CASE 1: Jacob
Jacob is a 41 year-old male who presented to the Emergency Department with a sore throat. The symptoms were present for a few days. Jacob complained of throat swelling, chills, productive cough, difficulty swallowing and odynophagia. Jacob was seen in the Emergency Department two days prior with similar symptoms. The initial visit was unremarkable and symptoms improved after he was given Toradol, IV fluids and a lidocaine gargle. Jacob was diagnosed with tonsillitis and discharged home. Jacob returned to ED with worsening throat pain, inability to swallow food, difficulty clearing oral secretions as well as chills and difficulty breathing.
Examination showed that Jacob had a fever of 38.7˚ F; otherwise, the rest of the vital signs were within normal limits. Inspection of the oropharynx was unremarkable. There was no erythema or exudates noted in the oropharynx. However, Jacob had difficulty swallowing his own secretions, and was uncomfortable with ranging his neck and had right anterior adenopathy. Jacob was also noted to have a muffled voice. His lungs were clear and no stridor was noted. A lateral neck film showed mild thickening of the epiglottis. Given the clinical presentation, there was a high suspicion for epiglottitis and ENT was consulted. Epiglottitis was diagnosed after the laryngoscopy. The larynx and pharynx showed a diffusely edematous lingual surface of the epiglottis without erythema or exudates. The vocal cords could not be visualized.
CASE 2: Christopher
Christopher is a 36 year old male with a past medical history of an infected neck cyst status post incision and drainage three months prior who presented to the Emergency department for sore throat. He reported that he awoke that morning with sore throat and had trouble swallowing. Christopher denied cough, fever, runny nose or sick contacts. As the day progressed, Christopher became increasingly short of breath. He appeared uncomfortable but was able to maintain his airway. Given the clinical suspicion of epiglottitis, Christopher agreed to an examination of the larynx with a Glidescope. After the patient was anesthetized with topical Cetacaine, a swollen erythematous epiglottis was noted. ENT was consulted and a flexible endoscopic exam showed a classically beefy-red epiglottis with a strawberry appearance, verifying the diagnosis of epiglottitis. Furthermore, the epiglottis was markedly edematous, and the edema extended to the arytenoids bilaterally, but the airway was patent.
In general, patients with epiglottitis have the following findings: fever, drooling, inability to handle secretions, cervical adenopathy, muffled voice, severe palpation over the larynx, and mild cough.
Epiglottitis can be a life-threatening condition caused by an acute inflammation of the epiglottis, vallecula, arytenoids, and aryepiglottic folds. Once thought to be a disease of children, the introduction of the Haemophilus influenzae vaccine as part of childhood immunization has made epiglottitis less common at 0.6 per 10000010 in the pediatric population. This decrease in incidence, however, is not observed in adults.12 Prior to the introduction of the vaccine, the ratio of incidence in children to adults was 2.6:1.1 In 1993 this ratio has decreased to 0.4:1; therefore, making the disease more prevalent in adults.1 The incidence in the adult population ranges from 0.97 to 3.1 in 100000 per year,3 with a mortality of 7%.4 In contrast, the mortality in children with epiglottitis has decreased to <1%.1
Mortality in the adult population can be attributed to sudden airway obstruction and difficulty in intubation of patients with extensive swelling of these structures. Extenuating factors may also be attributed to the decreased awareness of epiglottitis. Many adult patients with epiglottitis may be misdiagnosed and fail to have appropriate treatment initiated.4 As illustrated in the two cases above, it may be easy to disregard these patients on initial presentation.
The above cases demonstrate (1) how adults with epiglottitis may present; and (2) how a high index of suspicion eventually led to a correct diagnosis. In general, patients with epiglottitis have the following findings: fever, drooling, inability to handle secretions, cervical adenopathy, muffled voice, severe palpation over the larynx, and mild cough. Late findings may include stridor, respiratory distress or a toxic appearance. Sore throat and dysphagia, often out of proportion to the exam, are each seen about 95% of adult cases of epiglottitis.1 A muffled voice may only be seen in 54% of adult cases of epiglottitis.1 Fever may also be part of the presentation in 80% of adults.5 The average age of presentation for adults is 45 years.1 In adults, epiglottitis is more prevalent in the male population, with a male to female ratio of 3:1.1 Moreover, abscess formation and extrapharyngeal complications are more common in adults than in children.2
The onset of symptoms in adults tends to be more gradual and less fulminant. Most children are seen within 12 hours of symptom onset, while symptom duration can range from 2 hours to 10 days in adults,5 with an average of 2-3 days.2 The onset and progression of symptoms is rapid in children and often involve respiratory system. On the contrary, respiratory symptoms can be as seen in as few as 20 to 33% of adults, which may be attributed to the larger, more rigid structure of the adult larynx. There is usually airway compromise if an adult presents within 12 hours of symptom onset, while compromise is only seen in 41% of those adults with symptom onset longer than 12 hours prior to presentation.
Moreover, if presentation is greater than 24 hours after symptom onset, no intubation has been required.5 The rapid appearance of respiratory symptoms in adults is ominous for a more serious illness; and those that present with an upright posture and drooling correlates with a higher percentage requiring airway intervention.5
Mechanism of obstruction
Sudden fatal obstruction may occur in both adults and children. Several factors may contribute to sudden airway obstruction. The first factor is laryngeal obstruction from swelling. As the epiglottis swells, it curls posteriorly and forms a horseshoe shape that obstructs the airway. Edema of the aryepiglottic folds may also contribute to this process. Furthermore, inspiration draws these swollen structures downward and increases the degree of obstruction. Counter-intuitively, there is a lack of correlation between the size of the epiglottis and the degree of airway difficulty.2 Other factors that may be involved may be the rigidity of an inflamed epiglottis and supraglottic structures, which may prevent the epiglottis from keeping secretions out of the trachea. Subsequently, the secretions spilling into the vocal cords may precipitate laryngeal spasm or may contribute to airway obstruction.2 The position of the patient is also a factor for respiratory obstruction. Patients with airway obstruction find a position to maximize the ability to inhale and minimize airway resistance. Lying down may precipitate airwa
y obstruction. Finally, fatigue may contribute to what ultimately becomes respiratory arrest.
Diagnosis of epiglottitis involves both clinical and microbiologic approaches. The clinical diagnosis is based on history and physical exam. The history involves the presentation stated above. Part of the clinical diagnosis involves radiography and visualization. In the past, it was thought that a lateral soft tissue neck is sufficient for ruling out epiglottitis. However, its reliability, sensitivity and specificity have recently come in to question. It is noted that only 79% of cases are diagnosed with the lateral neck x-ray.6 The classical lateral neck radiograph findings are swollen epiglottis (thumb sign), thickened aryepiglottic folds and obliteration of the vallecula. Epiglottitis can be ruled out if the epiglottic width is less than 8mm (normal 3-5mm) and the aryepiglottic width is less than 7mm.5 Another proposed approach is to note the ratio of the width of the epiglottis to the anteroposterior width of the C4 vertebral body, which should not be greater than 0.33. This accounts for the wide variability in size of the soft tissue structures in the supraglottic region, and has a sensitivity and specificity of 96% and 100%, respectively.11 Laryngoscopy is noted to be the most sensitive and specific test for epiglottitis. Direct visualization may reveal a swollen, inflamed or pale, horseshoe-shaped epiglottis. The aryepiglottic folds are usually inflamed or edematous as well. Although noted to precipitate laryngeal spasm in children, there have been no reports of adults having spasms as a result of laryngoscopy.5
The microbiologic approach is important to ensure that the organism is sensitive to the antibiotic regimen being used. Pharyngeal swabs are inadequate; therefore, blood cultures should be utilized. If the cause is bacterial, adults have a more diverse microbiology and etiology for epiglottitis. Type B Hemophilus Influenzae type B (Hib) is almost always the cause in children, but it is only found in as few as 17% of adults.7 However, in adult cases where Hib is identified as the pathogen, the disease tends to be more severe. Other pathogens in adults include haemophilus parainfluenzae, streptococcus pneumoniae and group A streptococci. The largely negative blood cultures in adults suggest either a mechanical or viral etiology.7 Other non-infectious causes are thermal, caustic foreign body, or chemotherapy induced injuries.12
Successful treatment requires an early recognition of epiglottitis in adults. Up to 67% of adults could be misdiagnosed on their initial visit and may not receive appropriate therapy.5 Unlike in children, where the standard of practice is to intubate and protect the airway immediately regardless of the clinical condition;2 this approach is more controversial in adults. It is thought by some that adult epiglottitis is less fulminant, and many cases could be managed medically if airway obstruction is minimal or absent.2 However, there are some who think that sudden airway obstruction may occur in adults; and this could occur with minimal initial clinical findings, and that it is difficult to tell which patients would obstruct. Thus, some favor immediate airway intervention regardless of the clinical condition. Whatever the approach, all diagnosed patients should be admitted to the hospital and carefully monitored in an ICU setting.
If there is any indication of rapidly progressing symptoms and deterioration while in the ICU or ED, an airway must be established. Clinical deterioration occurs within hours. Signs of impending airway compromise include stridor, hoarseness, tripod position, and dyspnea. It would be inappropriate to continue an observational approach for these patients without endotracheal intubation because a seemingly decrease or improvement may indicate a worsening occlusion or fatigue rather than recovery. Roughly, no more than 10 to 30% of patients will require airway intervention.5 Nevertheless, if a definitive airway is needed, the mean duration of airway support has been reported to only be 40-60 hours.8
The mainstay of medical therapy is appropriate antibiotics. Medical therapy is appropriate in patients with a more insidious onset of symptoms without respiratory symptoms at presentation, and can be carefully monitored. Adult epiglottitis, as previously mentioned, is caused by a variety of organisms. Intravenous antibiotics should be initiated immediately and should cover for H influenzae, staph aureus, streptococcus and pneumococcus. Appropriate antibiotics would be ampicillin or a third generation cephalosporin, the preferred first-line agent.1 The regimen can be narrowed as soon as the sensitivities are available. Response to adequate antibiotic coverage is usually rapid; and prolonged toxicity may suggest an epiglottic abscess has formed.2 Complimentary treatment includes hydration, electrolyte and airway monitoring. There are anecdotal reports of benefits from the use of humidified oxygen, inhaled vasoconstrictors (racemic epinephrine), and steroids.5 While there are no case reports in the benefit of steroids, it presumably decreases the inflammatory response that may be responsible for airway obstruction.5
In patients without respiratory symptoms on presentation, mortality is very low; and in those with respiratory symptoms, it could be 21.7% and 14.3% in intubated patients.9 Overall, mortality in adults is also about 7%, as opposed to children which is <1%.1 Extraepiglottic infections have been reported and include pneumonia, and empyema, pericarditis, meningitis, epiglottic abscess, neck cellulites and noncardiogenic pulmonary edema. Case Resolution
Case 1: Decadron and ceftriaxone were started in the ED. Jacob was admitted to a stepdown unit for airway monitoring. Repeat laryngoscopy the morning after admission showed improvement, but not complete resolution. Antibiotics were eventually changed to augmentin ES PO BID to complete a 10 day course. Pt completed three doses of IV Decadron. On the day of discharge, Jacob denied any trouble swallowing, dyspnea, chest pain, vomiting, diarrhea, or fever..
Case 2: Christopher was admitted to the SICU for treatment and airway monitoring. He was given prednisone 60 mg po, ceftriaxone 2g IV, clindamycin 600 mg IV, decadron 10 mg IV, and racemic epinephrine in the ED. In the SICU, Christopher remained stable and there were no signs or symptoms or respiratory distress, no stridor. Antibiotics and steroids were continued. Christopher was eventually observed on the floor and started on Avelox per ID recommendations. Serial flexible laryngoscopy was performed during admission until improvement of epiglottic swelling was seen.
Although less prevalent in children, epiglottitis should still be part of the differential diagnosis in adults. The diagnosis of the disease is dependent on a high index of suspicion, clinical findings and microbiologic findings. Unlike in children, the disease in adults is less fulminant, has a diverse etiology and is less likely to need aggressive intervention. Treatment for the majority of adults with epiglottitis is careful observation and antibiotics. The key is to have the suspicion.
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