Epiglottitis

Epiglottitis is the inflammation of the epiglottis—the flap at the base of the tongue that prevents food entering the trachea (windpipe).[7] 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.[1][2] As the epiglottis is in the upper airway, swelling can interfere with breathing.[7] People may lean forward in an effort to open the airway.[1] As the condition worsens, stridor and bluish skin may occur.[1]

Epiglottitis
Other namesAcute supraglottitis
Neck X-ray showing thumbprint sign.
SpecialtyPulmonology
SymptomsTrouble swallowing, drooling, changes to the voice, fever, increased breathing rate, stridor[1][2]
Usual onsetRapid[1][2]
CausesH. influenzae type b, burns, trauma to the area[1]
Diagnostic methodMedical imaging, looking at the epiglottis[3][1]
PreventionHib vaccine, rifampin[4][5]
TreatmentEndotracheal intubation, intravenous antibiotics, corticosteroids[1][2][4]
Prognosis5% risk of death[3]
Frequency~2 per 100,000 per year[1][6]

Epiglottitis was historically mostly caused by infection by H. influenzae type b.[1] With vaccination it is now more often caused by other bacteria, most commonly Streptococcus pneumoniae, Streptococcus pyogenes, or Staphylococcus aureus.[1] Predisposing factors include burns and trauma to the area.[1] The most accurate way to make the diagnosis is to look directly at the epiglottis.[3] 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.[1]

An effective vaccine, the Hib vaccine, has been available since the 1980s.[4] 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.[5] The most important part of treatment involves securing the airway, which is often done by endotracheal intubation.[1] Intravenous antibiotics such as ceftriaxone and possibly vancomycin or clindamycin is then given.[2][4] Corticosteroids are also typically used.[1] With appropriate treatment, the risk of death among children with the condition is about one percent and among adults is seven percent.[3]

With the use of the Hib vaccine, the number of cases of epiglottitis has decreased by more than 95%.[8] While, historically, young children were mostly affected, it is now more common among older children and adults.[4] In the United States it affects about 1.3 per 100,000 children a year.[1] In adults between 1 and 4 per 100,000 are affected a year.[6] It occurs more commonly in the developing world.[9] In children the risk of death is about 6%; however, if they are intubated early it is less than 1%.[5]

Signs and symptoms

Epiglottitis is associated with fever, throat pain, difficulty in swallowing, drooling, hoarseness of voice, and stridor.[10] Onset is typically over a day.[10] The throat itself may appear normal.[10]

Stridor is a sign of upper airways 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. The early symptoms are usually insidious but rapidly progressive, and swelling of the throat may lead to cyanosis and asphyxiation.[11]

Causes

Epiglottitis is primarily caused by an acquired bacterial infection of the epiglottis.[1] Historically it was most often caused by Haemophilus influenzae type B, but with the availability of immunization this is no longer the case.[1] Presently the bacteria most often causing infection are Streptococcus pneumoniae, Streptococcus pyogenes, or Staphylococcus aureus.[1] While the overall incidence of epiglottitis has decreased, the incidence of cases caused by Streptococcus pneumoniae has increased in adults.[12] The exact strains of Streptococcus pneumoniae are often those that are covered by the PPV-23 vaccine,[12] 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.[13] 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.[13]

Alternate risk factors and causes associated with infection include burns and other trauma to the area.[1] Medical research has also identified a link between epiglottitis and crack cocaine usage.[14] Underlying health conditions, such as Graft-versus-host disease and Lymphoproliferative disorders, have also been identified as contributors of increased risk for developing the infection .[4] This is in part caused by the host's decreased immune response coupled with the common sites targeting the soft tissue of the lymphatic system, both of which are already negatively impacted by the pre-existing illnesses.[15]

Diagnosis

Diagnosis may be confirmed by direct inspection using a laryngoscope, although this may provoke airway spasm. 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. Imaging is rarely useful, and treatment should not be delayed for this test to be carried out.[16]

Imaging

On lateral C-spine X-ray, the thumbprint sign describes a swollen, enlarged epiglottis.[10] A normal X-ray, however, does not exclude the diagnosis.[10] An ultrasound may be helpful if specific changes are present, but its use (as of 2018) is in the early stages of study.[10]

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.[17]

Necrotizing Epiglottitis

If there is visual or radiologic evidence that the infection has caused tissue destruction, the disease is called "necrotizing epiglottitis" (NE).[18] The feared complication of NE is the bacteria spreading to the surrounding neck muscles and causing cervical necrotizing fasciitis which is a surgical emergency.[18]

Differential Diagnosis

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.[19][20]

Prevention

An effective vaccine, the Hib vaccine, has been available since the 1980s.[4] 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.[5]

Management

The most important part of treatment involves securing the airway.[1] Nebulized epinephrine may be useful to improve the situation temporarily.[10] Corticosteroids are also typically used.[1] Evidence for benefit, however, is poor.[10] Epiglottitis may require urgent tracheal intubation to protect the airway.[1] Tracheal intubation can be difficult due to distorted anatomy and profuse secretions. Spontaneous respiration is ideally maintained until tracheal intubation is successful.[10] A surgical airway opening (cricothyrotomy) may be required if intubation is not possible.[10]

Intravenous antibiotics such as ceftriaxone and possibly vancomycin or clindamycin are given once the airway is secure.[2][4] If the patient has a penicillin allergy, trimethoprim/sulfamethoxazole or clindamycin may be an alternative.

Necrotizing epiglottis is treated similarly to uncomplicated epiglottitis, but usually requires intubation in addition to standard IV antibiotic therapy.[18] If necrotizing fasciitis of the neck is suspected based on clinical exam or CT scan, patients are taken to the operating room for emergency debridement.[18]

Prognosis

With appropriate treatment, the risk of death among children with the condition is about one percent and among adults is seven percent.[3] Some people may develop pneumonia, lymphadenopathy, or septic arthritis.[21]

Epidemiology

While, historically, young children were mostly affected, it is now more common among older children and adults.[4] Before Hemophilus influenzae (Hib) immunization children of two to four were most commonly affected.[1] With immunization about 1.3 per 100,000 children are affected a year.[1] 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 one and over the age of 85 are also particularly vulnerable.[22]

Notable cases

Bill Bixby's 6-year-old son Christopher died of the condition in 1981.[23]

Jeannie Mai spent some time in an ICU with epiglottitis.[24]

Sarah Silverman spent a week in the ICU at Cedars Sinai Hospital with epiglottitis.[25][26][27]

George Washington is thought to have died of epiglottitis.[28] 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.[29]

References

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  2. Zoorob, R; Sidani, MA; Fremont, RD; Kihlberg, C (1 November 2012). "Antibiotic use in acute upper respiratory tract infections". American Family Physician. 86 (9): 817–22. PMID 23113461.
  3. Westerhuis, B; Bietz, MG; Lindemann, J (August 2013). "Acute epiglottitis in adults: an under-recognized and life-threatening condition". South Dakota Medicine. 66 (8): 309–11, 313. PMID 24175495.
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  5. Blacklow, Neil R. (2004). Infectious diseases (3rd ed.). Philadelphia: Lippincott Williams & Wilkins. p. 461. ISBN 9780781733717. Archived from the original on 2016-08-16.
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  7. Hamborsky, j (2015). "Haemophilus influenzae type b". Centers for Disease Control and Prevention. Epidemiology and Prevention of Vaccine-Preventable Diseases (13 ed.). Public Health Foundation. p. Chapter 8. ISBN 9780990449119. Archived from the original on 20 July 2016. Retrieved 14 July 2016.
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  11. Guerra AM, Waseem M (10 February 2021). "Epiglottitis". National Center for Biotechnology Information, U.S. National Library of Medicine. PMID 28613691. Retrieved 20 July 2021.
  12. Isakson, M.; Hugosson, S. (April 2011). "Acute epiglottitis: epidemiology and Streptococcus pneumoniae serotype distribution in adults". The Journal of Laryngology and Otology. 125 (4): 390–393. doi:10.1017/S0022215110002446. ISSN 1748-5460. PMID 21106138.
  13. Chen, Cheng; Natarajan, Mukil; Bianchi, David; Aue, Georg; Powers, John H (2018-02-17). "Acute Epiglottitis in the Immunocompromised Host: Case Report and Review of the Literature". Open Forum Infectious Diseases. 5 (3): ofy038. doi:10.1093/ofid/ofy038. ISSN 2328-8957. PMC 5846294. PMID 29564363.
  14. Mayo-Smith, Michael F.; Spinale, Joseph (1997). "Thermal epiglottitis in adults: A new complication of illicit drug use". The Journal of Emergency Medicine. 15 (4): 483–5. doi:10.1016/S0736-4679(97)00077-2. PMID 9279700.
  15. Dhainaut, Jean-François; Claessens, Yann-Erick; Janes, Jonathan; Nelson, David R. (2005-11-15). "Underlying Disorders and Their Impact on the Host Response to Infection". Clinical Infectious Diseases. 41 (Supplement_7): S481–S489. doi:10.1086/432001. ISSN 1058-4838. PMID 16237651.
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  17. Ito, Keiko; Chitose, Hiroko; Koganemaru, Masamichi (2011). "Four cases of acute epiglottitis with a peritonsillar abscess". Auris Nasus Larynx. 38 (2): 284–8. doi:10.1016/j.anl.2010.06.004. PMID 20800396.
  18. Villemure-Poliquin, Noémie; Chénard-Roy, Jade; Lachance, Sophie; Leclerc, Jacques E.; Lemaire-Lambert, Anthony (November 2020). "Necrotizing epiglottitis with necrotizing fasciitis in a child: A case report and review of literature". International Journal of Pediatric Otorhinolaryngology. 138: 110385. doi:10.1016/j.ijporl.2020.110385. ISSN 1872-8464. PMID 33152976.
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  24. Atad, Corey (2020-11-02). "Jeannie Mai Forced Off 'Dancing With The Stars' After Being Hospitalized For Rare Condition". ET Canada. Retrieved 2020-11-03.
  25. "Comedian Sarah Silverman 'lucky to be alive' after surgery". apnews.com. 2016-07-07. Archived from the original on 11 August 2017. Retrieved 2 June 2017.
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