Course Authors

Martin Carey, M.D.

Dr. Carey reports no conflict of interest.

Estimated course time: 1 hour(s).

Albert Einstein College of Medicine – Montefiore Medical Center designates this enduring material activity for a maximum of 1.0 AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

In support of improving patient care, this activity has been planned and implemented by Albert Einstein College of Medicine-Montefiore Medical Center and InterMDnet. Albert Einstein College of Medicine – Montefiore Medical Center is jointly accredited by the Accreditation Council for Continuing Medical Education (ACCME), the Accreditation Council for Pharmacy Education (ACPE), and the American Nurses Credentialing Center (ANCC), to provide continuing education for the healthcare team.

 

Learning Objectives

Upon completion of this Cyberounds^®, you should be able to:

• List the differential diagnoses of severe sore throat in the adult

• Discuss the investigation of an important cause of sore throat in the adult

• Manage the treatment of an important cause of sore throat in the adult.

 

An unusually quiet night in the ER. Your peaceful reading is suddenly disturbed by the triage nurse, who pops his head around the door and announces, "Sore throat in Room 4." You wonder whether to mention, yet again, that you dislike patients referred to in this way.

As you get to your feet to follow the nurse you hear, from across the hall, the sound of someone who clearly is having difficulty breathing. Entering the room, you see an African-American male, in his mid-thirties, sitting up on the edge of the bed. He has audible respiration, and seems to be having some trouble with swallowing - he is spitting into a kidney dish that he holds under his chin.

You approach the bed. The nurse has hooked him to the monitor -- you note a pulse rate of 120 beats per minute and a blood pressure of 115/75. His O₂ saturation is 97% on room air. You try to take a history.

In a soft voice, the patient states that he had first noted a sore throat about 24 hours earlier. Although he had slept well the previous night, the sore throat was worse on awaking, and had progressed through the day. He had noted increasing difficulty with eating, and had reverted to fluids only by the evening. He had noted little relief with over-the-counter analgesia.

The nurse has just finished taking his temperature, and reports it is 102°F via tympanic thermometer.

A quick review of the past history is negative for any significant illness. He is a non-smoker and drinks a beer a night. On no prescribed medications, the patient admits to taking over-the-counter vitamin supplements regularly. He has no allergies, manages a local fast food outlet and has never left his home state of Arkansas.

Q. Given this history, what would be your differential diagnosis at this point?

A. With the high fever and the symptom progression noted, it is probable that this represents some sort of infective process, although non-infectious causes cannot be completely excluded. The differential diagnosis must remain broad, but it would include the following conditions:

• Retropharyngeal or peri-tonsillar infections
• Infectious mononucleosis
• Diphtheria
• Ludwig's angina
• Epiglottitis
• Allergic drug reactions
• Foreign bodies
• Tumors or trauma to the larynx
• Inhalation or aspiration of toxic chemicals

We have no history suggestive of a foreign body aspiration, the history is probably too acute to be a tumor, and we have no history of toxic chemical exposure. Although a number of other systemic conditions should always be considered in the differential diagnosis of upper airway obstruction, for example, Wegener's granulomatosis, sarcoidosis, pemphigus, pemphigoid, and amyloidosis, the lack of a prolonged history or any other symptoms prior to the development of this acute episode make these conditions very unlikely.

Of the infectious etiologies, diphtheria is unlikely, as there has been no history of recent travel. The other possibilities should all be considered.

Q. Given the differential diagnosis at this stage, what you would expect to find on examination which may help with a definitive diagnosis?

A. Infectious mononucleosis is usually associated with younger patients, with up to 95% of the population displaying antibodies by the time they reach their thirties. Examination should reveal a reddened pharynx and tonsils, usually with an exudate. Retropharyngeal abscess and peri-tonsillar abscess are both usually associated with significant erythema of the oropharynx, perhaps with exudate. There may be swelling of the tissues of the back of the mouth, and a clear pointing abscess may, on occasion, be seen. Epiglottitis may present with remarkably few findings on examination of the oropharynx. Severe cases may be associated with protrusion of the tongue. One of the cardinal signs seems to be the presence of anterior neck tenderness, particularly over the larynx, or on movement of the laryngeal cartilage.

The patient described was sitting upright, exhibited dysphagia, had respiratory difficulty and his voice was muffled. Examination revealed tenderness on laryngeal movement. Chest examination was unremarkable, except for transmitted sounds. Aside from the marked sinus tachycardia, the cardiovascular examination was normal.

In view of these findings, a presumed diagnosis of epiglottitis was made. The symptoms noted in patients with epiglottitis are variable. A severe sore throat is almost universal. Dysphagia occurs in at least three-quarters of patients, fever in nearly 90%, muffling of the voice in about half. Other findings included drooling, respiratory distress, lymphadenopathy, anterior neck tenderness and hoarseness. The finding of a severe sore throat in an adult, especially if associated with anterior neck tenderness, is highly suggestive of epiglottitis.

Q. But epiglottitis is a disease of children, isn't it? How common is it in adults anyway?

A. Although epiglottitis did seem to be a disease of children, it is now much more commonly seen among adults. In the early 1980s, the ratio of children to adults with a diagnosis of epiglottitis was about 2.6 to 1. By the mid-90s, that ratio had dropped to 0.4 to 1. Despite these data, articles are still published which suggest that the disease is more common in children. It is important for emergency physicians treating adult patients to maintain a high index of suspicion for this disease.

Interestingly, epiglottitis was always considered, historically, a disease of adults, and it is only between the 50s and the 80s that it became more common in children, and was described as a childhood disease.

Q. What organisms are most often responsible for epiglottitis?

A. A wide range of organisms has been implicated in epiglottitis. In children, the commonest organism was Haemophilus influenzae. The introduction of the H 'flu vaccination is probably the reason that epiglottitis is now so rare in children. A study from Sheffield in the United Kingdom describes a sharp decline in the incidence after the introduction of the vaccine, the presence of the H flu epiglottitis only in unvaccinated children and the etiology being Streptococci in vaccinated children. It is worth noting, though, that case reports do exist of H flu epiglottitis occurring in children who have been vaccinated.

In adults, often no organism can be isolated. Blood cultures are positive in only a minority of cases. Throat cultures are positive in about a quarter of cases. Bacteria, viruses and fungi have all been implicated in the pathogenesis of the condition.

Organisms that have been isolated include:

• Haemophilus influenza and parainfluenza
• Pneumococcus
• Staphylococcus aureus
• Streptococcus
• Escherichia coli
• Klebsiella
• Neisseria
• Vibrio vulnificus

The isolation of Vibrio vulnificus from a patient with epiglottitis makes an interesting story. The patient was carrying his goldfish home in a bag, when the cigarette he was smoking somehow caught the bag on fire. In an attempt to save the fish, the person burnt his hand. He placed the wet hand in his mouth, and a day or two later developed the signs and symptoms of epiglottitis. Vibrio was isolated from a throat culture, and was the presumed cause of the infection.

The only virus proven to cause epiglottitis is Herpes simplex, although a viral etiology has been suggested for the disease, especially in mild cases, where no other organism could be identified.

In patients with pre-existing pathology, a wide range of organisms have been implicated in the development of the disease, including fungal causes, and Mycobacterium tuberculosis. Epiglottitis due to candidiasis, aspergillus and klebsiella have all been described in patients with malignant disease, or other conditions where the patient may be immunocompromised.

Q. If there is a high index of suspicion that the patient has epiglottitis, how can the diagnosis be made?

A. It is well known that in children, no attempt should be made to visualize the back of the oropharynx, or the epiglottis indirectly, because there is a risk of causing laryngeal spasm, and, thus, complete airway obstruction. In adults, however, this risk does not exist. Indirect laryngoscopy has consistently been demonstrated to be safe in adults. If an inflamed epiglottis is visualized - it is often described as looking like a strawberry - the diagnosis is made. Some departments may have access to flexible nasopharyngeal endoscopy, and this is the method of choice, if available. Radiology of the neck is often described as being needed to make the diagnosis, but, in fact, is neither very sensitive (around 40%) nor specific (about 75%) for the diagnosis.

Recently, some authors have described using CT imaging to make the diagnosis when the other investigations are not available. The CT displays thickening of epiglottitis, aryepiglottic folds false and true vocal cords. There may also be obliteration of the pre-epiglottic fat, thickening of the platysma and changes in the subcutaneous fat. As noted above, some patients cannot comfortably lie down. Thus, CT should probably only be employed in those patients in whom there is a degree of doubt about the diagnosis, who are stable and in no respiratory distress. It may also be useful in those cases where potential complications such as abscess formation are possible.

Q. The diagnosis is made. What is the initial treatment?

A. The first priority in management must be airway protection. In children, the standard of care was that the most experienced anesthetist available intubated all patients with epiglottitis, with otorhinolaryngology standing by to perform emergent tracheostomy if required. The patients were gradually sedated using inhalational agents, and then intubated once deeply sedated.

In adults, there is debate as to whether this approach is required in all cases. There is no argument that patients who have any degree of respiratory compromise should be intubated as soon as possible. Steps should be taken to ensure that the intubating physician could perform invasive airway protection, for example, an emergent criccothyrotomy, if oral intubation is unsuccessful. Patients with respiratory compromise should be intubated, as deterioration in their condition can occur rapidly and unpredictably. Although some physicians use inhalation anesthesia to sedate patients prior to intubation, others will use rapid sequence induction.

It should be mentioned that respiratory compromise can occur in epiglottitis patients who seem able to protect their airway and have no signs of respiratory compromise. There is little doubt that epiglottitis patients do need close observation in an ICU, with the means to provide a definitive airway at the bedside. However, there seems to be an increasing trend towards trying to manage these patients conservatively.

Although no organized controlled clinical trials seem to exist, management includes the use of inhaled racemic epinephrine, intramuscular epinephrine, intravenous steroids, analgesia and supplemental oxygen as required. Patients without respiratory compromise may represent patients with a 'milder' form of the disease, but it appears that those managed conservatively have a shorter length of stay, and have fewer complications than those managed with intubation. It must be stressed, though, that all patients with epiglottitis are at risk of respiratory compromise, and that this may occur rapidly and with little warning. Close observation is mandatory.

Staging systems have been developed to try and predict those patients who are likely to deteriorate. However, they have not been adopted universally. This is probably because no one sign seems to be predictive of deterioration.

Q. Given that airway protection is now assured, what antibiotic should be administered?

A. Antibiotics are required, despite the low yield of blood cultures and throat swabs. Typically, second or third generation cephalosporins are used. Agents include cefuroxime, ceftriaxone or cefotaxime. Other agents used include ampicillin-sulbactam or trimethoprim-sulfamethoxazole. All these agents should be administered intravenously. Lack of response to therapy should raise the possibility of the development of an epiglottic abscess.

Q. Should any measures be taken to prophylactically manage household contacts of a proven index case of epiglottitis?

A. If the organism isolated is proven to be H. influenzae, close household contacts of the index case should probably be managed with rifampicin. This is particularly important if there are unvaccinated contacts. The dose is 20 mg/kg/day, to a maximum of 600 mg daily, for four days. This regimen eradicates nearly 90% of carrier states.

Bonus Question

If the patient was a 25-year-old who admitted to regular use of crack cocaine, who presents acutely with signs and symptoms suggestive of epiglottitis, what is a possible mechanism for production of these symptoms?

A. Increasingly, thermal injury to the epiglottis is described in patients who smoke crack. This is because crack smokers often use small wads of wire wool in their crack pipes. These wads become very hot and can be inhaled when the pipe is sucked on. Thermal injury of the epiglottis can occur, with associated edema and possible respiratory compromise. These patients should be closely observed, and may require intubation if respiratory compromise progresses.

Who Wants to Be a Millionaire? Bonus Question

Which of the following American Presidents died of epiglottitis?

A. John Tyler
B. George Washington
C. Grover Cleveland
D. James Madison
>E. William Taft

A. George Washington is thought to have contracted epiglottitis as his terminal illness. Although his death, in December 1799, was recorded as due to this condition, the fact that his physicians bled him (as was the standard therapy of the day) of over two liters of blood in about 12 hours probably didn't help!