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UP TO DATE

Croup: Pharmacologic and supportive interventions


Author
Charles R Woods, MD, MS
Section Editor
Sheldon L Kaplan, MD
Deputy Editor
Carrie Armsby, MD, MPH
Disclosures: Charles R Woods, MD, MS Other Financial Interest: Cerexa [Epiglottitis (Data
Safety Monitoring Board for pediatric trials of the antibiotic agent ceftaroline)]. Sheldon L
Kaplan, MD Grant/Research/Clinical Trial Support: Pfizer [vaccine (PCV13)]; Forest Lab
[antibiotic (Ceftaroline)]; Optimer [antibiotic (fidaxomicin)]. Consultant/Advisory Boards: Pfizer
[vaccine (PCV13)]. Carrie Armsby, MD, MPH Nothing to disclose.

Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found,
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All topics are updated as new evidence becomes available and our peer review
process is complete.
Literature review current through: Mar 2015. | This topic last updated: Jan
30, 2014.

INTRODUCTION Croup (laryngotracheitis) is a respiratory illness


characterized by inspiratory stridor, barking cough, and hoarseness. It typically
occurs in children six months to three years of age and is caused by
parainfluenza virus. (See "Croup: Clinical features, evaluation, and diagnosis".)

The treatment of croup has changed significantly since the 1980s.


Glucocorticoids and nebulized epinephrinehave become the cornerstones of
therapy. Substantial clinical evidence supports the efficacy of these
interventions [1-5]. The impact also is evident in the decrease in annual hospital
admissions for croup in children in the United States between 1979 to 1982 and
1994 to 1997 (from 2.8 to 2.1 per 1000 for children <1 year and from 1.8 to 1.2
per 1000 children for children 1 to 4 years) [6].

Treatment of croup may involve a variety of pharmacologic and


nonpharmacologic interventions. It may occur entirely at home, or in the office,
emergency department (ED), or hospital setting. Supportive and pharmacologic
interventions will be discussed below. The clinical features and evaluation of
croup and the approach to management are discussed separately.
(See "Croup: Clinical features, evaluation, and diagnosis"and "Croup: Approach
to management".)

GLUCOCORTICOIDS Glucocorticoids provide long-lasting and effective


treatment of mild, moderate, and severe croup [3,7-9]. The antiinflammatory
actions of glucocorticoids are thought to decrease edema in the laryngeal
mucosa of children with croup. Improvement is usually evident within six hours
of administration but seldom is dramatic [7,10].

Treatment with glucocorticoids at various doses and by various routes has been
shown to improve croup scores and to decrease unscheduled medical visits,
length of stay in the emergency department or hospital, and the use
of epinephrine [7]. Among the available glucocorticoids, dexamethasone has
been used most frequently, is the least expensive, has the longest duration of
action, and is the easiest to administer.

Efficacy Intramuscular (IM), intravenous (IV), oral, and inhaled routes of


administration of glucocorticoids have been shown to be effective in croup of all
levels of severity [7,8]. Dexamethasone (oral or IM) andbudesonide (inhaled)
were the agents used in the majority of studies. A systematic review included
24 trials (with collective enrollment of 2878 children) that objectively measured
the effectiveness of glucocorticoid treatment for croup compared with placebo
[7]. Fourteen other trials compared different glucocorticoid agents or different
routes or dosages of the same agent [7]. Compared with treatment with
placebo, treatment with glucocorticoid was associated with:

Improvement in the croup score at six hours with a weighted mean


difference of -1.2 (95% CI -1.6 to -0.8) and at 12 hours -1.9 (95% CI -2.4
to -1.3); at 24 hours this improvement was no longer significant (-1.3, 95%
CI -2.7-0.2)
Fewer return visits and/or (re)admissions (relative risk 0.50, 95% CI 0.3-
0.7)
Decreased length of time spent in emergency department or hospital
(weighted mean difference 12 hours, 95% CI 5-19 hours)
Decreased use of epinephrine (risk difference 10 percent; 95% CI 1-20
percent)
There were no significant differences in clinical efficacy between the
routes or agents, and the combination of oral or IM dexamethasone with
inhaled budesonide was not superior to either agent alone [11,12].

Another systematic review of eight randomized controlled trials compared the


administration of nebulized glucocorticoids with placebo. Children treated with
nebulized glucocorticoids were significantly more likely to show improvement in
croup score at five hours (combined relative risk (RR) 1.48, 95% CI 1.27-1.74)
and significantly less likely to need hospital admission (combined RR 0.56, 95%
CI 0.42-0.75) [13].

Adverse effects Few serious adverse effects have been reported in the
studies evaluating the efficacy of asingle dose of glucocorticoids in croup [14].
However, most of these studies were too small to sufficiently evaluate rare (<1
percent) adverse effects [15,16].

The primary concern is potential risk of progressive viral infection or secondary


bacterial infection, which have been reported in patients who received
glucocorticoid treatment over several days [16], or received
nebulizeddexamethasone and had neutropenia [17]. These complications have
not been described in children who have received single doses of oral,
intramuscular, or intravenous glucocorticoids for croup.

Glucocorticoid use may exacerbate active varicella and tuberculosis and should
be avoided in children with these infections and in those recently exposed to,
and possibly incubating, varicella [18,19]. (See "Clinical features of varicella-
zoster virus infection: Chickenpox" and "Treatment of varicella-zoster virus
infection: Chickenpox".)

Administration of glucocorticoids may mask the presentation of steroid-


responsive upper airway lesions, such as hemangiomas, which also can
present with stridor, particularly during a viral upper respiratory tract infection
[20]. (See "Epidemiology, pathogenesis, clinical features, and complications of
infantile hemangiomas".)

Agents

Dexamethasone Dexamethasone may be administered IM, IV, or orally. To


date, no clinically significant difference in croup outcomes between IM or orally
administered dexamethasone has been demonstrated [7]. When
dexamethasone is administered IM or IV, a single dose of 0.6 mg/kg (maximum
dose of 10 mg) is used most frequently. Smaller doses appear to be equally
effective for mild croup when administered orally, as illustrated below:

In one study, 100 children with mild croup were randomly assigned to
receive oral dexamethasone (0.15mg/kg) or placebo in the emergency
department [21]. Eight children in the placebo group, and none in the
dexamethasone group, returned for medical care (a statistically significant
difference).
In another study, 120 hospitalized children with croup were randomly
assigned to receive a single oral dose
of dexamethasone (0.15 mg/kg, 0.3 mg/kg, 0.6 mg/kg) or placebo [22].
There was no difference in duration of hospitalization, reduction in croup
score, or epinephrine use among the three groups receiving
dexamethasone.

The second study described above [22] included a small number of children
with relatively mild croup and consequently may have been underpowered
(unable) to detect a clinically important difference, particularly in children with
more severe symptoms [14].

Children with mild croup who can tolerate oral medications can be given
either dexamethasone 0.15 mg/kg or dexamethasone 0.6 mg/kg orally, to a
maximum total dose of 10 mg. Although the lower 0.15 mg/kg dose appears to
be efficacious [21], we continue to suggest the higher dose [23,24].

The oral preparation of dexamethasone (1 mg per mL) has a foul taste. The
intravenous preparation is more concentrated (4 mg per mL) and can be given
orally mixed with syrup [11,25,26].

Studies of nebulized dexamethasone in children with croup have mixed results.


One study found nebulized dexamethasone to be less effective than oral
dexamethasone in preventing the need for subsequent treatment with
glucocorticoid or epinephrine in children with mild croup [27]. Another study
found that treatment with nebulized dexamethasone in children with moderate
croup improved croup scores at four hours but did not affect the rate of
hospitalization [17]. In addition, two patients with neutropenia who were treated
with dexamethasone developed bacterial tracheitis.

Budesonide Nebulized budesonide has been shown to be more effective


than placebo and as effective as IM or oral dexamethasone for the treatment of
croup [7,28]. However, nebulized budesonide is more expensive and more
difficult to administer than IM or oral dexamethasone and is not routinely
indicated in the treatment of croup. However, nebulized budesonide may
provide an alternative to IM or IV dexamethasone for children with vomiting or
severe respiratory distress [24]. In children with severe respiratory distress, a
single dose of budesonide may be mixed with epinephrine and administered
simultaneously. (See "Croup: Approach to management", section on 'Moderate
to severe croup'.)

Prednisolone Some authorities suggest that for children who are treated as
outpatients, oralprednisolone (2 mg/kg per day for three days) is an alternative
to oral dexamethasone [29]. The use of prednisolone in the treatment of croup
has been evaluated in a limited number of studies.

A 2011 meta-analysis of two trials [30,31] that compared a single dose of


oral dexamethasone (0.6 mg/kg or 0.15 mg/kg) with a single dose of
oral prednisolone (1 mg/kg) showed no difference in croup scores, but children
randomized to receive dexamethasone had fewer return
visits and/or subsequent admissions (9.6 versus 29.7 percent, RR 0.3, 95% CI
0.2-0.6) [7].

A subsequent randomized trial compared oral dexamethasone (0.6 mg/kg on


the first day followed by placebo on the next two days) with
oral prednisolone (2 mg/kg per day for three days) in 87 children with mild or
moderate croup who were treated as outpatients [29]. There were no
differences between groups in additional health care visits (2 versus 7 percent
[not significant]), duration of symptoms (2.8 versus 2.2 days), duration of
nonbarky cough (6.1 versus 5.9 days), nights with disturbed parental sleep (0.7
versus 1.2), or days with stress (1.6 versus 1.4).

Another study of 70 children compared prednisolone (1 mg/kg every 12 hours)


with placebo in children with croup who were already intubated [8]. Children
who received prednisolone had a shorter median duration of intubation than
those in the placebo group (98 versus 138 hours). In addition, fewer children in
the prednisolone group required reintubation (5 versus 34 percent).

Prednisone The use of prednisone in the management of croup has not


been evaluated in clinical trials. However, it has equivalent potency
to prednisolone and, in theory, should have similar effects. Despite its lack of
proven benefit, prednisone is widely used in the outpatient management of
croup [32].

If prednisone is to be used, it is important to administer a dose that is equivalent


in strength to the doses of glucocorticoids that have been better
studied. Dexamethasone has 6.67 times the glucocorticoid potency of
prednisone (4 mg/kg of prednisone = 0.6 mg/kg of dexamethasone; 2 mg/kg of
prednisone = 0.3 mg/kg of dexamethasone; and 1 mg/kg of prednisone =
0.15 mg/kg of dexamethasone). If choosing to use the higher dose (ie,
4 mg/kg of prednisone), the volume required may be prohibitive given that the
concentration of the oral solution of prednisone is 1 mg/1 mL.

Betamethasone A pilot study compared the effectiveness of a single oral


dose of betamethasone (0.4mg/kg) with a single dose of
IM dexamethasone (0.6 mg/kg) in 52 children (six months to six years) with mild
to moderate croup who were treated in the emergency department [33]. Despite
randomization, mean baseline croup scores were higher in the dexamethasone
group (3.6 versus 2). Croup scores declined significantly in both groups, and
there were no differences between groups in mean croup scores four hours
after treatment, rate of hospitalization, time to resolution of symptoms, need for
additional treatment, or number of return visits to the emergency department.

Repeated dosing The majority of clinical trials of oral and IM glucocorticoids


in croup have used a single dose. Repeat doses are not necessary on a routine
basis. Although repeat doses may be reasonable in the occasional child who
has persistent symptoms, they should be used with caution. The anecdotal
cases of progression of viral illness and secondary bacterial infection that have
been reported with use of glucocorticoids for croup occurred with repeated
dosing over several days [34], or in neutropenic patients [17]. (See 'Adverse
effects' above.)
Moderate to severe symptoms that persist for more than a few days should
prompt investigation for other causes of airway obstruction. (See "Croup:
Clinical features, evaluation, and diagnosis", section on 'Differential diagnosis'.)

NEBULIZED EPINEPHRINE The administration of nebulized epinephrine to


patients with moderate to severe croup often results in rapid improvement of
upper airway obstruction. Epinephrine constricts precapillary arterioles in the
upper airway mucosa and decreases capillary hydrostatic pressure, leading to
fluid resorption and improvement in airway edema [18]. Even a modest increase
in airway diameter can lead to significant clinical improvement.

Benefits Several small randomized controlled trials and a meta-analysis


have demonstrated the benefit of racemic epinephrine compared with placebo
in reducing the croup scores 30 minutes after treatment in children in the
emergency department, hospital, and intensive care unit [1,35-37]. The
magnitude of reduction in mean croup score from baseline ranged from 2.2 to
3.6 at 20 to 30 minutes (compared with approximately 1 in the placebo group).
However, by 120 minutes, croup scores returned to baseline or near baseline
[1,36]. In one trial, treatment with IM dexamethasone and nebulized
epinephrine was associated with decreased duration of hospitalization
compared with IM dexamethasone and placebo (-32 hours, 95% CI -59.1 to -
4.9) [38,39].

Administration of epinephrine does not alter the natural history of croup in the
short (>2 hours) or longer term (24 to 36 hours) [1,36,39].

In the studies described above, racemic epinephrine was administered either by


nebulization alone or by nebulization combined with intermittent positive
pressure breaths [1,35,36]. Another study compared these two methods of
administration and found them to be similarly effective [2].

Racemic versus L-epinephrine Racemic epinephrine, which is a 1:1


mixture of the D- and L-isomers, was initially thought to produce fewer systemic
side effects, such as tachycardia and hypertension [18]. However, a
randomized double-blind study comparing racemic epinephrine and L-
epinephrine in children with croup found no difference between the two
preparations in 30-minute croup score, heart rate, blood pressure, respiratory
rate, fraction of inspired oxygen, or oxygen saturation [40]. This finding is
particularly important outside of the United States, where racemic epinephrine
is not readily available. Either form of epinephrine is acceptable to use in the
United States.

Dose

Racemic epinephrine is administered as 0.05 mL/kg per dose (maximum


of 0.5 mL) of a 2.25 percent solution diluted to 3 mL total volume with
normal saline. It is given via nebulizer over 15 minutes.
L-epinephrine is administered as 0.5 mL/kg per dose (maximum of 5 mL)
of a 1:1000 dilution [40]. It is given via nebulizer over 15 minutes.

Nebulized epinephrine treatments may be repeated every 15 to 20 minutes if


warranted by the clinical course. Children who require repeated frequent dosing
(eg, three or more doses within two to three hours) to achieve stabilization of
their respiratory function generally should be admitted to an intensive care unit
or intermediate care setting (depending on the severity of persisting signs).

Precautions The clinical effects of nebulized epinephrine last for no more


than two hours. After the effects have worn off, symptoms may return to
baseline (an apparent worsening, sometimes referred to as the "rebound
phenomenon"). Children who receive even a single dose of nebulized
epinephrine should be observed in the emergency department or hospital
setting for at least three to four hours after administration to ensure that
symptoms do not return to baseline.

Serious adverse effects from nebulized epinephrine are exceedingly rare.


However, a case of myocardial infarction in a child with croup who received
three doses of racemic epinephrine within 60 minutes has been reported [41].
Thus, it seems prudent to place children who require ongoing epinephrine
treatments more frequently than every one to two hours on cardiac monitors
(both because of the severity of illness and the potential systemic impact of
nebulized epinephrine). Continuous electrocardiographic monitoring (or
equivalent cardiac monitoring) also should be considered in these cases.

OXYGEN Oxygen is not known to have any direct impact on the subglottic
edema or airway narrowing, but should be administered to children who are
hypoxemic (oxygen saturation of <92 percent in room air) and/or in moderate to
severe respiratory distress [14,24]. Supplemental oxygen should be humidified
to decrease drying effects on the airways, since drying may impede the
physiologic removal of airway secretions via mucociliary and cough
mechanisms. (See "Continuous oxygen delivery systems for infants, children,
and adults".)

Heliox Helium is inert, nontoxic, and of very low density. Heliox is a mixture
of helium (70 to 80 percent) and oxygen (20 to 30 percent). It can flow through
airways with less turbulence and resistance than pure oxygen.
(See "Physiology and clinical use of heliox".)

Heliox decreases the work of respiration in children with croup and theoretically
could be used as a temporizing measure, to prevent the need for intubation
while waiting for glucocorticoids to decrease airway edema [42]. However, in
clinical trials, heliox has not definitively been shown to be more effective than
humidified oxygen or racemic epinephrine in reducing croup scores [43-45]. A
2013 systematic review found only three methodologically limited trials (91
patients) evaluating heliox in children with croup and concluded that a larger
trial is needed before recommendations regarding the use of heliox in children
with croup can be made [45].

MIST THERAPY Humidified air is frequently used in the treatment of croup,


although there have been no studies supporting its efficacy in reducing
symptoms [46]. Two randomized trials (one comparing mist versus no mist and
the other comparing no mist, low humidity, and 100 percent humidity) among
children brought to an emergency department for croup demonstrated no
significant change in croup scores from baseline between the groups [47,48].

Although humidified air does not reduce subglottic edema, it may provide other
benefits. Inhalation of moist air, relative to dry air, may decrease drying of
inflamed mucosal surfaces and reduce inspissation of secretions [49]. In
addition, a mist source may provide a sense of comfort and reassurance to both
the child and family [50-52]. In medical settings, mist therapy may be provided
by blow-by or saline nebulization treatments. Croup tents should be avoided,
since they can aggravate a child's anxiety and make vital signs and other visual
assessments of the child more difficult. Some guidelines recommend against
the use of mist therapy for children who are hospitalized with croup [24].
Certainly if the child is agitated by the provision of mist, mist therapy should be
discontinued.
OTHER THERAPIES

Antibiotics Antibiotics have no role in the routine management of


uncomplicated croup, since most cases are caused by viruses [14]. Antibiotics
should be used only to treat specific bacterial complications, such as tracheitis.

Antitussives Nonprescription antitussive agents are of unproven benefit for


croup (or other respiratory tract infections). Codeine, which is a more potent
cough suppressant, can alter the child's sensorium, making it difficult to follow
the clinical course.

Decongestants Decongestants also are of unproven benefit for croup


[14,24].

Sedatives The routine use of sedative agents in effort to improve airway


obstruction by relieving anxiety and apprehension is not recommended.
Sedatives may treat the symptom of agitation while masking the underlying
causes of air hunger and hypoxia. They also may decrease respiratory effort
(and therefore croup scores), without improving ventilation [14,53].

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These articles are written at the 10th to 12th grade reading level and are best for
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encourage you to print or e-mail these topics to your patients. (You can also
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Basics topic (see "Patient information: Croup (The Basics)")


Beyond the Basics topic (see "Patient information: Croup in infants and
children (Beyond the Basics)")
SUMMARY

Treatment with glucocorticoids (oral, intramuscular, or nebulized) has


been shown to decrease croup scores, unscheduled medical visits, length
of stay in the emergency department or hospital, and the use
of epinephrine. A single dose of oral or intramuscular dexamethasone is
appropriate and adequate for most children. (See 'Glucocorticoids' above
and "Croup: Approach to management".)
Treatment with nebulized epinephrine results in rapid improvement of
upper airway obstruction, but the duration of effect is less than two hours.
Racemic epinephrine and L-epinephrine appear to be equally effective.
(See 'Nebulized epinephrine' above.)
Humidified air is frequently used as a supportive treatment for croup;
however, there have been no studies supporting its efficacy in reducing
symptoms. (See 'Mist therapy' above.)
Humidified oxygen should be administered to children who are
hypoxemic and/or in moderate to severe respiratory distress.
(See 'Oxygen' above.)
Heliox has not definitively been shown to be more effective than
humidified oxygen or racemicepinephrine in reducing croup scores.
(See 'Heliox' above.)
Antibiotics should be used only to treat specific bacterial complications of
croup. (See 'Antibiotics' above and "Croup: Approach to management",
section on 'Complications'.)
Antitussives and decongestants are of unproven benefit in the
management of croup. Sedatives may decrease the work of breathing and
improve agitation without actually improving ventilation or addressing the
underlying cause of agitation (hypoxemia). (See 'Other therapies' above.)
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pilot study. Acad Emerg Med 1998; 5:1130.
44. Weber JE, Chudnofsky CR, Younger JG, et al. A randomized comparison of
helium-oxygen mixture (Heliox) and racemic epinephrine for the treatment of
moderate to severe croup. Pediatrics 2001; 107:E96.
45. Moraa I, Sturman N, McGuire T, van Driel ML. Heliox for croup in children.
Cochrane Database Syst Rev 2013; 12:CD006822.
46. Skolnik NS. Treatment of croup. A critical review. Am J Dis Child 1989;
143:1045.
47. Neto GM, Kentab O, Klassen TP, Osmond MH. A randomized controlled trial of
mist in the acute treatment of moderate croup. Acad Emerg Med 2002; 9:873.
48. Scolnik D, Coates AL, Stephens D, et al. Controlled delivery of high vs low
humidity vs mist therapy for croup in emergency departments: a randomized
controlled trial. JAMA 2006; 295:1274.
49. Dulfano MJ, Adler K, Wooten O. Physical properties of sputum. IV. Effects of
100 per cent humidity and water mist. Am Rev Respir Dis 1973; 107:130.
50. Parks CR. Mist therapy: rationale and practice. J Pediatr 1970; 76:305.
51. Henry R. Moist air in the treatment of laryngotracheitis. Arch Dis Child 1983;
58:577.
52. Sasaki CT, Suzuki M. The respiratory mechanism of aerosol inhalation in the
treatment of partial airway obstruction. Pediatrics 1977; 59:689.
53. Fanconi S, Burger R, Maurer H, et al. Transcutaneous carbon dioxide pressure
for monitoring patients with severe croup. J Pediatr 1990; 117:701.

Topic 6008 Version 18.0


Croup: Approach to management
Author
Charles R Woods, MD, MS
Section Editor
Sheldon L Kaplan, MD
Deputy Editor
Carrie Armsby, MD, MPH
Disclosures: Charles R Woods, MD, MS Other Financial Interest: Cerexa [Epiglottitis (Data
Safety Monitoring Board for pediatric trials of the antibiotic agent ceftaroline)]. Sheldon L
Kaplan, MD Grant/Research/Clinical Trial Support: Pfizer [vaccine (PCV13)]; Forest Lab
[antibiotic (Ceftaroline)]; Optimer [antibiotic (fidaxomicin)]. Consultant/Advisory Boards: Pfizer
[vaccine (PCV13)]. Carrie Armsby, MD, MPH Nothing to disclose.

Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found,
these are addressed by vetting through a multi-level review process, and through requirements
for references to be provided to support the content. Appropriately referenced content is
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Conflict of interest policy

All topics are updated as new evidence becomes available and our peer review
process is complete.
Literature review current through: Mar 2015. | This topic last updated: Dec
14, 2013.

INTRODUCTION Croup (laryngotracheitis) is a respiratory illness


characterized by inspiratory stridor, barking cough, and hoarseness. It typically
occurs in children six months to three years of age and is caused by
parainfluenza virus. (See "Croup: Clinical features, evaluation, and diagnosis".)

The treatment of croup has changed significantly since the 1980s.


Corticosteroids and nebulized epinephrinehave become the cornerstones of
therapy. Substantial clinical evidence supports the efficacy of these
interventions [1-5]. The impact also is evident in the decrease in annual hospital
admissions for croup in children in the United States between 1979 and 1982,
and 1994 and 1997 (from 2.8 to 2.1 per 1000 for children <1 year and from 1.8
to 1.2 per 1000 children for children 1 to 4 years) [6].

The approach to the management of croup will be discussed below. The clinical
features and evaluation of croup and the evidence supporting the use of the
pharmacologic and supportive interventions included below are discussed
separately. (See "Croup: Clinical features, evaluation, and
diagnosis" and "Croup: Pharmacologic and supportive interventions".)
OVERVIEW The treatment of croup and the setting in which the child is
initially evaluated depend upon the severity of symptoms and the presence of
risk factors for rapid progression. There is no definitive treatment for the viruses
that cause croup. Pharmacologic therapy is directed toward decreasing airway
edema, and supportive care is directed toward the provision of respiratory
support and the maintenance of hydration. (See"Croup: Pharmacologic and
supportive interventions".)

Most children with croup who seek medical attention have a mild, self-limited
illness and can be successfully managed as outpatients. The clinician must be
able to identify children with mild symptoms, who can be safely managed at
home, and those with moderate to severe croup or rapidly progressing
symptoms, who require full evaluation and possible treatment in the office or
emergency department setting.

Severity assessment The severity of croup is often determined by the


clinical scoring systems. Although there are a number of validated croup
scoring systems, the Westley croup score [1] has been the most extensively
studied; it is described below. No matter which system is used to assess
severity, the presence of chest wall retractions and stridor at rest are the two
most critical clinical features [7].

Westley croup score The elements of the Westley croup score describe
key features of the physical examination [1]. Each element is assigned a score,
as illustrated below:

Level of consciousness: Normal, including sleep = 0; disoriented = 5


Cyanosis: None = 0; with agitation = 4; at rest = 5
Stridor: None = 0; with agitation = 1; at rest = 2
Air entry: Normal = 0; decreased = 1; markedly decreased = 2
Retractions: None = 0; mild = 1; moderate = 2; severe = 3

The total score ranges from 0 to 17.

Mild croup is defined by a Westley croup score of 2. Typically these


children have a barking cough, hoarse cry, but no stridor at rest. Children
with mild croup may have stridor when upset or crying (ie, agitated) and
either no, or only mild, chest wall/subcostal retractions [8,9].
Moderate croup is defined by a Westley croup score of 3 to 7. Children
with moderate croup have stridor at rest, at least mild retractions, and may
have other symptoms or signs of respiratory distress, but little or no
agitation [8,9].
Severe croup is defined by a Westley croup score of 8. Children with
severe croup have significant stridor at rest, although stridor may
decrease with worsening upper airway obstruction and decreased air
entry [8,9]. Retractions are severe (including indrawing of the sternum)
and the child may appear anxious, agitated, or fatigued. Prompt
recognition and treatment of children with severe croup are paramount.

Respiratory failure Croup occasionally results in significant upper airway


obstruction with impending respiratory failure, heralded by the following signs
[8,10]:

Fatigue and listlessness


Marked retractions (although retractions may decrease with increased
obstruction and decreased air entry)
Decreased or absent breath sounds
Depressed level of consciousness
Tachycardia out of proportion to fever
Cyanosis or pallor

PHONE TRIAGE The first contact with the health-care system regarding a
child with symptoms of croup may occur by phone. When assessing patients by
phone, the health-care provider must distinguish children who need immediate
medical attention or further evaluation from those who can be managed at
home. Children who need further evaluation include those who have:

Stridor at rest
An abnormal airway (eg, subglottic narrowing from care in the neonatal
intensive care unit)
Previous episodes of moderate to severe croup
Medical conditions that predispose to respiratory failure (eg,
neuromuscular disorders)
Rapid progression of symptoms (ie, symptoms of upper airway
obstruction after less than 12 hours of illness)
Inability to tolerate oral fluids
Parental concern that cannot be relieved by reassurance
Prolonged symptoms (more than three to seven days) or an atypical
course (perhaps indicating an alternative diagnosis) (see "Croup: Clinical
features, evaluation, and diagnosis", section on 'Differential diagnosis')

Patients who are assessed by phone and determined to have mild symptoms
and none of the above indications for further evaluation can be managed at
home. (See 'Home treatment' below.)

MILD CROUP Children with mild symptoms, defined by a Westley croup


score of 2, should be treated symptomatically with humidity, fever reduction,
and oral fluids. Many such children can be managed by phone, provided that
none of the criteria for further evaluation described above are present.

Home treatment The caregivers of children with mild croup who are
managed at home should be instructed in provision of supportive care including
mist, antipyretics, and encouragement of fluid intake.

In acute situations and for short periods of time, caregivers may try sitting with
the child in a bathroom filled with steam generated by running hot water from
the shower to improve symptoms. This may help reassure parents that
"something" is being done to reverse the symptoms, and anecdotal evidence
supports some value of this measure.

Exposure to cold night air also may lessen symptoms of mild croup, although
this has never been systematically studied. If parents or caregivers wish to use
humidifiers at home, only those that produce mist at room temperature should
be used to avoid the risk of burns from steam or the heating element.

Patients who are managed at home should receive a follow-up phone call;
caregivers should receive instructions regarding indications to seek medical
attention, including [8]:

Difficulty breathing
Pallor or cyanosis
Severe coughing spells
Drooling or difficulty swallowing
Fatigue
Worsening course
Fever (>38.5C)
Prolonged symptoms (longer than seven days)
Stridor at rest
Suprasternal retractions

Caregivers also should be provided with some guidance regarding when it is


safe for them to drive the child to the emergency department and when to call
for emergency medical services. Emergency medical services should provide
transportation for children who are severely agitated, cyanotic, struggling to
breathe, or lethargic [8].

Outpatient treatment Children who are seen in the office or emergency


department with mild croup may require little or no therapy, or may have
improvement with humidified air. (See "Croup: Pharmacologic and supportive
interventions", section on 'Mist therapy'.) Randomized controlled trials have
demonstrated that treatment with a single dose of oral dexamethasone (0.15 to
0.6 mg/kg, maximum dose 10 mg) may reduce the need for reevaluation,
shorten the course, improve duration of the child's sleep, and reduce parental
stress [11,12].

We suggest that children with mild croup who are seen in the outpatient setting
be treated with a single dose of oral dexamethasone (0.6 mg/kg). Treatment of
such children in the late morning or early afternoon hours may prevent
worsening of symptoms as evening approaches. However, anticipatory
guidance about potential worsening and when to seek care or return for follow-
up also is reasonable. (See "Croup: Pharmacologic and supportive
interventions", section on 'Dexamethasone'.)

Children with mild croup who are tolerating fluids and have not received
nebulized epinephrine can be sent home after specific follow-up (which may
occur by phone) has been arranged and the caregiver has received instructions
regarding home care and indications to seek medical attention as described
above. (See 'Home treatment' above.)

MODERATE TO SEVERE CROUP Children with moderate croup (Westley


croup score 3 to 7, stridor and retractions at rest without agitation) should be
evaluated in the emergency department or office (provided the office is
equipped to handle acute upper airway obstruction). Children with severe croup
(Westley croup score 8, stridor and retractions at rest with agitation, lethargy,
or cyanosis, marked sternal wall indrawing) should be evaluated in the
emergency department. Such children require aggressive therapy, monitoring,
and supportive care.

Supportive care Supportive care for children with moderate/severe croup


includes administration of humidified air or humidified oxygen as indicated for
hypoxemia (oxygen saturation <92 percent in room air) or respiratory distress.
(See "Croup: Pharmacologic and supportive interventions", section on
'Oxygen'.)

The child with severe croup must be approached cautiously, as any increase in
anxiety may worsen airway obstruction. The parent or caregiver should be
instructed to hold and comfort the child and to administer humidified oxygen.
Nebulized epinephrine should be added as quickly as possible, as described
below. In the meantime, health-care providers should continuously observe the
child and be prepared to provide bag mask ventilation and advanced airway
techniques if the condition worsens. (See "Emergent endotracheal intubation in
children".)

Monitoring Monitoring should include pulse oximetry and close observation


of respiratory status, including level of consciousness, stridor, cyanosis, air
entry, and retractions. Trends in ventilation can be monitored noninvasively with
capnography if capnography is available and the child will tolerate the nasal
prongs [13].

Fluids Administration of intravenous fluids may be necessary in some


children. Fever and tachypnea may increase fluid requirements, and respiratory
difficulty may prevent the child from achieving adequate oral intake.
(See "Maintenance fluid therapy in children".)

Intubation Endotracheal intubation is required in less than 1 percent of


those who are seen in the emergency department and 2 to 6 percent of those
who are hospitalized [14-17]. The need for intubation should be anticipated in
children with progressive respiratory failure so that the procedure can be
performed in as controlled a setting as possible. A tracheal tube that is 0.5 to 1
mm smaller than would typically be used may be required. (See 'Respiratory
failure' above and "Emergent endotracheal intubation in children", section on
'Endotracheal tube'.)

Pharmacotherapy The benefits of corticosteroids and


nebulized epinephrine for moderate to severe croup have been demonstrated in
meta-analysis and randomized controlled trials, respectively [1,18-20]. Specific
pharmacologic intervention depends upon the severity of symptoms:

For children with moderate stridor at rest and moderate retractions or


more severe symptoms, we recommend administration
of dexamethasone (0.6 mg/kg, maximum of 10 mg) by the least invasive
route possible: oral if oral intake is tolerated, intravenous if IV access has
been established, IM if oral intake is not tolerated and IV access has not
been established. The oral preparation of dexamethasone (1 mg per mL)
has a foul taste. The intravenous preparation is more concentrated (4 mg
per mL) and can be given orally mixed with syrup [8,21-23]. A single dose
of nebulized budesonide (as described below) is another option,
particularly for children who are vomiting. (See "Croup: Pharmacologic
and supportive interventions", section on 'Glucocorticoids'.)
For children with moderate stridor at rest and moderate retractions, or
more severe symptoms, we recommend nebulized epinephrine in addition
to dexamethasone:
Racemic epinephrine is administered as 0.05 mL/kg per dose
(maximum of 0.5 mL) of a 2.25 percent solution diluted to 3 mL total
volume with normal saline. It is given via nebulizer over 15 minutes.
L-epinephrine is administered as 0.5 mL/kg per dose (maximum of
5 mL) of a 1:1000 dilution. It is given via nebulizer over 15 minutes.

Nebulized epinephrine can be repeated every 15 to 20 minutes. The


administration of three or more doses within a two- to three-hour time period
should prompt initiation of close cardiac monitoring if this is not already
underway. (See "Croup: Pharmacologic and supportive interventions", section
on 'Nebulized epinephrine'.)

Children who receive nebulized epinephrine should also


receive dexamethasone by the least invasive route that can be accomplished,
as described above. (See "Croup: Pharmacologic and supportive interventions",
section on 'Glucocorticoids'.)
Although it is not routinely indicated in the treatment of croup, a single dose of
nebulized budesonide (2 mg [2 mL solution] via nebulizer) may provide an
alternative to IM or IV dexamethasone for children with vomiting or severe
respiratory distress [8]. In children with severe respiratory distress, budesonide
may be mixed withepinephrine and administered simultaneously [8].
(See "Croup: Pharmacologic and supportive interventions", section on
'Budesonide'.)

Observation Children with moderate/severe croup should be observed after


pharmacologic intervention. During the observation period, children should be
encouraged to drink.

Children who have received


nebulized epinephrine and dexamethasone with good response should be
observed for at least three to four hours [24-27]. Croup symptoms usually
improve within 30 minutes of administration of nebulized epinephrine
[28,29] but may return to baseline as the effects of epinephrine wear off
(usually by two hours).
Children who received dexamethasone and remain symptomatic should
be observed for at least four hours before deciding whether they require
hospital admission (as the effect of dexamethasone may not be apparent
for several hours) [8].

Discharge to home Many children with moderate/severe croup have


symptomatic improvement after treatment with
nebulized epinephrine and/or corticosteroids.

After three to four hours of observation, children who remain comfortable may
be discharged home if they meet the following criteria [24-27]:

No stridor at rest
Normal pulse oximetry
Good air exchange
Normal color
Normal level of consciousness
Demonstrated ability to tolerate fluids by mouth
Caregivers understand the indications for return to care and would be
able to return if necessary
Before discharge, follow-up with the primary care provider should be arranged
within the next 24 hours. Instructions regarding home treatment should be
provided. (See 'Home treatment' above.)

About 5 percent of children well enough for discharge from the emergency
department after receiving corticosteroids and
nebulized epinephrine treatments may be expected to return for care. Relapse
within 24 hours is unlikely in those who have minimal symptoms at the time of
discharge [30].

Hospitalization

Indications Children with moderate/severe croup whose condition worsens


or fails to improve as expected after treatment with nebulized epinephrine and
corticosteroids should be admitted to the hospital for repeated doses of
nebulized epinephrine, observation, and supportive care. Poor response to
nebulized epinephrine in conjunction with high fever and toxic appearance
should prompt consideration of bacterial tracheitis (picture 1) [8]. (See "Croup:
Clinical features, evaluation, and diagnosis", section on 'Bacterial
tracheitis' and "Bacterial tracheitis in children: Clinical features and diagnosis",
section on 'Clinical features'.)

Additional factors that influence the decision regarding admission include [8,31]:

Need for supplemental oxygen


Moderate retractions and tachypnea, indicating increased work of
breathing, which may lead to respiratory fatigue and failure
Degree of response to initial therapies
"Toxicity" or clinical picture suggesting serious secondary bacterial
infection
Poor oral intake and degree of dehydration
Young age, particularly younger than six months
Ability of the family to comprehend the instructions regarding recognition
of features that indicate the need to return for care
Ability of the family to return for care (eg, distance from home to care
site, weather/travel conditions)
Recurrent visits to the ED within 24 hours
Interventions Children who are admitted to the hospital should continue to
be monitored for heart rate and oxygen saturation and to receive humidified
oxygen as necessary. Capnography, if it is available, is a useful technique for
monitoring ventilation if the child will tolerate nasal prongs. If the child is unable
to tolerate oral intake, maintenance intravenous fluids should be administered.

Pharmacologic interventions for hospitalized patients may include


nebulized epinephrine for persisting severe respiratory distress. Nebulized
epinephrine can be repeated every 15 to 20 minutes, as described above.
(See'Pharmacotherapy' above.)

However, children who require repeated doses of epinephrine (eg, three or


more doses within two to three hours, or ongoing administration more frequently
than every one to two hours) should be admitted/transferredto an intensive care
unit or other setting where appropriately close monitoring can be accomplished.

Repeat doses of corticosteroids are not necessary on a routine basis and may
have adverse effects. Moderate to severe symptoms that persist for more than
a few days should prompt investigation for other causes of airway obstruction.
(See "Croup: Clinical features, evaluation, and diagnosis", section on
'Differential diagnosis'.)

Infection control Children who are admitted to the hospital with croup
should be managed with contact precautions (ie, gown and gloves for contact),
particularly if parainfluenza or respiratory syncytial virus is the suspected
etiology. If influenza is suspected, droplet isolation measures (ie, respiratory
mask within three feet) also should be followed. (See "General principles of
infection control".)

Discharge criteria Children who require hospital admission may be


discharged when they meet the following criteria:

No stridor at rest
Normal pulse oximetry
Good air exchange
Normal color
Normal level of consciousness
Demonstrated ability to tolerate fluids by mouth
FOLLOW-UP Any patient who was admitted to the hospital, received
nebulized epinephrine, or had a prolonged outpatient visit should have follow-
up scheduled with the primary care provider within 24 hours or as soon as can
be arranged. Although some children may continue to have mild to moderate
symptoms at the time of follow-up, there are no studies that support the routine
use of corticosteroid therapy beyond 24 hours.

Follow-up should continue until the child's symptoms have begun to resolve.
The child who does not improve as expected (over the course of approximately
seven days) may have an underlying airway abnormality or may be developing
a complication of croup. Further evaluation, particularly with a radiograph of the
soft tissues of the neck, or consultation with otolaryngology, may be warranted.
(See "Croup: Clinical features, evaluation, and diagnosis", section on
'Differential diagnosis'.)

PROGNOSIS Symptoms of croup resolve in most children within three days


but may persist for up to one week [32,33]. Less than 5 percent of children with
croup require hospital admission [34], and among those, 1 to 6 percent require
intubation [14-17,35]. Mortality is rare, occurring in <0.5 percent of intubated
children [36].

Complications Complications of croup are uncommon. Children with


moderate to severe croup are at risk for hypoxemia (oxygen saturation <92
percent in room air) and respiratory failure. Other complications include
pulmonary edema, pneumothorax, and pneumomediastinum [37]. These
complications can be anticipated and managed by aggressive monitoring and
intervention in the medical setting. Out-of-hospital cardiac arrest and death also
have been reported [38].

Secondary bacterial infections may arise from croup. Bacterial tracheitis,


bronchopneumonia, and pneumonia occur in a small number of patients
[10,15,33,39]. In most instances, the child has been relatively stable or
beginning to improve after several days of illness but then suddenly worsens,
with higher or recurrent fever, increased (and potentially productive)
cough, and/or respiratory distress. (See "Bacterial tracheitis in children: Clinical
features and diagnosis", section on 'Clinical features' and "Community-acquired
pneumonia in children: Clinical features and diagnosis", section on 'Clinical
presentation'.)
INFORMATION FOR PATIENTS UpToDate offers two types of patient
education materials, The Basics and Beyond the Basics. The Basics patient
education pieces are written in plain language, at the 5th to 6thgrade reading
level, and they answer the four or five key questions a patient might have about
a given condition. These articles are best for patients who want a general
overview and who prefer short, easy-to-read materials. Beyond the Basics
patient education pieces are longer, more sophisticated, and more detailed.
These articles are written at the 10th to 12th grade reading level and are best for
patients who want in-depth information and are comfortable with some medical
jargon.

Here are the patient education articles that are relevant to this topic. We
encourage you to print or e-mail these topics to your patients. (You can also
locate patient education articles on a variety of subjects by searching on
patient info and the keyword(s) of interest.)

Basics topics (see "Patient information: Croup (The Basics)")


Beyond the Basics topics (see "Patient information: Croup in infants and
children (Beyond the Basics)")

SUMMARY AND RECOMMENDATIONS

Overview

Children with croup who should be seen in the office or emergency


department include those who have stridor at rest, an abnormal airway,
previous episodes of moderate to severe croup, underlying conditions that
may predispose to respiratory failure, rapid progression of symptoms,
inability to tolerate fluids, prolonged symptoms, or an atypical course.
(See 'Phone triage' above.)

Mild symptoms

Children with mild symptoms can be managed at home. Families should


be instructed in provision of supportive care and indications to seek
medical attention. (See 'Home treatment' above.)
We suggest that a single dose of oral dexamethasone (0.6 mg/kg) be
used when electing to treat children with mild croup who are seen in the
outpatient setting (Grade 2A). (See 'Outpatient treatment' above
and"Croup: Pharmacologic and supportive interventions", section on
'Dexamethasone'.)
Children with moderate croup should be evaluated in the office or
emergency department, and those with severe croup should be evaluated
in the emergency department. Children with severe croup must be
approached cautiously, as any increase in anxiety may worsen airway
obstruction. (See 'Moderate to severe croup' above.)

Moderate to severe symptoms

Supportive care for the child with moderate or severe croup includes
administration of humidified air or oxygen as indicated by
hypoxemia and/or respiratory distress, provision of intravenous fluids, and
monitoring for worsening respiratory distress. (See 'Supportive
care' above and "Croup: Pharmacologic and supportive interventions",
section on 'Mist therapy'.)
We recommend that children with moderate to severe croup who have
moderate stridor at rest, moderate retractions, and/or more severe
symptoms be treated with nebulized epinephrine (Grade 1A) in addition
to dexamethasone. (See 'Pharmacotherapy' above and "Croup:
Pharmacologic and supportive interventions", section on 'Nebulized
epinephrine'.)
Racemic epinephrine is administered as 0.05 mL/kg per dose
(maximum of 0.5 mL) of a 2.25 percent solution diluted to 3 mL total
volume with normal saline. It is given via nebulizer over 15 minutes.
L-epinephrine is administered as 0.5 mL/kg per dose (maximum of
5 mL) of a 1:1000 dilution. It is given via nebulizer over 15 minutes.

Nebulized epinephrine can be repeated every 15 to 20 minutes. The


administration of three or more doses within a two- to three-hour time period
should prompt initiation of close cardiac monitoring if this is not already
underway.

We recommend that children with moderate to severe croup be treated


with dexamethasone (0.6 mg/kg,maximum of 10 mg), by the least invasive
route (Grade 1A). (See 'Pharmacotherapy' above and "Croup:
Pharmacologic and supportive interventions", section on
'Glucocorticoids'.)
Children with moderate to severe croup should be observed for three to
four hours after intervention. Those who improve may be discharged
home. (See 'Discharge to home' above.)
Children with moderate to severe croup whose condition worsens or fails
to improve as expected after treatment with nebulized epinephrine and
corticosteroids should be admitted to the hospital.
(See'Hospitalization' above.)
We suggest not using repeated doses of corticosteroids. (Grade 2C).
(See 'Hospitalization' above and"Croup: Pharmacologic and supportive
interventions", section on 'Repeated dosing'.)
Other causes of upper airway obstruction should be investigated in
children who have moderate to severe symptoms that persist for more
than a few days. (See "Croup: Clinical features, evaluation, and
diagnosis", section on 'Differential diagnosis'.)
Children who received nebulized epinephrine, had a prolonged
outpatient visit, or were admitted to the hospital should have follow-up
scheduled with the primary care provider within 24 hours of discharge or
as soon as follow-up can be arranged. (See 'Follow-up' above.)

Outcome

Most children with croup recover uneventfully. (See 'Prognosis' above.)


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laryngotracheobronchitis (croup). Use of intermittent positive-pressure breathing
and racemic epinephrine. Am J Dis Child 1975; 129:790.
21. Klassen TP, Craig WR, Moher D, et al. Nebulized budesonide and oral
dexamethasone for treatment of croup: a randomized controlled trial. JAMA
1998; 279:1629.
22. Paul RI. Oral dexamethasone for croup (commentary). AAP Grand Rounds
2004; 12:67.
23. Duggan DE, Yeh KC, Matalia N, et al. Bioavailability of oral dexamethasone.
Clin Pharmacol Ther 1975; 18:205.
24. Prendergast M, Jones JS, Hartman D. Racemic epinephrine in the treatment of
laryngotracheitis: can we identify children for outpatient therapy? Am J Emerg
Med 1994; 12:613.
25. Ledwith CA, Shea LM, Mauro RD. Safety and efficacy of nebulized racemic
epinephrine in conjunction with oral dexamethasone and mist in the outpatient
treatment of croup. Ann Emerg Med 1995; 25:331.
26. Kunkel NC, Baker MD. Use of racemic epinephrine, dexamethasone, and mist
in the outpatient management of croup. Pediatr Emerg Care 1996; 12:156.
27. Rizos JD, DiGravio BE, Sehl MJ, Tallon JM. The disposition of children with
croup treated with racemic epinephrine and dexamethasone in the emergency
department. J Emerg Med 1998; 16:535.
28. Waisman Y, Klein BL, Boenning DA, et al. Prospective randomized double-blind
study comparing L-epinephrine and racemic epinephrine aerosols in the
treatment of laryngotracheitis (croup). Pediatrics 1992; 89:302.
29. Fitzgerald D, Mellis C, Johnson M, et al. Nebulized budesonide is as effective
as nebulized adrenaline in moderately severe croup. Pediatrics 1996; 97:722.
30. Brown JC. The management of croup. Br Med Bull 2002; 61:189.
31. Kaditis AG, Wald ER. Viral croup: current diagnosis and treatment. Pediatr
Infect Dis J 1998; 17:827.
32. Thompson M, Vodicka TA, Blair PS, et al. Duration of symptoms of respiratory
tract infections in children: systematic review. BMJ 2013; 347:f7027.
33. Cherry JD. Croup (laryngitis, laryngotracheitis, spasmodic croup,
laryngotracheobronchitis, bacterial tracheitis, and
laryngotracheobronchopneumonitis) and epiglottitis (supraglottitis). In: Feigin
and Cherrys Textbook of Pediatric Infectious Diseases, 7th ed, Cherry JD,
Harrison GJ, Kaplan SL, et al (Eds), Elsevier Saunders, Philadelphia 2014.
p.241.
34. Johnson D. Croup. Clin Evid 2005; :310.
35. Dawson KP, Mogridge N, Downward G. Severe acute laryngotracheitis in
Christchurch 1980-90. N Z Med J 1991; 104:374.
36. McEniery J, Gillis J, Kilham H, Benjamin B. Review of intubation in severe
laryngotracheobronchitis. Pediatrics 1991; 87:847.
37. Travis KW, Todres ID, Shannon DC. Pulmonary edema associated with croup
and epiglottitis. Pediatrics 1977; 59:695.
38. Fisher JD. Out-of-hospital cardiopulmonary arrest in children with croup. Pediatr
Emerg Care 2004; 20:35.
39. Rosekrans JA. Viral croup: current diagnosis and treatment. Mayo Clin Proc
1998; 73:1102.

Topic 6004 Version 9.0


Croup: Clinical features, evaluation, and diagnosis
Author
Charles R Woods, MD, MS
Section Editors
Sheldon L Kaplan, MD
Gregory Redding, MD
Deputy Editor
Carrie Armsby, MD, MPH
Disclosures: Charles R Woods, MD, MS Other Financial Interest: Cerexa [Epiglottitis (Data
Safety Monitoring Board for pediatric trials of the antibiotic agent ceftaroline)]. Sheldon L
Kaplan, MD Grant/Research/Clinical Trial Support: Pfizer [vaccine (PCV13)]; Forest Lab
[antibiotic (Ceftaroline)]; Optimer [antibiotic (fidaxomicin)]. Consultant/Advisory Boards: Pfizer
[vaccine (PCV13)]. Gregory Redding, MD Nothing to disclose. Carrie Armsby, MD,
MPH Nothing to disclose.

Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found,
these are addressed by vetting through a multi-level review process, and through requirements
for references to be provided to support the content. Appropriately referenced content is
required of all authors and must conform to UpToDate standards of evidence.

Conflict of interest policy

All topics are updated as new evidence becomes available and our peer review
process is complete.
Literature review current through: Mar 2015. | This topic last updated: Feb
18, 2015.

INTRODUCTION Croup is a respiratory illness characterized by inspiratory


stridor, cough, and hoarseness. These symptoms result from inflammation in
the larynx and subglottic airway. A barking cough is the hallmark of croup
among infants and young children, whereas hoarseness predominates in older
children and adults. Although croup usually is a mild and self-limited illness,
significant upper airway obstruction, respiratory distress, and, rarely, death, can
occur.

The clinical features, evaluation, and diagnosis of croup will be discussed here.
The management of croup is discussed separately. (See "Croup: Approach to
management" and "Croup: Pharmacologic and supportive interventions".)

DEFINITIONS The term croup has been used to describe a variety of upper
respiratory conditions in children, including laryngitis, laryngotracheitis,
laryngotracheobronchitis, bacterial tracheitis, or spasmodic croup [1]. These
terms are defined below. In the past, the term croup also has been applied to
laryngeal diphtheria (diphtheritic or membranous croup), which is discussed
separately. (See "Epidemiology and pathophysiology of diphtheria" and "Clinical
manifestations, diagnosis and treatment of diphtheria".)

Throughout this review, the term croup will be used to refer to laryngotracheitis.
Laryngotracheobronchitis, laryngotracheobronchopneumonitis, bacterial
tracheitis, and spasmodic croup are designated specifically as such.

Laryngitis refers to inflammation limited to the larynx and manifests itself


as hoarseness [2]. It usually occurs in older children and adults and,
similar to croup, is frequently caused by a viral infection. The etiology,
management, and evaluation of other causes of hoarseness are
discussed in detail separately. (See "Hoarseness in children: Etiology and
management" and "Hoarseness in children: Evaluation".)
Laryngotracheitis (croup) refers to inflammation of the larynx and trachea
[2]. Although lower airway signs are absent, the typical barking cough will
be present.
Laryngotracheobronchitis (LTB) occurs when inflammation extends into
the bronchi, resulting in lower airway signs (eg, wheezing, crackles, air
trapping, increased tachypnea) and sometimes more severe illness than
laryngotracheitis alone [2]. This term commonly is used interchangeably
with laryngotracheitis, and the entities are often indistinct clinically. Further
extension of inflammation into the lower airways results in
laryngotracheobronchopneumonitis, which sometimes can be complicated
by bacterial superinfection. Bacterial superinfection can be manifest as
pneumonia, bronchopneumonia, or bacterial tracheitis.
Bacterial tracheitis (also called bacterial croup) describes bacterial
infection of the subglottic trachea, resulting in a thick, purulent exudate,
which causes symptoms of upper airway obstruction (picture 1). The
bronchi and lungs are typically involved, as well (ie, bacterial
tracheobronchitis). Bacterial tracheitis may occur as a complication of viral
respiratory infections (usually those which manifest themselves as LTB or
laryngotracheobronchopneumonitis) or as a primary bacterial infection.
(See "Bacterial tracheitis in children: Clinical features and diagnosis".)
Spasmodic croup is characterized by the sudden onset of inspiratory
stridor at night, short duration (several hours), and sudden cessation [2].
This is often in the setting of a mild upper respiratory infection, but without
fever or inflammation. A striking feature of spasmodic croup is its recurrent
nature, hence the alternate descriptive term, "frequently recurrent croup".
Because of some clinical overlap with atopic diseases, it is sometimes
referred to as "allergic croup".
We consider spasmodic croup to be distinct from atypical croup,
although the terms are sometimes used interchangeably. Atypical croup
may be defined as recurrent episodes of croup-like symptoms occurring
beyond the typical age range of six months to three years for viral croup
or recurrent episodes that do not appear to be simple spasmodic croup
[3].

ETIOLOGY Croup is usually caused by viruses. Bacterial infection may


occur secondarily, as described above.

Parainfluenza virus type 1 is the most common cause of acute laryngotracheitis,


especially the fall and winter epidemics [4-6]. Parainfluenza type 2 sometimes
causes croup outbreaks, but usually with milder disease than type 1.
Parainfluenza type 3 causes sporadic cases of croup that often are more
severe than those due to types 1 and 2. In multicenter surveillance of children
<5 years who were hospitalized with febrile or acute respiratory illnesses, 43
percent of children with confirmed parainfluenza infection were diagnosed with
croup [7]. Croup was the most common discharge diagnosis for children with
confirmed parainfluenza 1 (42 percent) and parainfluenza 2 (48 percent)
infections but was only diagnosed in 11 percent of children with confirmed
parainfluenza 3 infections.

The microbiology, pathogenesis, and epidemiology of parainfluenza infections


are discussed separately. (See"Parainfluenza viruses in children".)

A number of other viruses that typically cause lower respiratory tract disease
also can cause upper respiratory tract symptoms, including croup, as described
below [6].

Respiratory syncytial virus (RSV) and adenoviruses are relatively


frequent causes of croup. The laryngotracheal component of disease is
usually less significant than that of the lower airways. (See"Respiratory
syncytial virus infection: Clinical features and diagnosis", section on
'Clinical manifestations' and "Epidemiology and clinical manifestations of
adenovirus infection", section on 'Clinical presentation'.)
Human coronavirus NL63 (HCoV-NL63), first identified in 2004, has
been implicated in croup and other respiratory illnesses [8-10]. The
prevalence of HCoV-NL63 varies geographically. (See "Coronaviruses",
section on 'Respiratory'.)
Measles is an important cause of croup in areas where measles remains
prevalent. (See "Clinical manifestations and diagnosis of measles".)
Influenza virus is a relatively uncommon cause of croup. However,
children hospitalized with influenzal croup tend to have longer
hospitalization and greater risk of readmission for relapse of laryngeal
symptoms than those with parainfluenzal croup. (See "Seasonal influenza
in children: Clinical features and diagnosis".)
Rhinoviruses, enteroviruses (especially Coxsackie types A9, B4, and B5,
and echovirus types 4, 11, and 21), and herpes simplex virus are
occasional causes of sporadic cases of croup that are usually mild. (See
appropriate topic reviews).
Metapneumoviruses cause primarily lower respiratory tract disease
similar to RSV, but upper respiratory tract symptoms have been described
in some patients [11]. (See "Human metapneumovirus infections".)

Croup also may be caused by bacteria. Mycoplasma pneumoniae has been


associated with mild cases of croup. In addition, secondary bacterial infection
may occur in children with laryngotracheitis, laryngotracheobronchitis, or
laryngotracheobronchopneumonitis. The most common secondary bacterial
pathogens include Staphylococcus aureus, Streptococcus pyogenes, and S.
pneumoniae [1].

EPIDEMIOLOGY Croup most commonly occurs in children 6 to 36 months of


age. It is seen in younger infants (as young as three months) and in preschool
children, but it is rare beyond age six years [1,12]. It is more common in boys,
with a male:female ratio of about 1.4:1 [1,12-14].

Family history of croup is a risk factor for croup and recurrent croup. In a case-
control study, children whose parents had a history of croup were 3.2 times as
likely to have an episode of croup and 4.1 times as likely to have recurrent
croup as children with no parental history of croup [15]. Parental smoking, a
well-recognized risk factor for respiratory tract infections in children, does not
appear to increase the risk of croup [15,16]. (See"Secondhand smoke
exposure: Effects in children", section on 'Respiratory symptoms and illness'.)

Most cases of croup occur in the fall or early winter, with the major incidence
peaks coinciding with parainfluenza type 1 activity (often in October) and minor
peaks occurring during periods of respiratory syncytial virus or influenza virus
activity. (See "Respiratory syncytial virus infection: Clinical features and
diagnosis", section on 'Seasonality' and "Seasonal influenza in children: Clinical
features and diagnosis", section on 'Influenza activity'.)

Emergency department (ED) visits for croup are most frequent between 10:00
PM and 4:00 AM. However, children seen for croup between noon and 6:00 PM
are more likely to be admitted to the hospital [4,17]. A morning peak between
7:00 AM and 11:00 AM in ED visits for croup also has been noted [14].

Hospital admissions for croup have declined steadily since the late 1970s. In an
analysis of data from the National Hospital Discharge Surveys from 1979
through 1997, the estimated number of annual hospitalizations for croup
decreased from 48,900 to 33,500 [5]. Estimates of annual hospitalization rates
for croup caused by parainfluenza virus types 1 to 3 from 1994 to 1997 were
0.4 to 1.1 per 1000 children for children younger than one year and 0.24 to 0.61
per 1000 children for children between one and four years. Approximately one-
half of these hospitalizations were attributed to parainfluenza type 1.

In a six-year (1999 to 2005) population-based study, 5.6 percent of children


with a diagnosis of croup in the ED required hospital admission. Among those
discharged home, 4.4 percent had a repeat ED visit within 48 hours [14].

PATHOGENESIS The viruses that cause croup typically infect the nasal and
pharyngeal mucosal epithelia initially and then spread locally along the
respiratory epithelium to the larynx and trachea.

The anatomic hallmark of croup is narrowing of the trachea in the subglottic


region. This portion of the trachea is surrounded by a firm cartilaginous ring
such that any inflammation results in narrowing of the airway. In addition to this
"fixed" obstruction, dynamic obstruction of the extrathoracic trachea below the
cartilaginous ring may occur when the child struggles, cries, or becomes
agitated. The dynamic obstruction occurs as a result of the combination of high
negative pressure in the distal extrathoracic trachea and the floppiness of the
tracheal wall in children.

Laryngoscopic evaluation of patients during acute laryngotracheitis shows


redness and swelling of the lateral walls of the trachea. In severe cases, the
subglottic airway may be reduced to a diameter of 1 to 2 mm. In addition to
mucosal edema and swelling, fibrinous exudates and, occasionally,
pseudomembranes can build up on the tracheal surfaces and contribute to
airway narrowing. The vocal cords and laryngeal tissues also can become
swollen, and cord mobility may be impaired [2,18-20]. Autopsy studies in
children with laryngotracheitis show infiltration of histiocytes, lymphocytes,
plasma cells, and neutrophils into edematous lamina propria, submucosa, and
adventitia of the larynx and trachea [21-23].

In spasmodic croup, findings on direct laryngoscopy demonstrate


noninflammatory edema [18]. This suggests that there is no direct viral
involvement of the tracheal epithelium.

Patients with bacterial tracheitis have a bacterial superinfection that causes


thick pus to develop within the lumen of the subglottic trachea (picture 1).
Ulcerations, pseudomembranes, and microabscesses of the mucosal surface
occur. The supraglottic tissues usually are normal. (See "Bacterial tracheitis in
children: Clinical features and diagnosis", section on 'Pathogenesis'.)

Host factors Only a small fraction of children with parainfluenza virus


infections develop overt croup. This suggests that host (or genetic) factors play
a role in the pathogenesis. Host factors that may contribute to the development
of croup include functional or anatomic susceptibility to upper airway narrowing,
variations in immune response, and predisposition to atopy [14].

Underlying host factors that predispose to clinically significant narrowing of the


upper airway include:

Anatomic narrowing of the airway, from etiologies such as subglottic


stenosis, laryngeal webs, tracheomalacia, laryngomalacia, laryngeal
clefts, or subglottic hemangiomas [3]
Hyperactive airways, perhaps aggravated by atopy or gastroesophageal
reflux, as suggested in some children with spasmodic croup or recurrent
croup [24-26]
Acquired airway narrowing from respiratory tract papillomas (human
papillomavirus), post-intubation scarring, or irritation from aspirations
associated with gastroesophageal reflux

The potential role of the immune response was demonstrated in studies that
demonstrated increased production of parainfluenza virus-specific IgE and
increased lymphoproliferative response to parainfluenza virus antigen, and
diminished histamine-induced suppression of lymphocyte transformation
responses to parainfluenza virus in children with parainfluenza virus and croup
compared with those with parainfluenza virus without croup [27,28].

CLINICAL PRESENTATION The clinical presentation of croup depends


upon the specific croup syndrome and the degree of upper airway obstruction.
Although croup usually is a mild and self-limited illness, specific features of the
history and physical examination identify children who are seriously ill or at risk
for rapid progression of disease. (See 'Evaluation' below.)

Laryngotracheitis Laryngotracheitis typically occurs in children three


months to three years of age [2]. The onset of symptoms is usually gradual,
beginning with nasal irritation, congestion, and coryza. Symptoms generally
progress over 12 to 48 hours to include fever, hoarseness, barking cough, and
stridor. Respiratory distress increases as upper airway obstruction becomes
more severe. Rapid progression or signs of lower airway involvement suggests
a more serious illness. Cough usually resolves within three days [29]; other
symptoms may persist for seven days with a gradual return to normal [2].
Deviations from this expected course should prompt consideration of diagnoses
other than laryngotracheitis. (See 'Differential diagnosis'below.)

The degree of upper airway obstruction is evident on physical examination, as


described below. In mild cases, the child is hoarse and has nasal congestion.
There is minimal, if any, pharyngitis. As airway obstruction progresses, stridor
develops, and there may be mild tachypnea with a prolonged inspiratory phase.
The presence of stridor is a key element in the assessment of severity. Stridor
at rest is a sign of significant upper airway obstruction. As upper airway
obstruction progresses, the child may become restless or anxious.
(See'Severity assessment' below.)
When airway obstruction becomes severe, suprasternal, subcostal, and
intercostal retractions may be seen. Breath sounds can be diminished.
Agitation, which generally is accompanied by increased inspiratory effort,
exacerbates the subglottic narrowing by creating negative pressure in the
airway. This can lead to further respiratory distress and agitation.

Hypoxia and cyanosis can develop, as can respiratory fatigue from sustained
increased respiratory effort. High respiratory rates also tend to correlate with the
presence of hypoxia. Without intervention, the hypoxia or fatigue can
sometimes lead to death.

Spasmodic croup Spasmodic croup also occurs in children three months to


three years of age [2]. In contrast to laryngotracheitis, spasmodic croup always
occurs at night; the duration of symptoms is short, often with symptoms
subsiding by the time of presentation for medical attention; and the onset and
cessation of symptoms are abrupt. Fever is typically absent, but mild upper
respiratory tract symptoms (eg, coryza) may be present. Episodes can recur
within the same night and for two to four successive evenings [30]. A striking
feature of spasmodic croup is its recurrent nature, hence the alternate
descriptive term, "frequently recurrent croup". There may be a familial
predisposition to spasmodic croup, and it may be more common in children with
a family history of allergies [24].

Early in the clinical course, spasmodic croup may be difficult to distinguish from
laryngotracheitis. As the course progresses, the episodic nature of spasmodic
croup and relative wellness of the child between attacks differentiate it from
classic croup, in which the symptoms are continuous.

Although the initial presentation can be dramatic, the clinical course is usually
benign. Symptoms are almost always relieved by comforting the anxious child
and administering humidified air. Rarely, children may benefit from treatment
with corticosteroids and/or nebulized epinephrine [31]. Other therapies
generally are not indicated. (See "Croup: Approach to management".)

Bacterial tracheitis Bacterial tracheitis may present as a primary or


secondary infection [32]. In primary infection, there is acute onset of symptoms
of upper airway obstruction with fever and toxic appearance. In secondary
infection, there is marked worsening during the clinical course of viral
laryngotracheitis, with high fever, toxic appearance, and increasing respiratory
distress secondary to tracheal obstruction from purulent secretions. In both of
these presentations, signs of lower airway disease, such as crackles and
wheezes, may be present. However, the upper airway obstruction is the more
clinically significant manifestation [2,33]. (See"Bacterial tracheitis in children:
Clinical features and diagnosis", section on 'Clinical features'.)

Recurrent croup A child who has had recurrent episodes of classic viral
croup may have an underlying condition that predisposes him or her to develop
clinically significant narrowing of the upper airway. Recurrent episodes of croup-
like symptoms occurring outside the typical age range for viral croup (ie, six
months to three years) and recurrent episodes that do not appear to be simple
spasmodic croup should raise suspicion for large airway lesions,
gastroesophageal reflux or eosinophilic esophagitis, or atopic conditions [3,34-
38].

Children with recurrent croup may require radiographic evaluation or


bronchoscopy. (See 'Host factors' above and 'Imaging' below.)

EVALUATION

Overview The evaluation of children with suspected croup has several


objectives, including prompt identification of patients with significant upper
airway obstruction or at risk for rapid progression of upper airway obstruction. In
addition, there are some conditions with presentations similar to that of croup
that require specific evaluations and/or interventions; these too must be
promptly identified. (See 'Differential diagnosis' below.)

During the evaluation, efforts should be made to make the child as comfortable
as possible. The increased inspiratory effort that accompanies anxiety and fear
in young children can exacerbate subglottic narrowing, further diminishing air
exchange and oxygenation. (See 'Pathogenesis' above.)

Rapid assessment and initial management Rapid assessment of general


appearance (including the presence of stridor at rest), vital signs, pulse
oximetry, airway stability, and mental status is necessary to identify children
with severe respiratory distress and/or impending respiratory failure.
(See "Croup: Approach to management".)
Endotracheal intubation is required in less than 1 percent of children with croup
who are seen in the emergency department. However, the need for
endotracheal intubation should be anticipated in children with progressive
respiratory failure so that it can be performed in as controlled a setting as
possible. Respiratory failure is heralded by the following signs [1,39,40]:

Fatigue and listlessness


Marked retractions (although retractions may decrease with increased
obstruction and decreased air entry)
Decreased or absent breath sounds
Depressed level of consciousness
Tachycardia out of proportion to fever
Cyanosis or pallor

A tracheal tube that is 0.5 to 1 mm smaller than would typically be used may be
required. (See "Emergent endotracheal intubation in children", section on
'Endotracheal tube'.)

In addition to establishment of an airway, children who have severe respiratory


distress require immediate pharmacologic treatment, including administration of
nebulized epinephrine and systemic or nebulized corticosteroids. (See "Croup:
Approach to management", section on 'Moderate to severe croup'.)

Once control of the airway is established and pharmacologic treatment, if


necessary, is under way, the remainder of the evaluation can proceed.

History The history should include a description of the onset, duration, and
progression of symptoms. Factors that are associated with increased severity of
illness include:

Sudden onset of symptoms


Rapidly progressing symptoms (ie, symptoms of upper airway
obstruction after fewer than 12 hours of illness)
Previous episodes of croup
Underlying abnormality of the upper airway
Medical conditions that predispose to respiratory failure (eg,
neuromuscular disorders)
Aspects of the history that are helpful in distinguishing croup from other causes
of acute upper airway obstruction include [1,41]:

Fever The absence of fever from onset of symptoms to the time of


presentation is suggestive of spasmodic croup or a noninfectious etiology
(eg, foreign body aspiration or ingestion, acute angioneurotic edema).
Hoarseness and barking cough Hoarseness and barking cough,
characteristic findings in croup, are typically absent in children with acute
epiglottitis, foreign body aspiration, and angioneurotic edema.
Difficulty swallowing Difficulty swallowing may occur in acute epiglottitis
and foreign body aspiration. A large ingested foreign body may lodge in
the upper esophagus, where it distorts and narrows the upper trachea,
thus mimicking the croup syndrome (including barking cough and
inspiratory stridor).
Drooling Drooling may occur in children with peritonsillar or
retropharyngeal abscesses, retropharyngeal cellulitis, and epiglottitis. In
an observational study, drooling was present in approximately 80 percent
of children with epiglottitis, but only 10 percent of those with croup [41].
Throat pain Complaints of dysphagia and sore throat are more
common in children with epiglottitis than croup (approximately 60 to 70
percent versus <10 percent) [41].

The differential diagnosis of croup is discussed in greater detail below.


(See 'Differential diagnosis' below.)

Examination The objectives of the examination of the child with croup


include assessment of severity of upper airway obstruction and exclusion of
other infectious and non-infectious causes of acute upper airway obstruction,
both of which are necessary in making management decisions.

The initial examination often can be accomplished by observing the child in a


comfortable position with the caretaker. Every effort should be made to
measure the child's weight and vital signs.

Aspects of the examination that are helpful in assessing the degree of upper
airway obstruction and severity of illness include:
Overall appearance Is the child comfortable and interactive, anxious
and quiet, or obtunded? Is there stridor at rest? Stridor at rest is a sign of
significant upper airway obstruction. Children with significant upper airway
obstruction may prefer to sit up and lean forward in a "sniffing" position
(neck is mildly flexed, and head is mildly extended). This position tends to
improve the patency of the upper airway.
Quality of the voice Does the child have a hoarse or diminished cry? Is
the voice muffled? A muffled "hot potato" voice is suggestive of epiglottitis,
retropharyngeal abscess, or peritonsillar abscess.
Degree of respiratory distress Signs of respiratory distress include
tachypnea, hypoxemia, and increased work of breathing (intercostal,
subcostal, or suprasternal retractions; nasal flaring; grunting; use of
accessory muscles)
Tidal volume Does there appear to be good chest expansion with
inspiration, indicating adequate air entry?
Lung examination Are there abnormal respiratory sounds during
inspiration or expiration? Inspiratory stridor indicates upper airway
obstruction, whereas expiratory wheezing is a sign of lower airway
obstruction. If there is stridor, is it present at rest or only with agitation? As
discussed above, stridor at rest is a sign of significant upper airway
obstruction. Stridor will be more obvious on auscultation, since the
inspiratory noise is transmitted through the chest. The presence of
crackles (rales) also suggests lower respiratory tract involvement (eg,
laryngotracheobronchitis, laryngotracheobronchopneumonitis, or bacterial
tracheitis).
Assessment of hydration status Decreased oral intake and increased
insensible losses from fever and tachypnea may result in dehydration.
(See "Clinical assessment and diagnosis of hypovolemia (dehydration) in
children".)

These aspects of the examination are often used in clinical scoring systems to
evaluate the severity of illnessand/or in making decisions regarding the need for
hospital admission. (See 'Severity assessment' below and"Croup: Approach to
management".)
Components of the examination that are useful in distinguishing croup from
other causes of acute upper airway obstruction include [39,41]:

Preferred posture Children with epiglottitis usually prefer to sit up in the


tripod or sniffing position (picture 2A-B).
Examination of the oropharynx for the following signs:
Cherry red, swollen epiglottis, suggestive of epiglottitis
Pharyngitis, typically minimal in laryngotracheitis, may be more
pronounced in epiglottitis or laryngitis
Excessive salivation, suggestive of acute epiglottitis, peritonsillar
abscess, or retropharyngeal abscess
Diphtheritic membrane
Tonsillar asymmetry or deviation of the uvula suggestive of
peritonsillar abscess
Midline or unilateral swelling of the posterior pharyngeal wall
suggestive of retropharyngeal abscess
Concerns have been raised about safety of examining the pharynx in
children with upper airway obstruction and possible epiglottitis since
such efforts have been reported to precipitate cardiorespiratory
arrest. However, in two series, each including more than 200
patients with epiglottitis or viral croup, direct examination of the
oropharynx was not associated with sudden clinical deterioration
[32,42].
Examination of the cervical lymph nodes, which can be enlarged in
patients with retropharyngeal or peritonsillar abscesses.
Other physical findings may be present, depending on the particular
inciting virus. As an example, rash, conjunctivitis, exudative pharyngitis,
and adenopathy are suggestive of adenovirus infection.
Otitis media (acute or with effusion) may be present as a primary viral or
secondary bacterial process.

The differential diagnosis of croup is discussed in greater detail below.


(See 'Differential diagnosis' below.)

Severity assessment The severity of croup is often determined by the


clinical scoring systems. Although there are a number of validated croup
scoring systems, the Westley croup score [43] has been the most extensively
studied; it is described below. No matter which system is used to assess
severity, the presence of chest wall retractions and stridor at rest are the two
critical clinical features.

The elements of the Westley croup score describe key features of the physical
examination [43]. Each element is assigned a score, as illustrated below:

Level of consciousness: Normal, including sleep = 0; disoriented = 5


Cyanosis: None = 0; with agitation = 4; at rest = 5
Stridor: None = 0; with agitation = 1; at rest = 2
Air entry: Normal = 0; decreased = 1; markedly decreased = 2
Retractions: None = 0; mild = 1; moderate = 2; severe = 3

Mild croup is defined by a Westley croup score of 2. Typically, these children


have a barking cough and hoarse cry, but no stridor at rest. Children with mild
croup may have stridor when upset or crying (ie, agitated) and either no, or only
mild, chest wall/subcostal retractions [1,39].

Moderate croup is defined by a Westley croup score of 3 to 7. Children with


moderate croup have stridor at rest, at least mild retractions, and may have
other symptoms or signs of respiratory distress, but little or no agitation [1,39].

Severe croup is defined by a Westley croup score of 8. Children with severe


croup have significant stridor at rest, although stridor may decrease with
worsening upper airway obstruction and decreased air entry [1,39]. Retractions
are severe (including indrawing of the sternum) and the child may appear
anxious, agitated, or fatigued. Prompt recognition and treatment of children with
severe croup are paramount.

Imaging

Indications Radiographic confirmation of acute laryngotracheitis is not


required in the vast majority of children with croup. Radiographic evaluation of
the chest and/or upper trachea is indicated if the diagnosis is in question, the
course is atypical, an inhaled or swallowed foreign body is suspected (although
the majority are not radio-opaque), croup is recurrent, and/or there is a failure to
respond as expected to therapeutic interventions. (See 'Differential
diagnosis' below and "Croup: Approach to management".)
Findings In children with croup, a posterior-anterior chest radiograph
demonstrates subglottic narrowing, commonly called the "steeple sign" (image
1). The lateral view may demonstrate overdistention of the hypopharynx during
inspiration [44] and subglottic haziness (image 2). The epiglottis should have a
normal appearance.

In contrast, the lateral radiograph in virtually all children with epiglottitis


demonstrates swelling of the epiglottis, sometimes called the "thumb sign"
(image 3). (See "Epiglottitis (supraglottitis): Clinical features and diagnosis",
section on 'Radiographic features'.)

The lateral radiograph in children with bacterial tracheitis may demonstrate only
nonspecific edema or intraluminal membranes and irregularities of the tracheal
wall (image 4) [45].

Laboratory studies Laboratory studies, which are rarely indicated in


children with croup, are of limited diagnostic utility but may help guide
management in more severe cases.

Blood tests The white blood cell (WBC) count can be low, normal, or
elevated; WBC counts >10,000cells/microL are common. Neutrophil or
lymphocyte predominance may be present on the differential [46,47]. The
presence of a large number of band-form neutrophils is suggestive of primary or
secondary bacterial infection. Croup is not associated with any specific
alterations in serum chemistries.

Microbiology Confirmation of etiologic diagnosis is not necessary for most


children with croup, since croup is a self-limited illness that usually requires only
symptomatic therapy. When an etiologic diagnosis is necessary, viral
culture and/or rapid diagnostic tests that detect viral antigens are performed on
secretions from the nasopharynx or throat. (See 'Etiologic diagnosis' below.)

DIAGNOSIS

Clinical diagnosis The diagnosis of croup is clinical, based on the presence


of a barking cough and stridor, especially during a typical community epidemic
of one of the causative viruses. (See 'Etiology' above.)
Neither radiographs nor laboratory tests are necessary to make the diagnosis.
However, radiographs may be helpful in excluding other causes if the diagnosis
is in question. (See 'Differential diagnosis' below.)

Etiologic diagnosis Although not typically required in most cases of croup,


identification of a specific viral etiology may be necessary to make decisions
regarding isolation for patients requiring hospitalization or for
public health/epidemiologic monitoring purposes. Testing for influenza is
indicated if the results will influence decisions regarding treatment, prophylaxis
of contacts, or performance of other diagnostic tests; laboratory confirmation
should not delay the initiation of antiviral therapy for influenza when clinical and
seasonal considerations are compatible with influenza as the potential etiology
of croup. (See "Seasonal influenza in children: Clinical features and diagnosis",
section on 'Laboratory diagnosis' and "Seasonal influenza in children:
Prevention and treatment with antiviral drugs", section on 'Timing of treatment'.)

Diagnosis of a specific viral etiology can be made by viral culture of secretions


from the nasopharynx or throat. Rapid tests that detect viral antigens in these
secretions are commercially available for many respiratory viruses. The
diagnosis of specific viral infections is discussed in detail in individual topic
reviews:

Parainfluenza (see "Parainfluenza viruses in children", section on


'Diagnosis')
Influenza (see "Seasonal influenza in children: Clinical features and
diagnosis", section on 'Diagnosis')
Respiratory syncytial virus (see "Respiratory syncytial virus infection:
Clinical features and diagnosis", section on 'Laboratory diagnosis')
Adenovirus (see "Diagnosis, treatment, and prevention of adenovirus
infection", section on 'Diagnostic tests of choice for different adenovirus
syndromes')
Measles (see "Clinical manifestations and diagnosis of measles", section
on 'Diagnosis')
Enteroviruses (see "Clinical manifestations and diagnosis of enterovirus
and parechovirus infections", section on 'Laboratory diagnosis')
Metapneumovirus (see "Human metapneumovirus infections", section on
'Diagnosis')
Coronavirus (see "Coronaviruses", section on 'Diagnosis')

In addition, multiplex tests, which assess the presence of multiple agents at the
same time, and PCR-based tests are becoming more widely available [48].

DIFFERENTIAL DIAGNOSIS The differential diagnosis of croup includes


other causes of stridor and/orrespiratory distress. The primary considerations
are those with acute onset, particularly those that may rapidly progress to
complete upper airway obstruction, and those that require specific therapy.
Underlying anatomic anomalies of the upper airway also must be considered,
since they may contribute to more severe disease. (See 'Host factors' above.)

Important considerations include [1,12]:

Acute epiglottitis
Peritonsillar and retropharyngeal abscesses
Foreign body aspiration or ingestion
Allergic reaction
Acute angioneurotic edema
Upper airway injury
Congenital anomalies of the upper airway
Laryngeal diphtheria (see "Clinical manifestations, diagnosis and
treatment of diphtheria")

Acute epiglottitis Epiglottitis, which is rare in the era of vaccination


against Haemophilus influenzae type b, is distinguished from croup by the
absence of barking cough and the presence of anxiety that is out of proportion
to the degree of respiratory distress. Onset of symptoms is rapid, and because
of the associated bacteremia, the child is highly febrile, pale, toxic, and ill-
appearing. Because of the swollen epiglottis, the child will have difficulty
swallowing and is often drooling. The children usually prefer to sit up and
seldom have observed cough [41]. Epiglottitis occurs infrequently, and there is
no predominant etiologic pathogen. (See"Epiglottitis (supraglottitis): Clinical
features and diagnosis".)

Peritonsillar or retropharyngeal abscesses Children with deep neck space


abscesses, cellulitis of the cervical prevertebral tissues, or other painful
infections of the oropharynx may present with drooling and neck extension and
varying degrees of toxicity. Barking cough is usually absent. (See "Peritonsillar
cellulitis and abscess", section on 'Presentation' and "Retropharyngeal
infections in children", section on 'Presentation'.)

Foreign body In foreign body aspiration, there often is a history of the


sudden onset of choking and symptoms of upper airway obstruction in a
previously healthy child. If an inhaled foreign body lodges in the larynx, it will
produce hoarseness and stridor. If a large foreign body is swallowed, it may
lodge in the upper esophagus, resulting in distortion of the adjacent soft
extrathoracic trachea, producing a barking cough and inspiratory stridor.
(See "Airway foreign bodies in children" and "Foreign bodies of the esophagus
and gastrointestinal tract in children".)

Allergic reaction or acute angioneurotic edema Allergic reaction or acute


angioneurotic edema has rapid onset without antecedent cold symptoms or
fever. The primary manifestations are swelling of the lips and tongue, urticarial
rash, dysphagia without hoarseness, and sometimes inspiratory stridor [1,12].
There may be a history of allergy or a previous attack. (See "An overview of
angioedema: Clinical features, diagnosis, and management", section on
'Clinical features'.)

Upper airway injury Injury to the airway from smoke or thermal or chemical
burns should be evident from the history. The child typically does not have fever
or a viral prodrome.

Anomalies of the upper airway Hoarseness and stridor caused by


anomalies of the upper airway (eg, laryngeal webs, laryngomalacia, vocal cord
paralysis, congenital subglottic stenosis, and subglottic hemangioma) and
laryngeal papillomas have a more chronic course with absence of fever and
symptoms of upper respiratory tract illness, unless the presentation is due to
exacerbation of airway narrowing from the impact of a concomitant viral
infection. (See "Assessment of stridor in children" and "Hoarseness in children:
Etiology and management" and "Congenital anomalies of the larynx".)

Other potential mimickers of croup Other potential mimickers of croup


include bronchogenic cyst (which can cause airway compression) and early
Guillain-Barr syndrome (involvement of the laryngeal nerve may cause vocal
cord paralysis) [49,50]. (See "Congenital anomalies of the intrathoracic airways
and tracheoesophageal fistula", section on 'Bronchogenic
cyst' and "Epidemiology, clinical features, and diagnosis of Guillain-Barr
syndrome in children", section on 'Clinical features' and "Hoarseness in
children: Etiology and management", section on 'Vocal fold paralysis'.)

INFORMATION FOR PATIENTS UpToDate offers two types of patient


education materials, The Basics and Beyond the Basics. The Basics patient
education pieces are written in plain language, at the 5th to 6thgrade reading
level, and they answer the four or five key questions a patient might have about
a given condition. These articles are best for patients who want a general
overview and who prefer short, easy-to-read materials. Beyond the Basics
patient education pieces are longer, more sophisticated, and more detailed.
These articles are written at the 10th to 12th grade reading level and are best for
patients who want in-depth information and are comfortable with some medical
jargon.

Here are the patient education articles that are relevant to this topic. We
encourage you to print or e-mail these topics to your patients. (You can also
locate patient education articles on a variety of subjects by searching on
patient info and the keyword(s) of interest.)

Basics topic (see "Patient information: Croup (The Basics)")


Beyond the Basics topic (see "Patient information: Croup in infants and
children (Beyond the Basics)")

SUMMARY AND RECOMMENDATIONS

The term croup has been used to describe a variety of upper respiratory
conditions in children, including laryngitis, laryngotracheitis,
laryngotracheobronchitis, bacterial tracheitis, or spasmodic croup.
(See'Definitions' above.)
Croup is usually caused by viruses. Bacterial infection may occur
secondarily. Parainfluenza virus type 1 is the most common cause of
croup; other causes include respiratory syncytial virus and influenza virus.
(See 'Etiology' above.)
Croup most commonly occurs in children 6 to 36 months of age. Most
cases occur in the fall or early winter. (See 'Epidemiology' above.)
Host factors that may contribute to the development of croup include
functional or anatomic susceptibility to upper airway narrowing.
(See 'Pathogenesis' above.)
The clinical presentation of croup depends upon the specific croup
syndrome and the degree of upper airway obstruction. (See 'Clinical
presentation' above.)
The onset of symptoms in laryngotracheitis is gradual, beginning with
nasal irritation, congestion, and coryza. Fever, hoarseness, barking
cough, and stridor usually develop during the next 12 to 48 hours. Rapid
progression or signs of lower airway involvement suggest a more serious
illness. (See'Laryngotracheitis' above.)
The onset of symptoms in spasmodic croup is sudden and always
occurs at night. Fever is typically absent, but mild upper respiratory tract
symptoms may be present. (See 'Spasmodic croup' above.)
Bacterial tracheitis (picture 1 and image 4) may present acutely or as
marked worsening during the course of an antecedent viral upper
respiratory infection. Clinical manifestations of bacterial tracheitis include
fever, toxic appearance, and severe respiratory distress. (See 'Bacterial
tracheitis' above and"Bacterial tracheitis in children: Clinical features and
diagnosis".)
The objectives of the evaluation of the child with croup include
assessment of severity and exclusion of other causes of upper airway
obstruction. (See 'Overview' above.)
Rapid assessment of general appearance, vital signs, pulse oximetry,
airway stability, and mental status are necessary to identify children with
severe respiratory distress and/or impending respiratory failure.
(See 'Rapid assessment and initial management' above.)
The history should include a description of the onset, duration and
progression of symptoms, and ascertain whether there are any underlying
conditions that predispose to a more severe course. (See'History' above.)
Aspects of the examination that are useful in assessing the severity of
upper airway obstruction include overall appearance (including the
presence of stridor at rest or only with agitation), quality of voice, work of
breathing, tidal volume and air entry, and the presence of wheezing.
(See 'Examination' above.)
The diagnosis of croup is clinical, based upon the presence of a barking
cough and stridor. Neither radiographs nor laboratory tests are necessary
to make the diagnosis. However, radiographs may be helpful in excluding
other causes if the diagnosis is in question. (See 'Diagnosis' above.)
The differential diagnosis of croup includes other causes of
stridor and/or respiratory distress. The primary considerations are those
with acute onset, particularly those that may rapidly progress to complete
upper airway obstruction, and those that require specific therapy.
Important considerations include acute epiglottitis, peritonsillar and
retropharyngeal abscesses, foreign body aspiration, acute angioneurotic
edema, upper airway injury, and congenital anomalies of the upper
airway. (See'Differential diagnosis' above.)
Use of UpToDate is subject to the Subscription and License Agreement.
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