Croup BMJ

Croup
The right clinical information, right where it's needed
Last updated: Jun 28, 2018

Table of Contents
Summary 3
Basics 4
Definition 4
Epidemiology 4
Etiology 4
Pathophysiology 4
Prevention 5
Primary prevention 5
Secondary prevention 5
Diagnosis 6
Case history 6
Step-by-step diagnostic approach 6
Risk factors 7
History & examination factors 7
Diagnostic tests 9
Differential diagnosis 10
Diagnostic criteria 12
Treatment 13
Step-by-step treatment approach 13
Treatment details overview 15
Treatment options 16
Follow up 22
Recommendations 22
Complications 23
Prognosis 23
Guidelines 24
Diagnostic guidelines 24
Treatment guidelines 24
Evidence scores 25
References 28
Disclaimer 35
Summary
◊ Common cause of acute respiratory distress in children.
◊ Acute onset of seal-like barky cough, in moderate to severe cases accompanied by stridor and
sternal/intercostal indrawing.
◊ Careful history and physical exam sufficient for confirming clinical diagnosis and ruling out potentially
serious differentials.
◊ Orally administered corticosteroids are the mainstay for all levels of severity, combined with
nebulized epinephrine in moderate to severe croup to provide temporary relief of the symptoms of
upper-airway obstruction.
Croup Basics
Definition
Croup, also known as laryngotracheobronchitis, is a common respiratory disease of childhood, characterized
by the sudden onset of a seal-like barky cough, often accompanied by stridor, voice hoarseness, and
BASICS
respiratory distress. The symptoms are a result of upper-airway obstruction due to generalized inflammation
of the airways, as a result of viral infection (typically parainfluenza virus types 1 or 3).
Epidemiology
Croup is a frequent cause of acute respiratory distress in young children. Typically, it affects those between
6 months and 3 years of age, peaking in the second year of life. It has been reported to occur in infants
younger than 6 months, in adolescents and, more rarely, in adults.[1] [2] An observational study in a
pediatric group practice found it to be the confirmed diagnosis in 15% of all cases of lower respiratory
infection.[1] Boys are more commonly affected, with a ratio of 1.4:1 compared with girls.[1] There is no
evidence to suggest variations in ethnicity prevalence. In North America, admission rates peak in late
autumn (September through December), but cases occur all year round.[3] A peak in clinical presentations is
correlated with parainfluenza virus epidemics. These peaks typically occur in alternating years and result in a
50 % increase in the number of children admitted with croup.[3]
Etiology
The illness is due to viral infection (typically parainfluenza virus types 1 or 3).[3] Several other viral
pathogens have been recognized, including influenza A and B, adenovirus, respiratory syncytial virus,
metapneumovirus, coronavirus HCoV-NL63, and rarely measles.[1] [4] [5] [6] [7] [8] Distinctions have
been made between viral croup and spasmodic croup. However, it remains unclear as to whether these
entities represent different diseases or are merely a spectrum of the same disease. Clinically, it is difficult
to distinguish between the two, and is likely unnecessary as treatment decisions are based upon history
and clinical severity of the airway obstruction. Historically, laryngeal diphtheria was well known as a cause
of croup, but this is now rare in immunized populations. Reports of diphtheric croup have been published in
case series from India and Russia.[9] [10] [11] [12] A weak link between a history of previous intubation and
croup has been indicated.[13]
Pathophysiology
The symptoms result from upper-airway obstruction due to generalized inflammation and edema of the
airways. At the cellular level this progresses to necrosis and shedding of the epithelium. The narrowed
subglottic region is responsible for the symptoms of seal-like barky cough, stridor (from increased airflow
turbulence), and sternal/intercostal indrawing. If the upper-airway obstruction worsens, respiratory failure can
result, leading to asynchronous chest and abdominal wall motion, fatigue, hypoxia, and hypercarbia.[14] [15]
[16]
4 This PDF of the BMJ Best Practice topic is based on the web version that was last updated: Jun 28, 2018.
BMJ Best Practice topics are regularly updated and the most recent version
of the topics can be found on bestpractice.bmj.com . Use of this content is
subject to our disclaimer. © BMJ Publishing Group Ltd 2019. All rights reserved.
Croup Prevention
Primary prevention
No strategies for primary prevention are currently recommended. Work continues into the development of
an effective vaccine against the parainfluenza virus.[17] [18] If a vaccine were to become available, this may
lead to a significant reduction in croup caused by parainfluenza viruses.
Secondary prevention
In developing nations, vitamin A has been used as a preventive therapy for croup caused by severe
measles.[7] [8]
PREVENTION
This PDF of the BMJ Best Practice topic is based on the web version that was last updated: Jun 28, 2018.
5
Croup Diagnosis
Case history
Case history #1
A 2-year-old boy is brought to the emergency room by his parents in the middle of the night. He has had
mild symptoms of an upper respiratory infection for 48 hours, awoke with a sudden onset of seal-like
barky cough and has had inspiratory stridor when crying. The stridor disappeared at rest, but the seal-like
barky cough has persisted.
Case history #2
A 3-year-old boy is brought to the emergency room by his parents in the late evening. He has developed
a sudden onset of a seal-like barky cough, accompanied by clear nasal discharge. His parents became
alarmed when he developed stridor, which persists throughout the trip to the hospital. On examination, he
has a seal-like barky cough and inspiratory stridor when at rest, which worsens with agitation. Persistent
sternal indrawing is also evident at rest.
Step-by-step diagnostic approach

The diagnosis of croup depends upon a careful history and physical examination. The key features are the
characteristic sudden-onset, seal-like barky cough, often accompanied by stridor and chest wall (intercostal)
or sternal indrawing. Symptoms are typically worse at night and increase with agitation.
There may be a history of prior nonspecific upper respiratory tract symptoms (coryza, non-barky cough, mild
fever), although the seal-like barky cough may also present abruptly with no preceding illness. Although not
essential to the diagnosis, there is commonly a hoarse voice.
Clinical presentation
DIAGNOSIS
Presentations may range from mild symptoms to impending respiratory failure.[19] The physician should
look out for the following symptoms and signs according to severity:
• Mild: seal-like barky cough but no stridor or sternal/intercostal retractions at rest

• Moderate: seal-like barky cough with stridor and sternal retractions at rest; no agitation or lethargy
• Severe: seal-like barky cough with stridor and sternal/intercostal retractions, associated with
agitation or lethargy
• Impending respiratory failure: increasing upper airway obstruction, sternal/intercostal retractions,
asynchronous chest wall and abdominal movement, fatigue, and signs of hypoxia (pallor or
cyanosis) and hypercarbia (decreased level of consciousness secondary to rising PaCO2). The
degree of chest wall retractions may diminish with the onset of respiratory failure as the child tires.
The clinician must consider the differential diagnosis during the physical examination. In particular, if
epiglottitis is suspected, examination of the oropharynx or manipulation of the neck is contraindicated as it
may precipitate further airway obstruction.
Croup Diagnosis
Work-up
Croup is largely a clinical diagnosis. X-ray of the anteroposterior and lateral neck is not performed in
a child presenting with typical symptoms and signs of croup. The steeple sign (narrowed trachea) is a
classic finding on anteroposterior view, but is not always present. Radiologic studies are contraindicated
if there is clinical suspicion of epiglottitis or bacterial tracheitis, as manipulation of the neck region and
agitation may precipitate further airway obstruction. If the clinical picture is atypical for these conditions,
soft-tissue radiographs of the neck may provide helpful information to support an alternative diagnosis.
Any x-ray should be performed with considerable care and personnel equipped to support the airway in
the event of worsening obstruction.
Risk factors
Strong
age 6 months to 6 years
• Occurs in this age group. Typically, it affects those between 6 months and 3 years of age, peaking in
the second year of life.[1]
Weak
male gender
• Male to female ratio: 1.4:1.[1]
prior intubation
• Small observational study indicates a weak link between a history of previous intubation and croup.[13]
History & examination factors
DIAGNOSIS
Key diagnostic factors
symptoms increasing with agitation (common)
• Seen in all levels of severity.
distinctive seal-like bark y cough (common)

• Key feature, required to make a diagnosis of croup.
age 6 months to 6 years (common)

• Occurs in this age group. Typically, it affects children between 6 months and 3 years of age, peaking in
the second year of life.[1]
• Croup can be seen in infants as young as 3 months of age, and may also occur, although rarely, in
older children, adolescents, and adults.
Other diagnostic factors

male gender (common)
• Male to female ratio: 1.4:1.[1]
7
Croup Diagnosis
peak season late autumn (North America) (common)
• Cases peak in late autumn in North America (September to December), which correlates with the peak
prevalence of parainfluenza virus in the community.[3]
prodromal symptoms (common)

• Nonspecific upper respiratory tract symptoms (coryza, nonbarky cough, mild fever) for 12 to 48 hours
may be present. Not a key feature in all cases; seal-like barky cough may present abruptly with no
preceding illness.
abrupt onset of symptoms (common)

• Typical, but not essential to the diagnosis.
symptoms worse at night (common)

• Typical, but not essential to the diagnosis.
hoarse voice (common)

• Not essential to the diagnosis, but commonly seen.
respiratory distress (sternal/intercostal indrawing, stridor) (uncommon)

• In moderate/severe croup.
persistent agitation (uncommon)

• In severe croup.
lethargy (uncommon)
• In severe croup (more likely in impending respiratory failure).
asynchronous chest wall and abdominal movement (uncommon)

• Impending respiratory failure.
DIAGNOSIS
fatigue (uncommon)
signs of hypoxia (pallor or cyanosis) (uncommon)

signs of hypercarbia (decreased level of consciousness secondary to rising

PaCO2) (uncommon)
Croup Diagnosis
Diagnostic tests
1st test to order
Test Result
clinical exam typical features on
clinical exam
• Croup is largely a clinical diagnosis.
Other tests to consider
Test Result
x-ray anteroposterior and lateral neck steeple sign in
anteroposterior view or
• Croup is largely a clinical diagnosis. Therefore, x-ray should not be
normal
performed in a child presenting with typical symptoms and signs of
croup. The steeple sign (narrowed trachea) is a classic finding on
anteroposterior view, but is not always present.
• Radiologic studies are contraindicated if there is clinical suspicion of
epiglottitis or bacterial tracheitis, as manipulation of the neck region
and agitation may precipitate further airway obstruction. If the clinical
picture is atypical for these conditions, soft-tissue radiographs of
the neck may provide helpful information to support an alternative
diagnosis. Any x-ray should be performed with considerable care and
personnel equipped to support the airway in the event of worsening
obstruction.
DIAGNOSIS
9
Croup Diagnosis
Differential diagnosis
Condition Differentiating signs / Differentiating tests

symptoms
Bacterial tracheitis • May or may not have • Radiologic studies are
antecedent symptoms contraindicated if there is
consistent with croup; clinical suspicion of bacterial
sudden deterioration tracheitis, as manipulation of
following 2 to 7 days of a the neck region and agitation
mild to moderate croup or may precipitate further
other mild viral illness;[14] airway obstruction.
fever, toxic appearance (child • Bronchoscopy, performed at
appears systemically ill and the time of intubation, shows
does not interact normally erythematous tracheal
with his/her surroundings) mucosa, with thick, purulent
may be present; painful tracheal secretions.[24]
cough; poor response to • The most frequently
treatment with nebulized isolated pathogens
epinephrine.[20] [21] [22] from tracheal secretions
[23] include Staphylococcus
aureus , group A
streptococcus, Moraxella
catarrhalis , Streptococcus
pneumoniae , Haemophilus
influenzae , and anaerobic
organisms.[14] [21] [22] [25]
[26] [27]
Epiglot titis • Rarely seen since • Radiologic studies are

widespread immunization contraindicated if there
against Haemophilus is clinical suspicion of
influenzae B;[28] [29] epiglottitis, as manipulation
[30] sudden onset of high of the neck region and
fever, dysphagia, drooling, agitation may precipitate
DIAGNOSIS
and anxiety; preferred further airway obstruction.

posture: sitting upright with • Visualization of the
head extended; nonbarky airway (prior to controlled
cough.[14] endotracheal intubation)
confirms the diagnosis
showing an edematous,
erythematous epiglottis,
often obstructing the view of
the vocal cords.
Foreign body in the upper • Sudden onset of dyspnea • Many foreign bodies are not
airway and stridor; usually a clear radiopaque, thus x-rays may
history of foreign body not confirm the diagnosis.
inhalation or ingestion;[14] • Direct visualization and
no prodrome or symptoms of removal of foreign body in
viral illness; no fever (unless the operating room confirms
secondary infection).[31] the diagnosis.
Croup Diagnosis
Condition Differentiating signs / Differentiating tests

symptoms
Retropharyngeal abscess • Dysphagia, drooling, • Lateral neck radiograph may
occasionally stridor, demonstrate retroflexion
dyspnea, tachypnea, neck of cervical vertebrae and
stiffness, unilateral cervical posterior pharyngeal
adenopathy; onset is edema.[32]
typically more gradual, often
accompanied by fever.[31]
Peritonsillar abscess • Dysphagia, drooling, • No differentiating tests.

occasionally stridor,
dyspnea, tachypnea, neck
stiffness, unilateral cervical
adenopathy; onset is
typically more gradual, often
accompanied by fever.[31]
Angioneurotic edema • May present at any age; • No differentiating tests.

acute swelling of the upper
airway may cause dyspnea
and stridor; fever uncommon.
Swelling of face, tongue, or
pharynx may be present.
Allergic reaction • May present at any age; • Allergy testing (skin prick
rapid onset of dysphagia, or RAST) may determine
stridor, and possible underlying allergen.
cutaneous manifestations
(urticarial rash); often
personal or family history of
prior episodes or allergy.
Laryngeal diphtheria • Extremely rare clinical • No differentiating tests.

emergency. May present
DIAGNOSIS
at any age; history of
inadequate immunization;
prodrome with symptoms
of pharyngitis for 2
to 3 days; low-grade
fever, voice hoarseness,
potentially barky cough;
dysphagia, inspiratory
stridor; characteristic
membranous pharyngitis on
examination.[31]
Congenital or acquired • Extremely rare. Usually • Upper airway endoscopy

tracheal or laryngeal presents at <3 months of or bronchoscopy will allow
abnormalities age. direct visualization of the
• Abnormally prolonged underlying abnormality.
or recurrent stridor. Poor However, these tests should
response to croup treatment. be delayed until after the
acute illness.
11
Croup Diagnosis
Diagnostic criteria
Clinical classification of severity[19]
• Mild: seal-like barky cough but no stridor or sternal/intercostal retractions at rest
• Moderate: seal-like barky cough with stridor and sternal retractions at rest; no agitation or lethargy
• Severe: seal-like barky cough with stridor and sternal/intercostal retractions associated with agitation
or lethargy
• Impending respiratory failure: increasing upper airway obstruction, sternal/intercostal retractions,
asynchronous chest wall and abdominal movement, fatigue, and signs of hypoxia (pallor or cyanosis)
and hypercarbia (decreased level of consciousness secondary to rising PaCO2). The degree of chest
wall retractions may diminish with the onset of respiratory failure as the child tires.
Westley croup score: research classification

Total score ranging from 0 to 17 points. Five component items make up the score [33]:
• Stridor (0 = none, 1 = with agitation only, 2 = at rest)

• Retractions (0 = none, 1 = mild, 2 = moderate, 3 = severe)
• Cyanosis (0 = none, 4 = cyanosis with agitation, 5 = cyanosis at rest)
• Level of consciousness (0 = normal - including asleep, 5 = disoriented)
• Air entry (0 = normal, 1 = decreased, 2 = markedly decreased).
The Westley croup score has been used in numerous clinical research studies to classify croup into mild,
moderate, and severe categories. However, substantial interobserver variability exists when the score is used
in clinical practice, thus limiting its use in the clinical setting.[34]
DIAGNOSIS
Croup Treatment
Step-by-step treatment approach

In mild and moderate croup, the main goals of treatment are symptomatic relief; this is achieved with
supportive care and oral or nebulized corticosteroids. In moderate croup these should be combined with
nebulized epinephrine. Children may be safely discharged home after 2 to 4 hours of observation following
epinephrine administration.[35] [36] [37] [38] [39] [40] [41]
In severe croup, the main treatment aim is to prevent further airway compromise. In addition to the
combination treatment of nebulized or parenteral corticosteroids plus nebulized epinephrine, oxygen is
given to children demonstrating marked respiratory distress.[19] [42] [43] [44] [45] Intubation is indicated for
impending respiratory failure.[46] [47] [48] [49]
General care
Care should be taken to avoid frightening the child, as agitation may cause worsening of symptoms.[19]
To ensure comfort, the child should be seated comfortably in the caregiver's lap during assessment and
treatment. Although there is little research regarding the use of oxygen in croup, the clinical rationale is
clear in a child with significant respiratory distress. The mechanism by which patients with severe croup
become hypoxic is secondary to relative hypoventilation. Therefore, close monitoring and reassessment
should occur continuously. Humidified oxygen may be administered via a plastic hose with the opening
held within a few centimeters of the nose or mouth to minimize the chance of causing agitation.[19] [42]
[43] [44] [45]
Especially in mild croup, parental assurance and education to the self-limited nature of the illness is
important.
Corticosteroids
Corticosteroids are the mainstay of medical treatment in mild,1[A]Evidence moderate,2[B]Evidence and
severe3[A]Evidence croup.[36] [51] [52] [53] [54] [55] [50] A systematic review of children with moderate
to severe croup treated with corticosteroids showed a 10% reduction in absolute proportion requiring
nebulized epinephrine, an average 12-hour reduction in length of stay in hospital or emergency room, and
a 50% reduction in both number of admissions for treatment or return visits.[56]
The usual administration is a single oral dose of dexamethasone, with treatment effect evident within 2
hours, and further beneficial effects noted up to 10 hours following initial dose.[36] Traditionally, a dose of
0.6 mg/kg/dose was used for croup; however, evidence now supports the use of a smaller dose of 0.15
mg/kg/dose.4[B]Evidence Adding inhaled budesonide does not appear to provide additional benefit.[61]
5[B]Evidence There is inadequate evidence comparing single versus multiple doses of corticosteroids.
With most croup symptoms showing resolution within 3 days of the onset, and the anti-inflammatory effect
of dexamethasone thought to last between 2 to 4 days, a second dose is unlikely to be beneficial in the
majority of children with croup.[62]
Both oral and intramuscular routes of administration have been shown to be equivalent or superior
to inhaled corticosteroids in moderate to severe croup.[36] [53] [63] [64] [65] Alternative routes of
TREATMENT
administration will be necessary in children who do not tolerate or absorb oral medication (e.g., children
with persistent vomiting or severe respiratory distress). Inhaled budesonide may be preferable in children
with severe hypoxia, in whom reduced gut and tissue perfusion can impair oral and intramuscular
absorption. Establishing IV access can increase distress and potentially precipitate respiratory failure.
Extreme care should be taken when considering IV administration.
13
Croup Treatment
To date, no adverse effects have been attributed to the use of corticosteroids in children with croup.
Theoretical concerns include a possible increased risk of complications of varicella (bacterial
superinfection, disseminated varicella) in a child with recent exposure. 6[C]Evidence
Adding nebulized epinephrine

In moderate and severe croup, nebulized epinephrine should be administered with dexamethasone
as it provides temporary relief of symptoms of airway obstruction. [68] [69] 7[B]Evidence A clear
reduction in stridor and sternal/intercostal retractions should be evident within 10 to 30 minutes following
administration.[36] The clinical effects of nebulized epinephrine last on average at least 1 hour, but
usually subside 2 hours after administration. On average, symptoms return to their baseline, without
evidence of a rebound effect.[72] [71] [33] [73] [74] [75] [76]
Although racemic epinephrine has traditionally been used to treat children with croup, L-epinephrine is as
effective in moderate to severe croup.[77] In North America, L-epinephrine availability may be limited. The
same dose of nebulized epinephrine is used regardless of weight, as the effective dose of drug delivered
to the airway is regulated by individual tidal volume.[78] [79] [80] [81] No adverse effects have been noted
when given one dose at a time. [77] [70] [71] [72] [57] [82] [83] Caution should be used with multiple
doses of nebulized epinephrine.8[C]Evidence There have been no reports of complications associated
with the use of L-epinephrine in children with known cardiac conditions. However, careful observation is
advisable if epinephrine treatment is deemed necessary.
In children who do not respond to combination treatment within a few hours following administration, a
refocused assessment should take place to rule out alternate diagnoses.
Impending respiratory failure

In children progressing to asynchronous chest wall and abdominal movement, fatigue, and signs of
hypoxia (pallor or cyanosis) and hypercarbia (decreased level of consciousness secondary to rising
PaCO2), endotracheal intubation may be necessary to secure the airway.
Treatments with no added benefit

Historically mist or humidified air have been widely employed, but there is now convincing evidence that
these are ineffective[19] [85] [86] [87] [88] [89] [90] 9[B]Evidence and even harmful in some instances.
For example, hot humidified air carries an increased risk of scald injuries[91] and mist tents promote mold
growth if improperly cleaned.[90] Additionally, being enclosed in a cold, wet space separated from the
caregiver may increase a child's agitation.
Antibiotics, beta-2 agonists and decongestants have not been studied and their use should be
discouraged.[19] [42] [43] [44] [45]
Heliox (a defined mixture of helium and oxygen) has been studied as an adjunctive therapy in severe
airway obstruction.[82] [92]Helium is an inert gas that has no recognized pharmaceutical properties.
Heliox usually contains 70% helium, limiting the fractional concentration of oxygen to maximal 30%.
Compared with nitrogen, the major gas found in room air, the lower-density helium gas decreases the
TREATMENT
turbulence of airflow over the narrowed airways, which theoretically should result in decreased work of
breathing. However, heliox has not yet been shown to confer improvements over standard therapies,[93]
10[B]Evidence limits the fractional concentration of inhaled oxygen which can be provided and can be
challenging to use in unskilled hands.[82] [92] [94] [95] [96] [97] [98] [99] It is not currently recommended
for use in children with severe croup.
Croup Treatment
Tracheostomy is a rare intervention reserved for cases of unsuccessful endotracheal intubation (e.g.,
in severe epiglottitis) and is not indicated in croup. Its complications include risk of bleeding, damage
to adjacent structures in the neck, air leak (pneumomediastinum or pneumothorax), obstruction of the
tracheotomy tube, infection, and tracheal injury.
Treatment details overview

Please note that formulations/routes and doses may differ between drug names and brands, drug
formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer
Acute ( summary )
mild (no stridor at rest)
1st corticosteroids + supportive care
moderate (stridor at rest; no agitation

or lethargy)
plus nebulized epinephrine
severe (stridor at rest with agitation

or lethargy)
plus supplemental ox ygen
with impending adjunct intubation

respiratory failure
TREATMENT
15
Croup Treatment
Treatment options
Please note that formulations/routes and doses may differ between drug names and brands, drug
formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer
Acute
mild (no stridor at rest)

Primary options
» dexamethasone: 0.15 to 0.6 mg/kg orally as

a single dose
» A single dose of oral dexamethasone is given

as soon as the clinical diagnosis of croup has
been made. Its effect in reducing the clinical
signs of croup is seen within 2 hours, with further
beneficial effects noted up to 10 hours following
administration.[36]
» Traditionally, a dose of 0.6 mg/kg/dose

was used for croup; however, evidence now
supports the use of a smaller dose of 0.15 mg/
kg/dose.4[B]Evidence
» Care should be taken to avoid frightening

the child, as agitation may cause worsening of
symptoms.[19] Especially in mild croup, parental
assurance and education to the self-limited
nature of the illness is important.
» Historically mist or humidified air have been

widely employed, but there is now convincing
evidence that these are ineffective[19] [85] [86]
[87] [88] [89] [90] 9[B]Evidence and even harmful
in some instances.
moderate (stridor at rest; no agitation
or lethargy)

Primary options

a single dose
OR
» budesonide inhaled: 2 mg nebulized as a

single dose
TREATMENT
OR
» dexamethasone sodium phosphate: 0.6 mg/

kg intramuscularly as a single dose
Croup Treatment
Acute
signs of croup is seen within 2 hours, with further
beneficial effect noted up to 10 hours following
administration.[36]

» Nebulized budesonide is preferable in severe

hypoxia, persistent vomiting, or respiratory
distress preventing administration of an oral
dose.
» Intramuscular dexamethasone is another

alternative.
» Care should be taken to avoid frightening

the child, as agitation may cause worsening of
symptoms.[19] Historically mist or humidified
air have been widely employed, but there
is now convincing evidence that these are
ineffective[19] [85] [86] [87] [88] [89] [90]
9[B]Evidence and even harmful in some
instances.
Treatment recommended for ALL patients in
selected patient group
Primary options
» racepinephrine (racemic epinephrine)

inhaled: (2.25% solution) 0.5 mL diluted to
2-4 mL with normal saline nebulized as a
single dose
OR
» epinephrine (adrenaline): (1:1000 solution

of L-epinephrine) 5 mL undiluted nebulized as
a single dose
» In children presenting with stridor, sternal

indrawing at rest and persistent or increasing
agitation, nebulized epinephrine should be
administered in addition to dexamethasone.
It provides temporary relief of the airway
obstruction while awaiting the effects of
TREATMENT
corticosteroid treatment.[69]
» The clinical effects of nebulized epinephrine

last on average at least 1 hour, but usually
subside 2 hours after administration.[33]
17
Croup Treatment
Acute
» The use of one dose at a time of nebulized
epinephrine has not been associated with any
clinically significant increases in BP or heart rate,
neither has it been associated with any adverse
events.[77] [70] [71] [72] [57] [82] [83] Caution
should be used with multiple doses of nebulized
epinephrine.8[C]Evidence Careful observation
is advisable if epinephrine treatment is deemed
necessary.
» Although racemic epinephrine has traditionally

been used to treat children with croup, L-
epinephrine is as effective in moderate to severe
croup.[77] In North America, L-epinephrine
availability may be limited. The same dose is
used regardless of weight, as the effective dose
of drug delivered to the airway is regulated by
individual tidal volume.[78] [79] [80] [81]
severe (stridor at rest with agitation
or lethargy)

Primary options

a single dose
OR
» budesonide inhaled: 2 mg nebulized as a

single dose
OR
» dexamethasone sodium phosphate: 0.6 mg/

kg intramuscularly as a single dose
Secondary options
» dexamethasone sodium phosphate: 0.15 to

0.6 mg/kg intravenously as a single dose

signs of croup is seen by 2 hours, with further
beneficial effect noted up to 10 hours following
administration.[36]

TREATMENT

» Nebulized budesonide is preferable in severe

hypoxia, persistent vomiting, or respiratory
Croup Treatment
Acute
distress preventing administration of an oral
dose.
» Intramuscular or IV dexamethasone is another

alternative. However, there is significant potential
to increase agitation and respiratory distress
when an IV line is inserted in a child with severe
croup.
» Wherever possible, the child should be kept

in a calm environment with his/her caregiver.
Care should be taken to minimize interventions
that would increase the child’s agitation.
Historically mist or humidified air have been
widely employed, but there is now convincing
evidence that these are ineffective[19] [85] [86]
[87] [88] [89] [90] 9[B]Evidence and even harmful
in some instances.
Primary options
» racepinephrine (racemic epinephrine)

inhaled: (2.25% solution) 0.5 mL diluted to
2-4 mL with normal saline nebulized as a
single dose
OR
» epinephrine (adrenaline): (1:1000 solution

of L-epinephrine) 5 mL undiluted nebulized as
a single dose
» In children presenting with stridor, sternal/

intercostal indrawing at rest and persistent or
increasing agitation, nebulized epinephrine
should be administered in addition to
dexamethasone. It provides temporary relief of
the airway obstruction while awaiting the effects
of corticosteroid treatment.[69]
» The clinical effects of nebulized epinephrine

last on average at least 1 hour, but usually
subside 2 hours after administration.[33]
» The use of one dose at a time of nebulized

epinephrine has not been associated with any
clinically significant increases in BP or heart rate,
neither has it been associated with any adverse
TREATMENT
events.[77] [70] [71] [72] [57] [82] [83] Caution

should be used with multiple doses of nebulized
epinephrine.8[C]Evidence Careful observation
is advisable if epinephrine treatment is deemed
necessary.
19
Croup Treatment
Acute
» Although racemic epinephrine has traditionally
been used to treat children with croup, L-
epinephrine is as effective in moderate to severe
croup.[77] In North America, L-epinephrine
availability may be limited. The same dose is
used regardless of weight, as the effective dose
of drug delivered to the airway is regulated by
individual tidal volume.[78] [79] [80] [81]
plus supplemental ox ygen
Primary options
» oxygen: 8 to 10 L/min blow-by
Secondary options
» oxygen: 100% by nonrebreather mask
» Humidified oxygen is given to children

demonstrating significant signs and symptoms
of respiratory distress, preferably as blow-by
oxygen via tubing held a few centimeters from
the child's nose and mouth.
» In the event that oxygenation is insufficient

using this method, 100% oxygen via a
nonrebreather mask is administered. However,
the application of the mask to the face carries
the potential for increasing agitation and
preparations (experienced personnel, intubation
equipment, medications) should be made to
secure the airway if the clinical situation worsens
to impending respiratory failure.
» Oxygen saturation monitoring should occur,

providing this does not increase the child's level
of agitation.
with impending adjunct intubation
respiratory failure
Treatment recommended for SOME patients in
» Indicated in children progressing to
asynchronous chest wall and abdominal
movement, fatigue, and signs of hypoxia (pallor
or cyanosis) and hypercarbia (decreased level of
consciousness secondary to rising PaCO2).
» Becoming increasingly uncommon (in only

1% to 3% of children admitted with croup) and
TREATMENT
performed as rapid sequence induction in a

controlled setting with experienced personnel
and equipment.[46] [47] [48] [49]
» Advisable to have a selection of endotracheal

tubes of smaller sizes at hand, as subglottic
Croup Treatment
Acute
edema may cause difficulty when intubating with
a standard sized endotracheal tube.
TREATMENT
21
Croup Follow up
Recommendations
Monitoring
FOLLOW UP
Children with moderate to severe croup responding well to combination therapy with corticosteroids and
nebulized epinephrine (plus oxygen) may be safely discharged home after 2 to 4 hours of observation
following epinephrine administration.
Children admitted to the hospital with significant respiratory distress despite therapy require continuous
monitoring and observation of respiratory status and vital signs.
In children who have undergone intubation, there is no need for subsequent follow-up after extubation,
once the respiratory distress and symptoms of upper-airway obstruction have resolved.
In the rare case of a child with persistent symptoms of upper-airway obstruction, re-evaluation should
occur to assess for pre-existing upper-airway anatomical abnormalities.
Patient instructions
Parents should be made aware of the symptoms and signs of croup:
• Hoarse voice
• Seal-like barking cough
• Stridor (a high-pitched crowing sound heard as child breathes in)
• Fever (although not all children will have a fever).
Most children with mild croup can be observed at home.
Parents should be advised to go to the hospital if:
• The crowing sound (stridor) can be heard continually

• The skin between the ribs is pulling in with every breath
• The child is restless or agitated.
Parents should be instructed to call an ambulance if:
• The child's face is very pale, blue, or gray (includes blue lips) for more than a few seconds
• The child is unusually sleepy or is not responding
• The child is having a lot of trouble breathing (e.g., the belly is sinking in while breathing, or the skin
between the ribs or over the windpipe is pulling in with each breath; the nostrils may also be flaring
in and out)
• The child is upset (agitated or restless) while struggling to breathe and cannot be calmed down
quickly
• The child wants to sit instead of lie down
• The child cannot talk, is drooling, or having trouble swallowing.
Croup Follow up
Complications
Complications Timeframe Likelihood
FOLLOW UP
bacterial tracheitis short term low
Postulated mechanism is bacterial superinfection related to previously unknown immune dysfunction,

requiring treatment with broad-spectrum IV antibiotics and, in severe cases, endotracheal intubation.[103]
pneumonia short term low
Postulated mechanism is bacterial superinfection, requiring treatment with broad-spectrum antibiotics.
Prognosis
Although most children with the condition suffer a mild and self-limited illness of short duration, the stress
and disruption experienced by the child and family are well documented.[102]
Mild
Self-limited without treatment but shorter time to resolution with dexamethasone treatment.
Moderate
Reasonable outlook. While symptoms of obstruction may be frightening, symptoms resolve without significant
complications.
Severe
Before corticosteroids became standard treatment, children with severe croup were 5 times more likely
to receive endotracheal intubation,[50] and remained intubated for 30% longer.[51] Introduction of routine
corticosteroid treatment has dramatically decreased numbers of children intubated,3[A]Evidence reduced
number of days spent in ICU and shortened length of hospital stay.[54] Since combination treatment with
dexamethasone and nebulized epinephrine became standard care, prognosis for severe croup has been
excellent.
Impending respiratory failure

Very rare, with intubation required in only 1% to 3% of all cases.[46] [47] [48] [49]
23
Croup Guidelines
Diagnostic guidelines
International
Acute management of croup in the emergency department

Published by: Canadian Paediatric Society Last published: 2017
Guideline for the diagnosis and management of croup [19]

Published by: Alberta Medical Association Last published: 2015
Treatment guidelines
International
GUIDELINES
Acute management of croup in the emergency department

Published by: Canadian Paediatric Society Last published: 2017
Children’s Mercy Hospitals and Clinics evidence based practice clinical

practice guide: croup [100]
Published by: Children's Mercy Hospital's Office of Evidence Based Last published: 2016
Practice
Guideline for the diagnosis and management of croup [19]

Published by: Alberta Medical Association Last published: 2015
Clinical practice guidelines: croup (laryngotracheobronchitis) [101]

Published by: Royal Children's Hospital Melbourne Last published: 2011
Croup Evidence scores
Evidence scores
1. Need for additional medical attention and symptom severity: there is good-quality evidence that
compared with placebo, a single dose of oral dexamethasone reduces the need for additional medical
attention in children with mild croup. There is medium-quality evidence that it reduces symptom
severity, compared with placebo, in the same group.
Evidence level A: Systematic reviews (SRs) or randomized controlled trials (RCTs) of >200
participants.
2. Symptom severity: there is medium-quality evidence that compared with placebo, oral or intramuscular
dexamethasone reduces the severity of symptoms after 12 to 24 hours in children with moderate to
severe croup.
Evidence level B: Randomized controlled trials (RCTs) of <200 participants, methodologically
flawed RCTs of >200 participants, methodologically flawed systematic reviews (SRs) or good quality
observational (cohort) studies.
3. Reduction in rate and duration of endotracheal intubation: there is good-quality evidence that
corticosteroid treatment has a positive impact on the need for endotracheal intubation. A meta-
analysis of 10 RCTs involving 1286 children with severe croup and impending respiratory failure
demonstrated that patients who received corticosteroid treatment experienced a 5-fold reduction in
the rate of endotracheal intubation.[50] In another study, 70 children with severe croup who underwent
endotracheal intubation and corticosteroid treatment were associated with a reduction in length
of intubation by one third, and a 7-fold lower risk for reintubation compared with those receiving
placebo.[51]
Evidence level A: Systematic reviews (SRs) or randomized controlled trials (RCTs) of >200
participants.
4. Response to treatment: there is medium-quality evidence that higher doses of corticosteroids may be
no more effective than lower doses at inducing treatment response. A meta-analysis of hospitalized
children showed that a higher dose of hydrocortisone equivalents was associated with a higher
proportion of children responding to treatment compared with placebo (there were methodologic
issues with the analysis).[50] Four small RCTs comparing different doses of oral dexamethasone
consistently demonstrated no significant differences between groups treated with doses ranging
between 0.15 to 0.6 mg/kg.[57] [58] [59] [60]
EVIDENCE SCORES
5. Symptom severity and admission to the hospital: there is medium-quality evidence that compared
with nebulized budesonide alone and oral dexamethasone alone, oral dexamethasone plus nebulized
budesonide is no more effective at reducing symptom severity after 4 hours or reducing hospital
admission rates after 1 week in children with moderate to severe croup.
25
6. Risk of varicella complications: there is poor-quality evidence from 2 case-control studies addressing
this risk that presented conflicting results. One study noted an increased risk of complicated varicella
in immunocompetent children treated with corticosteroids,[66] while the second study did not show the
same finding.[67]
Evidence level C: Poor quality observational (cohort) studies or methodologically flawed randomized
controlled trials (RCTs) of <200 participants.
7. Symptom severity: there is medium-quality evidence that compared with placebo or no treatment,
nebulized epinephrine reduces symptom severity after 10 to 30 minutes in children with moderate to
severe croup, though the benefit seems to be short term only.[70] [71] [33]
8. Risk of adverse effects associated with multiple doses of nebulized epinephrine: there is poor-quality
evidence concerning the increased risk of adverse effects with multiple dose of nebulized epinephrine
from a case report documenting the onset of ventricular tachycardia and myocardial infarction in a
previously healthy child with severe croup following 3 doses of nebulized epinephrine within a 1-hour
period.[84]
Evidence level C: Poor quality observational (cohort) studies or methodologically flawed randomized
controlled trials (RCTs) of <200 participants.
9. Symptom improvement: there is medium-quality evidence that humidified air is no more effective at
improving symptoms in children with moderate to severe croup when compared with nonhumidified or
low-humidity air.
10. Symptom improvement: there is medium-quality evidence from a small RCT comparing heliox
versus racemic epinephrine in 29 children with moderate to severe croup (concurrently treated with
intramuscular dexamethasone and oxygen) that there are similar rates of improvement in clinical croup
score, oxygen saturation, heart rates, and respiratory rates with each treatment.[82] A second small
RCT in 15 children with mild croup did not show a significant difference in croup score improvement
EVIDENCE SCORES
between treatment groups.[92]
EVIDENCE SCORES
27
Croup References
Key articles
• Johnson D, Klassen T, Kellner J. Diagnosis and management of croup: Alberta Medical
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100. Children's Mercy Hospital's Office of Evidence Based Practice. Children’s Mercy Hospitals and Clinics
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101. Royal Children's Hospital Melbourne. Clinical practice guidelines: croup (laryngotracheobronchitis).
http://www.rch.org.au (last accessed 22 October 2016). Full text
102. Johnson D, Williamson J. Croup: duration of symptoms and impact on family functioning. Pediatr Res.
2001;49:83A.
103. Johnson DW, Schuh S, Koren G, et al. Outpatient treatment of croup with nebulized dexamethasone.
Arch Pediatr Adolesc Med. 1996;150:349-355. Abstract
Croup Disclaimer
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Contributors:
// Authors:
Candice Bjornson, MSc, MD, FRCPC

Associate Professor
University of Calgary, Calgary, Canada
DISCLOSURES: CB declares that she has no competing interests. CB is the author of several references in
this monograph.
David Johnson, MD
Professor
Department of Pediatrics and Physiology and Pharmacology, University of Calgary, Calgary, Canada
DISCLOSURES: DJ declares that he has no competing interests. DJ is the author of several references in
this monograph.
// Peer Reviewers:
Jeffrey Chapman, MD
Staff
Department of Pulmonary and Critical Care Medicine, Cleveland Clinic, Cleveland, OH
DISCLOSURES: JC declares that he has no competing interests.
Ken Farion, MD
Assistant Professor
Pediatrics and Emergency Medicine, University of Ottawa, Ottawa, Canada
DISCLOSURES: KF declares that he has no competing interests.
Doreen Matsui, MD, FRCPC

Associate Professor
Departments of Paediatrics and Medicine, Children's Hospital of Western Ontario, London, Ontario, Canada
DISCLOSURES: DM declares that she has no competing interests.
Jeremy Hull, MBBS

Consultant Paediatrician
Children's Hospital and West Wing, John Radcliffe Hospital, Oxford, UK
DISCLOSURES: JH declares that he has no competing interests.
Steve Cunningham, MBBS, PhD

Consultant Respiratory Paediatrician
Department of Respiratory & Sleep Medicine, Royal Hospital for Sick Children, Edinburgh, UK
DISCLOSURES: SC declares that he has no competing interests.

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Croup

The right clinical information, right where it's needed

Last updated: Jun 28, 2018

◊ Common cause of acute respiratory distress in children.

Step-by-step diagnostic approach

• Mild: seal-like barky cough but no stridor or sternal/intercostal retractions at rest

History & examination factors

distinctive seal-like bark y cough (common)

age 6 months to 6 years (common)

Other diagnostic factors

prodromal symptoms (common)

abrupt onset of symptoms (common)

symptoms worse at night (common)

hoarse voice (common)

respiratory distress (sternal/intercostal indrawing, stridor) (uncommon)

persistent agitation (uncommon)

asynchronous chest wall and abdominal movement (uncommon)

signs of hypoxia (pallor or cyanosis) (uncommon)

signs of hypercarbia (decreased level of consciousness secondary to rising

Other tests to consider

Condition Differentiating signs / Differentiating tests

Epiglot titis • Rarely seen since • Radiologic studies are

and anxiety; preferred further airway obstruction.

Condition Differentiating signs / Differentiating tests

Peritonsillar abscess • Dysphagia, drooling, • No differentiating tests.

Angioneurotic edema • May present at any age; • No differentiating tests.

Laryngeal diphtheria • Extremely rare clinical • No differentiating tests.

Congenital or acquired • Extremely rare. Usually • Upper airway endoscopy

Westley croup score: research classification

• Stridor (0 = none, 1 = with agitation only, 2 = at rest)

Step-by-step treatment approach

Adding nebulized epinephrine

Impending respiratory failure

Treatments with no added benefit

Treatment details overview

1st corticosteroids + supportive care

moderate (stridor at rest; no agitation

1st corticosteroids + supportive care

plus nebulized epinephrine

severe (stridor at rest with agitation

1st corticosteroids + supportive care

plus nebulized epinephrine

plus supplemental ox ygen

with impending adjunct intubation

1st corticosteroids + supportive care

» dexamethasone: 0.15 to 0.6 mg/kg orally as

» A single dose of oral dexamethasone is given

» Traditionally, a dose of 0.6 mg/kg/dose

» Care should be taken to avoid frightening

» Historically mist or humidified air have been

1st corticosteroids + supportive care

» dexamethasone: 0.15 to 0.6 mg/kg orally as

» budesonide inhaled: 2 mg nebulized as a

» dexamethasone sodium phosphate: 0.6 mg/

» Traditionally, a dose of 0.6 mg/kg/dose

» Nebulized budesonide is preferable in severe

» Intramuscular dexamethasone is another

» Care should be taken to avoid frightening

» racepinephrine (racemic epinephrine)

» epinephrine (adrenaline): (1:1000 solution

» In children presenting with stridor, sternal

» The clinical effects of nebulized epinephrine

» Although racemic epinephrine has traditionally

1st corticosteroids + supportive care