American 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 Center,
Seattle, WA.

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. [1] His is probably the first recorded death from epiglottitis. Roland of Palma is reported to have described an epiglottic abscess in the 13th century. [2] Theisen [3] 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. [4] Epiglottitis was considered ``rare in children.'' [5] 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, [6] [7] 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. [8] The mortality rate for children is now less than 1%, but the mortality in adults is in the range of 6% to 7%, [9] with series reporting between 0 and 20% mortality rates. [10] [11] [12] 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. [13] [14] [15] [16] 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).

 

EPIDEMIOLOGY

The incidence of epiglottitis in adults is about 1/100,000 per annum. [9] [12] [17] The average age is between 42 [18] and 47 [12] years. Kass et al [19] have noted an increase in cases occurring during the summer months, though others have not been able to confirm this. [9] [18] Male to female ratios of between 1.8:1 and 4:1 have been reported. [12] [20]

 

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.'' [21] In an analysis of 158 cases from the literature, Khilanani and Khatib [22] 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. [16] [23] [24] [25] The signs include lymphadenopathy, drooling, and respiratory distress. [16] Gentle palpation of the larynx is frequently extremely painful, which should immediately raise the suspicion of epiglottitis. [13] A recent study by Frantz et al [12] 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.

 

INVESTIGATION

In adults, in distinct contrast to the situation in children, the use of indirect laryngoscopy to visualize the epiglottis is considered a safe procedure. [24] When available, the flexible nasoendoscope has been described as the method of choice for investigation in the ED. [26] There is little to be gained from radiology of the soft tissues of the neck . Stankiewicz and Bowes [27] reported a sensitivity of 38% and a specificity of 76% for diagnosing epiglottitis from a radiograph of the lateral soft tissues of the neck.

 

DIFFERENTIAL DIAGNOSIS

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. [16] Conversely, allergic drug reactions, [28] angioneurotic edema, foreign bodies, tumors or trauma of the larynx, reflex laryngospasm, and inhalation and aspiration of toxic chemicals such as hydrocarbons [25] 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. [29] Morton and Barr [30] reported a case of hyperventilation mimicking the signs of acute epiglottitis. Epiglottitis has been related to the use of crack cocaine [31] [32] ; 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.

 

MICROBIOLOGY

A variety of microorganisms have been implicated in epiglottitis [7] [12] [33] [34] [35] [36] [37] [38] [39] [40] [41] [42] [43] [44] [45] [46] [47] [48] [49] [50] [51] [52] (Table 1) . In the majority of cases, however, no definite organism can be identified. [12] [15] [21] 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. [6] A viral etiology has been postulated for some cases of adult epiglottitis, especially the milder cases. [21] However, of the viruses, only herpes simplex has been positively identified on histology. [48] Anaerobic organisms are major constituents of the microflora of the upper respiratory tract. However, they have rarely been reported as causing epiglottitis in adults. [41]

 

TABLE 1 -- Organisms Responsible for Epiglottitis in Adults

Haemophilus influenzae

H parainfluenzae

Pneumococcus

Staphylococcus aureus

beta-Hemolytic streptococcus

Streptococcus pyogenes

S milleri

S viridans

Escherichia coli

Bacteroides melanogenicus

Klebsiella pneumoniae

Neisseria spp

Kingella kingae

Vibrio vulnificus

Serratia marcescens

Pastuerella multocida

Citrobacter diversus

Branhamella catarrhalis

Aspergillus

Candida albicans

Herpes simplex

Mycobacterium tuberculosis


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. [50] Candida epiglottitis was the presenting illness for a patient with acute myelogenous leukemia [49] and has been described in other patients immunocompromised as a result of chemotherapy. [53] A study by Farley et al [54] 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), [55] alcoholism, steroid use, use of immunosuppressive drugs, history of splenectomy, and malignancy. Lederman et al [36] 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.

 

TREATMENT

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. [18] The presence of a tachycardia out of proportion to other symptoms is considered to be an early indicator of potential airway compromise and hypoxemia. [56] The treatment of adults without a rapidly progressive history and without significant respiratory compromise is controversial. Friedman et al [57] 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. [58]

 

Antibiotics should be administered urgently. A second- or third-generation cephalosporin, active against ampicillin-resistant

 

Haemophilus influenzae, is the drug of choice. [59] A combination of ampicillin and chloramphenicol was previously recommended. [23] The proportion of Haemophilus influenzae resistant to ampicillin varies by institution and may be as high as 36%. [54]

 

A number of authors advise the use of steroids, but there are no controlled trials that prove any benefit from this therapy. [12] [59] Rivron and Murray [60] 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. [14] [61] A rapid-sequence induction is recommended by some, with endotracheal intubation initially attempted, followed by cricothyrotomy if unsuccessful. [57] A proportion of adults with epiglottitis can be managed conservatively with very close observation in the intensive care unit, without intubation. [13] [62] There are case reports of sudden respiratory compromise occurring, including cases in patients who appeared to be stable or even improving. [57] [63] [64] 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, [65] but the emergency procedure is associated with a complication rate five times greater than that of elective surgical airway intervention. [66] The reported complication rates vary between 6% and 39%. [67] [68] The immediate complications of the procedure include hemorrhage, mediastinal or subcutaneous emphysema, and tube misplacement. [65] [69] 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. [69] 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. [55] 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. [70] [71] 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. [72] [73] In these cases the patient should definitely be evaluated for underlying disorders, such as collagen vascular disease, sarcoidosis, or occult malignancy.

 

PREVENTION

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, [74] and vice versa, [75] 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. [76] 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. [77]

 

CONCLUSION

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.

 

REFERENCES

1. Lewis FO: Washington's last illness. Ann Med History 1932;4:245-248

2. Howard RE: Laryngeal abscess. Laryngoscope 1931;41:344-347

3. Theisen CF: Angina epiglottidea anterior. Report of three cases. Albany Med Ann 1900;21:395-405

4. Brown JM: Acute infections of the epiglottis. Arch Otolaryngol 1940;32:631-641

5. Lederman MD: Acute phlegmonous epiglottiditis. Laryngoscope 1914;24:25-32

6. Takala AK, Petola H, Eskola J: Disappearance of epiglottitis during large scale vaccination with Haemophilus influenzae Type B conjugate vaccine among children in Finland. Laryngoscope 1994;104:731-735

7. Gorelick MH, Baker MD: Epiglottitis in children, 1979 through 1992. Effects of Haemophilus influenzae type b immunization. Arch Pediatr Adolesc Med 1994;148:47-50

8. Frantz TD, Rasgon BM: Acute epiglottitis: Changing epidemiological patterns. Otolaryngol Head Neck Surg 1993;109:457-460

9. Mayo-Smith MF, Hirsch PJ, Wodzinski SF, Schiffman FJ: Acute epiglottitis in adults: an eight-year experience in the State of Rhode Island. N Engl J Med 1986;314:1133-1139

10. Mustoe T, Strome M: Adult epiglottitis. Am J Otolaryngol 1983;4:393-399

11. Carenfelt C, Sobin A: Acute infectious epiglottitis in children and adults: Annual incidence and mortality. Clin Otolaryngol 1989;14:489-493

12. Frantz TD, Rasgon BM, Quesenberry CP: Acute epiglottitis in adults. Analysis of 129 cases. JAMA 1994;272:1358-1360

13. Andreassen UK, Baer S, Nielsen TG, et al: Acute epiglottitis--25 years experience with nasotracheal intubation, current management policy and future trends. J Laryngol Otol 1992;106:1072-1075

14. Crosby E, Reid D: Acute epiglottitis in the adult: Is intubation mandatory? Can J Anaesth 1991;38:914-918

15. Sheik KH, Mostow SR: Epiglottitis--an increasing problem for adults. Western J Med 1989;151:520-524

16. Fontanarosa PB, Polsky SS, Goldman GE: Adult epiglottitis. J Emerg Med 1989;7:223-231

17. Wurtele P: Acute epiglottitis in children and adults: A large scale incidence study. Otolaryngol Head Neck Surg 1990;103:902-908

18. Barrow HN, Vastola AP, Wang RC: Adult supraglottitis. Otolaryngol Head Neck Surg 1993;109:474-477

19. Kass EG, McFadden EA, Jacobsen S, Toohill RJ: Acute epiglottitis in the adult: Experience with a seasonal presentation. Laryngoscope 1993;103:841-844

20. Shih L, Hawkins DB, Stanley RB: Acute epiglottitis in adults: A review of 48 cases. Ann Otol Rhinol Laryngol 1988;97:527-529

21. Shapiro J, Eavey RD, Baker AS: Adult supraglottitis: A prospective analysis. JAMA 1988;259:563-567

22. Khilanani U, Khatib R: Acute epiglottitis in adults. Am J Med Sci 1984;287:65-70

23. Cohen EL: Epiglottitis in adults. Ann Emerg Med 1984;13:620-623

24. Denholm S, Rivron RP: Acute epiglottitis in adults: A potentially lethal cause of sore throat. J R Coll Surg Edinb 1992;37:333-335

25. Stair TO, Hirsch BE: Adult supraglottitis. Am J Emerg Med 1985;3:512-518

26. Cox GJ, Bates GJ, Drake-Lee AB, Watson DJ: The use of flexible nasoendoscopy in adults with acute epiglottitis. Ann R C Surg Engl 1988;70:361-362

27. Stankiewicz JA, Bowes AK: Croup and epiglottitis: A radiologic study. Laryngoscope 1985;95:1159-1160

28. Luetzow TJ: Complications in the use of prochlorperazine. Wis Med J 1991;90:64-65

29. Lerner DM, Deeb Z: Acute upper airway obstruction resulting from systemic diseases. Southern Med J 1993;86:623-627

30. Morton NS, Barr GW: Stidor in an adult. Anaesthesia 1989;44:232-234

31. Sataloff RT: Upper airway distress and crack cocaine use. Otolaryngol Head Neck Surg 1994;111:115 (letter)

32. Savitt DL, Colagiovanni S: Crack cocaine-related epiglottitis. Ann Emerg Med 1991;20:322-323 (letter)

33. Key SN: Angina epiglottidea anterior: Report of a case caused by the bacillus influenzae. JAMA 1916;67:116

34. Takala AK, Eskola J, van Alphen L: Spectrum of invasive Haemophilus influenzae type b disease in adults. Arch Intern Med 1990;150:2573-2576

35. Trollfors B, Brorson JE, Claesson B, Sandberg T: Invasive infections caused by Haemophilus species other than Haemophilus influenzae. Infection 1985;13:12-14

36. Lederman MM, Lowder J, Lerner PI: Bacteremic pneumococcal epiglottitis in adults with malignancy. Am Rev Respir Dis 1982;125:117-118

37. Shalit M, Gross DJ, Levo Y: Pneumococcal epiglottitis in systemic lupus erythematosus on high-dose corticosteroids. Ann Rheum Dis 1982;41:615-616

38. Glock JL, Morales WJ: Acute epiglottitis during pregnancy. Southern Med J 1993;86:836-838

39. Rothstein SG, Persky MS, Edelman BA, et al: Epiglottitis in AIDS patients. Laryngoscope 1989;99:389-392

40. Chong WH, Woodhead MA, Millard FJ: Mediastinitis and bilateral thoracic empyemas complicating adult epiglottitis. Thorax 1990;45:491-492

41. Devita MA, Wagner IJ: Acute epiglottitis in the adult. Crit Care Med 1986;14:1082-1083

42. Stanley RE, Liang TS: Acute epiglottitis in adults (The Singapore experience). J Larygol Otol 1988;102:1017-1021

43. Berthiaume JT, Pien FD: Acute Klebsiella epiglottitis: Considerations for initial antibiotic coverage. Laryngoscope 1982;92:799-800

44. Kennedy CA, Rosen H: Kingella kingae bacteremia and adult epiglottitis in a granulocytopenic host. Am J Med 1988;85:701-702

45. Mehtar S, Bangham L, Kalmanovitch D, Wren M: Adult epiglottitis due to Vibrio vulnificus. BMJ 1988;296:827-828

46. Parment PA, Hagberg L: Fatal Serratia marcescens epiglottitis in a patient with leukaemia. J Infect 1987;14:280 (letter)

47. Stuart MJ, Hodgetts TJ: Adult epiglottitis: Prompt diagnosis saves lives. BMJ 1994;308:329-330

48. D'Angelo AJ, Zwillenberg S, Olekszyk JP, et al: Adult supraglottitis due to herpes simplex virus. J Otolaryngol 1990;19:179-181

49. Cole S, Zawin M, Lundberg B, et al: Candida epiglottitis in an adult with acute nonlymphocytic leukemia. Am J Med 1987;82:662-664

50. Bolivar R, Gomez LG, Luna M, et al: Aspergillus epiglottitis. Cancer 1983;51:367-370

51. Vernham GA, Crowther JA: Acute myeloid leukaemia presenting with acute Branhamella catarrhalis epiglottitis. J Infect 1993;26:93-95

52. Navarrete ML, Quesada P, Garcia M, Lorente J: Acute epiglottitis in the adult. J Laryngol Otol 1991;105:839-841

53. Walsh TJ, Gray WC: Candida epiglottitis in immunocompromised patients. Chest 1987;91:482-485

54. Farley MM, Stephens DS, Brachman PS, et al: Invasive Haemophilus influenzae disease in adults. Ann Intern Med 1992;116:806-812

55. Rothstein SG, Persky MS, Edelman BA, et al: Epiglottitis in AIDS patients. Laryngoscope 1989;99:389-392

56. Deeb ZE: Acute epiglottitis in adults. JAMA 1995;273:920 (letter)

57. Friedman M, Toriumi DM, Grybauskas V, Applebaum EL: A plea for uniformity in the staging and management of adult epiglottitis. Ear Nose Throat J 1988;67:873-880

58. Dort JC, Frohlich AM, Tate RB: Acute epiglottitis in adults: Diagnosis and treatment in 43 patients. J Otolaryngol 1994;23:281-285

59. Baker AS, Eavey RD: Adult supraglottitis (epiglottitis). N Engl J Med 1986;314:1185-1186

60. Rivron RP, Murray JA: Adult epiglottitis: Is there a consensus on diagnosis and treatment? Clin Otolaryngol 1991;16:338-344

61. Love JB, Phelan DM, Runciman WB, et al: Acute epiglottitis in adults. Anaesth Intens Care 1984;12:264-269

62. Arndal H, Andreassen UK: Acute epiglottitis in children and adults. Nasotracheal intubation, tracheostomy or careful observation? Current status in Scandinavia. J Laryngol Otol 1988;102:1012-1016

63. Gerrish SP, Jones AS, Watson DM, Wight RG: Adult epiglottitis. BMJ 1987;295:1183-1184

64. Mayo-Smith M: Fatal respiratory arrest in adult epiglottitis in the intensive care unit. Chest 1993;104:964-965

65. Clancy MJ: A study of the performance of cricothyroidotomy on cadavers using the Minitrach II. Arch Emerg Med 1989;6:143-145

66. Esses BA, Jafek BW: Crycothyroidotomy: A decade of experience in Denver. Ann Otol Rhinol Laryngol 1987;96:519-524

67. McGill J, Clinton JE, Ruiz E: Cricothyrotomy in the emergency department. Ann Emerg Med 1982;11:361-364

68. Erlandson MJ, Clinton JE, Ruiz E, Cohen J: Cricothyrotomy in the emergency department revisited. J Emerg Med 1989;7:115-118

69. Burkey B, Esclamado R, Morganroth M: The role of cricothyrotomy in airway management. Clin Chest Med 1991;12:561-571

70. Lang SA, Duncan PG, Shephard DA, Ha HC: Pulmonary oedema associated with airway obstruction. Can J Anaesth 1990;37:210-218

71. Sternbach GL, Goldschmid D: Adult respiratory distress syndrome associated with adult epiglottitis. J Emerg Med 1993;11:23-26

72. Guss DA, Jackson JE: Recurring epiglottitis in an adult. Ann Emerg Med 1987;16:441-444

73. Wilson JF, Coutras S, Tami TA: Recurrent adult acute epiglottitis: the role of lingual tonsillectomy. Ann Otol Rhinol Larygol 1989;98:602-604

74. Glode MP, Halsey NA, Murray M, et al: Epiglottitis in adults: Association with Haemophilus influenzae type b colonization and disease in children. Pediatr Infect Dis 1984;3:548-551

75. Takala AK, Eskola J, van Alphen L: Spectrum of invasive Haemophilus influenzae type b disease in adults. Arch Int Med 1990;150:2573-2576

76. Vaught WW: Oxygen mask acquired supraglottitis. Arch Otolaryngol Head Neck Surg 1989;115:394 (letter)

77. Jadavji T, Cheung R, Bannatyne RM, Prober CG: Rifampicin alone or with trimethoprim for contacts of children with Haemophilus influenzae type b infections. Can Med Assoc J 1986;135:328-331