Epiglottitis: Diagnosis and Treatment

Jonathan R. Bates, MD

Epiglottitis is a rapid and poten- patients with meningitis, these pa- tially fatal of the supra- tients may resist rotation as EDUCATIONAL glottic that causes death by well as flexion. OBJECTIVES upper and Gram- A toxic child with and signs negative bacteremia with Haemophi- of pharyngeal tenderness with a nor- Evaluate the child with fever and and be able to diagnose and lus influenzae type B (HIB). The ma- mal throat inspection may well have manage: (1) acute viral ; jonity of patients (80%) are less than epiglottitis. A soft tissue lateral neck (2) laryngotracheobronchitis; (3) 5 years of age, and the remaining x-ray will usually demonstrate char- diphtheria; (4) foreign body; (5) 20% are scattered over the remain- actenistic radiographic features even ; (6) epig- ing life span. Although sporadically at this stage. lottitis; and (7) adenoiditis. recognized since the early 1 6th cen- As the swelling increases, respi- tury, the disease has only become ratory distress gradually develops well-described since the late 1 940s. over the next few hours. Slight Initial mortality rates approaching tachypnea with flaring of the alae 1 00% have steadily declined to nasi, retractions of the chest wall, about 2% with the use of artificial and use of the accessory muscles of airways and . respiration progresses with the ap- The key to obtaining a good out- pearance of characteristic raspy, come with available effective then- coarse inspiratory breath sounds apy in this disease is prompt recog- quite distinct from the almost musi- nition and rapid initiation of therapy. cal tone heard in patients with croup. Delays of even a few minutes can be The voice may be noticeably muffled disastrous. Advanced planning for as if the child has a mouthful of the handling of such patients in the mashed potatoes, but this may be a office, clinic, or emergency room subtle sign, apparent only to the has been effective in avoiding death mother. At this stage the child as- from such delays. Decisive action sumes the classic posture of epi- can make the difference between glottitis; sitting upright, leaning for- death or anoxic damage as an out- ward, open-mouthed, and finally come, and complete recovery with- drooling as the swollen be- out sequelae. comes too painful even for swallow- ing saliva. Although some children CLINICAL PICTURE at this stage are obviously air-hun- The disease typically begins ab- gry and anxious, others are remark- ruptly with the onset of fever greater ably still and seem intent on holding than 102#{176}F (rectal) and sore throat their position to maximize ventila- or difficulty swallowing. These two tion. With the onset of respiratory features of epiglottic distress, the diagnosis can virtually signal the onset of this illness and always be made from the history and occur simultaneously or within a few observation of the position of the hours of each other. If seen at this patient (Table). stage, the diagnosis is not simple, From this point onward, the child but is suggested by absent or mini- should have supplemental oxygen mal pharyngeal findings and toxicity. (humidified, if possible), proper air- About 20% of patients have a cough way equipment, and experienced and, in some, vomiting is present. cardiopulmonary resuscitation per- Respiratory distress is absent ini- sonnel at his side until an endotra- tially and this makes the diagnosis cheal tube or tracheostomy can be ___ I- more obscure. Some patients are placed. Once tachypnea begins, the Dr Bates is Director, Emergency Medical Ser- reluctant to move their neck and this child begins to tire and respiratory vices, Children’s Hospital Medical Center, may mimic nuchal rigidity, but Ken- and cardiac arrest will follow, within and Instructor, Department of Pediatrics, Har- nig’s sign is absent. In contrast to minutes to a few hours. vard Medical School, Boston.

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whose airway is obstructed may re- tion. In a modest number of in- quire pressures greater than 50 mm stances, the child can voluntarily TABLE. Diagnosis of Epiglottitis Hg to achieve ventilation. Thus, yen- open his mouth wide enough to en- Early symptoms tilation may be impossible using a able the examiner to see the swollen Fever bag with a pressure-release or cherry-red epiglottis without using a : Sore throat with minimal on absent ‘‘ ‘ . pharyngeal findings pop-off’ valve. Many chambers stick or changing the child’s posi- that are used to humidify oxygen are tion. Swallowing difficulty equipped with a similar pressure- The lateral neck radiograph must Toxicity release valve. If ventilation is not be reviewed promptly since the de- Later symptoms-signs Limited neck mobility, both flexion achieved when using equipment with cision to perform a tracheotomy or and notation pressure-release valves, change to to intubate the patient may be influ- Muffled voice either mouth-to-mouth ventilation or enced by the interpretation. Fig 1 is Tachypnea, tachycandia use a limp bag that inflates by incom- a lateral neck film typical of those Flaring of alae nasi ing gas pressure connected directly seen with epiglottitis. There are five Retractions of the chest wall to the oxygen outlet without a humi- hallmarks: (1) an open and/or pro- Raspy inspiratony sound difying chamber. Both techniques truding jaw, (2) a dilated hypophar- Late signs can produce the higher pressures ynx, (3) encroachment of the lingual Drooling needed to overcome the upper air- aspect of the epiglottis on the vallec- Air hunger way obstruction. ula, (4) a doughy, thickened appear- Sitting forward Transport, even within the hospi- ance of the enlarged epiglottis, any- tal, should follow these guidelines. If tenoids, and aryepiglottic folds transporting from the office or clinic which resemble the shadow of an TRANSPORTATION to a hospital, specific information adult thumb, and (5) a straight or Once the diagnosis is made or (age, heart rate, respiratory rate, es- kyphotic cervical column as op- suspected, transportation to a hos- timated arrival time) should be trans- posed to the usual lordosis. The ap- pital or to a place within a hospital mitted early on to ensure maximal pearance of the supraglottic struc- where definitive care can be ren- preparedness at the center. tunes (‘ ‘thumb sign’ ‘) is most critical, dered must be accomplished rapidly while the other findings (1 , 2, 3, and and safely. The child should be DIAGNOSIS 5) may be variable depending on the closely monitored and constantly at- Confirmation of the diagnosis in- degree of obstruction. For compari- tended by someone trained in car- volves two considerations: (1) Is it son, a normal lateral neck film is diopulmonary resuscitation (CPR). epiglottitis? and (2) What is the shown in Fig 2, and a case with Administration of humidified oxygen causative organism and its croup (laryngotnacheobronchitis) is will temporarily benefit the child sensitivities? shown in Fig 3 where the subglottic while in transit. The child should be If the patient presents the typical haze and narrowing are apparent. disturbed as little as possible (eg, no picture as described, the diagnosis An anterior-posterior film done at the venipuncture) and usually is most is virtually certain. If significant res- same time as the lateral view allows calm if held in an upright position in piratory distress is present, as for the best discrimination between his mother’s arms. shown by more than a 50% increase epiglottitis and croup. If the child appears in extremis above the normal respiratory rate, Identification of a bacterial etiol- and the transport time is prolonged further diagnostic efforts, especially ogy for epiglottitis is best done by (greater than 30 minutes), emer- throat culture, should be deferred blood culture, after the airway is se- gency intubation or tracheostomy until someone with expertise suita- cured. If drawn shortly after intuba- prior to transporting should be con- ble to secure the airway is present. tion or tracheostomy, more than sidered seriously. Alternatively, me- If the patient is not having respira- 80% of such cultures will be positive ticulous and forceful mouth-to- tory distress, a soft tissue lateral for HIB. Other pathogens are iso- mouth or bag and mask ventilation neck x-ray is most helpful. The pa- lated from the blood in less than 1 % should be the initial therapeutic ap- tient should be attended by CPR per- of the reported cases. Typically, proach to sudden airway obstruc- sonnel, and humidified oxygen three or four days are necessary for tion, since these techniques are should be provided continuously adequate growth in the laboratory, generally available and familiar to while obtaining the roentgenograms. and thus decisions about antibiotics pediatricians. Although only anecdotal information must be made before the results are Ambu bags, Hope resuscitation may be available, most authorities available. If counten-immunoelectro- bags, and similar self-inflating bags consider examination of the poste- phoresis or latex fixation antigen are equipped with pressure-release rior with a throat stick or studies on the blood are available, valves which open when ventilation mirror to be too dangerous since added certainty of the presence of pressures exceed approximately 50 even minor alterations of the child’s HIB can be available immediately. mm Hg. Patients with epiglottitis position may produce total obstruc- These studies are often positive

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even in patients who fail to demon- strate the organism in blood cul- tures. Nose, throat, or epiglottal surface swabs yield HIB in about half the cases, but are also often positive for Streptococcus, Neisseria and other pathogens from the oropharynx which do not correlate positively with the blood cultures. Thus, these cul- tunes cannot be assumed to be di- agnostic. They should be obtained only with airway immediately avail- able. Antibiotics should be adminis- tered intravenously immediately Fig 1. Lateral radiograph of the neck of a after the blood cultures have been patient with epiglottitis: A, dilated hypophar- drawn as detailed below. ynx; B, reduced size of vallecula due to en- - 2. Lateral radiograph of the neck of a croaching swollen epiglottis; C, rounded, normal child. The vallecula is spacious and thickened epiglottis (“thumb sign”) with swol- the supraglottic structures are delicate. TREATMENT len aryepiglottic folds extending posteriorly and inferiorly; D, reversed cervical curvature. The primary therapy before and This radiograph does not demonstrate an en route to securing the airway is open mouth and protruded mandible charac- humidified oxygen administered teristic of more advanced obstruction. continuously. Children often sense the anxiety of their parents and staff and become frightened by the oxy- tnansnasally, particularly if retention gen mask or funnel. Generally, the sutures are in place to assist in ele- child has better acceptance of oxy- vation of the . gen therapy when it is administered Endotracheal tubes should be by the mother. suctioned hourly, and regular chest The choice of method for securing physical therapy should be em- the airway depends primarily on the ployed to mobilize secretions. Intu- resources available. If appropriate bated patients are usually placed in intubation expertise, nursing skills, humidified tents to be sure that all and prompt replacement capability inspired air is moist, whether inhaled in the event of accidental extubation through the tube or around it, since are all present, then intubation is the these patients seem to have appre- preferred method, having slightly ciable leakage of air around the lower morbidity and the advantage tube. of avoiding a surgical procedure. Oral endotracheal tubes are However, if the available skills favor treacherous and should be avoided tracheostomy, it is entirely appropri- if at all possible in favor of nasal ate. The intubated child with epiglot- endotracheal tubes. Orally placed Fig 3. Lateral radiograph of the neck of a titis requires experienced nursing tubes are irritating to the patient, child with laryngotracheobronchitis (croup). attention to prevent plugging of the easily dislodged by and jaw The hypopharynx is dilated, but the vallecula tube by secretions, and accidental motions, and may be associated with and epiglottis are normal. Note the haziness and blurring of the air column at the level of or self-extubation. The ever-present life-threatening aspiration should the the fourth cervical vertebra indicating sub- hazard of accidental extubation also patient vomit, since the oral airway glottic swelling. requires the availability of skilled (placed to prevent biting through the personnel to re-intubate on short no- endotracheal tube) occludes the or- tice. Accidental decannulation of a opharynx. The placement of a na- extubation, the patient should be tracheostomy tube with soft tissue sogastnic tube helps to reduce this closely monitored for several hours. collapse into the stoma can likewise risk during the period of intubation, An additional 24 hours of humidifi- create an emergency condition, but even when nasal endotracheal tubes cation following extubation is advis- it is technically easier to pass an are used. able. endotracheal tube into the stoma The airway tube is left in place Antibiotics are essential in ther- than to re-intubate transorally or between 24 and 72 hours, and after apy, but their initiation should not

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delay securing the airway. Since the child with an endotnacheal tube in the literature, HIB epiglottitis is more than 99% of cases are due to or tracheostomy, the possibility of known to have recurred only once. HIB, the choice of antibiotics de- hypoxic agitation must be constantly pends on the local sensitivity pat- considered before ordering sedation CONCLUSION terns of HIB. If ampicillin-resistant since the airway appliance may be- Epiglottitis is a life-threatening in- HIB are present locally, chloram- come blocked with secretions at any fectious disease characterized by phenicol, 25 mg/kg IV every six time. the abrupt onset of fever and dys- hours (100 mg/kg/24 hours), is Although steroids have been ad- phagia followed shortly by respira- preferred. This dose is maximal, and vocated in the treatment of epiglot- tory distress. Initial management fo- close monitoring of blood counts, titis to reduce the swelling associ- cuses on maintaining oxygenation and/or blood levels, if available, is ated with the inflammation, there is needed, especially for patients un- no persuasive evidence that they are and placing an artificial airway as quickly as possible. Soft tissue ra- der 2 years of age. Once sensitivities of benefit. Racemic epinephnine, diographs of the neck can help es- are available, it may be possible to perhaps of some value in croup, is tablish the diagnosis in questionable change from chloramphenicol to am- not recommended in epiglottitis. cases. Intravenous antibiotics to picillin. If no ampicillin-resistant HIB Most clinicians feel that the in- cover HIB are instituted after the air- have been demonstrated locally, creased oxygen consumption in- way is secured and blood cultures ampicillin, 100 mg/kg IV every six duced by the drug and the risks of obtained. An excellent outcome can hours (400 mg/kg/24 hours), may manipulating a child with a tenuous be achieved if patients are given ox- be used initially, although many cli- airway are sufficient reasons not to ygen promptly and attended closely nicians prefer to use chlonampheni- use racemic epinephnine. from presentation to placement of col in all cases until sensitivity re- Other foci of HIB infection have an airway. sults are reported. been reported in epiglottitis patients, Treatment with these dosages of but their incidence is low. Once the ampicillin or chloramphenicol given airway is secured and antibiotics be- RECOMMENDED READING intravenously for seven days has gun, a careful examination for evi- Berenberg W, Kevy 5: Acute epiglottitis in been completely effective and the dence of , meningitis, childhood: A serious emergency, readily patient can be discharged without , and pericarditis is in recognizable at the bedside. N EngI J Med 258:870, 1958 any additional medication. order. Although there is increasing Cantrell AW, Bell AA, Morioka WT: Acute Children with epiglottitis should concern that HIB may be epiglottitis: Intubation versus tracheos- not be sedated before the airway is associated significantly with sec- tomy. Laryngoscope 88:944, 1978 secured by nasotracheal intubation ondary cases, prophylaxis is not Daum AS, Bates JA, Smith AL: Epiglottitis or tracheostomy. After the airway is currently recommended for siblings (supraglottitis), in Eeigan AO, cherry JO (eds): Textbook of Pediatric Infectious Dis- secured, the intubated child may re- and other contacts. However, should eases. Philadelphia, WB Saunders, in press quire sedation while the endotra- siblings or contacts develop fever 1980 cheal tube is in place. Chloral hy- (greater than 102 F) they should be Myers M: More on treatment of epiglottitis, drate given by the nasogastric tube evaluated by their physician in light letter. JPediatr83:168, 1973 Aapkin RH: The diagnosis of epiglottitis: Sim- or rectally at a dosage of 25 to 40 of their exposure to a child with HIB plicity and reliability of radiographs in the mg/kg/day divided into four equal epiglottitis. neck in the differential diagnosis of the doses is commonly used. Even in In more than 500 reported cases croup syndrome. J Pediatr 80:96, 1972

Abstract

Nebulized Racemic Epinephrine by IPPB for the Treatment of Croup. Estley CR, et al. Am J Dis Child 1 32:484, 1978. In a well controlled study of viral croup, patients who received nacemic epinephnine by intermittent positive-pressure (IPPB) were helped compared to controls. The help was short-lived; ie, there was only a short response to therapy and then return to distress. Therefore, therapy will need to be repeated and the patient should be hospitalized. Whether repeat therapy remains effective has not been studied; whether IPPB with epi- nephnine shortens hospitalization has not been studied. Comment: If a child with croup is hospitalized and if he does not have epiglottitis and if he looks like he may need to be intubated (rising Pco2s) then I would try IPPB with epinephnine (racemic on 1 % aqueous are equivalent). If there was a response I would repeat as often as necessary to prevent intubation. If no response I would intubate. (R.H.R.)

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Pediatrics in Review is the official journal of the American Academy of Pediatrics. A monthly publication, it has been published continuously since 1979. Pediatrics in Review is owned, published, and trademarked by the American Academy of Pediatrics, 345 Park Avenue, Itasca, Illinois, 60143. Copyright © 1979 by the American Academy of Pediatrics. All rights reserved. Print ISSN: 0191-9601.

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