate laryngospasm in any patient. The epiglottis appeared swollen and erythematous in each case. Documentation of supraglottic involvement varied, but it was specifically noted in 9 patients. All 28 patients had standard lateral cervical soft tissue x-ray studies performed. The films of 25 patients were interpreted by the staff radiologist as being consistent with epiglottitis, a sensitivity of 89%. The films of one patient were considered abnormal, with “anterior soft tissue swelling,” but the characteristic findings of epiglottitis were not specified. In two other patients, the radiologist interpreted the films as normal. In each of these three cases, laryngoscopy was performed and revealed epiglottitis. The soft tissue x-ray studies of 24 of 28 patients were available for independent retrospective analysis. Of the 24 radiographs reviewed, 22 were considered positive and demonstrated soft tissue swelling characteristic of acute epiglottitis, a sensitivity of 92%. The mean epiglottic width in this group was 14 mmh3.0 mm (SD), range 10 to 22 mm. The radiographs of two patients were considered normal by retrospective analysis, with a mean epiglottic width of 7.0 mm+1.4mm(SD),range6to8mm. The retrospective analysis was in agreement with the radiologists’s prospective interpretation in 23 of 24 cases. The x-ray studies considered positive for epiglottitis by retrospective review had also been considered positive by the radiologist. The films that had been interpreted as normal by the radiologist were also considered normal, or negative for epiglottitis, upon retrospective analysis. Review of the radiograph that had been considered abnormal but not characteristic of epiglottitis by the radiologist revealed swelling of the anterior supraglottic soft tissues and an epiglottic width of 12 mm, and was considered consistent with epiglottitis. All patients had been admitted to either the intensive care unit or a closely monitored medical unit, where they received mist oxygen, hydration, and intravenous antibiotics. Antibiotics used included cefoperazone (19), ceftriaxone (3), cefoxitin (l), cefamandole (l), ceftazidime (l), ampicillin plus sulbactam (l), nafcillin and cefaperazone (l), and timentin and
Adult Epiglottitis
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Table 1. Symptoms and Signs in 28 Patients with Adult Supraglottitls
Historical Factors Sore throat Dysphagia Voice change Dyspnea Duration < 24 hrs 24-72 hrs > 72 hrs Taking antibiotics Physical Findings Temperature <37.8X 37.8O-39.0% >39.0% Cervical adenopathy Drooling Respiratory distress Stridor
No.
Percent
28 28 18 6
100 100 64 21
11 8 9 12
39 29 32 43
12 13 3 22 8 6 0
43 46 11 79 29 21 0
cefoxitin (1). Sixteen patients (57%) had received intravenous steroids, but specific indications for steroid administration could not be determined. Twenty-six patients (93 W) were considered to have had a “mild” clinical course, defined as clinical improvement after admission and no need for airway support. Two patients had required respiratory assistance and had been defined as having a “severe” clinical course. Both patients needing establishment of an artificial airway had been orotracheally intubated in the operating room and both remained intubated for 48 hours. One patient was intubated 6 hours after admission because of respiratory distress and subsequently had blood cultures positive for Hemophilus parainj7uenza. The second patient was intubated 24 hours after admission because of increasing respiratory difficulty, but had negative blood cultures. Both patients requiring intubation had presented within 24 hours of onset of symptoms. There was no apparent relationship between the need for airway support and other historical factors, vital signs, physical findings, presence of drooling, or leukocyte count. All patients improved during hospitalization, with the majority of patients recovering completely in 3 to 5 days. The length of hospital stay ranged from 3 to 7 days with a mean of 4.5 days. Both patients with positive blood cultures were hospitalized for 6 days. The nonbacteremic patient requiring intubation was hospitalized for 5 days. Steroid administration did not appear to benefit or worsen the clinical course. Except for the two patients requiring intubation, there were few complications. One patient had an uneventful hospital course, showed clinical improve-
ment and was discharged, but had relapse of symptoms within 48 hours and required readmission. He was successfully treated with intravenous antibiotics, steroids, hydration, inhaled mist, oxygen, and observation; had an uncomplicated additional 3-day hospital course; and was considered a single episode of epiglottitis. Another patient developed mild, self-limited antibiotic-induced diarrhea. There were no cases of frank airway obstruction or respiratory arrest. No tracheostomies were performed and there were no deaths.
DISCUSSION Epiglottitis in adults occurs regularly, though uncommonly, in clinical practice (16). Although prior studies (7,11,18) have emphasized the high incidence of airway obstruction and the associated mortality of the disease, recent reports (13,14,16) suggest that adult epiglottitis may frequently follow a “less pernicious” clinical course than classically described. The findings in the present study, designed to evaluate the presenting features and characterize the clinical course of adults with epiglottitis, also demonstrate a relatively benign clinical course for the majority of patients. In 28 cases of adult epiglottitis, no deaths occurred and only two patients required airway support.
Adult Epiglottitis in Perspective It is speculated that the death of George Washington in 1796 represented the earliest recorded case of epiglottitis (19). However, the report of an adult with H influenza septicemia and fatal airway obstruction by LeMierre and coworkers (20) in 1936 is recognized as the first published case of adult epiglottitis. Prior to 1940, when Brown (21) reported the first series of cases of the disease, epiglottitis had been primarily considered an adult illness (7). During the 195Os, epiglottitis as a disease entity in children became widely publicized, while epiglottitis in adults was seldom mentioned (7). Consequently, by 1960, there were less than 40 reported cases of adult epiglottitis (22). In more recent years, epiglottitis in adults has been increasingly discussed in the medical literature. By 1977, there were over 100 cases documented (12). In 1985, Stair and Hirsch (22) reviewed all previous reports of adult epiglottitis and tabulated 389 cases. From 1986 to 1988, seven additional series (13, 14,16,17,23-25) including 168 additional patients have been published, bringing the total number of
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cases of adult epiglottitis to over 500. The present study adds 28 patients. A possible explanation for the increased number of reports of epiglottitis in adults is an actual increase in the incidence of the disease, estimated at 9.7 cases per million adults per year (17). However, it may be more likely that enhanced physician awareness and improved recognition of the disorder accounts for the apparent growing number of cases (15,17). Regardless of the reason for the increase, large hospitals can expect several cases of adult epiglottitis per year (16).
Morbidity and Mortality In the past, epiglottitis in adults was considered a medical rarity (16) that frequently necessitated tracheostomy and was associated with high mortality (7,9,11,13,26). From 1958 to 1973, the mortality from adult epiglottitis was 32%, and tracheostomy was required in 45% of patients who recovered (7). Khilanani and Khatib (4), in a review of adult epiglottitis up to 1980, calculated an overall mortality rate of 10.8 %, with a 30% incidence of tracheostomy. In recent years, although the number of cases of adult epiglottitis has been increasing, mortality and morbidity associated with the disease appear to have been decreasing. The overall mortality for adult epiglottitis is now estimated at 4 to 7% (16,17), (and exceeds the mortality rate from childhood epiglottitis, estimated at less than 1X) (17,28). However, several current series of adult epiglottitis have reported 0% mortality rates. In 1983, Mustoe and Strome (13) reported 75 patients with adult epiglottitis with no fatalities and only 6 patients requiring tracheostomy. Deeb and coworkers (27), Chaisson and coworkers (16), Shapiro and coworkers (14), and Singer and McCabe (24) have recently published four additional series consisting of 112 patients with adult epiglottitis with no reported deaths. In the present study, there were no fatalities. Possible reasons for the decrease in mortality may include enhanced awareness, improved recognition, and prompt treatment of adult epiglottitis by clinicians, thereby potentially increasing the detection of more patients with “benign” disease as well as making an earlier diagnosis in cases that may have become severe if untreated. Pathophysiology Epiglottitis is an acute inflammatory disease affecting both the epiglottis and supraglottic soft tissues. Since the aryepiglottic folds, arytenoid soft tissues,
Phil l3. Fontanarosa, S. Scott Polsky, George E. Goldman
lingual tonsils, vallecula, and base of the tongue are also commonly involved, the term supraglottitis more accurately describes the process (1,2,14,22,29). Organisms may directly invade the epiglottis and the supraglottic tissues (2). The pathologic process can consist of localized cellulitis of the epiglottis or supraglottic structures, epiglottic abscess, or systemic infection with bacteremia (2,6). At times, the epiglottis may actually be spared, although surrounding tissues are markedly inflamed (14,15,29). Epiglottitis may be less severe in adults than in children partly because of the larger diameter and more rigid nature of the adult airway (30). More inflammatory edema at the glottic opening is necessary to produce symptoms of airway compromise in adults than in children (5,26,27,31). Although the adult airway is not as easily occluded as the pediatric airway (26,30), continued inflammation of the epiglottis, progressive swelling of the supraglottic structures, and increased respiratory secretions, combined with the potential for laryngospasm, may precipitate sudden upper airway obstruction (3,6).
Bacteriology The most common bacterial cause of adult epiglottitis is H influenza type b (2), isolated from blood cultures in only the minority of patients (14,30). Between 1958 and 1973, only 19% of documented cases of adult epiglottitis were caused by H influenza (33). Pooled data from previously published series demonstrate H influenza in 27% of blood cultures (16,34). Recent reports, however, have documented H influenza bacteremia in 0% (14), 15% (22), and 23% (17) of patients with adult epiglottitis. The frequency of bacteremia in adult epiglottitis is considerably lower than that in pediatric epiglottitis, where 65% to 90% have positive cultures, usually for H influenza (16,17,32). The incidence of bacteremia in the present study was 8% : 2 positive cultures in 24 patients. Four patients had no documentation of bIood cultures being obtained. Possible reasons accounting for the low incidence of bacteremia may include pretreatment with antibiotics (as had occurred in 43 % of patients), early recognition in mild stages of disease, and viral etiology. Recent evidence suggests that epiglottitis among adults is not a uniform disease. There appear to be two populations of patients: those with a fulminant clinical course, acute respiratory compromise, and blood cultures positive for H influenza; and those with a milder, benign course, no respiratory compromise, and no definable pathogen (14).
Adult Epiglottitis
Severe disease in adults is frequently associated with H influenza infection (17). Mustoe and Strome (13) noted an association between recovery of H influenza in blood cultures and a severe clinical course. Deeb and coworkers (27) found that the majority of patients who required airway intervention had positive blood cultures for W influenza. Mayo Smith and coworkers (17) reported a correlation between isolation of H influenza in blood cultures and increased morbidity and mortality, noting that 56% of patients with acute respiratory compromise had H influenza recovered. In our study, the single patient with positive blood cultures for H influenza did not require intubation and recovered completely with observational management. Conversely, the clinical course in adult epiglottitis is usually less severe when a pathogen other than H influenza is isolated or when no organism is cultured (14,30). Mayo Smith and coworkers (17) found that the majority of patients with milder disease had no defined pathogen. Shapiro and coworkers (14) reported 8 patients with a benign clinical course and no isolation of H influenza from blood, pharyngeal, vallecula, or suprahyoid cultures. However, some patients with non-H-influenza epiglottitis will develop respiratory distress and require airway support (14). In the present study, H infruenza was not isolated from either patient requiring intubation; one patient had blood cultures positive for H parainfluenza, the other had sterile blood cultures. Though the association of severity of clinical course and H influenza is suggested, the precise correlation is undefined and requires further evaluation (14). Other reported organisms in adult epiglottitis include streptococci, staphylococci, Branhamella catarrhalis, Klebsiella pneumoniae, Candida, Pasturella multocida, H parainfluenza, and herpes (1,2, 22,30). Frequently, no organism can be cultured, leading to the speculation that respiratory viruses have a role in milder cases of adult epiglottitis (1417,30).
Clinical Presentation Adults with epiglottitis may experience a longer prodrome and may present for medical attention only after several days of symptoms. Typically, adults present 1 to 2 days after onset of illness, although prodromes lasting up to 7 days have been reported (16). In a review of 101 cases of adult epiglottitis, the mean duration of illness before admission was 40 hours, with 58% of patients presenting fewer than 24 hours after symptom onset (4). In the present study,
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39% of patients presented within 24 hours of illness, while 32% delayed hospital presentation until after 4 days of symptoms. Previous studies suggest that the time from onset of symptoms to time of presentation may correlate with severity of disease and the need for airway support (17,27). Some cases of adult epiglottitis may be rapidly progressive, and patients may present within hours after symptom onset. Deeb and coworkers (27) found that patients presenting within 8 hours of symptoms had a more malignant clinical course and often required airway support for upper airway compromise, while patients presenting after 12 hours of symptoms generally had a more benign clinical course and did not require airway intervention. They postulate that for patients with symptoms of more than 12 hours duration along with the absence of drooling, the inflammatory process has probably peaked and airway obstruction is unlikely. Mayo Smith (17) also found that the risk of airway obstruction exists primarily early in the course of the disease. In the current study, although there were no episodes of airway obstruction, both patients requiring airway intervention presented within 24 hours of symptom onset. Other authors (3,9,18,26) emphasize the unpredictable nature of airway obstruction associated with adult epiglottitis. Furthermore, a report of fatal, delayed airway obstruction in an adult with epiglottitis and the resulting medical-legal ramifications of the case has recently been published (35). As in previous reports (7,25,36), the majority of patients in this study presented with dysphagia and sore throat. Although patients may complain of “the worst sore throat of my life” (29), physical examination of the oropharnyx is usually unimpressive (4,30). Pharyngeal pain out of proportion to clinical findings is consistent with acute epiglottitis (2,12,25). Temperature elevation (over 37.8”C) was noted in 57% of the patients in this study. Fever, not always present (16) in patients with adult epiglottitis, may be absent in up to 30% of cases (25) or may develop only later in the course of the disease (24). A voice change or “muffled” voice, documented in 18 patients (64%) in this study, is a common finding in adult epiglottitis (25). True hoarseness is not typical (6,30,37) because the supraglottic edema usually does not extend below the vocal cords (2). Aphonia, however, may signal imminent airway obstruction (24, 38). Drooling may indicate the need for airway protection (6,27), although Stair and Hirsch (22) noted that 4 of 5 patients with drooling in their series recovered without airway support. In the present study, 9 pa-
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tients had some degree of drooling or difficulty with secretions, but only one required intubation. No patient in this series presented with stridor, considered an indicator of a more fulminant course (6,16) and usually heralding the need for airway support (4). Other signs of respiratory difficulty: use of accessory muscles, retractions, air hunger, are considered ominous findings in adult epiglottitis, but do not always occur, particularly in the early stages of the disease (6,24).
Differential Diagnosis It has been suggested that “every acute sore throat with dysphagia is epiglottitis until proven otherwise” (6), especially if symptoms are disproportionate to pharyngeal examination (25,30). While acute epiglottitis should be readily recognized in the adult presenting with acute sore throat, dysphagia, fever, systemic toxicity, and signs of airway compromise (24), milder cases of adult epiglottitis are commonly misdiagnosed (6,39,40). Evidence suggests that it is frequently not recognized on initial visit to a physician (2,7,30). Cohen (30), Shabel (40), and Procino (39) reported incorrect diagnosis of acute epiglottitis in over 50% of cases in each of their series. Mayo Smith (17) noted that 32% of patients presenting with adult epiglottitis had been seen by a physician within 48 hours of hospital admission, and 21% had been given antibiotics. In our series, nearly half (43%) of patients had been previously evaluated by a physician and had been misdiagnosed. The most common diagnosis erroneously made when epiglottitis is present but missed is strep pharyngitis (41). The differential diagnosis of adult epiglottitis includes infectious processes such as mononucleosis, diptheria, pertussis, croup, and Ludwig’s angina, along with retropharyngeal, peripharyngeal, and peritonsillar abscesses, as well as noninfectious causes including allergic drug reactions, angioneurotic edema, foreign body aspiration, reflex laryngospasm, laryngeal trauma, tumors, hydrocarbon aspiration, systemic lupus erythematosis, and inhalation Of toxic fumes or superheated steam (1,2,6,22, 38).
Diagnostic Procedures For adult patients suspected of having acute epiglottitis, the diagnosis can be established by either lateral soft tissue cervical radiographs or laryngoscopy (25,42), provided there is no respiratory distress and
Phil 6. Fontanarosa, S. Scott Polsky, George E. Goldman
acute airway intervention is not required. Upright lateral neck films should be taken in the emergency department under the supervision of the physician with airway management equipment readily available (2). Indirect mirror laryngoscopy or flexible fiberoptic laryngoscopy may be performed only if personnel are prepared for establishment of a definitive airway if necessary (1). Lateral soft tissue cervical x-ray studies are a sensitive method for diagnosing adult epiglottitis and usually display characteristic findings (8,40). Marked edema of the epiglottis, with a “thumb shaped” appearance (3,25) is pathognomonic. Schumaker and coworkers (8) evaluated radiologic measurements in adult epiglottitis and demonstrated that an epiglottic width greater than 8 mm is highly suggestive of the diagnosis. Other radiographic findings seen with adult epiglottitis may include swelling of the aryepiglottic folds, aryetnoids, uvula, and supraglottic tissues; narrowing of the airway adjacent to the epiglottis; obliteration of the vallecula (5,6,30), edema of the prevertebral and retropharyngeal soft tissues, and “ballooning” of the hypopharynx (6,30,40,43). Xeroradiographs may provide even clearer details of the epiglottis and supraglottic structures (38). The correlation between lateral neck x-rays and laryngoscopic findings in adult epiglottitis has been studied. Shapiro and coworkers (14) and Mustoe and Strome (13) noted nearly perfect correlation between laryngoscopic and radiographic findings. Schabel and coworkers (40) noted characteristic x-ray changes in all patients with laryngoscopically documented epiglottitis. Schumaker and coworkers (8) demonstrated that the presence of radiographic findings indicative of epiglottitis correlates with positive laryngoscopy, and that normal films correspond with normal laryngoscopy. Simon (5) maintains that if supraglottic swelling is present on lateral cervical radiographs, the diagnosis of epiglottitis is confirmed. Lateral soft tissue cervical radiographs are useful for diagnosing adult epiglottitis only if characteristic changes are present. Negative lateral soft tissue films do not rule out epiglottitis (6,29,43). Chaisson and coworkers (16) reported radiographs to be 90% sensitive for diagnosing adult epiglottitis. Mayo Smith and coworkers (17) found characteristic radiographic findings in only 79% of patients with documented epiglottitis. In the present study, lateral cervical radiographs were 89% sensitive. Two patients with normal radiographs had characteristic findings of epiglottitis on laryngoscopy. If epiglottitis is confirmed by x-ray study, laryngoscopic instrumentation is unnecessary for diag-
Adult Epiglottitis
nosis (5). Since normal soft tissue x-ray studies do not safely exclude the diagnosis, patients with negative lateral cervical radiographs in whom adult epiglottitis is suspected should have indirect or direct laryngoscopy performed. In patients with mild symptoms and no sign of respiratory distress, indirect laryngoscopy is “not only indicated but should be mandatory to establish the diagnosis early on” in the evaluation of patients with suspected epiglottitis (6,3 1). Laryngoscopy provides critical information and typically reveals swelling of the epiglottis and supraglottic structures. The epiglottis may appear “cherry-red” in color, but is often pale and edematous (38). Indirect laryngoscopy is considered safe in adult patients without signs of respiratory distress or airway compromise (15,17). Shapiro and coworkers (14) contend that the laryngeal examination does not appear to precipitate airway obstruction as can occur in pediatric epiglottitis. Stair and Hirsch (22) noted that in 389 previously reported cases of adult epiglottitis, indirect laryngoscopy had not precipitated airway obstruction. Mayo Smith and coworkers (17) cited 52 laryngoscopic examinations without complication. Despite these findings, it is essential to have available the necessary equipment for endotracheal intubation and emergency cricothyrotomy or tracheotomy prior to performing laryngoscopy (1,30), and to be capable of, and willing to use them. If epiglottitis is suspected and respiratory distress or signs of upper airway compromise such as drooling or stridor are present, indirect laryngoscopy is considered dangerous and is contraindicated (6,44).
Management While it is agreed that signs of airway compromise or progressive respiratory distress mandate immediate establishment of a definitive airway, instituting a prophylactic airway in adult epiglottitis with no evidence of airway distress is controversial (15). Early reports (9,18) recommended tracheotomy for all patients with acute epiglottitis because of the suggestion that upper airway obstruction occurred in 50% of adults with the disease. More recently, Mayo Smith and coworkers (17) advocated that “an airway should be established at the time of presentation of any adult with epiglottitis.” Other studies indicate that the majority of adults with epiglottitis will not have airway obstruction and do not require intubation or tracheostomy (16,22,30). For stable patients with no respiratory distress a reasonable approach is admission to the intensive care
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unit with continuous monitoring, close observation of respiratory status, and the capability for immediate airway support if needed (1,15,22,29). At the first sign of respiratory distress, a definitive airway must be established (30). In the present study, if prophylactic treatment was instituted in each case, 93% of patients would have received an unnecessary artificial airway. The procedure of choice for airway management in adults with epiglottitis is also controversial. Prior to 1960, tracheostomy was the preferred method of airway support in both childhood and adult epiglottitis (45). A recent major trend in both populations is to avoid tracheostomy and substitute intubation (22,32,38,45). Since the natural history of epiglottitis in adults is brief, the need for an endotracheal tube is usually less than five days (11,45,46). In the present study the two patients who were intubated required airway support for 48 hours each. Endotracheal intubation in experienced hands along with the capability for more invasive airway control measures such as cricothyrotomy or emergency tracheostomy appears to be a reasonable approach in cases where acute airway support is required. Orotracheal and nasotracheal intubation under direct vision have been successfully utilized in adult epiglottitis (47,48). Blind nasotracheal intubation should be avoided because of the potential risk for trauma to the already swollen epiglottis (45). The fiberoptic laryngoscope and fiberoptic bronchoscope are also useful both for airway management and diagnosis in adult epiglottits (49). Fiberoptic instruments have the advantage of a single manipulation for visualization, diagnosis, and airway control, but require skill and expertise in their use (22). Fiberoptic intubation is not appropriate for patients with severe respiratory distress or airway obstruction (49,50). In an acutely obstructed airway from adult epiglottitis, crichothyrotomy is the simplest, most rapid, and most efficacious method to establish an emergency airway (6,22,30). For adults with epiglottitis and only mild symptoms, minimal breathing difficulty and no sign of airway compromise, medical therapy is often effective (1,4,5,16,29). Mainstays of medical management include intravenous antibiotics, steroids, and hydration, humidified mist oxygen, and intensive care unit admission (1,6). Once treatment has begun, most patients recover completely in 2 to 4 days (2). Even though the majority of cases of adult epiglottitis are blood culture negative, intravenous antibiotic therapy should be instituted in the emergency department and must provide coverage for H inji’uenzauntil
Phil 13.Fontanarosa, S. Scott Polsky, George E. Goldman
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culture and sensitivity results and beta lactamase testing are available. Initial therapy includes either a second or third generation cephalosporin with activity against H infruenza or a combination of ampicillin and chloramphenicol(1,14,15,23,31). The role of corticosteroids in the treatment of adult epiglottitis is unclear (22,29). Steroids are advocated to reduce edema associated with epiglottitis (2,6,22,51) but, to date, there have been no controlled studies examining their effectiveness. In this study, there was no apparent relationship of steroid use or lack thereof with the need for airway support, incidence of complications, length of hospital stay, or clinical course.
Limitations of the Study Because this study was a review and analysis of patients’ records, the limitations inherent in the retrospective design, as outlined by Singer and McCabe (24), are applicable. Potentially important information and useful data may be absent or inadequately documented. For example, six patients in the study were judged by the examiner to be in “mild” respiratory distress. However, except for respiratory rate, there was no documentation of more objective parameters characterizing the distress, such as flaring of the nasal alae, intercostal retractions, use of accessory muscles, or aphonia. Similarly, the documentation of the laryngoscopic findings was variable with respect to the incidence and degree of supraglottic structure involvement. Other potentially useful data would have included specific reasons for the administration of steroids and for the choice of a particular antibiotic
regimen. These limitations are not considered to be significant and do not substantially impact the conclusion of the study. SUMMARY Epiglottitis in adults is an uncommon, increasingly recognized, but frequently misdiagnosed infection. The emergency physician should consider the disorder in every patient with sore throat and dysphagia, especially if symptoms are disproportionate to physical examination findings. Lateral cervical radiographs are helpful if positive, displaying an edematous epiglottis, but normal x-ray studies do not exclude the diagnosis. For patients with no respiratory distress, laryngoscopic examination is safe and provides diagnostic information. The majority of adults with epiglottitis may have a less fulminant clinical course than classically described, with lower incidence of bacteremia. Nonetheless, the potential for respiratory compromise is ever-present, and does not appear to be consistently related to onset of symptoms, presenting findings, or bacteriologic data. If respiratory difficulty develops, a definitive airway must be established. Tracheal intubation, ideally performed in the operating room, is preferred over tracheostomy. Medical management may be effective for adults with epiglottitis, providing there is no respiratory distress. Medical therapy should include careful monitoring in an intensive care setting, intravenous antibiotics with activity against H influenza, hydration, inhaled mist, supplemental oxygen, and possibly corticosteroids. (2,22).
REFERENCES 1. Mace, SE. Acute epiglottitis in adults. Amer J Emerg Med. 1985;3:543-50. 2. Slay RD. Upper respiratory tract infection. In: Rosen P, Baker FJ, Barkin RM, et al. (eds). Emergency Medicine-Concepts and Clinical Practice. 2nd ed. St Louis: CV Mosby Co; 1988: 1071-89. 3. Marlowe FL Gtolaryngologic emergencies. In: Tintinalli JE, Krome RL, Ruiz E, eds. Emergency Medicine-A Comprehensive Study Guide, 2nd ed. New York: McGraw-Hill: 1988:58995. 4. Khilanani U, Khatib R. Acute epiglottitis in adults. Am J Med Scien. 1984;287:65-70. 5. Simon HB. Infections of the upper respiratory tract. In: Rubenstein E, Federman DD, eds. Scientific American-Meditine. New York: Scientific American Inc; 1986:7: 19: l-10. 6. Sarant G. Acute epiglottitis in adults. Ann Emerg Med. 1981; 10:58-61. 7. Hawkins DB, Miller AH, Sachs GB, Benz RB. Acute epiglottitis in adults. Laryngoscope. 1973;83:1211-20. 8. Schumaker HM, Doris PE, Birnbaum G. Radiographic parameters in adult epiglottitis. Ann Emerg Med. 1984;13:588-90.
9. Bass JW, Steele RW, Riebe RA. Acute epiglottitis: a surgical emergency. JAMA. 1974;229:671-5. 10. Warner JA, Finlay WE. Fulminating epiglottitis in adults. Anaesthesia. 1985;40:348-52. 11. Gorfinkel HJ, Brown R, Kabins SA. Acute infectious epiglottitis in adults. Ann Intern Med. 1969;70:289-94. 12. Bishop MJ. Epiglottitis in the adult. Anesthesiology. 1981;55: 701-2. 13. Mustoe T, Strome M. Adult epiglottitis. Amer J Otolaryngol. 1983;4:393-9. 14. Shapiro J, Roland DE, Baker AS. Adult supraglottitis. JAMA. 1988;4:563-8. 15. Baker AS, Eavey RD. Adult supraglottitis (epiglottitis) [Editorial]. N Engl J Med. 1986;314:1185-6. 16. Chaisson RE, Ross J, Gerberding JL, Sande MA. Clinical aspects of adult epiglottitis. West J Med. 1986;144:700-3. 17. Mayo Smith MF, Hirsch PJ, Wodzinski SF, Schiffman FJ. Acute epiglottitis in adults. N Engl J Med. 1986;314:113386. 18. Morgenstern KM, Abramson AL. Acute epiglottitis in adults. Laryngoscope. 1971;81:1066-73.
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19. Scheidemandel HHE. Did George Washington die of quinsy. Arch Otolaryngol. 1976;102:519-21. 20. LeMierre A, Meyer A, LaPlane R. Les septicemes a bacille de Pfeiffer. Ann Med. 1936;39:97-119. 21. Brown JM. Acute infections of the epiglottitis. Arch Otolaryngol. 1940;32:631-41. 22. Stair ‘IO, Hirsch BE. Adult supraglottitis. Amer J Emerg Med. 1985;3:512-18. 23. Warshinski J. Havas TE. McShane DP. Gullane PJ. Adult epiglottitis. JOtolaryngol: 1986;15:362-5. 24. Singer J, McCabe J. Epiglottitis at the extremes of age. Amer J Emerg Med. 1988;6:228-31. 25. Swedo SE, Lelyveld SS, Hageman JR. Epiglottitis: a recurrent episode in a youth. Am J Emerg Med. 1985;3:534-5. 26. Kander PL, Richards SH. Acute epiglottitis in adults. J Laryngo1 Otol. 1977;91:295-302. 27. Deeb ZE, Yenson AC, DeFries HO. Acute epiglottitis in the adult. Laryngoscope. 1985;95:289-91. 28. Cantrell RW, Bell RA, Moriaka WT. Acute epiglottitis: intubation versus tracheostomy. Laryngoscope. 1978;88:994-1005. 29. Kimmitt TP, Defries HO. Throat emergencies. In: Stair TO, ed. Practical management of eye, ear, nose, mouth and throat emergencies. Rockville, Maryland: Aspen Publishers Inc; 1986: 137-a. 30. Cohen EL. Epiglottitis in adults. Ann Emerg Med. 1984;13: 620-3. 31. Dailey RH. Acute upper airway obstruction. Emerg Med Clin North Amer. 1983;2:261-77. 32. Wetmore RF, Handler SD. Epiglottitis: evolution in management during the last decade. Ann Otol Rhino1 Laryngol. 1979; 88:822-6. 33. Heeneman H, Ward K. Epiglottic abscess: its occurrence and management. J Otolaryngol. 1977;6:31-6. 34. Chaisson RE. Epiglottitis in adults. In: Sande A, Trunkey DD, Root RK, eds. Contemporary issues in infectious diseases. Vol 5. Respiratory Infections. New York: Churchill Livingstone; 1986.
35. McNelis FL. Medical and legal management of adult acute epiglottitis. Laryngoscope. 1985;95:125-7. 36. Black MJ, Harbour J, Remsen KA, Baxter JD. Acute epiglottitis in adults. J Otolaryngol. 1981;10:23-7. 37. Jones HM. Acute epiglottitis: a personal study over 20 years. Proc R Sot Med. 1970;63:706-12. 38. Guillozet N, Barclary AM. Obstructive epiglottitis in adults. J Fam Pratt. 1982;15:895-7. 39. Procino ND. Acute epiglottitis in adults. Ear Nose Throat J. 1978:57:30-4. 40. Schabel SI, Katzberg RW, Burgener FA. Acute inflammation of epiglottitis and supraglottic structures in adults. Diagnostic Radiology. 1977;122:601-4. 41. Cohen EL. Epiglottitis in the adult. Prostgrad Med. 1984;75: 309-11. 42. Ossoff RH, Wolff AP. Acute epiglottitis in adults. JAMA. 1980;244:2639-40. 43. Kimmitt TP, Defries HO. Pharyngeal emergencies. Topics in Emergency Medicine. 1984;6:66-80. 44. Scheidemandel HHE, Page RS. Special considerations in epiglottitis in children. Laryngoscope. 1975;85:1738-45. 45. Ward CF, Benumof JL, Shapiro HM. Management of adult acute epiglottitis by tracheal intubation. Chest. 1977;71:93-5. 46. Robbins JP, Fitz-Hugh GS. Epiglottitis is the adult. Laryngoscope. 1971;81:700. 47. Wurtele P. Nasotracheal intubation: a modality in the management of acute epiglottitis in adults. J Otolaryngol. 1984;13: 118-22. 48. Tos M. Nasotracheal intubation in acute epiglottitis. Arch Otolaryngol. 1973;97:373-5. 49. Phelan DM, Love JB. Adult epiglottitis: Is there a role for the fiberoptic bronchoscope? Chest. 1984;86:783-4. 50. Guidice JC, Komansky HJ. Acute epiglottitis: the use of a fiberoptic bronchoscope in diagnosis and therapy. Chest. 1979; 75:21 l-12. 5 1. Strome M, Jaffee B. Epiglottitis - individualized management with steroids. Laryngoscope. 1974:84:921-g.
Printed in the USA ??Copyright 0 1989 Pergamon Press plc
ADULT EPIGLOTTITIS Phil B. Fontanarosa,
MD, FACEP,
S. Scott Polsky,
MD, FACEP,
George E. Goldman,
MD, FACEP
Akron City Hospital, Northeastern Ohio Universities College of Medicine, Akron, Ohio Reprin,ntaddress: Phil B. Fontanarosa, MD, FACEP, Department of Emergency Medicine, Akron City Hospital, 525 East Market Street, Akron, Ohio 44309
?? Abstract-Adult epiglottitis (or “supraglottitis”) is an uncommon but increasingly recognized entity. Though prior studies emphasized the fulminant nature of the disease, recent evidence suggests that epiglottitis in adults may follow a relatively less severe clinical course, especially if Hemophilus influenza is not isolated. The records of 28 patients with adult epiglottitis were retrospectively analyzed to characterize the presenting features and clinical course of the disease. The diagnosis was established by laryngoscopy, lateral cervical radiographs, or both. Laryngoscopy did not precipitate airway obstruction in any patient. The majority of patients experienced a relatively benign clinical course and improved with medical management that consisted of ICU admission, intravenous antibiotics, hydration, inhaled mist, and corticosteriods. Only two patients (7%) required airway support with orotracheal intubation because of respiratory difficulty. There were no instances of respiratory arrest or airway obstruction. No tracheostomies were performed, and there were no deaths. It was concluded that adult epiglottitis can follow a less severe course than classically described. 0 Keywords -epiglottitis,
more benign clinical course than pediatric epiglottitis (13,14), and the majority of cases have no definable pathogen (13,14,15,16,17). It was the presentation of six cases of adult epiglottitis at our institution over a recent 8-month period that prompted this review. A retrospective study was undertaken to evaluate the clinical presentation and to characterize the clinical course of adults with epiglottitis. METHODS Akron City Hospital is a 540-bed acute care hospital with an annual emergency department (ED) census of 60,000 adult patients. The medical records of all adult patients treated at Akron City Hospital between January, 1982, and July, 1988, with a primary or secondary final diagnosis of acute epiglottitis (or supraglottitis) were obtained. The emergency department chart and hospital records were retrospectively reviewed and analyzed for presenting symptoms, physical findings, diagnostic procedures, radiologic findings, laboratory data, treatment, clinical course, complications, and outcome. The diagnosis of epiglottitis was established in all cases by direct laryngoscopic findings or lateral cervical soft tissue radiographs that demonstrated an edematous epiglottis. The original prospective interpretation of the lateral neck films was performed by an attending radiologist. The radiographs were then reviewed by the investigators without knowledge of the initial radiologic impression. The films were retrospectively interpreted as
in adults; supraglottitis
INTRODUCTION Acute epiglottitis is a potentially life-threatening infectious disease that can cause sudden, unpredictable, upper airway obstruction (l-4). While most commonly seen in children, acute epiglottitis or “supraglottitis” in adults is now recognized with increasing frequency (2,3,5,6,7,8). Although previous studies have emphasized the fulminant nature of the disorder (9,10,11,12), recent evidence suggests that adult epiglottitis may follow a =
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focusing primarily
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is coordinated
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223
by Ron Walls,
MD,
of Van-
0736-4679/89$3.00 + .OO
Phil B. Fontanarosa, S. Scott Polsky, George E. Goldman
224
positive or negative for epiglottitis, and measurements of the width of the epiglottis at its widest point were made. Films demonstrating an epiglottic width greater than 8 mm (8) were considered positive.
RESULTS Our review identified 28 cases of adult epiglottitis that had presented during the study period. There were 18 females and 10 males. Patients ranged in age from 18 to 70 years, with a mean age of 39.5 years. No medical diseases predisposing to epiglottitis were found. Historical factors and presenting physical findings are summarized in Table 1. The mean length of time from onset of symptoms to emergency department presentation was 2.6 days, range 0 to 5 days. Thirtynine percent (1 l/28) of patients presented within 24 hours of symptoms, while 32% (9128) patients were not seen until 4 days after initial symptoms occurred. Twelve patients (43 %) had been seen by a physician prior to ED evaluation and admission and had been placed on antibiotics for treatment of pharyngitis (11 cases) and bronchitis (1 case). Of the nine patients with delayed ED presentation- over 4 days after symptom onset -7 were on a prescribed antibiotic regimen. All patients complained of sore throat and dysphagia. Other common symptoms included voice change (64%) and shortness of breath (21%). The most common physical findings were anterior cervical adenopathy (79%), excess salivation and secretions or some degree of drooling (29%), and “mild” respiratory distress (21%). The absence of stridor was noted in all cases. Other specific indicators of respiratory distress, such as retractions and use of accessory muscles, were not documented. The mean temperature was 38.O”C. (range 37.039.5). Sixteen patients (57%) had temperatures in excess of 37.8”C. The mean respiratory rate was 20.8/ min with a range of 12 to 32/min. Tachypnea (respiratory rate > 20) was noted in 11 patients. The pulse rate ranged from 72 to 140 beats/min, mean 102 beats/ min, with tachycardia (> 100 beats/min) present in 15 patients (54%). Leukocyte counts ranged from 6,300 to 31,000 cells/mm3 with a mean of 14,700 cells/mm’. Seventyfive percent of patients (21/28) had white cell counts over 13,000 cells/mms, and 5 patients (18%) had counts under 10,000 cells/mm3. Blood cultures were obtained in 24 patients with 2 positive results (8%): one for Hemophilus influenza type b and one for Hemophilus parainfluenza. Throat cultures were performed in 13 patients with
two positive for non-group-A beta streptococci. Both patients with bacteremia had negative throat cultures. No viral studies were obtained. Laryngoscopic visualization of the epiglottis was performed in 18 patients (64%): 14 by indirect laryngoscopy, 2 by direct laryngoscopy with a fiberoptic laryngoscope, and 2 by direct laryngoscopy during endotracheal intubation. Laryngoscopy was performed by an otolaryngologist (8 cases), emergency physician (7 cases), anesthesiologist (2 cases), and intensivist (1 case). Laryngoscopy did not precipitate laryngospasm in any patient. The epiglottis appeared swollen and erythematous in each case. Documentation of supraglottic involvement varied, but it was specifically noted in 9 patients. All 28 patients had standard lateral cervical soft tissue x-ray studies performed. The films of 25 patients were interpreted by the staff radiologist as being consistent with epiglottitis, a sensitivity of 89%. The films of one patient were considered abnormal, with “anterior soft tissue swelling,” but the characteristic findings of epiglottitis were not specified. In two other patients, the radiologist interpreted the films as normal. In each of these three cases, laryngoscopy was performed and revealed epiglottitis. The soft tissue x-ray studies of 24 of 28 patients were available for independent retrospective analysis. Of the 24 radiographs reviewed, 22 were considered positive and demonstrated soft tissue swelling characteristic of acute epiglottitis, a sensitivity of 92%. The mean epiglottic width in this group was 14 mmh3.0 mm (SD), range 10 to 22 mm. The radiographs of two patients were considered normal by retrospective analysis, with a mean epiglottic width of 7.0 mm+1.4mm(SD),range6to8mm. The retrospective analysis was in agreement with the radiologists’s prospective interpretation in 23 of 24 cases. The x-ray studies considered positive for epiglottitis by retrospective review had also been considered positive by the radiologist. The films that had been interpreted as normal by the radiologist were also considered normal, or negative for epiglottitis, upon retrospective analysis. Review of the radiograph that had been considered abnormal but not characteristic of epiglottitis by the radiologist revealed swelling of the anterior supraglottic soft tissues and an epiglottic width of 12 mm, and was considered consistent with epiglottitis. All patients had been admitted to either the intensive care unit or a closely monitored medical unit, where they received mist oxygen, hydration, and intravenous antibiotics. Antibiotics used included cefoperazone (19), ceftriaxone (3), cefoxitin (l), cefamandole (l), ceftazidime (l), ampicillin plus sulbactam (l), nafcillin and cefaperazone (l), and timentin and
Adult Epiglottitis
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Table 1. Symptoms and Signs in 28 Patients with Adult Supraglottitls
Historical Factors Sore throat Dysphagia Voice change Dyspnea Duration < 24 hrs 24-72 hrs > 72 hrs Taking antibiotics Physical Findings Temperature <37.8X 37.8O-39.0% >39.0% Cervical adenopathy Drooling Respiratory distress Stridor
No.
Percent
28 28 18 6
100 100 64 21
11 8 9 12
39 29 32 43
12 13 3 22 8 6 0
43 46 11 79 29 21 0
cefoxitin (1). Sixteen patients (57%) had received intravenous steroids, but specific indications for steroid administration could not be determined. Twenty-six patients (93 W) were considered to have had a “mild” clinical course, defined as clinical improvement after admission and no need for airway support. Two patients had required respiratory assistance and had been defined as having a “severe” clinical course. Both patients needing establishment of an artificial airway had been orotracheally intubated in the operating room and both remained intubated for 48 hours. One patient was intubated 6 hours after admission because of respiratory distress and subsequently had blood cultures positive for Hemophilus parainj7uenza. The second patient was intubated 24 hours after admission because of increasing respiratory difficulty, but had negative blood cultures. Both patients requiring intubation had presented within 24 hours of onset of symptoms. There was no apparent relationship between the need for airway support and other historical factors, vital signs, physical findings, presence of drooling, or leukocyte count. All patients improved during hospitalization, with the majority of patients recovering completely in 3 to 5 days. The length of hospital stay ranged from 3 to 7 days with a mean of 4.5 days. Both patients with positive blood cultures were hospitalized for 6 days. The nonbacteremic patient requiring intubation was hospitalized for 5 days. Steroid administration did not appear to benefit or worsen the clinical course. Except for the two patients requiring intubation, there were few complications. One patient had an uneventful hospital course, showed clinical improve-
ment and was discharged, but had relapse of symptoms within 48 hours and required readmission. He was successfully treated with intravenous antibiotics, steroids, hydration, inhaled mist, oxygen, and observation; had an uncomplicated additional 3-day hospital course; and was considered a single episode of epiglottitis. Another patient developed mild, self-limited antibiotic-induced diarrhea. There were no cases of frank airway obstruction or respiratory arrest. No tracheostomies were performed and there were no deaths.
DISCUSSION Epiglottitis in adults occurs regularly, though uncommonly, in clinical practice (16). Although prior studies (7,11,18) have emphasized the high incidence of airway obstruction and the associated mortality of the disease, recent reports (13,14,16) suggest that adult epiglottitis may frequently follow a “less pernicious” clinical course than classically described. The findings in the present study, designed to evaluate the presenting features and characterize the clinical course of adults with epiglottitis, also demonstrate a relatively benign clinical course for the majority of patients. In 28 cases of adult epiglottitis, no deaths occurred and only two patients required airway support.
Adult Epiglottitis in Perspective It is speculated that the death of George Washington in 1796 represented the earliest recorded case of epiglottitis (19). However, the report of an adult with H influenza septicemia and fatal airway obstruction by LeMierre and coworkers (20) in 1936 is recognized as the first published case of adult epiglottitis. Prior to 1940, when Brown (21) reported the first series of cases of the disease, epiglottitis had been primarily considered an adult illness (7). During the 195Os, epiglottitis as a disease entity in children became widely publicized, while epiglottitis in adults was seldom mentioned (7). Consequently, by 1960, there were less than 40 reported cases of adult epiglottitis (22). In more recent years, epiglottitis in adults has been increasingly discussed in the medical literature. By 1977, there were over 100 cases documented (12). In 1985, Stair and Hirsch (22) reviewed all previous reports of adult epiglottitis and tabulated 389 cases. From 1986 to 1988, seven additional series (13, 14,16,17,23-25) including 168 additional patients have been published, bringing the total number of
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cases of adult epiglottitis to over 500. The present study adds 28 patients. A possible explanation for the increased number of reports of epiglottitis in adults is an actual increase in the incidence of the disease, estimated at 9.7 cases per million adults per year (17). However, it may be more likely that enhanced physician awareness and improved recognition of the disorder accounts for the apparent growing number of cases (15,17). Regardless of the reason for the increase, large hospitals can expect several cases of adult epiglottitis per year (16).
Morbidity and Mortality In the past, epiglottitis in adults was considered a medical rarity (16) that frequently necessitated tracheostomy and was associated with high mortality (7,9,11,13,26). From 1958 to 1973, the mortality from adult epiglottitis was 32%, and tracheostomy was required in 45% of patients who recovered (7). Khilanani and Khatib (4), in a review of adult epiglottitis up to 1980, calculated an overall mortality rate of 10.8 %, with a 30% incidence of tracheostomy. In recent years, although the number of cases of adult epiglottitis has been increasing, mortality and morbidity associated with the disease appear to have been decreasing. The overall mortality for adult epiglottitis is now estimated at 4 to 7% (16,17), (and exceeds the mortality rate from childhood epiglottitis, estimated at less than 1X) (17,28). However, several current series of adult epiglottitis have reported 0% mortality rates. In 1983, Mustoe and Strome (13) reported 75 patients with adult epiglottitis with no fatalities and only 6 patients requiring tracheostomy. Deeb and coworkers (27), Chaisson and coworkers (16), Shapiro and coworkers (14), and Singer and McCabe (24) have recently published four additional series consisting of 112 patients with adult epiglottitis with no reported deaths. In the present study, there were no fatalities. Possible reasons for the decrease in mortality may include enhanced awareness, improved recognition, and prompt treatment of adult epiglottitis by clinicians, thereby potentially increasing the detection of more patients with “benign” disease as well as making an earlier diagnosis in cases that may have become severe if untreated. Pathophysiology Epiglottitis is an acute inflammatory disease affecting both the epiglottis and supraglottic soft tissues. Since the aryepiglottic folds, arytenoid soft tissues,
Phil l3. Fontanarosa, S. Scott Polsky, George E. Goldman
lingual tonsils, vallecula, and base of the tongue are also commonly involved, the term supraglottitis more accurately describes the process (1,2,14,22,29). Organisms may directly invade the epiglottis and the supraglottic tissues (2). The pathologic process can consist of localized cellulitis of the epiglottis or supraglottic structures, epiglottic abscess, or systemic infection with bacteremia (2,6). At times, the epiglottis may actually be spared, although surrounding tissues are markedly inflamed (14,15,29). Epiglottitis may be less severe in adults than in children partly because of the larger diameter and more rigid nature of the adult airway (30). More inflammatory edema at the glottic opening is necessary to produce symptoms of airway compromise in adults than in children (5,26,27,31). Although the adult airway is not as easily occluded as the pediatric airway (26,30), continued inflammation of the epiglottis, progressive swelling of the supraglottic structures, and increased respiratory secretions, combined with the potential for laryngospasm, may precipitate sudden upper airway obstruction (3,6).
Bacteriology The most common bacterial cause of adult epiglottitis is H influenza type b (2), isolated from blood cultures in only the minority of patients (14,30). Between 1958 and 1973, only 19% of documented cases of adult epiglottitis were caused by H influenza (33). Pooled data from previously published series demonstrate H influenza in 27% of blood cultures (16,34). Recent reports, however, have documented H influenza bacteremia in 0% (14), 15% (22), and 23% (17) of patients with adult epiglottitis. The frequency of bacteremia in adult epiglottitis is considerably lower than that in pediatric epiglottitis, where 65% to 90% have positive cultures, usually for H influenza (16,17,32). The incidence of bacteremia in the present study was 8% : 2 positive cultures in 24 patients. Four patients had no documentation of bIood cultures being obtained. Possible reasons accounting for the low incidence of bacteremia may include pretreatment with antibiotics (as had occurred in 43 % of patients), early recognition in mild stages of disease, and viral etiology. Recent evidence suggests that epiglottitis among adults is not a uniform disease. There appear to be two populations of patients: those with a fulminant clinical course, acute respiratory compromise, and blood cultures positive for H influenza; and those with a milder, benign course, no respiratory compromise, and no definable pathogen (14).
Adult Epiglottitis
Severe disease in adults is frequently associated with H influenza infection (17). Mustoe and Strome (13) noted an association between recovery of H influenza in blood cultures and a severe clinical course. Deeb and coworkers (27) found that the majority of patients who required airway intervention had positive blood cultures for W influenza. Mayo Smith and coworkers (17) reported a correlation between isolation of H influenza in blood cultures and increased morbidity and mortality, noting that 56% of patients with acute respiratory compromise had H influenza recovered. In our study, the single patient with positive blood cultures for H influenza did not require intubation and recovered completely with observational management. Conversely, the clinical course in adult epiglottitis is usually less severe when a pathogen other than H influenza is isolated or when no organism is cultured (14,30). Mayo Smith and coworkers (17) found that the majority of patients with milder disease had no defined pathogen. Shapiro and coworkers (14) reported 8 patients with a benign clinical course and no isolation of H influenza from blood, pharyngeal, vallecula, or suprahyoid cultures. However, some patients with non-H-influenza epiglottitis will develop respiratory distress and require airway support (14). In the present study, H infruenza was not isolated from either patient requiring intubation; one patient had blood cultures positive for H parainfluenza, the other had sterile blood cultures. Though the association of severity of clinical course and H influenza is suggested, the precise correlation is undefined and requires further evaluation (14). Other reported organisms in adult epiglottitis include streptococci, staphylococci, Branhamella catarrhalis, Klebsiella pneumoniae, Candida, Pasturella multocida, H parainfluenza, and herpes (1,2, 22,30). Frequently, no organism can be cultured, leading to the speculation that respiratory viruses have a role in milder cases of adult epiglottitis (1417,30).
Clinical Presentation Adults with epiglottitis may experience a longer prodrome and may present for medical attention only after several days of symptoms. Typically, adults present 1 to 2 days after onset of illness, although prodromes lasting up to 7 days have been reported (16). In a review of 101 cases of adult epiglottitis, the mean duration of illness before admission was 40 hours, with 58% of patients presenting fewer than 24 hours after symptom onset (4). In the present study,
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39% of patients presented within 24 hours of illness, while 32% delayed hospital presentation until after 4 days of symptoms. Previous studies suggest that the time from onset of symptoms to time of presentation may correlate with severity of disease and the need for airway support (17,27). Some cases of adult epiglottitis may be rapidly progressive, and patients may present within hours after symptom onset. Deeb and coworkers (27) found that patients presenting within 8 hours of symptoms had a more malignant clinical course and often required airway support for upper airway compromise, while patients presenting after 12 hours of symptoms generally had a more benign clinical course and did not require airway intervention. They postulate that for patients with symptoms of more than 12 hours duration along with the absence of drooling, the inflammatory process has probably peaked and airway obstruction is unlikely. Mayo Smith (17) also found that the risk of airway obstruction exists primarily early in the course of the disease. In the current study, although there were no episodes of airway obstruction, both patients requiring airway intervention presented within 24 hours of symptom onset. Other authors (3,9,18,26) emphasize the unpredictable nature of airway obstruction associated with adult epiglottitis. Furthermore, a report of fatal, delayed airway obstruction in an adult with epiglottitis and the resulting medical-legal ramifications of the case has recently been published (35). As in previous reports (7,25,36), the majority of patients in this study presented with dysphagia and sore throat. Although patients may complain of “the worst sore throat of my life” (29), physical examination of the oropharnyx is usually unimpressive (4,30). Pharyngeal pain out of proportion to clinical findings is consistent with acute epiglottitis (2,12,25). Temperature elevation (over 37.8”C) was noted in 57% of the patients in this study. Fever, not always present (16) in patients with adult epiglottitis, may be absent in up to 30% of cases (25) or may develop only later in the course of the disease (24). A voice change or “muffled” voice, documented in 18 patients (64%) in this study, is a common finding in adult epiglottitis (25). True hoarseness is not typical (6,30,37) because the supraglottic edema usually does not extend below the vocal cords (2). Aphonia, however, may signal imminent airway obstruction (24, 38). Drooling may indicate the need for airway protection (6,27), although Stair and Hirsch (22) noted that 4 of 5 patients with drooling in their series recovered without airway support. In the present study, 9 pa-
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tients had some degree of drooling or difficulty with secretions, but only one required intubation. No patient in this series presented with stridor, considered an indicator of a more fulminant course (6,16) and usually heralding the need for airway support (4). Other signs of respiratory difficulty: use of accessory muscles, retractions, air hunger, are considered ominous findings in adult epiglottitis, but do not always occur, particularly in the early stages of the disease (6,24).
Differential Diagnosis It has been suggested that “every acute sore throat with dysphagia is epiglottitis until proven otherwise” (6), especially if symptoms are disproportionate to pharyngeal examination (25,30). While acute epiglottitis should be readily recognized in the adult presenting with acute sore throat, dysphagia, fever, systemic toxicity, and signs of airway compromise (24), milder cases of adult epiglottitis are commonly misdiagnosed (6,39,40). Evidence suggests that it is frequently not recognized on initial visit to a physician (2,7,30). Cohen (30), Shabel (40), and Procino (39) reported incorrect diagnosis of acute epiglottitis in over 50% of cases in each of their series. Mayo Smith (17) noted that 32% of patients presenting with adult epiglottitis had been seen by a physician within 48 hours of hospital admission, and 21% had been given antibiotics. In our series, nearly half (43%) of patients had been previously evaluated by a physician and had been misdiagnosed. The most common diagnosis erroneously made when epiglottitis is present but missed is strep pharyngitis (41). The differential diagnosis of adult epiglottitis includes infectious processes such as mononucleosis, diptheria, pertussis, croup, and Ludwig’s angina, along with retropharyngeal, peripharyngeal, and peritonsillar abscesses, as well as noninfectious causes including allergic drug reactions, angioneurotic edema, foreign body aspiration, reflex laryngospasm, laryngeal trauma, tumors, hydrocarbon aspiration, systemic lupus erythematosis, and inhalation Of toxic fumes or superheated steam (1,2,6,22, 38).
Diagnostic Procedures For adult patients suspected of having acute epiglottitis, the diagnosis can be established by either lateral soft tissue cervical radiographs or laryngoscopy (25,42), provided there is no respiratory distress and
Phil 6. Fontanarosa, S. Scott Polsky, George E. Goldman
acute airway intervention is not required. Upright lateral neck films should be taken in the emergency department under the supervision of the physician with airway management equipment readily available (2). Indirect mirror laryngoscopy or flexible fiberoptic laryngoscopy may be performed only if personnel are prepared for establishment of a definitive airway if necessary (1). Lateral soft tissue cervical x-ray studies are a sensitive method for diagnosing adult epiglottitis and usually display characteristic findings (8,40). Marked edema of the epiglottis, with a “thumb shaped” appearance (3,25) is pathognomonic. Schumaker and coworkers (8) evaluated radiologic measurements in adult epiglottitis and demonstrated that an epiglottic width greater than 8 mm is highly suggestive of the diagnosis. Other radiographic findings seen with adult epiglottitis may include swelling of the aryepiglottic folds, aryetnoids, uvula, and supraglottic tissues; narrowing of the airway adjacent to the epiglottis; obliteration of the vallecula (5,6,30), edema of the prevertebral and retropharyngeal soft tissues, and “ballooning” of the hypopharynx (6,30,40,43). Xeroradiographs may provide even clearer details of the epiglottis and supraglottic structures (38). The correlation between lateral neck x-rays and laryngoscopic findings in adult epiglottitis has been studied. Shapiro and coworkers (14) and Mustoe and Strome (13) noted nearly perfect correlation between laryngoscopic and radiographic findings. Schabel and coworkers (40) noted characteristic x-ray changes in all patients with laryngoscopically documented epiglottitis. Schumaker and coworkers (8) demonstrated that the presence of radiographic findings indicative of epiglottitis correlates with positive laryngoscopy, and that normal films correspond with normal laryngoscopy. Simon (5) maintains that if supraglottic swelling is present on lateral cervical radiographs, the diagnosis of epiglottitis is confirmed. Lateral soft tissue cervical radiographs are useful for diagnosing adult epiglottitis only if characteristic changes are present. Negative lateral soft tissue films do not rule out epiglottitis (6,29,43). Chaisson and coworkers (16) reported radiographs to be 90% sensitive for diagnosing adult epiglottitis. Mayo Smith and coworkers (17) found characteristic radiographic findings in only 79% of patients with documented epiglottitis. In the present study, lateral cervical radiographs were 89% sensitive. Two patients with normal radiographs had characteristic findings of epiglottitis on laryngoscopy. If epiglottitis is confirmed by x-ray study, laryngoscopic instrumentation is unnecessary for diag-
Adult Epiglottitis
nosis (5). Since normal soft tissue x-ray studies do not safely exclude the diagnosis, patients with negative lateral cervical radiographs in whom adult epiglottitis is suspected should have indirect or direct laryngoscopy performed. In patients with mild symptoms and no sign of respiratory distress, indirect laryngoscopy is “not only indicated but should be mandatory to establish the diagnosis early on” in the evaluation of patients with suspected epiglottitis (6,3 1). Laryngoscopy provides critical information and typically reveals swelling of the epiglottis and supraglottic structures. The epiglottis may appear “cherry-red” in color, but is often pale and edematous (38). Indirect laryngoscopy is considered safe in adult patients without signs of respiratory distress or airway compromise (15,17). Shapiro and coworkers (14) contend that the laryngeal examination does not appear to precipitate airway obstruction as can occur in pediatric epiglottitis. Stair and Hirsch (22) noted that in 389 previously reported cases of adult epiglottitis, indirect laryngoscopy had not precipitated airway obstruction. Mayo Smith and coworkers (17) cited 52 laryngoscopic examinations without complication. Despite these findings, it is essential to have available the necessary equipment for endotracheal intubation and emergency cricothyrotomy or tracheotomy prior to performing laryngoscopy (1,30), and to be capable of, and willing to use them. If epiglottitis is suspected and respiratory distress or signs of upper airway compromise such as drooling or stridor are present, indirect laryngoscopy is considered dangerous and is contraindicated (6,44).
Management While it is agreed that signs of airway compromise or progressive respiratory distress mandate immediate establishment of a definitive airway, instituting a prophylactic airway in adult epiglottitis with no evidence of airway distress is controversial (15). Early reports (9,18) recommended tracheotomy for all patients with acute epiglottitis because of the suggestion that upper airway obstruction occurred in 50% of adults with the disease. More recently, Mayo Smith and coworkers (17) advocated that “an airway should be established at the time of presentation of any adult with epiglottitis.” Other studies indicate that the majority of adults with epiglottitis will not have airway obstruction and do not require intubation or tracheostomy (16,22,30). For stable patients with no respiratory distress a reasonable approach is admission to the intensive care
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unit with continuous monitoring, close observation of respiratory status, and the capability for immediate airway support if needed (1,15,22,29). At the first sign of respiratory distress, a definitive airway must be established (30). In the present study, if prophylactic treatment was instituted in each case, 93% of patients would have received an unnecessary artificial airway. The procedure of choice for airway management in adults with epiglottitis is also controversial. Prior to 1960, tracheostomy was the preferred method of airway support in both childhood and adult epiglottitis (45). A recent major trend in both populations is to avoid tracheostomy and substitute intubation (22,32,38,45). Since the natural history of epiglottitis in adults is brief, the need for an endotracheal tube is usually less than five days (11,45,46). In the present study the two patients who were intubated required airway support for 48 hours each. Endotracheal intubation in experienced hands along with the capability for more invasive airway control measures such as cricothyrotomy or emergency tracheostomy appears to be a reasonable approach in cases where acute airway support is required. Orotracheal and nasotracheal intubation under direct vision have been successfully utilized in adult epiglottitis (47,48). Blind nasotracheal intubation should be avoided because of the potential risk for trauma to the already swollen epiglottis (45). The fiberoptic laryngoscope and fiberoptic bronchoscope are also useful both for airway management and diagnosis in adult epiglottits (49). Fiberoptic instruments have the advantage of a single manipulation for visualization, diagnosis, and airway control, but require skill and expertise in their use (22). Fiberoptic intubation is not appropriate for patients with severe respiratory distress or airway obstruction (49,50). In an acutely obstructed airway from adult epiglottitis, crichothyrotomy is the simplest, most rapid, and most efficacious method to establish an emergency airway (6,22,30). For adults with epiglottitis and only mild symptoms, minimal breathing difficulty and no sign of airway compromise, medical therapy is often effective (1,4,5,16,29). Mainstays of medical management include intravenous antibiotics, steroids, and hydration, humidified mist oxygen, and intensive care unit admission (1,6). Once treatment has begun, most patients recover completely in 2 to 4 days (2). Even though the majority of cases of adult epiglottitis are blood culture negative, intravenous antibiotic therapy should be instituted in the emergency department and must provide coverage for H inji’uenzauntil
Phil 13.Fontanarosa, S. Scott Polsky, George E. Goldman
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culture and sensitivity results and beta lactamase testing are available. Initial therapy includes either a second or third generation cephalosporin with activity against H infruenza or a combination of ampicillin and chloramphenicol(1,14,15,23,31). The role of corticosteroids in the treatment of adult epiglottitis is unclear (22,29). Steroids are advocated to reduce edema associated with epiglottitis (2,6,22,51) but, to date, there have been no controlled studies examining their effectiveness. In this study, there was no apparent relationship of steroid use or lack thereof with the need for airway support, incidence of complications, length of hospital stay, or clinical course.
Limitations of the Study Because this study was a review and analysis of patients’ records, the limitations inherent in the retrospective design, as outlined by Singer and McCabe (24), are applicable. Potentially important information and useful data may be absent or inadequately documented. For example, six patients in the study were judged by the examiner to be in “mild” respiratory distress. However, except for respiratory rate, there was no documentation of more objective parameters characterizing the distress, such as flaring of the nasal alae, intercostal retractions, use of accessory muscles, or aphonia. Similarly, the documentation of the laryngoscopic findings was variable with respect to the incidence and degree of supraglottic structure involvement. Other potentially useful data would have included specific reasons for the administration of steroids and for the choice of a particular antibiotic
regimen. These limitations are not considered to be significant and do not substantially impact the conclusion of the study. SUMMARY Epiglottitis in adults is an uncommon, increasingly recognized, but frequently misdiagnosed infection. The emergency physician should consider the disorder in every patient with sore throat and dysphagia, especially if symptoms are disproportionate to physical examination findings. Lateral cervical radiographs are helpful if positive, displaying an edematous epiglottis, but normal x-ray studies do not exclude the diagnosis. For patients with no respiratory distress, laryngoscopic examination is safe and provides diagnostic information. The majority of adults with epiglottitis may have a less fulminant clinical course than classically described, with lower incidence of bacteremia. Nonetheless, the potential for respiratory compromise is ever-present, and does not appear to be consistently related to onset of symptoms, presenting findings, or bacteriologic data. If respiratory difficulty develops, a definitive airway must be established. Tracheal intubation, ideally performed in the operating room, is preferred over tracheostomy. Medical management may be effective for adults with epiglottitis, providing there is no respiratory distress. Medical therapy should include careful monitoring in an intensive care setting, intravenous antibiotics with activity against H influenza, hydration, inhaled mist, supplemental oxygen, and possibly corticosteroids. (2,22).
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