Epiglottitis is an inflammatory condition of the epiglottis and nearby structures like the arytenoids, aryepiglottic folds, and vallecula. Epiglottitis is a life-threatening infection that causes profound swelling of the upper airways which can lead to asphyxia and respiratory arrest.
Before the development of the Haemophilus influenzae type b vaccine, the majority of cases were caused by H.influenzae. The epiglottis is the area where the swelling is the greatest and can lead to airway obstruction, respiratory distress, and death.
The cause of epiglottitis is more commonly infectious rather than noninfectious. It can be bacterial, viral, or fungal in origin. In children, Haemophilus influenzae type B (HIB) is the most common cause. However, this has decreased since the widespread use of immunization. Other agents such as Streptococcus pyrogenes, S. pneumoniae, and S. aureus have been implicated. In immunocompromised hosts, Pseudomonas aeruginosa and Candida have been named. Noninfectious causes can be traumatic such as thermal, caustic, or foreign body ingestion.
While viruses do not cause epiglottitis, a prior viral infection may allow bacterial superinfection to develop. Viruses that may allow a superinfection include varicella-zoster, herpes simplex, and Epstein Barr virus.
Since the addition of the HIB vaccine to the infant immunization schedule, the annual incidence of epiglottitis in children has decreased overall. However, the incidence in adults has remained stable. Additionally, the age of children who have had epiglottitis has increased from 3 years old to about 6 to 12 years old.
While in the past epiglottis has been only reported in children, over the past few decades many cases in adults have been published.
The airways in the pediatric population are markedly different compared to those of adults. In a young child, the epiglottis is located more superiorly and anteriorly than in an adult. There is also a more oblique angle with the trachea. Further, the infant epiglottis is floppy compared to an adult's, whose epiglottis is more rigid. These anatomical differences are why airway compromise is more common in infants than adults.
H.influenzae infection of the epiglottis can lead to marked edema and swelling. This edema can rapidly spread to adjacent structures leading to rapid development of airway obstruction symptoms.
Other complications of epiglottitis include:
The history will reveal that this was a sudden onset. It will usually have occurred within the last 24 hours, or sometimes the last 12 hours. The patient will appear toxic. Most children have no prodromal symptoms. In the emergency department, the child will likely be sitting upright with the mouth open in a tripod position and possibly have a muffled voice. Drooling, dysphagia, and distress, or anxiety in children, are present. These are often referred to as the 3 Ds. Swelling of the upper airway results in turbulent airflow during inspiration or stridor. Signs of severe upper airway obstruction such as intercostal or suprasternal retractions, tachypnea, and cyanosis are concerning for impending respiratory failure and should signal the provider to act quickly. Avoid an exam of the throat with a tongue blade as it may result in the loss of the airway.
Anterior examination of the neck may reveal lymphadenopathy. If cyanosis is present, it is indicative of advanced infection and poor prognosis.
An oropharyngeal exam is usually not performed to evaluate a suspected case of epiglottitis because manipulation of the throat may lead to respiratory arrest. This diagnosis is primarily one of clinical suspicion. A lateral neck radiograph will show swelling of the epiglottis, also referred to as the “thumb sign.” It is not necessary to make the diagnosis but can be used to narrow down the provider’s differential diagnosis. A flexible fiberoptic laryngoscopy can be performed, but only in a very controlled setting such as the operating room due to the risk of inducing laryngospasm. Prior to any throat exam, it is vital that the airway has been secured.
Ultrasonography has been mentioned as another way to evaluate these patients, revealing an “alphabet P sign” in a longitudinal view. A complete blood count with differential, a blood culture, and an epiglottal culture should only be obtained in patients with a secured endotracheal tube.
CT scan of the neck is rarely needed but if done, placing the patient in a supine position can trigger respiratory crises. Once a diagnosis of the epiglottis is entertained, the child should not be left alone in the radiology suite.
The chest x-ray may reveal concomitant pneumonia in 10-15% of patients.
In some patients, percutaneous transtracheal ventilation may be required for temporary oxygenation of the patient. This may be converted to a tracheostomy under controlled circumstances.
The mainstay of treatment is to secure the airway first. Experienced providers should intubate these patients since their airways are regarded as difficult. An individual capable of performing a tracheotomy should be available if needed. The patient should be admitted to the intensive care unit after the airway is secured. The use of corticosteroids to reduce edema has been cited, with an overall shorter intensive care unit stay for these patients. Empiric antimicrobials should be initiated. Once culture and sensitivity results are available, the regimen should be adjusted.
All non-intubated patients must be admitted for observation and a tracheostomy tray must be available at the bedside. The ENT surgeon and the anesthesiologist must be notified of the admission in case an emergency airway is required. Nurses should be warned not to place the child in a supine position.
If an emergency airway is needed, it should ideally be obtained in the operating room. The airway should be visualized with a laryngoscope first. if endotracheal intubation is not an option, then a tracheostomy should be performed.
Antibiotics usually administered include cefuroxime, ceftriaxone, and cefotaxime. All close contacts should receive rifampin.
Because of the availability of the HIB vaccine, acute epiglottitis due to H. influenzae is not common. Thus, most health care providers may have less insight into the disorder. This lack often leads to delays in starting antibiotics. It can also delay sending the patient to a regular medical floor in an unmonitored room or even the radiology department. Acute epiglottitis can result in sudden airway obstruction. It is never wise to send the patient anywhere without proper monitoring and resuscitative equipment.
Other conditions that can mimic the presentation include an airway obstruction from a foreign object, acute angioedema, caustic ingestion causing airway compromise, diphtheria, or peritonsillar and retropharyngeal abscesses.
For most patients with epiglottitis, the prognosis is good when the diagnosis and treatment are prompt. Even those who require intubation are usually extubated in a few days without any residual sequelae. However, when the diagnosis is delayed in children, airway compromise can occur, and death is not uncommon.
The cause of death is usually due to sudden upper airway obstruction and difficulty intubating the patient, with extensive swelling of the laryngeal structures. Thus, every patient admitted with a diagnosis of acute epiglottitis must be seen by an ear, nose, and throat surgeon or anesthesiologist, and a tracheostomy tray must be made available at the bedside. Globally, a mortality rate of 3% to 7% has been reported in patients with unstable airways.
Complications of epiglottitis include the following:
Once the patient is admitted, the following care is necessary:
With appropriate treatment, most patients improve within 48-72 hours but antibiotics are still required for 7 days. Only afebrile patients should be discharged home.
Once a patient has been diagnosed with acute epiglottitis, the following professionals should be consulted:
Close contacts of patients with H. influenzae should be prescribed rifampin prophylaxis. One may opt to administer the HIB vaccine, but it is not 100% effective.
Patients who have recurrent episodes of acute epiglottitis warrant investigation of the immune system.
To prevent epiglottitis, vaccination should be encouraged. Children more than 24 months old do not need vaccination as the disease does provide long term immunity.
Clinical Negligence Leading to Malpractice
Epiglottitis is a relatively common presentation to the emergency department. Because of its high morbidity and mortality, it is highly recommended that the disorder is managed by an interprofessional team that includes an intensivist, pulmonologist, infectious disease consult, anesthesiologist and an ENT surgeon. Since most patients present to the emergency room, it is important that the triage nurse and emergency room physician know the signs and symptoms of the disorder. The condition can rapidly lead to respiratory distress and death.
Once the patient has been admitted, the emergency department physician should consult with an infectious disease expert and the ENT surgeon. Nurses should be educated about not placing the patient supine and the importance of monitoring oxygenation. Nurses should also be aware that the patient should never be allowed to go to a radiology suite alone and without monitoring equipment.
Some patients may require mechanical ventilation for a few days. However, all patients with acute symptoms must be admitted, and a tracheostomy tray must be available at the bedside. The oral cavity should not be probed, and the patient must not be stressed. The moment the patient is admitted, an anesthesiologist and the ENT surgeon must be notified in case there is a need for an airway.
The way to prevent epiglottitis is with vaccination and it is important for the public health nurse to check up on all close contacts including daycare workers. Rifampin is recommended for all close contacts as chemoprophylaxis.
Today, most patients with acute epiglottitis have a good outcome.
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