Epiglottitis
Template:Short description Template:Cs1 config Template:Infobox medical condition (new) Epiglottitis is the inflammation of the epiglottis—the flap at the base of the tongue that prevents food entering the trachea (windpipe).<ref name=Pink2015>Template:Cite book</ref> Symptoms are usually rapid in onset and include trouble swallowing which can result in drooling, changes to the voice, fever, and an increased breathing rate.<ref name=Ric2016/><ref name=AFP2012>Template:Cite journal</ref> As the epiglottis is in the upper airway, swelling can interfere with breathing.<ref name=Pink2015/> People may lean forward in an effort to open the airway.<ref name=Ric2016/> As the condition worsens, stridor and bluish skin may occur.<ref name=Ric2016/>
Epiglottitis was historically mostly caused by infection by H. influenzae type b (commonly referred to as "Hib").<ref name="Ric2016">Template:Cite journal</ref> Following the introduction of the Hib vaccine, pediatric cases of epiglottitis fell from 3.47 cases per 100,000 children in 1980 to 0.63 cases in 1990<ref name="StroudFriedman">Template:Cite journal</ref> such that it is now more often caused by other bacteria, most commonly Streptococcus pneumoniae, Streptococcus pyogenes, or Staphylococcus aureus.<ref name=Ric2016/> Predisposing factors include burns and trauma to the area.<ref name=Ric2016/> The most accurate way to make the diagnosis is to look directly at the epiglottis.<ref name=West2013>Template:Cite journal</ref> X-rays of the neck from the side may show a "thumbprint sign" but the lack of this sign does not mean the condition is absent.<ref name=Ric2016/>
An effective vaccine, the Hib vaccine, has been available since the 1980s.<ref name=Sch2015>Template:Cite book</ref> The antibiotic rifampicin may also be used to prevent the disease among those who have been exposed to the disease and are at high risk.<ref name=Black2004>Template:Cite book</ref> The most important part of treatment involves securing the airway, which is often done by endotracheal intubation.<ref name=Ric2016/> Intravenous antibiotics such as ceftriaxone and possibly vancomycin or clindamycin is then given.<ref name=AFP2012/><ref name=Sch2015/> Corticosteroids are also typically used.<ref name=Ric2016/> With appropriate treatment, the risk of death among children with the condition is about one percent and among adults is seven percent.<ref name=West2013/>
With the use of the Hib vaccine, the number of cases of epiglottitis has decreased by more than 95%.<ref name=Ter2015>Template:Cite book</ref> Historically, young children were mostly affected, but it is now more common among older children and adults.<ref name=Sch2015/> In the United States, pediatric cases of epiglottitis fell from 3.47 cases per 100,000 children in 1980 to 0.63 cases in 1990 following the introduction of the Hib vaccinae,<ref name="StroudFriedman">Template:Cite journal</ref> and it now affects about 1.3 per 100,000 children a year.<ref name=Ric2016/> In adults, between 1 and 4 per 100,000 are affected a year.<ref name=Text2016>Template:Cite book</ref> It occurs more commonly in the developing world.<ref>Template:Cite book</ref> In children the risk of death is about 6%; however, if they are intubated early, it is less than 1%.<ref name=Black2004/>
Signs and symptomsEdit
Epiglottitis is associated with fever, throat pain, difficulty in swallowing, drooling, hoarseness of voice, and stridor.<ref name=Got2018/> Onset is typically over a day.<ref name=Got2018>Template:Cite journal</ref> The throat itself may appear normal.<ref name=Got2018/>
Stridor is a sign of upper airway obstruction and is a surgical emergency. The child often appears acutely ill, anxious, and will have very quiet shallow breathing often keeping the head held forward and insisting on sitting up in bed, commonly called the "tripod position."<ref name="pmid35489220" /> The early symptoms are usually insidious but rapidly progressive, and swelling of the throat may lead to cyanosis and asphyxiation.<ref>Template:Cite journal</ref>
Adults commonly present with less dramatic breathing symptoms than children due to them having wider airways to begin with, so their main symptoms are usually a severe sore throat and difficulty swallowing.<ref name="pmid35489220" /> The back of the throat appears normal in 90% of adult patients, so epiglottitis should considered when there is pain out of proportion to exam or when pain is caused by pressing on the external windpipe.<ref name="pmid35489220" /> Adult epiglottitis is often referred to as supraglottitis. In contrast to children, the symptoms are non-specific, sub-acute and can be unpredictable. <ref name="pmid38230383">Template:Cite journal</ref>
CausesEdit
Epiglottitis is primarily caused by an acquired bacterial infection of the epiglottis.<ref name=Ric2016/> Historically it was most often caused by Haemophilus influenzae type B, but with the availability of immunization this is no longer the case.<ref name=Ric2016/> H. influenzae type B contains a capsule which helps it avoid being destroyed by macrophages and also contains surface proteins that allow it to stick to the lining of the upper respiratory tract.<ref>Template:Citation</ref> Presently, the bacteria most often causing infection are other encapsulated organisms including Streptococcus pneumoniae, Streptococcus pyogenes, and Staphylococcus aureus.<ref name=Ric2016/> These bacteria spread in respiratory droplets or aerosols produced from coughing and sneezing.<ref>{{#invoke:citation/CS1|citation |CitationClass=web }}</ref><ref>Template:Cite book</ref> While the overall incidence of epiglottitis has decreased, the incidence of cases caused by Streptococcus pneumoniae has increased in adults.<ref name="pmid21106138">Template:Cite journal</ref> The exact strains of Streptococcus pneumoniae are often those that are covered by the PPV-23 vaccine,<ref name="pmid21106138" /> but there is no evidence that this vaccine prevents epiglottitis.
There have been many cases of epiglottitis reported in immunocompromised patients, including those undergoing cancer treatment and those who are HIV positive.<ref name="pmid29564363">Template:Cite journal</ref> While a variety of different bacteria can cause disease in these patients, cases often involve the Candida species of fungus, though it is unknown if the fungus causes significant disease on its own.<ref name="pmid29564363" />
Alternate risk factors and causes associated with infection include burns and other trauma to the area.<ref name=Ric2016/> Medical research has also identified a link between epiglottitis and crack cocaine usage.<ref>Template:Cite journal</ref> Underlying disorders of the immune system, such as graft-versus-host disease and lymphoproliferative disorders, have also been identified as contributors of increased risk for developing the infection .<ref name=Sch2015/>
DiagnosisEdit
Diagnosis may be confirmed by direct inspection using a laryngoscope, although this may provoke airway spasm.<ref name="gpnotebook.co.uk" /> If epiglottitis is suspected, attempts to visualize the epiglottis using a tongue depressor are discouraged for this reason; therefore, diagnosis is made on basis of indirect fiberoptic laryngoscopy carried out in a controlled environment like an operating room.<ref name="gpnotebook.co.uk" /> An infected epiglottis appears swollen and is described as having a "cherry-red" appearance.<ref name="Levinson-2020" /> Imaging is rarely useful, and treatment should not be delayed for this test to be carried out.<ref name="gpnotebook.co.uk">{{#invoke:citation/CS1|citation |CitationClass=web }}</ref>
- Halloweensign.jpg
CT imaging showing the "halloween sign"
- Compare epiglottis.jpg
Left column: Normal epiglottis. Right column: Epiglottitis.
- Epiglottitis endoscopy.jpg
Swollen epiglottis in laryngoscopy
- Larynx normal.jpg
Normal appearance of epiglottis (label 3) as seen on laryngoscopy
ImagingEdit
On lateral C-spine X-ray, the thumbprint sign describes a swollen, enlarged epiglottis.<ref name=Got2018/> A normal X-ray, however, does not exclude the diagnosis.<ref name=Got2018/> An ultrasound may be helpful if specific changes are present, but its use (as of 2018) is in the early stages of study.<ref name=Got2018/>
On CT imaging, the "Halloween sign" describes an epiglottis of normal thickness. It can safely exclude the acute epiglottitis. Furthermore, CT imaging can help to diagnose other conditions such as peritonsillar abscess or retropharyngeal abscess which have similar clinical features.<ref>Template:Cite journal</ref>
Necrotizing EpiglottitisEdit
If there is visual or radiologic evidence that the infection has caused tissue destruction, the disease is called "necrotizing epiglottitis" (NE).<ref name="pmid33152976">Template:Cite journal</ref> The feared complication of NE is the bacteria spreading to the surrounding neck muscles and causing cervical necrotizing fasciitis which is a surgical emergency.<ref name="pmid33152976" />
Differential DiagnosisEdit
The differential diagnosis includes other infectious causes of acute airway obstruction, as well as acute or subacute mechanical causes. It includes, but is not limited to, the conditions below.<ref>Template:Cite journal</ref><ref>Template:Cite journal</ref>
- Retropharyngeal abscess
- Peritonsillar abscess
- Croup
- Infectious mononucleosis
- Diphtheria
- Pertussis
- Bacterial tracheitis
- Ludwig's angina
- Granulomatosis with polyangiitis
- Foreign body obstruction
- Obstructing tumor
- Allergic reaction, including angioedema
- Inhalation injury
- Laryngospasm
- Congenital structural defects in pediatric patients, including laryngomalacia
PreventionEdit
An effective vaccine, the Hib vaccine, has been available since the 1980s.<ref name=Sch2015/> Modern Hib vaccines are mainly conjugate vaccines, with the key component being the polysaccharide found in the bacteria's capsule which is its primary virulence factor.<ref>{{#invoke:citation/CS1|citation |CitationClass=web }}</ref> Currently, the CDC recommends that children receive a two or three-dose primary series with an additional booster dose.<ref>{{#invoke:citation/CS1|citation |CitationClass=web }}</ref> The countries of the world who have included the Hib vaccine in their immunization schedules typically begin the series at the age of two or three months with subsequent doses administered at four or eight week intervals.<ref name="pmid18582836">Template:Cite journal</ref> Routine vaccination in these nations has led to a dramatic decrease in the incidence of invasive diseases caused by H. influenzae type b such as epiglottitis, meningitis and pneumonia.<ref name="pmid18582836" /> It has been reported that epiglottitis cases have decreased by 95% since the 1980s following the introduction of the first Hib vaccine.<ref name="Ter2015" />
The antibiotic rifampicin may also be used to prevent the disease among those who have been exposed to the disease and are at high risk.<ref name="Black2004" /><ref name="Levinson-2020">Template:Cite book</ref>
ManagementEdit
The most important part of treatment involves securing the airway.<ref name=Ric2016/> Nebulized epinephrine may be useful to improve the situation temporarily.<ref name="Got2018" /> Corticosteroids are also typically used.<ref name="Ric2016" /> However, there is poor evidence for whether steroids actually improve patient outcomes.<ref name="Got2018" /> Epiglottitis may require urgent tracheal intubation to protect the airway.<ref name=Ric2016/> Tracheal intubation can be difficult due to distorted anatomy and profuse secretions. Spontaneous respiration is ideally maintained until tracheal intubation is successful.<ref name=Got2018/> A surgical airway opening (cricothyrotomy) may be required if intubation is not possible.<ref name=Got2018/> The management of epiglottitis is different in adults compared to children.<ref name="pmid38230383" /> Emergent tracheal intubation with general anesthesia (inhalational induction to preserve spontaneous ventilation) in the operating theater is standard.<ref name="pmid38230383" /> However only 10% of adults require airway intervention, which means a selective approach is required.<ref name="pmid38230383" /> Tracheal intubation is a high risk scenario with a 1 in 25 failure rate in adults.<ref name="pmid38230383" /> Multiple airway management techniques have described for adults and include: awake tracheostomy, awake fibreoptic intubation, general anesthesia with spontaneous breathing preserved or ablated with paralysis.<ref name="pmid38230383" /> The optimal technique is controversial and likely determined by contextual factors such as the severity of epiglottitis and the clinical location (ie emergency department or intensive care or the operating room).<ref name="pmid38230383" /> Ideally airway intervention should occur in the operating room with an otolaryngology surgeon present to perform an emergency tracheostomy in the event of complete airway obstruction or failed intubation.<ref name="pmid38230383" />
Intravenous antibiotics such as ceftriaxone and possibly vancomycin or clindamycin are given once the airway is secure.<ref name=AFP2012/><ref name=Sch2015/> A third-generation cephalosporin such as ceftriaxone is usually sufficient since it is usually effective against H. influenzae and S. pneumoniae.<ref name="Bradley-2020">Template:Cite book</ref> If S. aureus is suspected to be causing the disease, then the treatment should include ceftaroline or clindamycin as these would provide coverage against antibiotic resistant strains of that bacteria (MRSA).<ref name="Bradley-2020" /> Vancomycin can also be considered for its MRSA coverage, but it may be less safe than ceftaroline in children older than two months.<ref name="Bradley-2020" /> If the patient has a penicillin allergy, trimethoprim/sulfamethoxazole, clindamycin, or levofloxacin may be appropriate choices.<ref name="Bradley-2020" /><ref name="pmid35489220" />
Necrotizing epiglottitis is treated similarly to uncomplicated epiglottitis, but usually requires intubation in addition to standard IV antibiotic therapy.<ref name="pmid33152976" /> If the tissue damage continues to spread and necrotizing fasciitis of the neck is suspected, patients are taken to the operating room for emergency debridement.<ref name="pmid33152976" />
PrognosisEdit
With appropriate treatment, the risk of death among children with the condition is about one percent and among adults is seven percent.<ref name=West2013/> Elsewhere, it has been reported that only one percent of adults diagnosed with epiglottitis die from the disease.<ref name="pmid35489220" /> Some people may develop pneumonia, lymphadenopathy, or septic arthritis.<ref>{{#invoke:citation/CS1|citation |CitationClass=web }}</ref>
Between 1998 and 2006, there were an average of 36 deaths per year in the United States attributed to epiglottitis, giving a case-fatality rate of 0.89% during that time period.<ref name="pmid20513048" />
Patients who recover from necrotizing epiglottitis often regain their ability to swallow foods and liquids despite the tissue damage.<ref name="pmid33152976" />
EpidemiologyEdit
While, historically, young children were mostly affected, it is now more common among older children and adults.<ref name=Sch2015/> Before Haemophilus influenzae (Hib) immunization children of two to four were most commonly affected.<ref name=Ric2016/> With immunization about 1.3 per 100,000 children are affected a year.<ref name=Ric2016/> It has been reported that only 0.5 per 100,000 American children are diagnosed every year, while the incidence in American adults is about 1 to 4 per 100,000.<ref name="pmid35489220">Template:Cite journal</ref> A 2010 retrospective study revealed the average age of patients admitted to American hospitals for epiglottitis was about 45, but patients under the age of 1 and over the age of 85 are also particularly vulnerable.<ref name="pmid20513048">Template:Cite journal</ref>
Notable casesEdit
- Bill Bixby's 6-year-old son Christopher died of the condition in 1981.<ref>Template:Cite news</ref>
- Jeannie Mai spent some time in an ICU with epiglottitis.<ref>{{#invoke:citation/CS1|citation
|CitationClass=web }}</ref>
- Sarah Silverman spent a week in the ICU at Cedars Sinai Hospital with epiglottitis.<ref>{{#invoke:citation/CS1|citation
|CitationClass=web }}</ref><ref>{{#invoke:citation/CS1|citation |CitationClass=web }}</ref><ref>{{#invoke:citation/CS1|citation |CitationClass=web }}</ref>
- George Washington is thought to have died of epiglottitis.<ref>Template:Cite book</ref> The treatments given to Washington, such as severe bloodletting, an enema, vinegar, sage, molasses, butter, blistering his throat with Spanish fly, requiring him to swallow mercurous chloride and antimony potassium tartrate, and applying wheat poultices to various parts of the body, are no longer used.<ref>{{#invoke:citation/CS1|citation
|CitationClass=web }}</ref>
- Jin of BTS announced in a 2022 video that he had been diagnosed with epiglottitis.<ref>Template:Citation</ref>
- Wes Moore's father died of epiglottitis when Moore was four years old.<ref>{{#invoke:citation/CS1|citation
|CitationClass=web }}</ref>