Overview
Epiglottitis, or supraglottitis, is an inflammation of the epiglottis and/or the supraglottic tissues surrounding the epiglottis. This includes the aryepiglottic folds, arytenoid soft tissue, and, occasionally, the uvula. Epiglottitis is a form of cellulitis.
Epiglottitis is usually an infectious process of bacterial etiology directly or from bacteremia, but it can be caused by caustic ingestion, thermal injury, or direct trauma. Historically caused by Haemophilus influenzae type B, it was also a disease of children 2-7 years of age. With the introduction of the HiB vaccine in the 1980s, not only has the incidence of the disease lessened, but also the age prevalence and the bacteriology have changed. In the United states, prior to Hib vaccine the annual incidence rate for children under 5 years was 5 per 100,000.[1] The median age of children with epiglottitis has increased from 3 years to 6-12 years.[2] Risk factors for epiglottitis include incomplete or lack of immunizations for Hib or immune deficiency.[3] In addition, a review of pediatric upper airway infections suggests that bacterial tracheitis is more prevalent now than epiglottitis or croup.[4]
The changes in age distribution have changed the clinical presentation. The classic triad of dysphagia, drooling, and distress (3 "D's") can still be seen in the younger patients. Patients younger than 1 year may have an unclear course of fever and respiratory distress. The distinction between croup, laryngotracheitis, and epiglottitis is more difficult in younger children. Older children and adults may have subtler signs of respiratory difficulties, i.e. inability to lay flat, voice changes, and dysphagia out of proportion to clinical findings. The classical signs of ‘‘tripod position’’ are drooling, stridor, dyspnea, tachypnea and a child sitting forward assuming the sniffing position in order to maximize the airway. These classical signs may not be seen in older children, adults, or those early in the disease process.[4, 5, 6] There may also be pain with movement of the larynx. In comparison to retropharyngeal abscess presentation, another differential for upper airway obstruction in children, epiglottitis usually has a more acute-onset presentation.[7] An analysis of 19,374 croup patients and 236 epiglottitis patients from 146 Korean Emergency Departments found that epiglottitis patients presented with dyspnea, sore throat, and vomiting more often than croup patients.[8] In a case series of 134 children with epiglottitis from one state, the primary symptoms were noted in the following order in regards to percentage of symptomatology; difficulty breathing (80%), stridor (80%), muffled or hoarse voice (79%), pharyngitis (73%), fever (57%), sore throat (50%), tenderness of the anterior neck (38%), cough (30%), difficulty swallowing (26%), and change in voice (20%).[9] Another case series that evaluated 102 children with croup and 101 children with epiglottitis found that cough was more common in the children with croup while drooling, preferring to sit, refusal to swallow and dysphagia were more common in the patients with epiglottitis.[5]
Other than type B H influenzae, Streptococcus pneumoniae and the Streptococcus species (including group A streptococcus), and Staphylococcus aureus (including methicillin resistant) are the most common bacterial causes.[10] Nontypeable Haemophilus influenzae have also been reported as causes of epiglottitis.[11]
Children with viral infections can also present with epiglottitis secondary to subsequent superinfection with bacteria, including Epstein-Barr, varicella-zoster, parainfluenza, and herpes simplex viruses.[11]
Epiglottitis can occur in fully immunized children, thus it should not be removed from the differential of children presenting with typical symptoms (see image below).[12]
View Image | Postintubation image in a child with acute epiglottitis. Note the cherry red epiglottis. This image was taken in 2008; the child was completely immuni.... |
As untreated, epiglottitis can rapidly lead to upper airway obstruction and death, it must be considered in children presenting with upper airway compromise and fever in younger children and sore throat out of proportion to physical findings in older children.
As with many other aspects of the pediatric airway, the epiglottis is significantly different in children and adults. 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. It is also more omega shaped and floppy than the more rigid, U-shaped epiglottis in adults. All of these anatomical differences contribute to the rapid progression of upper airway obstruction in cases of epiglottitis.
In addition to infectious causes, numerous other causes of the epiglottic inflammation exist such as thermal inhalational injury or thermal ingestion,[7, 13] trauma to the upper airway, such as foreign bodies, and chemical irritation.[14] Other etiologies include absence of immunization, an immunocompromised state, and smoking.[15] All of these diverse causes share the most serious complication of airway occlusion caused by the tissue edema; when untreated, this can lead to respiratory failure and death. In the young child, this can take place in hours. As the disease becomes more rare, its existence and its quick progression may be forgotten; therefore, it is crucial for clinicians to promptly recognize and appropriately treat epiglottitis.[16, 17] See the image below.
View Image | Comparison of a normal pediatric airway (bottom) and airway from a child who died from epiglottitis (top). |
If epiglottitis is suspected, an otolaryngologist or general surgeon, as well as an anesthesiologist, should be consulted immediately. While in the emergency department (ED), the child should be kept as calm as possible. Laryngoscopy in the ED is discouraged. Ideally, the child should be taken to the operating room to secure the airway; no diagnostic tests are required before proceeding to the operating room. In addition, because the child should be admitted to an intensive care unit, the intensivist must be consulted.
Other causes of upper airway obstruction can mimic epiglottitis, such as bacterial tracheitis, croup, retropharyngeal abscess, foreign body, diphtheria, laryngeal papillomas, angioedema and even Guillain-Barré syndrome with bilateral vocal cord paralysis and neurological compromise. It is important to manage and protect the airway in all situations involved.[18]
See also the following:
Prehospital Care
If there is any concern for epiglottitis in the field, optimally the child should initially be given 100% oxygen via non-rebreather. In a stable child, oxygen may be administered by other routes, in order to keep the child calm during transport (e.g. nasal cannula, blow-by). If the child has adequate oxygenation, urgent transport to the nearest appropriate facility is required. The facility should have airway subspecialists (anesthesiologists, ear nose and throat (ENT), and intensive care physicians), preferentially familiar with managing a pediatric airway. Everything should be done to keep the child calm, often in the arms of a parent. The conscious and stable child should be allowed to assume a position of comfort. Obtaining vital signs or any other diagnostic procedures are secondary to ensuring an adequate airway.
If the child is not stable or has a signs of respiratory distress or pending respiratory arrest, first attempt ventilation with a bag-valve mask, with correct positioning, correct size face mask, and adequate ventilation with use of end-tidal CO2. Long, slow ventilations are best. Oral airway should NOT be placed. Orotracheal intubation should be attempted if emergency service personnel are unable to ventilate the child. Needle cricothyroidotomy is used only if the airway is unable to be secured. Pre-hospital providers should alert the receiving hospital staff about the concern for airway stability and the need for airway expertise with subspecialists on arrival to the ED.
Initial ED Management
In order to correctly manage acute epiglottitis, one must first consider it as a potential diagnosis.[19] In addition to the decreased frequency, the demographic changes lead to a less than "typical" presentation. If the diagnosis is possible, the child should have respiratory and cardiac monitoring placed, and he or she should be kept in plain view of medical staff at all times. Furthermore, the child's condition must be constantly evaluated in order to determine need for emergency airway management. Minor procedures, such as intravenous access, may precipitate respiratory distress and can be performed more safely after intubation.
Epiglottitis requires immediate airway management and the assistance of airway subspecialists should be called immediately upon awareness of epiglottitis as a diagnosis. Preparation with the aide of airway subspecialists is critical in airway management for this disease.
Make sure all airway equipment is open and ready including oxygen, BVM, endotracheal tube (ETT) of the correct size and a needle cricothyroidotomy kit. Unnecessary blood tests, intravenous access, and tongue depression with a tongue blade should be avoided. Initial airway management should begin with BVM with the appropriate size face mask seal, positioning of the patient, good chest rise, and normal range of end-tidal carbon dioxide (End tidal CO2) after using the bag-valve mask (BVM). As with all airway management, if unable to provide appropriate oxygenation and ventilation with BVM, the physician should be prepared to perform intubation or continue down the difficult airway algorithm and place a surgical airway (Please see below under laryngoscopy). The patient should be taken immediately to the operating room to get an fiberoptic placement of an airway.
Precautions
Any manipulation of the upper airway with direct or fiberoptic laryngoscopy may precipitate laryngospasm; thus, visualization of the epiglottis should not be performed unless staff members capable of securing an airway are present, although in reality, no cases of laryngospasm due to such visualization have ever been reported in the literature.
Lateral neck radiographs[20] are useful when evaluating a child with symptoms suggestive of epiglottitis, but these should not delay taking the patient to the operating room for definitive management. If radiographs are ordered, the child should be in constant attendance by the medical staff with available airway equipment.
Laryngoscopy and Emergent Ventilation
If respiratory arrest occurs, the patient should be ventilated using a bag-valve mask device, and intubation would be the next step in airway management. Intubation can be performed using video laryngoscopy or direct laryngoscopy. Video laryngoscopy is the preferred method of intubation compared to direct, with the most skilled airway provider to use technology to allow for a single attempt at intubation. In a patient with respiratory distress, "rescue" airway equipment should be prepared before rapid sequence intubation. The patient should be allowed to breath on their own, and should not be given a paralytic as part of rapid sequence intubation. As noted before, airway subspecialists should be notified of the airway risk, and a collective decision should be made regarding intervention. The provider with the most airway experience should manage the airway. Supraglottic airway such as a laryngeal mask airway should NOT be used for airway management, these devices are not effective for upper airway obstruction. Again optimally the patient should be taken immediately to the operating room to get an fiberoptic placement of an airway.
Orotracheal intubation or needle cricothyroidotomy (also known as percutaneous transtracheal ventilation or translaryngeal ventilation) may be necessary in emergent situations. Pediatric epiglottitis is one of the few instances in which the emergency physician may need to rapidly perform needle cricothyrotomy. See Percutaneous Transtracheal Ventilation in Pediatric Epiglottitis. Needle cricothyrotomy is a temporizing method used to treat cases of severe epiglottitis when the patient cannot be intubated before a formal tracheostomy. This procedure 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.
Tracheostomy should be reserved for patients in which endotracheal intubation is unsuccessful due to severe laryngeal edema.
Oxygen wall ports: pressure-controlled versus volume-controlled
Most physicians are not aware that normal, volume-controlled, oxygen wall ports are incapable of delivering the pressure needed to adequately oxygenate an adult through a 14-gauge catheter (50 psi). In some trauma centers, needle jet setups are specifically preinstalled to address this concern, with a pressure-controlled port (instead of the volume-controlled port). For children, a setting of 1 psi/kg is recommended, although literature data are lacking.
Transtracheal jet insufflation does little for ventilation; however, it may salvage enough time in cases of complete inspiratory airway occlusion to perform tracheostomy or begin extracorporeal bypass maneuvers.
Antibiotic Therapy
Antibiotic therapy is necessary but should be initiated after securing the airway. Empiric antimicrobial therapy must cover all likely pathogens in the context of the clinical setting.
Before culture results, use antibiotic agents that will cover the most likely organisms. Antibiotics should include third generation cephalosporin (e.g. Cefotaxime) AND an antistaphylococcal agent (e.g vancomycin).[20] The cause of infection is currently more likely to be Staphylococcus aureus (especially following trauma to the epiglottitis) or group A streptococci[21, 22, 23] as opposed to Haemophilus influenzae type B (Hib) because of widespread vaccine use against this organism,[24, 4] and antibiotic therapy should be directed as such. With the presence of white patches, Candida albicans should be suspected.[25] Sedation for comfort is also required.
In general, antibiotic treatment should continue for 7-10 days.
PICU Admission
The child should be admitted to the pediatric intensive care unit (PICU) and, if intubated, should be sedated and/or paralyzed.
No controlled studies exist on the use of intravenous steroids for reduction of airway structure edema due to pediatric epiglottitis. Nonetheless, some clinicians routinely use them in cases with adults.
Laryngoscopy should be repeated 24-48 hours after treatment to evaluate the degree of inflammation,[26] and a decision should be made when to consider extubation. Criteria for extubation include air leaks around the endotracheal tube or decreased edema and erythema of the epiglottis.
After observation of 24-48 hours postextubation, the patient can be discharged on oral antibiotics.
Transfer
If the hospital is unable to care for critically ill children, transfer should be arranged to the nearest appropriate facility, which, ideally, would be a hospital with a pediatric intensive care unit (PICU).
The airway should always be secured. Only then should an intravenous line be placed. In addition, the child should be sedated and given antibiotics before the transfer.
Transport of patients with epiglottitis may be a concern, especially for patients who are maintaining an airway in the emergency department but who could lose airway protection during transport. A survey done at the 1990 Pediatric Critical Care Transport Leadership Conference showed that 49% of physicians recommend intubation before interhospital transfer. The other 49% made decisions on a patient-to-patient basis.[27] Therefore, the physician should use clinical judgment in making this decision.
Author
Sylvia Owusu-Ansah, MD, MPH, FAAP, Fellow in Emergency Medical Services, Johns Hopkins University School of Medicine
Disclosure: Nothing to disclose.
Specialty Editors
Mary L Windle, PharmD, Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference
Disclosure: Nothing to disclose.
Wayne Wolfram, MD, MPH, Professor, Department of Emergency Medicine, Mercy St Vincent Medical Center; Chairman, Pediatric Institutional Review Board, Mercy St Vincent Medical Center, Toledo, Ohio
Disclosure: Nothing to disclose.
Chief Editor
Kirsten A Bechtel, MD, Associate Professor of Pediatrics, Section of Pediatric Emergency Medicine, Yale University School of Medicine; Co-Director, Injury Free Coalition for Kids, Yale-New Haven Children's Hospital
Disclosure: Nothing to disclose.
Additional Contributors
James Li, MD, Former Assistant Professor, Division of Emergency Medicine, Harvard Medical School; Board of Directors, Remote Medicine
Disclosure: Nothing to disclose.
Robert Allan Felter, MD, FAAP, CPE, FACPE, Professor of Clinical Pediatrics, Department of Pediatrics, Georgetown University School of Medicine; Assistant Professor of Pediatrics, George Washington University School of Medicine and Health Sciences; Attending Physician, Emergency and Trauma Center, Children's Hospital National Medical Center
Disclosure: Nothing to disclose.
Acknowledgements
Ron D Waldrop, MD, MS, FAAP, FACEP, FACPE Attending Physician, Pediatric Emergency Medicine, Huntsville Women’s and Children’s Hospital; Assistant Clinical Professor, Georgetown University School of Medicine
Ron D Waldrop, MD, MS, FAAP, FACEP, FACPE is a member of the following medical societies: American Academy of Pediatrics, American College of Emergency Physicians, American College of Physician Executives, and National Association for Healthcare Quality
Disclosure: Nothing to disclose.
){kind=link}
){kind=link}