Pediatric Epiglottitis

As with many other aspects of the pediatric airway, the epiglottis is significantly different in the child than in the adult. In the infant, the epiglottis is located more anteriorly and superiorly than in the adult, and it is at a greater angle with the trachea. The infant epiglottis is also more omega shaped and floppy than the more rigid, U-shaped epiglottis in the adult.

Haemophilus influenzae type b (Hib) or Streptococcus pneumoniae (see Etiology) can colonize the pharynges of otherwise healthy children through respiratory transmission from intimate contact. These bacteria may penetrate the mucosal barrier, invading the bloodstream and causing bacteremia and seeding of the epiglottis and surrounding tissues. Bacteremia may also lead to infection of the meninges, skin, lungs, ears, joints, and other structures.

Hib infection of the epiglottis leads to acute onset of inflammatory edema, beginning on the lingual surface of the epiglottis where the submucosa is loosely attached. Swelling significantly reduces the airway aperture. Edema rapidly progresses to involve the aryepiglottic folds, the arytenoids, and the entire supraglottic larynx. The tightly bound epithelium on the vocal cords halts edema spread at this level. Frank airway obstruction, aspiration of oropharyngeal secretions, or distal mucous plugging can cause respiratory arrest.

Noninfectious inflammation of any of the structures around the epiglottis may also result from thermal or chemical injury or from local trauma, including blunt trauma to the neck.[1]

Historically, Haemophilus influenzae type b (Hib) was the predominant organism (>90%) in pediatric epiglottitis cases. Since the widespread use of the Hib vaccine, the incidence and causative agents of epiglottitis have changed; however, even vaccinated children can develop epiglottitis due to non – type b H influenzae.[2] Clearly, epiglottitis due to Hib persists in parts of the world where Hib vaccination is not used.

The following are other known bacterial causes of pediatric epiglottitis:

• Streptococcus pneumoniae

• Group A and group C (ie, beta-hemolytic) streptococci

• Staphylococcus aureus

• Moraxella catarrhalis

• Haemophilus parainfluenzae

• Neisseria meningitidis

• Pseudomonas species

• Candida albicans, especially in immunocompromised patients

• Klebsiella pneumoniae

• Pasteurella multocida

Although viruses normally do not cause epiglottitis, a previous viral infection may allow bacterial superinfection to occur. Viral agents may include herpes simplex virus (HSV), parainfluenzae virus, varicella-zoster virus (VZV), human immunodeficiency virus (HIV),[3] and Epstein-Barr virus (EBV). Varicella can cause a primary or secondary infection often with group A beta-hemolytic streptococci.

Noninfectious etiologies include thermal injuries, trauma-causing blind finger sweeps to remove a foreign body from the pharynx, angioneurotic edema, hemophagocytic lymphohistiocytosis,[4] and acute leukemia. Lymphoproliferative diseases may also cause epiglottic swelling.

Historically, acute epiglottitis was most common in children aged 2-4 years. The use of the Haemophilus influenzae type b (Hib) vaccine has reduced incidence of epiglottitis in the United States, making this a rare condition in children.[5] Introduction of the polysaccharide vaccine in 1985, followed by the highly effective conjugate vaccine, dramatically reduced the incidence of epiglottitis, with concomitant declines in hospital admissions. Studies have shown an annual incidence rate of 0.63 cases per 100,000 persons,[6] and studies of children of all ages with epiglottitis report a seasonal variation in incidence.

A comparison made between a large US children's hospital's chart review from 1995 to 2003 and a previous report from the same hospital completed 27 years earlier, showed a 10-fold decline in acute epiglottitis admissions, with streptococci becoming the major pathogens.[7] Epiglottitis incidence in adults has remained constant.

A retrospective case series of 107 patients admitted to a pediatric hospital's intensive care unit (ICU) from 1997 to 2006 concluded that bacterial tracheitis is now 3 times more likely to be the cause of pediatric respiratory failure compared with viral croup and epiglottitis combined. The authors attributed this change in the epidemiology of acute infectious upper airway disease to Hib vaccine as well as the use of corticosteroids for the treatment of viral croup.[8]

The international incidence of epiglottitis widely varies, with a significantly greater prevalence in countries without universal immunization. Among countries with mandatory immunization, the reported incidences are 0.9 cases per 100,000 persons in Sweden and 0.6 cases per 100,000 in the United Kingdom, for example. However, in recent years, epiglottitis has been increasing in frequency in the United Kingdom.[9] The reason for this is unclear and may be due to the administration of 3 vaccines rather than 4. Other studies suggest that bacterial tracheitis is now the most common serious airway infection in children.[8, 10]

A retrospective review of a Danish population demonstrated a mean national incidence of epiglottitis in children of 4.9 cases per 100,000 per year in the decade before Hib vaccination. From 1996 to 2005, with the introduction of widespread Hib vaccination, an incidence of only 0.02 cases per 100,000 per year was seen. During this period, the incidence of acute epiglottitis in adults remained constant, at 1.9 cases per 100,000 per year.[9]

Racial, sexual, and age differences in incidence

Most studies show no racial predominance for epiglottitis, although a recent study showed higher incidence among Black and Hispanic individuals. There also appears to be a 60% male predominance, which has remained true even with the changing epidemiology of epiglottitis.

Epiglottitis was once believed to occur exclusively in children. In the past, this condition occurred most commonly in children aged 2-4 years; however, it may occur at any age. Adult cases have been reported in recent years, and some evidence suggests the incidence in adults is increasing.

The prognosis is good for patients with epiglottitis whose airways have been secured. The mortality rate is less than 1% in these patients. However, mortality rates as high as 10% can occur in children whose airways are not protected by endotracheal intubation.

An analysis by Allen et al of US mortality trends from 1979 to 2017 showed that deaths from acute epiglottitis decreased following the widespread use of the Hib vaccine. Of a total 1187 epiglottitis-related deaths during the 39-year period, 443 occurred among children and adolescents. Mortality rates in this age group fell from 0.064 per 100,000 individuals (41 deaths) in 1979 to 0.001 per 100,000 individuals (1 death) in 2017.[11]

Complications

During the bacteremic phase of the disease, other foci of infection are possible. Pneumonia is the most commonly cited associated illness, followed by otitis media. Meningitis has also been reported in association with epiglottitis.

As with other causes of upper airway obstruction, pulmonary edema can be observed after the airway has been secured. Accidental extubation and respiratory arrest are the 2 most common complications, and accidental extubation can cause additional complications. Cervical adenitis, tonsillitis, and otitis media have also been documented.

In summary, complications associated with a swollen epiglottis and surrounding tissues include airway obstruction, which can lead to respiratory arrest and death from hypoxia as well the following:

• Aspiration

• Endotracheal tube dislodgement

• Extubation

• Tracheal stenosis

• Pneumothorax or pneumomediastinum

• Epiglottic abscess

• Adenitis

• Cervical cellulitis

• Septic shock

• Pulmonary edema (rare)

• Cerebral anoxia

• Death from asphyxia

In classic cases involving bacteremia with Haemophilus influenzae, other structures may have concomitant infectious processes. These may include the following:

• Meningitis

• Pneumonia

• Septicemia

• Cellulitis

• Septic arthritis

• Otitis media

• Pericarditis (rare)

For patient education information, see Cold & Flu Center. Also see the WebMD patient education resource Epiglottis.

Epiglottitis is characterized by the abrupt onset of severe symptoms. Without airway control and medical management, symptoms may rapidly progress to respiratory obstruction and death in a matter of hours.

Usually, no prodromal symptoms occur in children. Fever is usually the first symptom, and temperatures often reach 40°C. This is rapidly followed by stridor and labored breathing. Dysphagia, refusal to eat, muffled (ie, guttural) or hoarse voice, sore throat, and anxiety are common. Cough and ear pain are less frequent.

The clinical triad of drooling, dysphagia, and distress is the classic presentation. Fever with associated respiratory distress or air hunger occurs in most patients. Drooling occurs in up to 80% of cases.

If the cause of epiglottitis is not infectious, the presentation may vary. A child presenting with upper airway respiratory distress without an obvious source or fever should be questioned regarding the possibility of ingestion of a toxic or hot liquid, or a traumatic event such as falling on an object with an open mouth or swallowing or having a foreign body removed.[12]

The child appears toxic; shock may occur early in the course of the disease. Marked restlessness, irritability, and extreme anxiety are common.

The child may sit with his or her chin hyperextended and body leaning forward (ie, tripod or sniffing position) to maximize air entry and improve diaphragmatic excursion (see the following image).

Child assuming the sniffing position with upper airway obstruction.

The mouth may be open wide and the tongue may protrude; an affected child often drools, because swallowing is difficult or painful.

An erythematous and classic swollen, cherry red epiglottis can often be seen during careful examination of the oropharynx, although this examination should not be attempted if it may compromise respiratory effort.

Early on, the child may have stridulous respirations, but as the disease progresses, airway sounds may diminish. Stridor can occur with marked suprasternal, subcostal, and intercostal retractions.

Anterior neck examination may reveal tender adenopathy. In the older child, pain may be noted on movement of the hyoid bone.[13]

Cyanosis, which occurs late in the course of the condition, indicates a poor prognosis.

Securing an airway is the overriding priority. An expert in pediatric airway management should always perform an endotracheal intubation on any child with suspected epiglottitis before radiography or blood work is performed.

Laryngoscopy is the best way to confirm the diagnosis, but it is not advised to attempt any procedures without securing the airway. Simply depressing the child's tongue with a tongue blade may visualize the epiglottitis in some situations. Some concern exists regarding the safety of such procedures, which can provoke anxiety and increased respiratory effort during examination leading to airway obstruction.

Laboratory evaluation is nonspecific in patients with epiglottitis and should be performed once the airway is secured. The white blood cell (WBC) count may be elevated from 15,000-45,000 cells/µL with a predominance of bands. Histologic examination reveals massive infiltration with polymorphonuclear leukocytes and inflammatory edema.

Classic cases of epiglottitis require no radiographic evaluation; however, radiography may be needed in some cases to confirm the diagnosis and to exclude other potential causes of acute airway obstruction. When radiography is required to exclude other diagnoses, perform portable radiography at the patient's bedside.

Recommendations for computed tomography (CT) scanning of the neck in early or unusual cases have been suggested,[15] although great care should be used because of the positioning of the patient.

If epiglottitis is in the differential diagnosis, the child should never be left alone even if imaging studies are being obtained. The child should always be accompanied by personnel who are able to achieve rapid airway access if needed.

Blood cultures and culture of the epiglottis should be performed only after the airway is secured.

Blood cultures may show Haemophilus influenzae type b (Hib) between 12-15% and 90% of cases.

Cultures of the surface of the epiglottis obtained during endotracheal intubation are positive in 50-75% of cases.[16]

If epiglottitis is seriously considered, no imaging studies are required. In less-clear cases, imaging studies are occasionally helpful in establishing the diagnosis or excluding epiglottitis.

Lateral neck radiography

Never obtain a lateral neck radiograph before achieving definitive airway control. If radiography is required, the safest procedure is to perform portable radiography at the bedside.

In classic epiglottitis, a lateral soft-tissue radiograph of the neck reveals a swollen epiglottis protruding from the anterior wall of the hypopharynx (ie, thumbprint sign),[17] thickened aryepiglottic folds, obliteration of the vallecula, and dilation of the hypopharynx (see the following images). Note that negative findings on lateral radiographs do not exclude the diagnosis, especially in the early stages of presentation.

Swollen epiglottis with characteristic thumbprint sign.

Radiograph of a child with acute epiglottitis; note the hypopharyngeal dilatation, obliteration of the vallecula, and thickened aryepiglottic folds—a positive thumb sign.

Chest radiography

Chest radiography may reveal concomitant pneumonia in as many as 15% of patients. Postintubation chest radiographs occasionally show pulmonary edema.

Laryngoscopy can help exclude other diagnoses in an older child who is cooperative. However, do not perform a laryngoscopy if the procedure might increase anxiety, which can exacerbate the airway obstruction.

The naris can be anesthetized with lidocaine jelly before inserting the fiberoptic laryngoscope. Insert the laryngoscope through the naris, advancing it slowly into the supraglottic region. The epiglottis should be easily visualized to determine the presence of swelling.

A study performed in Germany recommended laryngoscopy to aid in the diagnosis in patients with atypical presentations or with crouplike coughs. This study also showed that fiberoptic endoscopy is especially useful in cooperative older children with moderate respiratory distress.

Also termed needle cricothyrotomy or translaryngeal ventilation, percutaneous transtracheal ventilation is a temporizing method used to treat cases of severe epiglottitis when the patient cannot be intubated before a formal tracheostomy.

Percutaneous transtracheal ventilation involves inserting a needle through the cricothyroid membrane, which lies inferior to the thyroid cartilage and superior to the cricoid cartilage. The cricothyroid arteries typically course through the superior portion of the membrane.

The procedure is performed as follows:

• Begin by localizing the cricothyroid membrane. This can be accomplished by finding the thyroid cartilage prominence (ie, Adam's apple) and running a finger down until the depression of the cricothyroid membrane is felt. Another method is to find the trachea and run the fingers up along the tracheal rings until a more prominent bulge representing the cricoid ring is felt; the cricothyroid membrane is above this bulge. The latter technique has been shown to be more useful in small infants.

• Prepare the membrane area with antiseptic solution. If the patient is awake, use lidocaine to anesthetize the skin overlying the membrane.

• Use the thumb and middle finger of the nondominant hand to hold the trachea in place. In the dominant hand, hold a 3-mL or 5-mL syringe containing 2 mL of saline or lidocaine, which is attached to the needle and 16-gauge or 18-gauge catheter.

• Place the needle through the inferior portion of the cricothyroid membrane at the midline, caudally directing the needle at a 45° angle, and puncture the skin and subcutaneous tissue. A small incision with a No. 11 scalpel facilitates needle and catheter insertion.

• Apply continuous negative pressure while advancing the needle. The needle should be inside the trachea when bubbles become visible in the syringe.

• Advance the catheter off the needle until its hub rests against the skin surface. Remove the needle and syringe.

• Connect high-pressure tubing to the catheter and administer 100% oxygen at 25-35 pounds per square inch for small children.

Perform ventilations at a rate of 1 second of inhalation to 4-5 seconds of exhalation. Exhalation is easily accommodated by cutting a small hole in the distal end of the high-pressure tubing to create a side hole. Inhalation occurs when the hole is covered with a finger, exhalation when the hole is left open.

Treatment in patients with epiglottitis is directed toward relieving the airway obstruction and eradicating the infectious agent. Optimally, initial treatment is provided by a pediatric anesthesiologist and either a pediatric surgeon or a pediatric otolaryngologist. Once the airway is controlled, a pediatric intensivist is required for inpatient management.

Avoid procedures that might increase the child's anxiety until after the child's airway is secured. Procedures such as venipuncture and intravenous access, although appropriate in most cases involving children with acute epiglottitis, may heighten anxiety and precipitate airway compromise.

Do not underestimate the potential for sudden deterioration. As soon as epiglottitis is suspected, initiating and mobilizing a medical and surgical team capable of securing the airway is imperative.

Never place a child in a supine position (other than during the endotracheal intubation procedure), because immediate respiratory arrest in this position has been reported.

See also Epiglottitis and Emergent Management of Pediatric Epiglottitis.

When a child has respiratory arrest, the first step is to administer bag-valve-mask ventilation with 100% oxygen. All of these children can be oxygenated and ventilated with good bag-valve-mask technique. Once the child is oxygenated and ventilated, the airway can be secured with an endotracheal tube, cricothyrotomy, or tracheostomy. These treatments should prevent cerebral anoxia, arrest, and death, the most feared complications.

Once an airway is established, admit the child with epiglottitis to an intensive care unit (ICU), where the patient should be sedated and/or paralyzed to prevent inadvertent extubation.

Medical treatment begins by evaluating airway, breathing, and circulation. Supplemental oxygen administration, a nonthreatening initial step, is easily accomplished with blow-by oxygen administered by a parent.

Place the equipment needed for emergent airway management at the bedside, and keep the patient in view at all times.

As discussed in Managing Respiratory Arrest, if acute respiratory arrest occurs, ventilate the child with 100% supplemental oxygen, using a bag-valve-mask device, and arrange for intubation. When a child has a respiratory arrest and appropriate surgical personnel are unavailable, the attending physician may attempt intubation.

Alternative methods to gain immediate control of the airway, such as needle cricothyrotomy, are considered temporary until a more permanent procedure (eg, tracheostomy) can be performed.

Once supplemental oxygen is provided, the next crucial step is to mobilize a team to establish an appropriate airway via endotracheal intubation. Mortality rates for children who receive endotracheal intubation are less than 1%. Children who do not receive intubation have mortality rates as high as 10%.

At a minimum, the team should include an anesthesiologist and a surgeon capable of establishing a pediatric surgical airway (ie, tracheostomy). Ideal team members would be a pediatric anesthesiologist and a pediatric surgeon or pediatric otolaryngologist.

The best setting for an endotracheal intubation is in an operating room with the patient under general anesthesia.

Endotracheal intubation procedure details are as follows:

• Move the patient to the operating room and prepare the equipment needed for a tracheostomy and bronchoscopy.

• Place the precordial stethoscope and electrocardiograph and pulse oximetry leads; then, with the patient in a sitting position, induce anesthesia using a mask with oxygen and halothane.

• Once the child is anesthetized, place the patient in a supine position and insert an intravenous line.

• Perform a laryngoscopy while the patient is under deep halothane anesthesia, inserting an orotracheal tube 0.5-1.0 mm smaller than predicted for the child.

When the endotracheal tube is in place, an otolaryngologist should examine the supraglottic structures using direct laryngoscopy and obtain appropriate surface cultures of the epiglottis. A secured nasotracheal tube usually replaces the orotracheal tube.

Tracheostomy

If endotracheal intubation is unsuccessful, perform a tracheostomy with percutaneous translaryngeal ventilation used as a temporizing measure (see Percutaneous Transtracheal Ventilation).

Racemic epinephrine administration plays little role in the management of infectious or thermal epiglottitis and is not indicated.

Appropriate antibiotics include ceftriaxone, cefotaxime, and cefuroxime (for nonmeningitic infections) (see Medications). As in all invasive Haemophilus influenzae type b (Hib) infections, contacts should receive rifampin chemoprophylaxis. For epiglottitis due to other organisms, antibiotics should be tailored to the cause of the infection.

Corticosteroid administration, although advocated in the past based on anecdotal reports, remains controversial. These agents have no proven efficacy for treating epiglottitis.

Although epiglottitis has declined with the use of the Haemophilus influenzae type b (Hib) conjugate vaccine, epiglottitis can still occur, albeit rarely, in a child who is adequately vaccinated.

All close contacts (including daycare center staff and children) who are exposed to a child with epiglottitis should receive a 4-day prophylactic course of treatment with rifampin at 20 mg/kg (not to exceed 600 mg/d).

Children older than 2 years with epiglottitis do not need vaccination, because the disease provides immune protection.

Patients recovering from epiglottitis may be extubated when repeated direct laryngoscopy at 24- to 48-hour intervals indicates reduced size and inflammation of the epiglottis. Criteria for extubation include decreased erythema and edema of the epiglottis and air leaks around the endotracheal tube.

The intravenous catheter may be removed when the patient can tolerate oral fluids and antibiotics. The total duration of antibiotic treatment is 7-10 days.

After further observation for 24-36 hours, patients who are afebrile and doing well may be discharged.

Antibiotic therapy is necessary in the management of epiglottitis but should be initiated after the airway is secured. Before obtaining culture results, use antibiotics for the most likely organisms. Following trauma to the epiglottis, Staphylococcus aureus should be suspected. With the presence of white patches, Candida albicans should be suspected. Sedation for comfort is also required.

Class Summary

Initiate antibiotics to provide empiric coverage of the most likely bacterial pathogens in the context of the clinical setting. Treatment should continue for 7-10 days, in general.

Ceftriaxone (Rocephin)

Ceftriaxone is a third-generation cephalosporin antibiotic with broad-spectrum activity against gram-negative bacteria, including Haemophilus influenzae, Enterobacteriaceae, and Neisseria species, and variable activity against gram-positive bacteria. This agent binds to penicillin-binding proteins and inhibits the final transpeptidation step of peptidoglycan synthesis, resulting in cell wall destruction and death of the organism.

Cefotaxime (Claforan)

Cefotaxime is another third-generation cephalosporin antibiotic with broad-spectrum activity against gram-positive and gram-negative bacteria. This agent binds to penicillin-binding proteins and inhibits the final transpeptidation step of peptidoglycan synthesis, resulting in cell wall destruction and death of the organism.

Cefuroxime (Zinacef , Kefurox)

Cefuroxime is a second-generation cephalosporin antibiotic with activity against gram-positive and some gram-negative bacteria, including Haemophilus influenzae. Cefuroxime binds to penicillin-binding proteins and inhibits the final transpeptidation step of peptidoglycan synthesis, resulting in cell wall destruction and death of the organism.

Chloramphenicol

When combined with ampicillin, chloramphenicol is an alternative agent if cephalosporins are unable to be used. This agent elicits activity against some gram-positive, gram-negative, and anaerobic organisms by inhibiting protein synthesis via reversibly binding to the 50S ribosomal subunit. Although unavailable in the United States, this antibiotic remains in use in parts of the world.

Clindamycin (Cleocin)

Clindamycin is a semisynthetic antibiotic produced by the 7(S)-chloro-substitution of the 7(R)-hydroxyl group of the parent compound, lincomycin. It is useful for gram-positive infections, including most community-associated MRSA disease. This agent inhibits bacterial growth, possibly by blocking dissociation of peptidyl tRNA from ribosomes, causing RNA-dependent protein synthesis to arrest. Clindamycin widely distributes in the body without penetration of the central nervous system. This drug is protein bound and excreted by the liver and kidneys.

Ampicillin and sulbactam (Unasyn)

Ampicillin with sulbactam is the drug combination of a beta-lactamase inhibitor with ampicillin. This combination interferes with bacterial cell wall synthesis during active replication, causing bactericidal activity against susceptible organisms.

Ampicillin with sulbactam is used as an alternative to amoxicillin when the patient is unable to take medication orally. This combination covers skin, enteric flora, and anaerobes but is not ideal for nosocomial pathogens.

Rifampin (Rifadin)

Rifampin is used for chemoprophylaxis in Hib infections.

Class Summary

Analgesic-antipyretic agents are helpful in relieving the lethargy, malaise, and fever associated with epiglottitis.

Acetaminophen (Tylenol, Acephen, Aspirin-Free Anacin)

Acetaminophen is the drug of choice (DOC) for treating pain in patients with documented hypersensitivity to aspirin or nonsteroidal anti-inflammatory drugs (NSAIDs), those with upper gastrointestinal disease, or those who take oral anticoagulants. This agent reduces fever by a direct action on the hypothalamic heat-regulating centers, which increases dissipation of body heat via vasodilation and sweating.

Ibuprofen (NeoProfen, Advil, Motrin)

Ibuprofen is usually the DOC for treating mild to moderate pain, if no contraindications exist. This agent inhibits inflammatory reactions and pain, probably by decreasing the activity of the cyclooxygenase enzyme, which inhibits prostaglandin synthesis. Ibuprofen is one of the few nonsteroidal anti-inflammatory drugs (NSAIDs) indicated for fever reduction.

Class Summary

These agents are used to induce active immunization against Haemophilus influenza type b.

Haemophilus b conjugate vaccine (ActHIB, Hiberix, PedvaxHIB)

This vaccine is used for routine immunization of children against invasive diseases caused by H influenzae type b by decreasing nasopharyngeal colonization. The CDC's Advisory Committee on Immunization Practices (ACIP) recommends that all children receive one of the conjugate vaccines licensed for infant use beginning routinely at age 2 months.