Croup: Straight To The Point of Care
Croup: Straight To The Point of Care
Croup: Straight To The Point of Care
Theory 4
Epidemiology 4
Aetiology 4
Pathophysiology 4
Case history 4
Diagnosis 6
Approach 6
History and exam 6
Risk factors 8
Investigations 8
Differentials 9
Criteria 10
Management 12
Approach 12
Treatment algorithm overview 14
Treatment algorithm 15
Primary prevention 21
Secondary prevention 21
Patient discussions 21
Follow up 22
Monitoring 22
Complications 22
Prognosis 22
Guidelines 24
Diagnostic guidelines 24
Treatment guidelines 24
References 25
Disclaimer 34
Croup Overview
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/
OVERVIEW
intercostal indrawing.
Careful history and physical examination sufficient for confirming clinical diagnosis and ruling out potentially
serious differentials.
Orally administered corticosteroids are the mainstay for all levels of severity, combined with nebulised
epinephrine (adrenaline) in moderate to severe croup to provide temporary relief of the symptoms of upper-
airway obstruction.
Definition
Croup, also known as laryngotracheobronchitis, is a common respiratory disease of childhood, characterised
by the sudden onset of a seal-like barky cough, often accompanied by stridor, voice hoarseness, and
respiratory distress. The symptoms are a result of upper-airway obstruction due to generalised inflammation
of the airways, as a result of viral infection (typically parainfluenza virus types 1 or 3).
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Croup Theory
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
THEORY
younger than 6 months, in adolescents, and, more rarely, in adults.[1] [2] An observational study in a US
paediatric 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. 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]
Aetiology
The illness is due to viral infection (typically parainfluenza virus types 1 or 3).[3] Several other viral
pathogens have been recognised, 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 to be 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 immunised 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 generalised inflammation and oedema 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 hypercapnia.[14] [15]
[16]
Case history
Case history #1
A 2-year-old boy is brought to the emergency department 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.
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Croup Theory
Case history #2
A 3-year-old boy is brought to the emergency department 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
THEORY
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.
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Croup Diagnosis
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 non-specific 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
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:
Work-up
Croup is largely a clinical diagnosis.[20] 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
DIAGNOSIS
classic finding on anteroposterior view, but is not always present. Radiological 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.
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Croup Diagnosis
• 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.[1]
DIAGNOSIS
persistent agitation (uncommon)
• In severe croup.
lethargy (uncommon)
• In severe croup (more likely in impending respiratory failure).
fatigue (uncommon)
• Impending respiratory failure.
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Croup Diagnosis
• Impending respiratory failure.
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]
• 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.[1]
Weak
male sex
• 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]
Investigations
1st test to order
Test Result
clinical exam typical features on
clinical exam
• Croup is largely a clinical diagnosis.
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.
• Radiological 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.
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Croup Diagnosis
Differentials
DIAGNOSIS
and anxiety; preferred further airway obstruction.
posture: sitting upright with • Visualisation of the
head extended; non-barky airway (prior to controlled
cough.[14] endotracheal intubation)
confirms the diagnosis
showing an oedematous,
erythematous epiglottis,
often obstructing the view of
the vocal cords.
Foreign body in the upper • Sudden onset of dyspnoea • Many foreign bodies are
airway and stridor; usually a clear not radio-opaque, thus x-
history of foreign body rays may not confirm the
inhalation or ingestion;[14] diagnosis.
no prodrome or symptoms of • Direct visualisation and
viral illness; no fever (unless removal of foreign body in
secondary infection).[32] the operating room confirms
the diagnosis.
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Croup Diagnosis
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.
to 3 days; low-grade
fever, voice hoarseness,
potentially barky cough;
dysphagia, inspiratory
stridor; characteristic
membranous pharyngitis on
examination.[32]
Criteria
Clinical classification of severity[19]
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Croup Diagnosis
DIAGNOSIS
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Croup Management
Approach
In mild and moderate croup, the main goals of treatment are symptomatic relief; this is achieved with
supportive care and oral or nebulised corticosteroids. In moderate croup these should be combined
with nebulised epinephrine (adrenaline). Children may be safely discharged home after 2 to 4 hours of
observation following epinephrine administration.[37] [38] [39] [40] [41] [42] [43]
In severe croup, the main treatment aim is to prevent further airway compromise. In addition to the
combination treatment of nebulised or parenteral corticosteroids plus nebulised epinephrine, oxygen is
given to children demonstrating marked respiratory distress.[19] [44] [45] [46] [47] Intubation is indicated for
impending respiratory failure.[48] [49] [50] [51]
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 carer'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 re-assessment
should occur continuously. Humidified oxygen may be administered via a plastic hose with the opening
held within a few centimetres of the nose or mouth to minimise the chance of causing agitation.[19] [44]
[45] [46] [47]
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, moderate, and severe croup.[38] [53]
[54] [55] [56] [57] [52] [58] In a systematic review, corticosteroids were found to improve symptoms of
moderate to severe croup within 2 hours, with the effect lasting for at least 24 hours.[59] Corticosteroid
were associated with an average 15-hour reduction in length of stay in hospital or emergency department,
and a 50% reduction in number of admissions for treatment and return visits.[59] However, most studies
were at high or unclear risk of bias.[59]
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.[38] 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. Adding inhaled budesonide does not appear to provide additional benefit.[64]
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.[65]
Both oral and intramuscular routes of administration have been shown to be equivalent or superior
MANAGEMENT
to inhaled corticosteroids in moderate to severe croup.[38] [55] [66] [67] [68] Alternative routes of
administration will be necessary in children who do not tolerate or absorb oral medicine (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
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Croup Management
absorption. Establishing intravenous access can increase distress and potentially precipitate respiratory
failure. Extreme care should be taken when considering intravenous administration.
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.
Although racemic epinephrine has traditionally been used to treat children with croup, L-epinephrine is
as effective in moderate to severe croup.[79] In some countries, L-epinephrine availability may be limited.
The same dose of nebulised epinephrine is used regardless of weight, as the effective dose of drug
delivered to the airway is regulated by individual tidal volume.[80] [81] [82] [83] No adverse effects have
been noted when given one dose at a time.[79] [73] [74] [84] [60] [85] [86] Caution should be used with
multiple doses of nebulised epinephrine. 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.
Antibiotics, beta-2 agonists, and decongestants have not been studied and their use should be
discouraged.[19] [44] [45] [46] [47]
Heliox (a defined mixture of helium and oxygen) has been studied as an adjunctive therapy in severe
airway obstruction.[85] [95] Helium is an inert gas that has no recognised pharmaceutical properties.
MANAGEMENT
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
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,[96]
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Croup Management
limits the fractional concentration of inhaled oxygen that can be provided and can be challenging to use in
unskilled hands.[85] [95] [97] [98] [99] [100] [101] [102] It is not currently recommended for use in children
with severe croup.
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.
Acute ( summary )
mild (no stridor at rest)
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Croup Management
Treatment algorithm
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
MANAGEMENT
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Croup Management
Acute
mild (no stridor at rest)
OR
OR
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Croup Management
Acute
» 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.
OR
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Croup Management
Acute
» Although racemic adrenaline (epinephrine)
has traditionally been used to treat children
with croup, L-adrenaline (epinephrine) is as
effective in moderate to severe croup.[79] In
some countries, L-adrenaline (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.[80] [81] [82] [83]
severe (stridor at rest with agitation
or lethargy)
OR
OR
Secondary options
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Croup Management
Acute
» Wherever possible, the child should be kept
in a calm environment with his/her caregiver.
Care should be taken to minimise 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] [88] [89]
[90] [91] [92] [93] and even harmful in some
instances.
plus nebulised adrenaline (epinephrine)
Treatment recommended for ALL patients in
selected patient group
Primary options
OR
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Croup Management
Acute
plus supplemental ox ygen
Treatment recommended for ALL patients in
selected patient group
Primary options
Secondary options
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Croup Management
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]
Patient discussions
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).
• The child's face is very pale, blue, or grey (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.
MANAGEMENT
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Croup Follow up
Monitoring
Monitoring
FOLLOW UP
Children with moderate to severe croup responding well to combination therapy with corticosteroids and
nebulised epinephrine (adrenaline) (plus oxygen) may be safely discharged home after 2 to 4 hours of
observation following epinephrine administration.
Children admitted to 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.
Complications
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.[103]
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.
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Croup Follow up
Severe
Before corticosteroids became standard treatment, children with severe croup were 5 times more likely
to receive endotracheal intubation,[52] and remained intubated for 30% longer.[53] Introduction of routine
FOLLOW UP
corticosteroid treatment has dramatically decreased numbers of children intubated, reduced number of days
spent in ICU, and shortened length of hospital stay.[56] Since combination treatment with dexamethasone
and nebulised epinephrine (adrenaline) became standard care, prognosis for severe croup has been
excellent.
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Croup Guidelines
Diagnostic guidelines
International
Management of the child with cough or difficult breathing: a guide for low-
income countries (ht tps://www.theunion.org/what-we-do/publications/
technical)
Published by: International Union Against TB and Lung Disease Last published: 2005
North America
Treatment guidelines
International
North America
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Croup References
Key articles
• Johnson D, Klassen T, Kellner J. Diagnosis and management of croup: Alberta Medical Association
REFERENCES
clinical practice guidelines. Alberta: Alberta Medical Association; 2015 [internet publication]. Full text
(https://www.topalbertadoctors.org/cpgs.php?sid=12&cpg_cats=35)
References
1. Denny FW, Murphy TF, Clyde WA Jr, et al. Croup: an 11-year study in a pediatric practice.
Pediatrics. 1983 Jun;71(6):871-6. Abstract (http://www.ncbi.nlm.nih.gov/pubmed/6304611?
tool=bestpractice.bmj.com)
2. Tong MC, Chu MC, Leighton SE, et al. Adult croup. Chest. 1996 Jun;109(6):1659-62. Full text
(https://journal.publications.chestnet.org/data/Journals/CHEST/21733/1659.pdf) Abstract (http://
www.ncbi.nlm.nih.gov/pubmed/8769531?tool=bestpractice.bmj.com)
3. Marx A, Torok TJ, Holman RC, et al. Pediatric hospitalizations for croup (laryngotracheobronchitis):
biennial increases associated with human parainfluenza virus 1 epidemics. J Infect
Dis. 1997 Dec;176(6):1423-7 Abstract (http://www.ncbi.nlm.nih.gov/pubmed/9395350?
tool=bestpractice.bmj.com)
4. Chapman RS, Henderson FW, Clyde WA Jr, et al. The epidemiology of tracheobronchitis in
pediatric practice. Am J Epidemiol. 1981 Dec;114(6):786-97. Abstract (http://www.ncbi.nlm.nih.gov/
pubmed/6797294?tool=bestpractice.bmj.com)
5. Williams JV, Harris PA, Tollefson SJ, et al. Human metapneumovirus and lower respiratory tract
disease in otherwise healthy infants and children. N Engl J Med. 2004 Jan 29;350(5):443-50. Abstract
(http://www.ncbi.nlm.nih.gov/pubmed/14749452?tool=bestpractice.bmj.com)
6. Van der Hoek L, Sure K, Ihorst G, et al. Human coronavirus NL63 infection is associated with croup.
Adv Exp Med Biol. 2006;581:485-91. Abstract (http://www.ncbi.nlm.nih.gov/pubmed/17037582?
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Contributors:
// Authors:
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 topic.
// 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.