Medical Management of Epiglottitis

Epiglottitis is defined as inflammation of the epiglottis and/or neighboring supraglottic structures including the hypopharynx. Epiglottitis is typically caused by a bacterial infection. Historically, epiglottitis has been an infection mostly prevalent in children ages 2 to 6 years old. However, since the introduction of the Haemophilus influenzae B (HiB) vaccine, there has been a shift with increasing incidence within the adult population. Common signs and symptoms upon clinical presentation include odynophagia, dysphagia, use of the tripod position, and stridor. Consultation of airway specialists including otolaryngologists, anesthesiologists, or intensivists is imperative so that early interventions can be pursued if necessary. Generally, it is recommended that patients be observed in a high acuity, closely monitored environment, such as an intensive care unit (ICU), until signs and symptoms resolve. The following is a case report involving a 63-year-old female who presented with epiglottitis, which required medical management and emergent procurement of a surgical airway.

CASE PRESENTATION

The patient was a 63-year-old African-American female with a past medical history of hepatitis C with liver cirrhosis, hypertension, gastroesophageal reflux, osteoarthritis of the lumbar spine, alcohol addiction, and obesity. Her reported medications included rifaximin 550 mg twice daily, acetaminophen 650 mg twice daily, losartan 25 mg daily, hydrochlorothiazide 25 mg daily, and ranitidine 150 mg twice daily. The patient reported allergies to sulfamethoxazole/trimethoprim and lisinopril. The patient had a height of 165 cm (5′5″), a weight of 89.8 kg (198 lb), and a body mass index of 32.95 kg/m². Her social history included alcohol abuse and 27-pack per year smoking history. She presented to the emergency department (ED) at approximately 3:00 pm with complaints of a sore throat that had reportedly started the previous evening. Her detailed complaint consisted of a bilateral sore throat, which was more severe on the left size and radiated to her left ear. The patient had attempted to take acetaminophen the night before but was unable to actually swallow the pills secondary to persisting dysphagia. Upon initial intake, the patient was noted to be afebrile, despite reporting the presence of a fever while at home. Physical examination in the ED revealed the notable presence of mild erythema involving the patient's left temporomandibular joint without swelling or drainage. There was no evidence of vocal changes or trismus, her tonsils were not appreciable, and the posterior oropharynx was normal in appearance. The patient was also noted to be in mild emotional distress with noted anxiety regarding the severity of her sore throat. There were no appreciable signs of swelling upon examination of the patient's head or neck, nor any tenderness to palpation. The patient did not display any signs or symptoms suggestive of labored breathing, stridor, or respiratory distress at the time of initial presentation.

The ED course included a computed tomography (CT) scan of her head and neck to evaluate for a deep space infection as the patient was adamant that she was unable to tolerate swallowing anything, including her own saliva. The official read of the CT included the following statement, “a lesion protruding out into the hypopharynx and piriform sinus region on the left side. There are small air bubbles nearby. Theoretically this could be related to infection. Might be related to an infected diverticulum. It is not a classic tumor but is certainly possible” (Figure 1). A rapid streptococcus test was run to assess for streptococcal pharyngitis but was negative. A complete blood count was drawn producing results all within normal ranges.

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The decision was made to admit the patient at roughly 9:00 pm for observation and to obtain an ear, nose, and throat (ENT) consult as she was symptomatic and unwilling to be discharged at that time. Overnight, she continued to deteriorate with continued complaints of difficulty swallowing and increasing pain. At 4:50 am it was noted that the patient's peripheral oxygen saturation (SpO₂) had dropped to 88% on room air; supplemental oxygen was administered via nasal cannula at a rate of 2 l/min producing a modest improvement of the patient's SpO₂ to 94%.

At 7:50 am a resident from the admitting medicine team examined the patient at bedside during pre-rounds. Of note, the patient was able to walk around with mild work of breathing. However, upper airway stridor was noted when in close proximity to the patient, which had not been noted in any previous examinations.

At 9:45 am the entirety of the medicine team rounded on the patient, and it became apparent that the patient was now experiencing significant respiratory distress. She was no longer able to speak above a whisper and unable to open her mouth more than 1 cm due to severe pain. However, there was still no appreciable edema noted at this time. Stridor was audible when entering the room, and the patient was seated in the tripod position with increased work of breathing and shortness of breath. The decision was made to administer intravenous (IV) methylprednisolone 125 mg and nebulized racemic epinephrine. An ICU attending was called to the patient's room where he immediately advocated the patient be transferred to the ICU. The ICU attending noted that the airway was unsafe to attempt intubation and that an emergent tracheostomy was needed. The ENT attending was notified immediately.

When the ENT team arrived in the ICU, an examination of the airway was performed via awake flexible fiberoptic laryngoscopy using topical/local anesthesia only. Severe edema and erythema of the epiglottis was noted upon visualization of the airway anatomy. A “minimal airway” was noted, and the larynx was unable to be visualized due to the substantial edema. The mass or lesion, which was previously noted on the CT scan, clinically measured 1.6 × 1.4 cm. The diagnosis of epiglottitis was subsequently made by the ENT attending; however, epiglottic surface cultures were not taken due to the urgency of the situation. Immediately following the clinical assessment and diagnosis, the patient was taken to the operating room where an emergent awake tracheostomy was performed under local anesthesia without complications. She returned to the ICU for close postoperative monitoring where she remained on supplemental O₂ overnight. IV dexamethasone 4 mg every 8 hours and IV ceftriaxone 2 g daily were started.

The patient was monitored in the ICU for 48 hours after the tracheostomy during which time she remained afebrile. A repeat CT scan was obtained, which confirmed correct placement of the tracheostomy and found extensive upper lung opacification, indicative of new-onset pneumonia (Figure 2). Thickening of the hypopharyngeal soft tissues was present bilaterally but noted to be more prominent on the left, measuring 11 mm, which was less prominent than noted on the prior CT scan. The epiglottis at 4 mm was noted to be thicker than on the initial scan, which was 2 mm, both measured in the antero-posterior view. The patient's symptoms, such as difficulty and pain with swallowing, started to resolve, and the patient was evaluated by the speech and language pathologist for swallowing and placement of a Passy-Muir speaking valve to facilitate speech. Repeat flexible nasolaryngoscopy was performed on postoperative day 5 and revealed resolving edema and erythema of the epiglottis, which was anatomically within normal limits, as well as a resolving exudate present overlying the arytenoids. It was also noted that there was paralysis of the left true vocal cord in the paramedian position with a good airway, meaning cord paralysis did not occur in the adductor position, and partial approximation of the cords. No signs of soft tissue obstruction were noted. Three days later, the tracheostomy was subsequently capped and planned for decannulation. The patient was discharged 2 days later with a prescription of ceftriaxone to complete her 10-day course as well as a dexamethasone taper and a plan to decannulate after the completion of her antibiotic treatment.

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EPIDEMIOLOGY

Epiglottitis is defined as inflammation of the epiglottic or adjacent supraglottic structures including the hypopharynx. If left untreated, the progression of epiglottitis and the resulting edema can be devastating leading to complete or partial airway obstruction. While there are several causes of epiglottitis, the most common is bacterial infection. In the pediatric population, this bacterial infection is typically seen in children ages 2 to 6 years old; incidence of epiglottitis in children due to bacterial infection has decreased by as much as 90% since the introduction of the HiB vaccine.^1–4 By comparison, the annual incidence epiglottitis due to bacterial infection in adults is approximately 1 to 4 in 100,000, which is 2.5 times the incidence in children.^5–7 The male to female ratio of infection ranges from 1.2 to 4.9 : 1. Thirty-five to fifty percent of cases of adult epiglottitis cases initially go misdiagnosed.⁵ In the case presented here, for example, the initial thought was that the patient had strep throat, and rapid step test was negative. In adults, concomitant chronic diseases such as hypertension and chronic obstructive respiratory diseases increase the risk for epiglottitis, and cigarette smokers are more commonly afflicted.^5,7 Mortality is estimated at 4 to 7% in adults and 2 to 3% in children for all cases of epiglottitis.⁸ The higher degree of mortality for adults is likely primarily related to initial misdiagnosis as commonly noted with adult cases. Failure to intervene prior to loss of the airway carries a six-fold increase in mortality.¹ Increased risk of mortality from epiglottitis include advanced age and male sex.⁹ Other bacterial causes of epiglottitis include Streptococcus pneumonia, Streptococcus pyogenes, Staphylococcus aureus, Streptococcus viridans, and Neisseria meningitides.⁶ Surface cultures of the epiglottis and supra glottic structures are typically negative, and blood cultures are not completely sensitive for identifying pathogens, especially when antibiotics have already been administered.⁶

CLINICAL PRESENTATION

When a patient presents with anxiety, sore throat, dysphagia, muffled voice, respiratory distress, stridor, or posturing in the tripod position, there should be high suspicion for an upper airway infection and attempts should be made to secure the airway prior to obtaining any sort of invasive clinical (ie, laryngoscopy) or radiographic diagnostic evaluation.^1,9 The tripod position is demonstrated when the patient is sitting upright with their neck extended, using the arms to support their trunk, and maximal jaw thrust effort in an attempt to open the airway. Epiglottitis is typically seen more acutely in young children with symptoms occurring less than 24 hours before admission when compared with adolescents and adults who tend to present 1 to 2 days after the onset of symptoms.^10,11 Adults are more likely to present with sore throat, dysphagia, and odynophagia (painful swallowing that occurs in nearly 100% of cases).^5–8 The difference in obstructive incidence is likely due to the anatomical structures of the adult, which are more rigid and less likely to gravitationally and mechanically obstruct the glottic opening.⁵ Disproportional severity of the sore throat symptoms combined with the physical findings of the oropharyngeal examination are an additional indicators suggestive of epiglottitis. Stridor is generally a late sign and indicates that the airway is partially obstructed.^2,12

Complications can occur other than impending airway obstruction. In such a scenario it is possible to have complications caused by treatment such as allergic reaction to medications. An additional complication of epiglottitis is the formation of an epiglottic abscess, which increases mortality to 30%.⁵

DIAGNOSIS

Deterioration can occur rapidly and quite unexpectedly; therefore, early involvement of airway specialists is imperative. Specialists that warrant consideration for consultation can include: otolaryngologists (ENT), anesthesiologists, or intensivists. Although their involvement may ultimately prove to be unnecessary, early consultation may provide sufficient time for securing the airway under more ideal clinical conditions.¹³ Definitive diagnosis can be made during nasolaryngoscopy, typically utilizing a flexible fiberoptic scope, performed in the emergency room for a compliant patient, but should be delayed for a child until general anesthesia has been attained as the added stress of the procedure could increase the risk of airway loss.¹⁴ If a cherry red edematous epiglottis is visualized during the nasolaryngoscopy, a diagnosis of epiglottitis can be made. Immediate steps should be taken to secure the patient's airway either by intubation or placement of a tracheostomy.¹² If intubation is deemed necessary, pressing down on the patient's chest may allow for an air bubble to form in the glottic opening, which may aid in the placement of the endotracheal tube. Prophylactic intubation should be strongly considered in all pediatric patients found to have epiglottitis for airway protection as the clinical progression can be unpredictable and has the potential to evolve quickly.^7,12 In adult patients, some authors advocate for early prophylactic intubation in all cases, whereas others believe that a more conservative approach can be taken in adults due to the larger diameter of the adult larynx.¹⁰

Although obtaining radiographic studies upon admission is not always advisable due to the rapid progression of epiglottitis, such studies can be helpful in diagnosing for patients who are otherwise clinically stable. A single, lateral, upright view of the neck in extension can help diagnose epiglottitis when the diagnosis is not established upon physical examinations. Lateral neck radiographs are most useful in facilities where CT imaging and flexible bronchoscopy are not readily available.^7,15 CT scans or magnetic resonance imaging are helpful in evaluating for complications such as the spread of infection or abscess formation, although a magnetic resonance imaging can take a considerable amount of time and may take away from time that could be used to secure the airway.⁷ Care should be taken in patient positioning as manipulation of the neck or inflicting discomfort can lead to further airway obstruction. In a healthy patient, the lateral plain radiograph of the neck will reveal the epiglottis as a thin, curved soft tissue opacity separated from the tongue by the vallecula, which is represented as radiolucency.¹⁵ In a patient with epiglottitis, the epiglottis is often noted to be quite edematous similar in appearance to a thumbprint (Figure 3A).^1,15 Other radiographic findings may include thickening of the aryepiglottic folds, prevertebral soft tissue swelling, and expansion of the hypopharynx. Sensitivity and specificity of radiography of epiglottitis in adults varies from 38 to 98%.^7,8 In approximately 25% of adult cases, a chest radiograph will reveal a concurrent pneumonia.¹⁶

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TREATMENT

Antimicrobial therapy is preferably based upon results from blood and epiglottic cultures when possible. The most commonly encountered bacterial organisms are HiB, Streptococcus pneumonia, Group A Streptococcus, and Staphylococcus aureus including methicillin-resistant S aureus strains.^11,13,17 Empiric combination antibiotic therapy with a third-generation cephalosporin and an antistaphylococcal agent is usually recommended.^11,14 Vancomycin is the antistaphylococcal agent of choice in patients with epiglottitis complicated by sepsis, those with concomitant meningitis, or those from areas with an increased prevalence of clindamycin-resistant methicillin-resistant S aureus.¹³ Patients with a penicillin allergy should be treated with vancomycin and a quinolone antibiotic agent. Antibiotics should be altered as culture sensitivities are identified by lab and adjusted to ensure completion of a 10-day course. Routine vaccination for HiB is not recommended in adults. However, older children or adults at an elevated risk of contracting an HiB infection includes those with a history of functional or anatomic asplenia, immunodeficiency, immunosuppression from cancer chemotherapy, infection with HIV, and receipt of a hematopoietic stem cell transplant.⁵ For high-risk patients who are also previously unvaccinated, the administration of at least 1 pediatric dose of an HiB conjugate vaccine should be considered.

There are a variety of adjuvant therapies available that can be utilized on a case-by-case basis. Administration of supplemental humidified oxygen along with IV hydration can help to limit the risk of sudden airway obstruction.⁹ Glucocorticoids are not universally recommended for initial treatment because evidence of a clear benefit, such as reduced length of stay or shorter duration of intubation in the ICU, is lacking. Some studies have indicated when steroids are given that hospital stays are typically longer; however, that correlation may be complicated by the fact that steroids are more likely to be given in more extreme or severe cases.¹⁸ If corticosteroids are to be used, the recommended course of treatment in adults is IV dexamethasone 4 to 10 mg as an initial bolus with a repeated IV dose of 4 mg every 6 hours along with close observation of the airway.³ Other steroids, such as IV methylprednisolone 125 mg, can also be considered for administration in adults.⁹ Steroids should be tapered as the signs and symptoms resolve. Administration of epinephrine, racemic, or subcutaneous may help to improve upper airway edema.¹

Patients should be monitored in the ICU regardless of the placement of an airway, such as their natural airway, intubation, or tracheostomy, so they can be monitored more closely for airway deterioration.¹⁹ Ideally, daily examination of the supraglottic structures should be performed with fiberoptic nasolaryngoscopy to assess the patient's response to therapy and evaluate for any complications such as epiglottic abscesses. For patients in whom the airway has been secured, care must be taken to avoid dislodgement of the endotracheal tube or tracheostomy, as reintubation may not be feasible leading to significant morbidity or mortality depending upon the severity of the clinical situation. The patient may require additional respiratory care and periodic suctioning to maintain airway patency. Extubation criteria include resolution of the patient's fever, odynophagia/dysphagia symptoms, and airway edema as assessed by nasolaryngoscopy or a positive cuff leak test. Edema typically improves within 2 to 3 days of initiating antimicrobial treatment.²

DIFFERENTIAL DIAGNOSES

Stridor is a high-pitched wheezing sound that results from disrupted airflow, a key sign of partial airway obstruction that can be associated with several different disease states. Stridor itself is caused by restrictions to airflow most commonly resulting from inflammation in the tissues surrounding the airway. In a healthy patient, airflow in the upper respiratory tract approximates laminar flow. In said patient, the moving column of air will create a slightly negative pressure on the wall of the airway.²⁰ But narrowing of the airway, often due to inflammation from infection or trauma, can cause an increase in airway resistance and turbulent air flow along the narrowed segment, as described by the Venturi effect. According to the Bernoulli principle, as the flow velocity increases, there is an increase in intraluminal negative pressure at the already narrowed lumen site, which promotes further collapse of the airway.²⁰ The increase in turbulent flow is demonstrated clinically as stridor. Stridor can be evident during the different stages of respiration. Stridor during inspiration can be attributed to supraglottic swelling due to collapse of unsupported soft tissues when negative pressure is generated during inspiration.²⁰ Biphasic stridor is characteristic of a fixed obstruction at the level of the cricoid. If a narrowing occurs at the intrathoracic trachea or bronchiole level, an expiratory stridor will likely develop due to the increased intrathoracic pressure generated during exhalation, which contributes to narrowing of the distal airways.²⁰

SUMMARY

Epiglottitis is defined as inflammation of the epiglottis and/or neighboring supraglottic structures and is generally caused by a bacterial infection. Although this type of infection was historically more prevalent in children ages 2 to 6 years, a decreasing incidence in pediatric patients has been observed following the introduction of the HiB vaccine. In contrast, there has recently been an increasing shift in incidence within the adult population. Clinical presentation of epiglottitis can include odynophagia, dysphagia, use of the tripod position, and stridor once the resulting edema begins to cause substantial airway obstruction. Early involvement of airway specialists such as otolaryngologists, anesthesiologists, or intensivists is warranted if epiglottitis is suspected. It is generally recommended that patients be managed and monitored in a high acuity environment, such as an ICU, until signs and symptoms resolve.