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BEST PRACTICE

Fifteen-minute consultation:
structured approach to management
of a child with recurrent croup
Vineeta Joshi,1 Vikas Malik,2 Omar Mirza,2 B Nirmal Kumar2

Department of Paediatrics,
Royal Albert Edward Infirmary,
Wrightington, Wigan & Leigh
NHS Foundation Trust, Wigan,
Lancashire, UK
2
Department of Otolaryngology
Otolaryngology and Head and
Neck Surgery, Royal Albert
Edward Infirmary, Wrightington,
Wigan & Leigh NHS Foundation
Trust, Wigan, Lancashire, UK
Correspondence to
Dr Vineeta Joshi, Department of
Paediatrics, Royal Albert Edward
Infirmary, Wrightington, Wigan
& Leigh NHS Foundation Trust,
Wigan, Lancashire WN1 2NN,
UK; vineetajoshi@doctors.org.uk
Received 25 April 2013
Revised 1 October 2013
Accepted 23 October 2013
Published Online First
14 November 2013

ABSTRACT
Recurrent croup is a distinct clinical entity from
viral croup. It is not a specific diagnosis and its
presence should alert the clinician to explore the
underlying cause. We discuss an evidence-based
structured approach to management of a child
with recurrent croup.

INTRODUCTION
Croup is a relatively common viral infection of larynx, trachea and/or bronchi that
is characterised by an acute onset of
barking cough, hoarseness and stridor with
or without dyspnoea, typically during Fall
and winter season. It is uncommon to have
more than one episode of croup in a year
in an immune competent child.1 Croupy
episodes which occur more frequently
(more than two episodes per year) have
been referred to as recurrent croup.2 3
Recurrent croup is not a specific diagnosis
and its presence should alert the clinician
to explore what is the real diagnosis4
(table 1). Recurrent croup can be the presentation of an underlying intrinsic and/or
extrinsic airway narrowing process (box 1).
A thorough history and physical examination is an essential preliminary step in
establishing the primary or contributory
cause of recurrent croup.

2.

3.

4.

WHAT SHOULD YOU ASK IN


HISTORY?

To cite: Joshi V, Malik V,


Mirza O, et al. Arch Dis Child
Educ Pract Ed 2014;99:
9093.

90

1. Previous episodes: Age of onset, presence/


absence of viral upper respiratory tract
infection symptoms, duration of each
episode, interval between episodes, presence of wheeze and importantly the
response to treatment should be enquired.
Severity of previous episodes should be
assessed based on age of presentation, frequency of episodes, need for urgent or
inpatient treatment and need for intubation. Recurrent croup in a child age more

5.

than 4 years has been suggested as a


marker of increased severity.5
Previous intubation: In the birth and neonatal
history, intubation may indicate acquired subglottic stenosis or subglottic cysts, which predispose to recurrent croup symptoms.
Gastro-oesophageal reflux (GOR): Ask
about symptoms suggestive of GOR in
each case and if already on antireflux treatment, any subsequent improvement in frequency or severity of croup episodes
should be enquired.
Studies report an evidence of gastrooesophageal reflux disease (GORD) in
47%100% of their patients with recurrent
croup.2 3 5 One study showed that children
with recurrent croup who had GOR presented at a younger age (mean age 6 months)
and had shorter interval between episodes
(mean interval period 3 months) as compared
with those who did not have reflux.5 GOR is
not a causative factor but may aggravate or
unmask pre-existing airway pathology.
Reactive airway disease (RAD) and atopy:
History of asthma, eczema and allergies
should be taken. Also, adequacy of treatment with respect to symptom control
should be assessed.
Recurrent croup has been referred to as
spasmodic croup in a number of earlier
studies that postulate recurrent croup to be a
spectrum of airway hyper-reactivity like
asthma. These studies report RAD to be
present in 40.4%82% of patients with
recurrent croup.68 However, a cause and
effect relationship between RAD and
recurrent croup has not been established.
Kwong et al2 reported history of asthma in
76.5% (n=17) of their patients but failed to
note any improvement in croup symptoms
despite optimum management of asthma.
Foreign body aspiration: Laryngeal/
tracheal foreign body can masquerade as
recurrent croup leading to a delayed
diagnosis.911

Joshi V, et al. Arch Dis Child Educ Pract Ed 2014;99:9093. doi:10.1136/archdischild-2013-303846

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Best practice
Table 1

Croup versus recurrent croup


Croup

Recurrent croup

Frequency
Aetiology

Usually one episode per year


Parainfluenza type-1, influenza, RSV

Age
Season
Symptoms

6 Months to 3 years, peak incidence at 2 years


During increased viral activityFall and winter season
Usually a prodrome of URTI followed by hoarseness,
barking cough, stridor,respiratory distress
Short lasting, usually 12 days
Symptoms completely resolve with standard treatment

Two or more episodes per year


Possible underlying predisposing cause that may be unmasked by viral
infection (see box 1)
Any age, can occur <6 months and >3 years
Throughout the year
Hoarseness, barking cough, stridor, respiratory distress (usually no
prodrome but symptoms made worse if concurrent URTI)
Relapsing, remitting course; episode can persist over weeks
Some or no response to treatment

Course
Response to
treatment
RSV, respiratory syncytial virus; URTI, upper respiratory tract infection.

6. Hoarseness or weak cry: Persistent hoarse voice or weak


cry in between episodes may indicate vocal cord pathology such as vocal cord paralysis or recurrent respiratory papillomatosis.12
7. Chronic symptoms: Persistent cough, dyspnoea, dysphagia and recurrent chest infections associated with recurrent stridor may suggest a pathology causing external
airway compression. Gormley et al13 reported a delay of
6 weeks to 10 years in the diagnosis of congenital

Box 1 Predisposing factors for recurrent croup


in children
Congenital
Congenital subglottic stenosis
Congenital cardiovascular abnormality
Tracheo-oesophageal fistula
Laryngotracheal cleft
Tracheobronchomalacia
Vocal cord paralysis
Congenital tracheal stenosis
Congenital goitre
Traumatic
Acquired subglottic stenosis
Subglottic cysts
Airway foreign body
Inflammatory
Gastro-oesophageal reflux disease
Asthma
Allergy
Infectious
Viral
Bacterial
Tumour
Subglottic haemangioma
Lymphangioma
Thyroid neoplasm
Recurrent respiratory papillomatosis
Thymoma
Lymphoma
Mediastinal mass

cardiovascular abnormalities that present with such


chronic symptoms.
8. Family history: Family history of croup, allergy and
atopy should be taken. Pruikkonen et al14 reported a
history of croup among parents and siblings to be the
most important predictive factor for croup and its recurrence in a child.

Apart from the above focused history, a full paediatric


history covering all aspects of growth and development should be taken to enable a comprehensive
management.
FOCUSED EXAMINATION OF A CHILD WITH
RECURRENT CROUP
1. Assessment of airway and breathing: In a child with acute
symptoms, immediately assess for severity of croup. Stridor
at rest, use of accessory muscles, chest wall retractions,
tachycardia, requirement of oxygen and increasing tiredness
suggest significant airway obstruction. Note the quality of
stridor, voice, cry, cough and presence of wheeze.
Type of stridor can help to localise the site of pathology.
Inspiratory stridor points towards a supraglottic problem,
for example, laryngomalacia; biphasic stridor points
towards a glottic or subglottic problem, for example, subglottic stenosis or vocal cord paralysis; whereas expiratory
stridor points towards a tracheal pathology, for example,
external compression of trachea or tracheomalacia.
2. General examination: Check if the child is pyrexial or
has a toxic appearance. No response to standard treatment of croup in an unwell child with pyrexia suggests
bacterial tracheitis.
Assess for failure to thrive. Presence of failure to thrive
may suggest an underlying significant GORD.
Look for any distinctive craniofacial features suggesting an
underlying genetic abnormality that may be associated
with an anatomic or functional airway abnormality.
Laryngospasm due to hypocalcaemia resulting in recurrent stridor has been reported in children with rickets.15
Look for presence of cutaneous haemangiomas.
Haemangioma particularly in the head and neck area
(beard distribution) may be associated with subglottic
haemangioma contributing to recurrent croup symptoms.16 Up to 50% of patients with subglottic

Joshi V, et al. Arch Dis Child Educ Pract Ed 2014;99:9093. doi:10.1136/archdischild-2013-303846

91

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Best practice
haemangioma have a cutaneous haemangioma in the head
and neck area.17
3. Chest auscultation: Reduced air entry may be present
due to an airway foreign body or due to external tracheal compression from a mediastinal mass.
4. Signs of allergy or atopy: Presence of nasal congestion,
mouth breathing, eczema and wheeze may suggest an
underlying atopy and airway reactivity predisposing to
recurrent croup.

Apart from above focused examination, a detailed


paediatric general and systems examination should be
carried out to complete the assessment.
WHAT MANAGEMENT SHOULD YOU PROVIDE?
1. Investigations: Appropriate investigation should be
carried out based on history and examination with the
aim of ruling out any underlying condition contributing
to the recurrence of croup symptoms.
A. Radiological investigations:
X-ray chest/neck: may be helpful to identify a
mediastinal mass or a radio-opaque foreign body.
A normal frontal and lateral chest radiograph has
been shown virtually to exclude the diagnosis of a
vascular ring as a cause of stridor.18
Cardiac/chest CT/MR scan: may be required if
there is suspicion of external tracheal compression by a vascular ring or a mediastinal mass.
B. Direct laryngo-tracheo-bronchoscopy (DLTB):
DLTB is the most important investigation to
exclude anatomic airway abnormality predisposing
to recurrent croup such as subglottic cysts and subglottic stenosis. It can also demonstrate findings
suggestive of GORD and extrinsic tracheal compression from causes such as double aortic arch,
innominate artery and mediastinal mass.
Anatomic airway abnormality has been reported in
25%100% of children with recurrent croup who underwent bronchoscopy.2 3 5 19 20 Therefore, a careful consideration of risk factors should be taken to guide
referral to otolaryngologist (box 2).
C Investigations for GOR: Testing for GOR will
depend on your own unit protocol.
D Blood tests: These are generally not helpful unless
bacterial tracheitis, mediastinal mass or hypocalcaemia is a consideration based on history,
examination and other investigations.
2. Treatment: Episodes of recurrent croup may or may not
respond to the standard treatment of viral croup that is,
oral dexamethasone with or without nebulised adrenaline. No response or a partial response to standard
treatment warrants further investigation. Specific treatment will depend on the underlying cause.

Bacterial tracheitis needs urgent attention and


treatment with broad-spectrum intravenous antibiotics, as there is a high likelihood of the need for
intubation.

92

Box 2 Indications for referral to an ENT surgeon

History of previous intubation


Age less than 1 year or more than 4 years
Multiple hospital admissions with croup
No improvement in symptoms despite appropriate
treatment for gastro-oesophageal reflux or reactive
airway disease and atopy
Suspicion of foreign body inhalation
Patients with congenital anomalies
Presence of cutaneous haemangioma
Patients with a persistent weak cry or hoarse voice
Patients who did not respond to standard treatment
of croup

Referral to a paediatric otolaryngologist is important for airway assessment. Subglottic stenosis is a


common finding on DLTB in children with recurrent
croup. Subglottic stenosis is diagnosed by conventional DLTB with the child breathing spontaneously
and the airway is sized using endotracheal tubes of
increasing diameter. Mild stenosis is found more commonly and is usually managed conservatively while
the higher grades require surgical intervention. The
most severe cases may even require laryngotracheal
reconstruction. DLTB should ideally be performed
when the child is free of respiratory tract infection.
A therapeutic trial of antireflux therapy can be
helpful if there are supporting clinical features.
Hoa et al reported a significant decline in the number
and duration of croup episodes in 87% of their
patients after 69 months of antireflux treatment.3
Even children who did not demonstrate reflux related
changes on endoscopy showed symptomatic improvement. Importantly, 50% of patients on antireflux
therapy who had a second endoscopic examination
showed clinically significant decrease in grading of
subglottic stenosis.
Associated RAD and atopy should be appropriately
managed with bronchodilators, steroids, antihistamine
and other specific drugs according to the condition. It
is suggested that if patients with recurrent croup are
considered to have hyper-reactive airway, it would be
justified to treat them with inhaled corticosteroids,
similar to patients with bronchial asthma.6 7
CONCLUSIONS
Recurrent croupy episodes should prompt a search for
an underlying cause that may be unmasked by a viral
infection. A thorough history and examination should
be undertaken to investigate the underlying cause.
Referral to paediatric otolaryngologist for DLTB
should be made after consideration of risk factors to
assist in diagnosis and management.

Joshi V, et al. Arch Dis Child Educ Pract Ed 2014;99:9093. doi:10.1136/archdischild-2013-303846

Downloaded from http://ep.bmj.com/ on January 29, 2015 - Published by group.bmj.com

Best practice
Contributors All the authors have helped in evidence-based
search and writing up the manuscript.
Competing interests None.
Provenance and peer review Commissioned; externally peer
reviewed.

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Joshi V, et al. Arch Dis Child Educ Pract Ed 2014;99:9093. doi:10.1136/archdischild-2013-303846

93

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Fifteen-minute consultation: structured


approach to management of a child with
recurrent croup
Vineeta Joshi, Vikas Malik, Omar Mirza and B Nirmal Kumar
Arch Dis Child Educ Pract Ed 2014 99: 90-93 originally published online
November 14, 2013

doi: 10.1136/archdischild-2013-303846
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