Croup

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6.7 Croup

Introduction

The term croup describes an acute clinical syndrome of hoarse voice, barking cough, and inspiratory stridor usually seen in young children. Croup results from swelling of the upper airway, in and around the larynx, usually as a result of a viral infection. Croup occurs seasonally, peaking in winter months due to the epidemics of upper respiratory viruses. Parainfluenza virus type 1 accounts for around half the cases during winter, with parainfluenza type 2, influenza type A, adenoviruses, respiratory syncytial virus, enteroviruses, and possibly Mycoplasma pneumoniae causing most of the other cases. Some of the viral exanthems, such as varicella, can cause concomitant croup by involvement of the upper airway in small children. Croup is a common childhood problem, with a peak incidence of 60 per 1000 child years in those aged between 1 and 2 years, although it may be seen up to the teen years. As such, it is by far the most common cause of acute upper airways obstruction likely to present to emergency departments.

The respiratory distress caused by obstruction tends to be most marked in younger children due to the small size of their larynx, the presence of loose submucous tissues, and the tight encirclement of the subglottic area by the cricoid cartilage. In children under 8 years of age, this is the narrowest region of the airway, hence any inflammatory swelling in this area results in a significant impingement of the airway. The younger child, who has a smaller diameter airway, requires an increased vigilance to assess the degree of airway compromise.

The lower airway involvement of laryngotracheobronchitis may also cause younger children to manifest wheeze due to concurrent inflammation producing mucus in the smaller peripheral airways. Likewise, occasionally older children known to have asthma may exhibit signs of asthma in the setting of croup.1

Presentation

History

The typical presentation of croup is in a preschool-aged child with a history of a recent onset of upper respiratory tract infection. The child subsequently develops a barking or seal-like cough, a hoarse voice and, if obstruction is severe enough, stridor. The stridor may initially be apparent only when a child is distressed, such as during crying. During crying or forced expiration the diameter of the upper airways physiologically narrows and, hence, stridor will manifest. Stridor, which is initially inspiratory, indicates obstruction at the laryngeal level or higher (i.e. upper airway). Expiratory stridor or biphasic stridor indicates more severe laryngeal obstruction or alternatively an obstruction occurring lower in the airway. The natural history of airway obstruction, when unmodified by steroids, is to increase slowly to peak over 24–48 hours. The airway compromise usually then resolves over a few days, although the laryngeal cough may persist longer.

Less common than infectious croup but usually more sudden in onset, older children may present with recurrent or spasmodic croup with no viral prodrome. This is thought to be allergic in origin. These children may have a history of atopy and suffer from asthma more than the general population. They should, however, be treated in the same manner as ‘viral’ croup. In the smaller child, particularly infants, problems with feeding, swallowing difficulties, and whether the child has been cyanosed should be ascertained.

It is important to enquire whether or not the child has had croup or other airway problems in the past and, specifically, whether the child has had any persistence of mild stridor in between acute attacks. This is important, as any child who has a pre-existing narrowing of the airway (infantile floppy larynx, laryngomalacia or other upper airway anatomical abnormalities) is more likely to proceed to severe obstruction with a superimposed acute obstruction. These children need to have a lower threshold for a period of observation as their obstruction may be more severe or persistent.

An immunisation history is important to check whether the child has had Hib vaccination if there is any suggestion that the condition could be epiglottitis; likewise, the very rare occurrence of diphtheria in the non-immunised.

Examination

Croup in children can generally be classified as mild, moderate, or severe (Table 6.7.1).

Table 6.7.1 Croup severity

Sign or symptom Mild Moderate or severe Stridor None or only if agitated Stridor at rest Respiratory rate Usually normal May be decreased Retractions None + to +++ Air entry Normal Normal to decreased Colour Normal May be pallor Cyanosis None Late sign only Conscious state Normal Restless or decreased

After Marks et al 2003.2

Most children with mild croup are not distressed and have only a barking cough with no stridor at rest or stridor audible only with physical activity, crying, or agitation. Crying causes physiological narrowing of the airway and will increase the respiratory distress. Hence the distressed, crying child’s obstruction will often defervesce by allowing the child to be cuddled in the parent’s arms. There may be signs due to viral illness, such as mild fever and nasal discharge. Children with mild cases can have their throats examined, but this should be deferred in more severe cases. A compromised but functioning airway should never be made worse by upsetting the child.

In more severe cases, the child may have a more pronounced stridor at rest. As airway obstruction progresses, increased work of breathing ensues and the child exhibits increasing substernal, intercostal, and subcostal retractions. Subtle signs of hypoxia causing altered consciousness may be reflected as anxiety or restlessness in a child. An obviously fatiguing child is a worrying sign. The child manifesting decreased air entry and respiratory effort, extreme pallor, and cyanosis requires immediate intervention.

The child’s preferred position may also give clues as to severity of obstruction or to a diagnosis other than croup. Hyperextension or other abnormal positioning of the neck may suggest epiglottitis or a retropharyngeal abscess. It is unusual for the child with croup to be saliva intolerant or have any tenderness of the neck.

The use of a croup severity score may be helpful for less-experienced staff to assess children with croup and communicate findings with a colleague when seeking advice. As previously mentioned, some children may have concomitant wheeze in addition to the upper airway stridor and hence their pattern of breathing may be ‘gas trapped’ and cause hyperinflation of the chest with slow expiration phase, in contrast to those with pure croup alone.

Investigations

Croup is usually an easy ‘spot diagnosis’ and requires no diagnostic tests.

Oximetry is of limited value, as children may maintain near-normal oxygen saturations even when they have significant airway obstruction.3 While it may have a role in cases of severe croup, this must be balanced with the distress caused by the monitoring probe in small children. In stable cases, where the diagnosis is unclear, a lateral soft tissue X-ray of the neck may be helpful to distinguish croup from epiglottitis or retropharyngeal abscess. However, the possible benefits of an X-ray need to be weighed against the risks of moving or disturbing the child when the obstruction is more than mild. A nasopharyngeal aspirate in croup is not necessary for diagnosis but may be useful for infection control for patients being admitted.

Differential diagnosis

It is important to establish that other, more sinister, causes of acute upper respiratory tract obstruction masquerading as croup are not present (Table 6.7.2). Especially in the younger child, one should enquire regarding longer-term symptoms preceding the present episode, such as low-grade stridor. This might suggest underlying congenital airway or vascular anomaly (e.g. tracheomalacia, subglottic stenosis, bilateral cord paralysis, laryngeal web, or vascular ring compression of the trachea). One should also enquire as to possible airway trauma or toxic ingestion. Dysphagia and drooling may suggest epiglottitis, peritonsillar or retropharyngeal abscess, or foreign body in the airway or oesophagus.

Table 6.7.2 Differential diagnoses of croup

Classic croup and epiglottitis are hard to confuse, as the latter usually presents as a pale, toxic, drooling child with a rapidly progressing course. Cough is generally not a prominent feature in epiglottitis. Children with epiglottitis may sit forwards, drooling saliva, and hold their neck in extension. In a child presenting with early epiglottitis, however, the distinction may be more difficult. Immunisation in developed countries, however, has made this distinction largely academic. Allergic angio-oedema may mimic croup after exposure to an allergen such as peanut. A child with ‘severe croup’ with a high fever, who does not respond to adrenaline (epinephrine) and steroids, may have tracheitis and will need consideration of a diagnostic laryngoscopy to provide a clear diagnosis. Likewise, the possibility of an inhaled foreign body should be kept in mind for children who don’t respond to treatment or have a prolonged course. While usually parents will volunteer a history of an acute obstruction or a sudden coughing fit, the history of an inhaled object may not always have been observed and therefore reported. A definitive diagnosis may need to be made by directly viewing the upper airway, but this should be performed only by an experienced paediatric anaesthetist, intensivist, or emergency physician in an appropriate clinical setting (see below).

Treatment and disposition

Mild or moderate croup

All children who present to an emergency department with croup should be treated with steroids.4 The mandatory use of steroids for croup in emergency departments results in a reduction in the relapse rate of those sent home,5 the average length of stay in hospital falling, and the number of children needing intensive care and intubation dramatically reducing.6,7 Prior to the regular use of steroids, a general rule of thumb was to admit children with stridor at rest (moderate) to hospital for observation, while allowing those with occasional stridor and barking cough only (mild) to be managed at home. As many children will improve within a few hours of taking steroids, often they may be discharged home after a short stay in the emergency department or an observation ward. Factors such as the distance from medical care, the availability of transport, the time of day, the child’s past history with regard to severe airway obstruction, and parental concern and attitude all need to be taken into account when making the decision to admit (Table 6.7.3).

Table 6.7.3 Possible Indications for admission

Recommended steroid doses are oral dexamethasone in a one-off dose of 0.15 mg kg–1,8 or an equivalent dose of prednisolone of 0.75 mg kg–1. Most children with croup will require only one dose, but if the upper airway obstruction symptoms persist (as opposed to upper respiratory tract infection symptoms), a further dose may be given 18 to 24 hours later. It is often more convenient to use prednisolone (rounded off to 1 mg kg–1) in the community, as it is more readily available. While one study9 suggested that children treated with prednisolone may re-present more commonly than those treated with dexamethasone, Fifoot et al10 did not confirm this finding. Steroids may be administered intramuscularly or intravenously in the child with severe obstruction, when there is concern that the child may aspirate or vomit, given their degree of respiratory difficulty.

Oral dexamethasone has been found to be as effective as inhaled steroids such as budesonide,11 and to work as fast, at a fraction of the cost. A blinded randomised trial, submitted for publication, of dexamethasone 0.15 mg kg–1 compared to placebo that looked at croup scores at 10-minute intervals after administration showed a significant difference at 30 minutes.12 Combining dexamethasone and budesonide is no more effective than dexamethasone alone.11,13 There is no place for antibiotics in a typical case of croup. The use of ‘steam’ or humidified air is unproven,14,15 despite its once common usage. The anecdotal report by parents of their child improving in the steam-filled bathroom at home is due to the defervescing of crying which occurs from cuddling in the room by the parent, rather than any steam effect.

Severe croup

Children with manifestations of severe obstruction should be given nebulised adrenaline (epinephrine). It is generally considered that adrenaline does not change the natural history of croup, such as length of stay in hospital or need to intubate, due to its short-lasting effects. It will, however, ‘buy time’ while waiting for the effect of steroids to occur. Rarely, in a worst-case scenario, adrenaline can be a useful temporising measure while organising the facilities and appropriate personnel for a child who may require intubation. The recommended dose (independent of age and weight) is 5 mL of 1:1000, nebulised with oxygen, which can be used for all children. This may be repeated after 10 minutes if needed and may help avoid the need for intubation in children who respond to steroids. ‘Rebound’ phenomenon may occur, where the upper airway obstruction may recur as the effect of the adrenaline wears off after 1–2 hours. While in the past it was recommended that any child who received adrenaline for croup should be admitted, a number of studies have now shown that children may be sent home safely if they have also received steroids and have improved to have no stridor at rest over a number of hours.16,17 Children receiving nebulised adrenaline require close clinical monitoring of their response, particularly the change in air entry, in order to detect any deterioration.

Intubation needs to be considered in the child who has increasing upper airway obstruction, hypoxia, decreasing conscious state, or fatigue despite nebulised adrenaline. These children should be discussed early with a paediatric intensivist in order to optimise management. The ideal setting for this to occur is in theatre or a paediatric intensive care unit environment via gaseous induction, using an endotracheal tube 1.0-mm smaller than predicted by the child’s size (see Chapter 2.3).

References

1 Denny F.W., Clyde W.A. Acute lower respiratory tract infections in nonhospitalized children. J Pediatr. 1986;108:635-645.

2 Marks M., Wilkinson D., Munro J. Paediatric Handbook, 6th ed. Victoria: Blackwell Science; 2003.

3 Stoney P.J., Chakrabarti M.K. Experience of pulse oximetry in children presenting with croup. J Laryngol Otol. 1991;105:295-298.

4 Geelhoed G.C. Croup. Pediatr Pulmonol. 1997;23(5):370-374.

5 Geelhoed G.C., Turner J., Macdonald W.B. Efficacy of a small single dose of oral dexamethasone for outpatient croup: A double blind placebo controlled clinical trial. Br Med J. 1996;313(7050):140-142.

6 Geelhoed G.C. Sixteen years of croup in a Western Australian teaching hospital: The impact of routine steroid treatment. Ann Emerg Med. 1996;28(6):621-626.

7 Dobrovoljac M., Geelhoed G.C. 27 years of croup: an update highlighting the effectiveness of 0.15 mg/kg of dexamethasone. Emerg Med Australas. 2009;21:309-314.

8 Geelhoed G.C., Macdonald W.G.B. Oral dexamethasone in the treatment of croup: 0.15 mg/kg is as effective as 0.3 mg/kg or 0.6 mg/kg. Pediatr Pulmonol. 1995;20:362-367.

9 Sparrow A., Geelhoed G.C. Prednisolone versus dexamethasone in croup: A randomised equivalence trial. Arch Dis Child. 2006;91:580-583.

10 Fifoot A.A., Ting J.Y.S. Comparison between single-dose oral prednisolone and oral dexamethasone in the treatment of croup: a randomized, double-blinded clinical trial. Emerg Med Australasia. 2007;19:51-58.

11 Klassen T.P., Craig W.R., Moher D., et al. Nebulized budesonide and oral dexamethasone for treatment of croup: A randomized controlled trial. JAMA. 1998;279(20):1629-1632.

12 Dobrovoljac M., Geelhoed G.C. How fast does oral dexamethasone work in croup patients? A randomised double blinded clinical trial. Emerg Med Australasia. 2009;21(4):309-314.

13 Geelhoed G.C. Budesonide offers no advantage when added to oral dexamethasone in the treatment of croup. Pediatr Emerg Care. 2005;21:359-362.

14 Neto G.M., Kentab O., Klassen T.P., et al. A randomized controlled trial of mist in the acute treatment of moderate croup. Acad Emerg Med. 2002;9:873-879.

15 Bouchier D., Dawson K.P., Fergusson D.M. Humidification in viral croup: A controlled trial. Aust Paediatr J. 1984;20:289-291.

16 Kelley P.B., Simon J.E. Racemic epinephrine use in croup and disposition. Am J Emerg Med. 1992;10(3):181-183.

17 Prendergast M., Jones J.S., Hartman D. Racemic epinephrine in the treatment of laryngotracheitis: Can we identify children for outpatient therapy? Am J Emerg Med. 1994;12(6):613-616.