Epiglottitis

Definition and Facts About Epiglottitis

Epiglottitis surgery
Epiglottitis is a medical emergency and anyone suspected of having epiglottitis should be taken to a hospital's emergency department immediately. Epiglottitis reconstruction surgery may be performed.
  • Epiglottitis is a potentially fatal medical emergency that occurs when the flap of tissue that covers the trachea (windpipe) during swallowing becomes infected or inflamed, resulting in swelling and obstruction that can close off the windpipe.
  • Epiglottitis may be caused by infection (such as with bacteria, viruses, or fungi), environmental agents (such as chemicals or heat damage), allergic reactions, or trauma to the neck or throat.
  • Epiglottitis is contagious.
  • Symptoms of epiglottitis include
  • A person with acute epiglottitis usually looks very ill.
  • Epiglottitis is a medical emergency and anyone suspected of having epiglottitis should be taken to a hospital's emergency department immediately.
  • Haemophilus influenzae type b (H. influenza), is a common bacteria that can cause epiglottitis. The Hib vaccine protects most children against these bacteria.
  • Epiglottitis is not always easy to diagnose and because it is so rare, it is commonly misdiagnosed as strep throat or croup. Tests for epiglottitis may include X-rays, laryngoscopy, blood tests, arterial blood gas, and blood cultures.
  • Whenever epiglottitis is suspected, immediate hospitalization is required. Antibiotics may be prescribed. Initial treatment may consist of close monitoring along with humidified oxygen and IV fluids, along with making a person comfortable and minimizing anxiety, which can cause the throat to close up. IV antibiotics may be prescribed to clear infection and control inflammation in the body.
  • If there are signs of airway obstruction due to epiglottitis, treatment requires laryngoscopy in an operating room. In severe cases, a cricothyrotomy (cutting the neck to insert a breathing tube directly into the windpipe) may be performed.
  • Epiglottitis may be prevented with childhood vaccination against H. influenza type b (Hib). For people who live with an unvaccinated child under age 4 years of age who is exposed to a person with H. influenza epiglottitis, preventive medication such as rifampin (Rifadin) is given to all household contacts to prevent the spread of the bacteria.
  • The prognosis for epiglottitis is good if the condition is caught early and treated in time. Most people with epiglottitis recover without problems. However, when epiglottitis is not diagnosed and treated early or properly, the prognosis is poor, and the condition can be fatal.
  • Epiglottitis also can occur with other infections in adults, such as pneumonia. Most commonly, it is misdiagnosed as strep throat or croup.
  • In July 2016, comedian and actor Sarah Silverman, made headlines when she was hospitalized for a case of epiglottitis.

What Is Epiglottitis?

  • Epiglottitis is a medical emergency that may result in death if not treated quickly. The epiglottis is a flap of tissue that sits at the base of the tongue that keeps food from going into the trachea (windpipe) during swallowing. When it becomes infected or inflamed, it can swell and obstruct or close off the windpipe, which may be fatal unless promptly treated.
  • With continued inflammation and swelling of the epiglottis, complete blockage of the airway may occur, leading to suffocation and death. Autopsies of people with epiglottitis have shown distortion of the epiglottis and its associated structures including the formation of abscesses (pockets of infection or pus). For unknown reasons, adults with epiglottic involvement are more likely than children to develop epiglottic abscesses.
  • Epiglottitis was first described in the 18th century and was accurately defined by Le Mierre in 1936. Although George Washington's death in 1796 was attributed to quinsy (abscess), which is a pocket of pus behind the tonsils, it was actually due to epiglottitis.

What Causes Epiglottitis?

Conditions that cause epiglottitis include infectious, chemical, and traumatic agents. Infectious causes are the most common. H influenzae type b was once the most common cause prior to vaccination. Currently, other organisms like bacteria, viruses, and fungi are the more common causes, especially among adults.

  • Organisms that can cause epiglottitis include Streptococcus pneumoniae, Haemophilus parainfluenzae, varicella-zoster (shingles), herpes simplex virus type 1 (oral herpes), and Staphylococcus aureus, among others.
  • Other types of epiglottitis that are environmental and not caused by infection include heat damage that may injure the epiglottis, called thermal epiglottitis. Thermal epiglottitis occurs from drinking hot liquids, eating solid foods, or using illicit drugs because of inhalation of metal pieces from crack cocaine pipes or the tip of marijuana cigarettes. In these cases the epiglottitis from thermal injury is similar to the illness caused by infection.
  • In very rare instances, epiglottitis may be caused by allergic reactions to food, insect stings or bites, or blunt trauma to the neck or throat.

What Are the Signs and Symptoms of Epiglottitis?

When epiglottitis strikes, it usually occurs quickly and its progression may range from just a few hours to a few days. The most common signs and symptoms include

  • sore throat,
  • muffling or changes in the voice,
  • difficulty speaking,
  • fever,
  • difficulty swallowing,
  • fast heart rate, and
  • difficulty in breathing.

A person with acute epiglottitis usually looks very ill. People with epiglottitis may appear restless and breathing with their neck, chest wall, and upper belly muscles. While they may be taking in less air with each breath, they may still manifest the high-pitched whistling sound, called inspiratory stridor. Both adults and children may have bluish discoloration of their skin from lack of oxygen after the airway becomes blocked.

Epiglottitis signs and symptoms in adults include

  • trouble breathing (respiratory distress),
  • drooling,
  • leaning forward to breathe,
  • taking rapid shallow breaths,
  • "pulling in" of muscles in the neck or between the ribs with breathing (retractions),
  • high-pitched whistling sound when breathing (stridor),
  • noisy breathing,
  • difficulty catching your breath,
  • sore throat,
  • fever,
  • raspy voice, and
  • trouble speaking.

Signs and symptoms of epiglottitis in children

In children, symptoms of epiglottitis are similar. Typically, a child who comes to the hospital with epiglottitis has a history of fever, difficulty talking, irritability, and problems swallowing for several hours. The child often sits forward and drools. Children may sit in a "sniffing position" with the body leaning forward and the head and nose tilted forward and upward as though they are sniffing a good smelling pie.

Epiglottitis signs and symptoms in children include

  • fever with chills,
  • high-pitched whistling sound when breathing (stridor),
  • difficulty breathing,
  • difficulty swallowing,
  • drooling,
  • refusing to eat,
  • muffled or hoarse voice,
  • scratchy and sore throat
  • anxiety or restlessness
  • symptoms reduced when leaning forward
  • and less commonly
  • cough, and
  • ear pain.

In infants younger than one year, signs and symptoms such as fever, drooling, and upright posturing may all be absent. The infant may have a cough and a history of an upper respiratory infection. It is very difficult to know if an infant has epiglottitis.

In contrast, adolescents and adults have a more generally ill appearance with sore throat as the main complaint along with fever, difficulty breathing, drooling, and stridor (noise with breathing).

Is Epiglottitis Contagious?

Epiglottitis itself is not contagious, but the common bacteria, Haemophilus influenzae type b (H. influenzae), that can cause it, are contagious. However, the Hib vaccine protects most children against these bacteria. Epiglottitis used to be more common among children age 2 through 6 years before the development of the Hib vaccine.

What Are the Categories of Epiglottitis?

Doctors have characterized adult epiglottitis into three categories:

Category 1: Severe respiratory distress with imminent or actual respiratory arrest. People typically report a brief history with a rapid illness that quickly becomes dangerous.

Category 2: Moderate-to-severe clinical symptoms and signs of considerable risk for potential airway blockage. Symptoms include sore throat, inability to swallow, difficulty in lying flat, muffled "hot potato" voice (speaking as if they have a mouthful of hot potato), stridor, and the use of accessory respiratory muscles with breathing.

Category 3: Mild-to-moderate illness without signs of potential airway blockage. These people often have a history of illness that has been occurring for days with complaints of sore throat and pain upon swallowing.

When to Seek Medical Care for Epiglottitis

Epiglottitis is a medical emergency. A person who is suspected of having epiglottitis should be taken to the hospital immediately. Any signs of difficulty in breathing should be reason enough to call 911 to take the person to a hospital's emergency department for evaluation by a doctor.

If the following signs and symptoms are present, an individual should go directly to a hospital's emergency department:

Sore throat associated with:

  • Muffled voice
  • Fever
  • Inability to swallow
  • Fast heartbeat
  • Irritability
  • Drooling
  • Respiratory distress characterized by shortness of breath, rapid shallow breathing, very ill-looking appearance, upright posturing with tendency to lean forward, and stridor (high-pitched sound when breathing in)

Which Specialties of Doctors Treat Epiglottitis?

Epiglottitis is generally uncommon, but it is severe and can be life-threatening. A person with epiglottitis initially may be diagnosed by a primary care provider (PCP), such as a family practitioner, internist, or a child's pediatrician. The person also may be seen and stabilized by an emergency medicine physician in a hospital emergency department. However, he or she should be referred to a specialist for further treatment, as epiglottitis is a serious disorder that can be fatal if not treated properly and promptly.

Specialists who can treat epiglottitis include otolaryngologists, also called ear, nose, and throat (ENT) doctors and anesthesiologists, specialists in airway management. If a person is sent to intensive care, he or she may be treated by a critical care specialist. An infectious disease specialist also may be involved in the person's care.

How Is Epiglottitis Diagnosed?

The doctor may order X-rays or simply look at the epiglottis and the windpipe by laryngoscopy-a procedure performed in an operating room.

  • The doctor may find that the pharynx is inflamed with a beefy cherry red, stiff, and swollen epiglottis.
  • Doctors often look for a "thumb sign" of epiglottitis on a lateral soft-tissue X-ray of the neck, which shows swelling and an enlarged epiglottis.
  • There should be no attempt at home to inspect the throat of a person suspected of having epiglottitis.
  • Because manipulation of the epiglottis may result in sudden fatal airway obstruction and because irregular slow heart rates have occurred with attempts at intubation (putting a tube down the throat and placing the person on a machine that helps with breathing), the doctor will use the controlled environment of an operating room to see the throat structures.

Other laboratory tests that doctors use to evaluate patients may include the following:

  • Blood tests to look for infection or inflammation
  • Arterial blood gas, which measures oxygenation of the blood and the severity of obstruction
  • Blood cultures, which may grow bacteria and indicate the cause of the epiglottitis
  • Other immunologic tests looking for antibodies to specific bacteria or viruses

These laboratory tests may not be useful in diagnosing epiglottitis until the person is stable. Also, the anxiety from having blood drawn or cultures taken from the throat may cause the unstable epiglottis to close off, completely obstructing the airway and creating an emergency with only a few minutes to correct.

Even with modern technology, epiglottitis is not easy to diagnose. Early in the disease, epiglottitis is commonly misdiagnosed as strep throat.

  • Other possible misdiagnoses include infectious causes such as croup, diphtheria, peritonsillar abscess, and infectious mononucleosis.
  • Noninfectious causes of epiglottitis have been mistaken as angioneurotic edema (swelling of the tissues in the airway), laryngeal inflammation or spasm, laryngeal trauma, cancerous growths, allergic reactions, thyroid gland infection, epiglottic hematoma, hemangioma, or inhalational injury.
  • It is often easy to mistake epiglottitis for croup. Epiglottitis differs clinically from croup by its progressive worsening, lack of a barking cough, and a cherry red swollen epiglottis versus a red/pink, nonswollen epiglottis in croup. One way doctors can tell epiglottitis from croup is with X-rays of the neck.

What Is the Treatment for Epiglottitis?

Currently, immediate hospitalization is required whenever the diagnosis of epiglottitis is suspected since the person is in danger of sudden and unpredictable closing of the airway. Doctors must establish a secure way for the person to breathe. Antibiotics may be prescribed to the patient.

  • Initial treatment of epiglottitis may consist of making the patient as comfortable as possible including placing an ill child in a dimly lit room with the parent holding the child, humidified oxygen, and close monitoring. If there are no signs of respiratory distress, IV fluids may be helpful. It is important to prevent anxiety because it may lead to an acute airway obstruction, especially in children.
  • People with possible signs of airway obstruction require laryngoscopy in the operating room with proper staff and airway intervention equipment. In severe cases, the doctor may need to perform a cricothyrotomy (cutting the neck to insert a breathing tube directly into the windpipe).
  • IV antibiotics may effectively clear infection and control inflammation in the body. Antibiotics usually are prescribed to treat the most common types of bacteria. Blood cultures usually are obtained with the premise that any organism found growing in the blood can be attributed as the cause of the epiglottitis.
  • Corticosteroids and epinephrine are used, but there is no good evidence these medications are helpful in cases of epiglottitis.

Patients should continue taking all antibiotics until the full course is completed. They should keep all follow-up appointments with the doctor. Most people improve significantly before leaving the hospital, so taking the antibiotics and returning to the hospital if there are any problems are the most important parts of follow-up.

How Can Epiglottitis Be Prevented?

Prevention of epiglottitis can be achieved with proper vaccination against H. influenza type b (Hib). It is important that children are vaccinated against Hib. Adult vaccination is not routinely recommended, except for people with immune-related medical conditions such as sickle cell anemia, splenectomy, cancers, or other diseases affecting the immune system.

When there is a member of a family with an unvaccinated child under age 4 years of age who is exposed to a person with H influenza epiglottitis, preventive medication such as rifampin (Rifadin) should be given to all household contacts to make sure that both the person with the illness and the rest of the household have the bacteria completely eradicated from their bodies. This prevents formation of a "carrier state" in which a person has the bacteria in the body but is not actively sick. Carriers can still spread the infection to other family members even though they are not ill.

What Is the Outlook for a Person With Epiglottitis?

A person with epiglottitis can recover very well if the condition is caught early and treated in time. A majority of people with epiglottitis do well and recover without problems. But if the person was not taken to the hospital early, and was not appropriately diagnosed and treated, the prognosis is poor with the possibilities of prolonged physical handicap and even death.

  • Before the Hib vaccine, mortality rates from epiglottitis were much higher. With current vaccination programs along with earlier recognition and treatment, the overall death rate from epiglottitis is estimated to be less than 0.89% - approximately 36 cases per year. The death rate from epiglottitis in adults is higher than that of children because the condition may be misdiagnosed.
  • Epiglottitis also can occur with other infections in adults, such as pneumonia. Most commonly, it is misdiagnosed as a strep throat. However, if it is suspected and treated appropriately, full recovery can be anticipated. Most of the deaths come from failure to diagnose epiglottitis in a timely fashion and resulting obstruction of the airway. As with any serious infection, bacteria may enter the blood, a condition called bacteremia, which may result in infections in other systems and sepsis (severe infection with shock, and often respiratory failure).

What Are the Signs and Symptoms of a Sore Throat?

Symptoms of sore throat from either a viral or bacterial infection can be generalized symptoms that occur throughout the body such as fever, headache, nausea, and malaise. Signs of sore throat include:

  • Pus on the surface of the tonsils can occur with bacteria or viruses
  • Tender and swollen lymph nodes in the neck
  • Drooling or spitting because swallowing is so painful
  • Difficulty breathing, especially inhaling
  • Bubbles of fluid on a red base in the oral cavity (may indicate the presence of coxsackie virus or herpes simplex virus)
References
REFERENCES:

"Haemophilus influenzae Disease (Including Hib)." CDC.gov. Updated Jun 14, 2016.
<http://www.cdc.gov/hi-disease/clinicians.html>

DiMuzio, B. et al. "Thumb sign of epiglottitis." Radiopaedia.org:
<http://radiopaedia.org/articles/thumb-sign-of-epiglottitis>

John Udeani, M. F., MD. "Pediatric Epiglottitis Clinical Presentation." Medscape. Updated Jan 14, 2016.
<http://emedicine.medscape.com/article/963773-clinical>