21/3/24, 21:54 Croup: Clinical features, evaluation, and diagnosis - UpToDate

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Crup: características clínicas, evaluación y diagnóstico.

AUTOR: Charles R Woods, MD, MS
EDITORES DE SECCIÓN: Anna H Messner, MD, Sheldon L Kaplan, MD, Joshua Nagler, MD, MHPEd
EDITOR ADJUNTO: Carrie Armsby, MD, MPH

Todos los temas se actualizan a medida que hay nueva evidencia disponible y nuestro proceso de revisión por pares se
completa.

Revisión de la literatura vigente hasta: febrero de 2024.

Este tema se actualizó por última vez: 23 de agosto de 2023.

INTRODUCCIÓN

El crup es una enfermedad respiratoria caracterizada por estridor inspiratorio, tos y

ronquera. Estos síntomas son el resultado de la inflamación de la laringe y las vías
respiratorias subglóticas. Una tos perruna es el sello distintivo del crup. Aunque el crup suele
ser una enfermedad leve y autolimitada, puede producirse una obstrucción importante de
las vías respiratorias superiores y dificultad respiratoria.

Aquí se analizarán las características clínicas, la evaluación y el diagnóstico del crup. El

tratamiento del crup se analiza por separado. (Ver "Manejo del crup" .)

DEFINICIONES

El término "crup" se ha utilizado para describir una variedad de afecciones de las vías
respiratorias superiores en los niños. A los efectos de esta revisión del tema, utilizaremos el
término "crup" para referirnos a la laringotraqueítis viral, como se define a continuación.

Laringotraqueítis (crup) : el crup es una enfermedad respiratoria caracterizada por

estridor inspiratorio, tos perruna y ronquera. Estos síntomas son el resultado de la
inflamación de la laringe y las vías respiratorias subglóticas.

Crup viral : el crup viral (también llamado crup clásico) se refiere al síndrome de crup
típico que ocurre comúnmente en niños de seis meses a tres años de edad. Como su nombre
lo indica, es causada por virus respiratorios y, por lo tanto, suelen estar presentes síntomas
virales (p. ej., congestión nasal, fiebre). El crup viral suele ser una enfermedad autolimitada;

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la tos normalmente se resuelve en tres días [ 1 ]. (Consulte 'Presentación clínica' a

continuación y 'Curso clínico' a continuación).

Crup espasmódico : el crup espasmódico también ocurre en niños de seis meses a tres
años de edad. El crup espasmódico siempre ocurre por la noche. El inicio y el cese de los
síntomas son abruptos y la duración de los síntomas es corta, y a menudo los síntomas
desaparecen en el momento de la presentación para recibir atención médica. Por lo general
no hay fiebre, pero pueden presentarse síntomas leves de las vías respiratorias superiores
(p. ej., coriza). Los episodios pueden repetirse dentro de la misma noche y durante dos a
cuatro noches sucesivas. Una característica sorprendente del crup espasmódico es su
naturaleza recurrente, de ahí el término descriptivo alternativo "crup frecuentemente
recurrente". Puede haber una predisposición familiar al crup espasmódico y parece ser más
común en niños con antecedentes familiares de alergias [ 2 ]. Debido a que existe cierta
superposición clínica con las enfermedades atópicas, a veces se le denomina "crup alérgico".

Al principio del curso clínico, el crup espasmódico puede ser difícil de distinguir del crup
viral. Con el tiempo, la naturaleza episódica de los síntomas y el bienestar relativo del niño
entre los ataques diferencian el crup espasmódico del crup viral, en el que los síntomas son
continuos.

Aunque la presentación inicial puede ser dramática, el curso clínico suele ser benigno. Los
síntomas casi siempre se alivian consolando al niño ansioso y administrándole aire
humidificado.

Los episodios recurrentes de crup también se denominan "crup atípico" o "crup recurrente",
con diferentes definiciones y consideraciones etiológicas [ 3 ]. (Ver 'Crup recurrente' a
continuación).

Otros términos relacionados : las siguientes afecciones están relacionadas con el crup,
pero consideramos estas entidades clínicas distintas:

● Laringitis : la laringitis se refiere a la inflamación limitada a la laringe que se manifiesta

como ronquera. Suele ocurrir en niños mayores y adultos y, al igual que el crup, con
frecuencia es causado por una infección viral. La laringitis se analiza por separado.
(Consulte "Causas comunes de ronquera en niños", sección sobre 'Laringitis' ).

● Laringotraqueobronquitis : la laringotraqueobronquitis ocurre cuando la inflamación

se extiende hacia los bronquios, lo que produce signos de las vías respiratorias
inferiores (p. ej., sibilancias, crepitantes). Tiende a ser una enfermedad más grave en
comparación con la laringotraqueítis sola. Sin embargo, las dos entidades se
superponen y el término laringotraqueobronquitis se usa comúnmente de manera
intercambiable con laringotraqueítis. La extensión de la inflamación hacia las vías

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respiratorias inferiores produce laringotraqueobronconeumonitis, que puede

complicarse con una sobreinfección bacteriana (es decir, neumonía).

● Bacterial tracheitis – Bacterial tracheitis (sometimes called "bacterial croup") is an

invasive exudative bacterial infection of the soft tissues of the trachea ( picture 1). In
some cases, there is extension to the subglottic laryngeal structures or the upper
bronchial tree. Bacterial tracheitis may occur as a primary infection or as a complication
of viral croup. With secondary infection, patients typically present with symptoms of
viral croup and then have marked worsening with high fevers, toxic appearance, and
severe respiratory distress. Bacterial tracheitis is discussed in greater detail separately.
(See "Bacterial tracheitis in children: Clinical features and diagnosis".)

EPIDEMIOLOGY

Croup is one of the most common respiratory illnesses in young children. It occurs mostly in
children ≤6 years old, with a peak incidence between six months to three years of age; it is
uncommon in children >6 years old [4,5]. A study of emergency department (ED) visits in the
United States from 2007 to 2014 estimated that there were approximately 350,000 to
400,000 croup-related ED visits each year, accounting for 1.3 percent of all ED visits [6].
Children <2 years old accounted for 43 percent of the visits, children ages two to seven years
made up 50 percent of visits, and children ≥7 accounted for only 7 percent. Croup is more
common in boys, with reported male:female ratios ranging from 1.4:1 to 2:1 [4-8].

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 [9]. Parental smoking, a well-recognized risk factor for other respiratory tract
infections in children, does not appear to increase the risk of croup [9,10]. (See "Secondhand
smoke exposure: Effects in children", section on 'Respiratory symptoms and illness'.)

In temperate climates, 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 in the
northern hemisphere) and minor peaks occurring during periods of respiratory syncytial
virus or influenza virus activity. (See "Seasonal influenza in children: Clinical features and
diagnosis", section on 'Influenza activity' and "Respiratory syncytial virus infection: Clinical
features and diagnosis in infants and children", section on 'Epidemiology'.)

ED visits for croup are most frequent between 10:00 PM and 4:00 AM [11]. A morning peak in
ED visits for croup between 7:00 AM and 11:00 AM also has been noted [8].

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Hospital admissions for croup have declined steadily since the late 1970s [12]. In studies
conducted in the contemporary era, approximately 5 to 10 percent of children with croup
presenting to the ED require hospitalization. This is discussed separately. (See "Management
of croup", section on 'Indications for hospital admission'.)

MICROBIOLOGY

Viral causes — Croup is usually caused by viruses [13]:

● Parainfluenza viruses – Parainfluenza virus type 1 is the most common cause of acute
laryngotracheitis, especially during fall and winter epidemics [13,14]. 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 virus infection were diagnosed with croup [15]. 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. Compared with types 1
to 3, infection caused by parainfluenza virus type 4 is less likely to be associated with
stridor and croup in children [16,17]. The microbiology, pathogenesis, and
epidemiology of parainfluenza infections are discussed separately. (See "Parainfluenza
viruses in children".)

● Rhinovirus, respiratory syncytial virus (RSV), and adenoviruses – Rhinovirus, RSV,

and adenoviruses are relatively frequent causes of croup. For children with RSV
infection, the laryngotracheal component of disease is usually less significant than that
of the lower airways. (See "Respiratory syncytial virus infection: Clinical features and
diagnosis in infants and children", section on 'Clinical manifestations' and
"Pathogenesis, epidemiology, and clinical manifestations of adenovirus infection",
section on 'Clinical presentation'.)

● Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) – SARS-CoV-2 has

become a relatively common cause of croup [14,18-22]. Croup was a more common
manifestation of infection with the Omicron variant than the earlier variants of SARS-
CoV-2 [22,23]. Children with croup caused by SARS-CoV-2 (especially the Omicron
variant) may have more severe symptoms, may require more intense treatment in the
emergency department, and may be more likely to need admission to the hospital than
children with croup caused by other viral etiologies [21-23]. (See "COVID-19: Clinical
manifestations and diagnosis in children".)

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● Influenza – 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", section on
'Pneumonia and respiratory tract complications'.)

● Other human coronaviruses – HCoV-NL63 has been associated with croup and other
respiratory illnesses in children [24-27], as have human coronaviruses OC43 HKU1 [28].
(See "Coronaviruses", section on 'Respiratory syndromes'.)

● Measles – Measles is an important cause of croup in areas where measles remains

prevalent. (See "Measles: Clinical manifestations, diagnosis, treatment, and
prevention".)

● Others – 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 "Enterovirus and parechovirus infections: Clinical features,
laboratory diagnosis, treatment, and prevention" and "Epidemiology, clinical
manifestations, and pathogenesis of rhinovirus infections".)

Human metapneumovirus causes primarily lower respiratory tract disease similar to

RSV, but upper respiratory tract symptoms have been described [29]. (See "Human
metapneumovirus infections".)

Bacterial infection — Croup is rarely caused by bacterial infection with the exception of
Mycoplasma pneumoniae, which can cause a mild croup-like illness [30]. (See "Mycoplasma
pneumoniae infection in children", section on 'Other respiratory manifestations'.)

However, bacterial infection may occur secondarily. The most common bacterial pathogens
in this setting include Staphylococcus aureus, Streptococcus pyogenes, and Streptococcus
pneumoniae [4]. This is discussed in detail separately. (See "Bacterial tracheitis in children:
Clinical features and diagnosis".)

PATHOGENESIS

Pathology

● Viral croup – 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 subglottic airway, the portion of the
larynx immediately below the vocal folds. The cricoid cartilage of the subglottis is a
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complete cartilaginous ring, unlike the tracheal rings, which are horseshoe shaped.
Because it is a complete ring, the cricoid cannot expand, causing significant airway
narrowing whenever the subglottic mucosa becomes inflamed. 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 is rarely necessary for patients with viral croup, but, when
performed, it typically shows redness and swelling in the area just below the vocal folds
( picture 2). 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 folds and laryngeal tissues also can become swollen,
and cord mobility may be impaired [31-33]. 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 [34-36].

● Spasmodic or recurrent croup – In spasmodic or recurrent croup, findings on direct

laryngoscopy may demonstrate noninflammatory edema, suggesting that there is no
direct viral involvement of the tracheal epithelium in children with this presentation
[31]. In a retrospective case series of 197 children with recurrent croup who underwent
endoscopy at a single center from 2002 to 2012, 21 percent had evidence of subglottic
stenosis and 20 percent had abnormal esophageal biopsies (including evidence of
reflux esophagitis, eosinophilic esophagitis, and candidal esophagitis) [37]. Children
with subglottic stenosis tended to be younger compared with those without (mean age
35 versus 58 months). In another case series of 103 children with recurrent croup who
underwent endoscopy at a single center from 2004 to 2013, 44 percent had a history of
prior intubation, subglottic stenosis, or previous airway procedure [38]. Other common
underlying conditions included asthma (64 percent), gastroesophageal reflux disease
(60 percent), and seasonal allergies (48 percent). Endoscopy was normal in 65 percent
of the children in this series; 9 percent of children had moderate to severe findings
(including subglottic stenosis, cyst, and hemangioma).

Though a causal relationship between gastroesophageal reflux disease and recurrent

croup has been postulated, the evidence to support this is limited. A systematic review
of observational studies found that a temporal association between treatment with
antireflux medication and reduction of croup symptoms was often reported; however,
the retrospective nature of the data and lack of control group made it difficult to draw
conclusions about causality [39].

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● Bacterial tracheitis – 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. This clinical entity is
discussed in greater detail separately. (See "Bacterial tracheitis in children: Clinical
features and diagnosis", section on 'Pathogenesis and pathology'.)

Host factors — Only a small subset of children with a parainfluenza viral infection 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
upper airway narrowing, variations in immune response, and predisposition to atopy [8].

Underlying host factors that predispose to clinically significant narrowing of the upper
airway include:

● Congenital and acquired airway abnormalities, such as laryngomalacia, laryngeal clefts,

subglottic stenosis, vallecular cysts, or subglottic hemangiomas [40]. Subglottic
hemangiomas grow in the first few months of life, and affected patients typically
present with symptoms mimicking croup (ie, stridor and barking cough). (See
"Congenital anomalies of the larynx" and "Infantile hemangiomas: Epidemiology,
pathogenesis, clinical features, and complications", section on 'Airway hemangiomas'.)

● Hyperactive airways, perhaps aggravated by atopy or gastroesophageal reflux, as

suggested in some children with spasmodic croup or recurrent croup [2,41,42]. (See
'Spasmodic croup' above.)

CLINICAL PRESENTATION

Croup typically occurs in children ≤6 years old, with a peak incidence between six months to
three years of age (see 'Epidemiology' above). The illness begins with typical viral upper
respiratory symptoms (rhinorrhea, cough, congestion) and progresses to signs of airway
inflammation and obstruction (barking cough, stridor, respiratory distress).

Presenting signs and symptoms — Symptoms usually begin with nasal discharge,
congestion, and coryza and progress over 12 to 48 hours to include fever, hoarseness,
barking cough, and stridor. There is minimal, if any, pharyngitis. As airway narrowing
progresses, stridor develops and there may be mild tachypnea with a prolonged inspiratory
phase. If upper airway obstruction worsens, severe respiratory distress can develop. Rapid
progression or signs of concurrent lower airway involvement suggests a more serious illness
(eg, bacterial tracheitis or pneumonia). (See 'Differential diagnosis' below.)

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

in the table ( table 1) and described below (see 'Severity assessment' below). Biphasic
stridor (stridor heard on both inspiration and expiration) at rest is a sign of significant airway
obstruction. As airway obstruction progresses, the child may become restless or anxious.
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.

Clinical course — Croup is usually a self-limited illness, and the cough typically resolves
within three days [1]. Other symptoms may persist for up to seven days, with a gradual
return to normal. Deviation from this expected course should prompt consideration of
diagnoses other than croup. (See 'Differential diagnosis' below.)

EVALUATION

Overview — The evaluation of children with suspected croup is aimed at promptly

identifying patients with severe upper airway obstruction or those at risk for rapid
progression of upper airway obstruction and excluding other conditions with presentations
similar to croup that require specific evaluations and/or interventions. (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 ( table 1). Children who have severe respiratory distress
require immediate treatment, as summarized in the figure ( algorithm 1) and discussed in
detail separately. (See "Management of croup", section on 'Moderate to severe croup' and
"Management of croup", section on 'Respiratory care'.)

In addition, the child's hydration status should be assessed. Moderate to severe croup may
be associated with decreased oral intake and increased insensible losses from fever and
tachypnea, resulting in dehydration. (See "Clinical assessment of hypovolemia (dehydration)
in children".)

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Once treatment is underway and the child is more stable, 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 [4,43]:

● Fever – The absence of fever from onset of symptoms to the time of presentation is
suggestive of spasmodic croup or other noninfectious etiology of stridor (eg, subglottic
cyst, subglottic hemangioma, foreign body aspiration).

● Barking cough – The classic physical finding in a patient with subglottic narrowing is a
barky, seal-like cough.

● Hoarseness – Hoarseness may be present in croup, particularly in older children,

whereas hoarseness is not a typical finding in epiglottitis or foreign body aspiration.

● Difficulty swallowing – Difficulty swallowing may occur in acute epiglottitis. Rarely, 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 more commonly occurs 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 [43].

● Throat pain – Complaints of dysphagia and sore throat are more common in children
with epiglottitis than croup (approximately 60 to 70 versus <10 percent) [43].

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

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noninfectious 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.

Severity assessment — The severity of croup is determined by the presence or absence of

stridor at rest, degree of chest wall retractions, air entry, presence or absence of pallor or
cyanosis, and mental status. There are a number of validated clinical scoring systems that
are used to assess croup severity. The Westley croup score has been the most extensively
studied ( table 1) (calculator 1) [44].

● Mild croup (Westley croup score ≤2) – Children with mild croup have a barking cough,
hoarse cry, no stridor at rest (although stridor may be present when upset or crying),
and either no or only mild chest wall/subcostal retractions [4,45,46].

● Moderate croup (Westley croup score 3 to 7) – Children with moderate croup have
stridor at rest. They have at least mild retractions and may have other mild to moderate
symptoms or signs of respiratory distress [4,45,46].

● Severe croup (Westley croup score ≥8) – Children with severe croup have significant
stridor at rest, although the intensity of the stridor may decrease with worsening upper
airway obstruction and poor air entry [4,45,46]. They have severe retractions (including
indrawing of the sternum) and other signs of significant distress. They may appear
anxious, agitated, or pale and fatigued.

● Impending respiratory failure (Westley croup score ≥12) – Croup occasionally results
in severe airway obstruction with impending respiratory failure, heralded by the
following signs [4,45,46]:

• Fatigue and listlessness

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

Croup clinical scores are widely used in clinical practice and in clinical trials evaluating the
efficacy of different treatments for croup. However, it is important to understand that these
scores are somewhat subjective and there can be substantial interobserver variability [47,48].
Nevertheless, the croup score remains a useful guide for assessing severity of illness and
responses to therapies.

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Prompt recognition and treatment of children with severe croup are paramount, as
discussed separately. (See "Management of croup", section on 'Moderate to severe croup'.)

Assessing for other causes — Components of the physical examination that are useful in
distinguishing croup from other causes of acute upper airway obstruction and respiratory
distress include [43,45]:

● Preferred posture – Children with epiglottitis usually prefer to sit up in the "tripod" or
"sniffing position" (neck is mildly flexed, and head is mildly extended) ( picture 3A-B).

● Quality of the voice – Children with croup may have a hoarse voice or diminished cry. A
muffled "hot-potato" voice is suggestive of epiglottitis, retropharyngeal abscess, or
peritonsillar abscess.

● Examination of the oropharynx for the following signs:

• Pharyngitis, typically minimal in croup, may be more pronounced in epiglottitis or

laryngitis
• Excessive salivation, suggestive of acute epiglottitis, peritonsillar abscess,
parapharyngeal 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
• Cherry-red, swollen epiglottis, suggestive of epiglottitis

For most patients who have a clinical picture consistent with viral croup, direct
visualization of the epiglottis is not necessary and cautious examination of the child's
throat is sufficient. The approach to diagnosing epiglottitis, including which patients
should undergo attempts at direct visualization, is discussed separately. (See
"Epiglottitis (supraglottitis): Clinical features and diagnosis", section on 'Signs of
impending airway obstruction'.)

● Examination of the cervical lymph nodes, which can be enlarged in patients with
retropharyngeal or peritonsillar abscesses.

● Lung examination – Expiratory wheezing suggests small or medium airway obstruction

(eg, asthma or bronchiolitis). Crackles (rales) suggests lower respiratory tract disease
(eg, pneumonia).

● 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.

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● 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.)

Radiographs

● 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 course is atypical and/or the diagnosis is in question

• The child has severe symptoms and does not respond as expected to therapeutic
interventions
• There is suspicion for an inhaled or swallowed foreign body (although the majority
are not radiopaque) (see "Airway foreign bodies in children")
• The child has recurrent episodes of croup and has not previously had airway
radiographs performed (see 'Recurrent croup' below)

● Findings – In children with croup, a posterior-anterior chest radiograph demonstrates

subglottic narrowing, commonly called the "steeple sign" ( image 1A). The lateral view
may demonstrate overdistention of the hypopharynx during inspiration and subglottic
haziness ( image 1B). The epiglottis should have a normal appearance.

In one study, greater degrees of narrowing of the trachea on a frontal or lateral plain
radiograph correlated with increased likelihood of hospitalization and longer hospital stay
[49].

Laboratory studies — Laboratory studies are rarely indicated in children with croup and are
of limited diagnostic utility.

Blood tests — The white blood cell count can be low, normal, or elevated; white blood cell
counts >10,000 cells/microL are common. 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, but children with dehydration may have low serum
bicarbonate and/or elevated blood urea nitrogen. (See "Clinical assessment of hypovolemia
(dehydration) in children", section on 'Laboratory testing'.)

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.
However, identification of a specific viral etiology may be necessary to make decisions
regarding isolation. When an etiologic diagnosis is necessary, rapid diagnostic tests are

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performed on secretions from the nasopharynx, as discussed below. (See 'Etiologic

diagnosis' below.)

DIAGNOSIS

Clinical diagnosis — Croup is diagnosed clinically, based upon the characteristic barking
cough and stridor, especially during a typical community epidemic of one of the causative
viruses. (See 'Clinical presentation' above and 'Viral causes' 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. In
children with croup, the characteristic radiographic finding is subglottic narrowing ("steeple
sign") ( image 1A). (See 'Radiographs' above and '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. In
particular, SARS-CoV-2 testing may be appropriate given the infection control and quarantine
implications. (See "COVID-19: Diagnosis" and "COVID-19: Infection prevention for persons
with SARS-CoV-2 infection".)

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: Management", section on 'Timing' and "Seasonal influenza in
children: Clinical features and diagnosis", section on 'Whom to test'.)

Diagnosis of a specific viral etiology can be made with rapid polymerase chain reaction (PCR),
rapid antigen testing, or viral culture of secretions from the nasopharynx. Multiplex tests (eg,
respiratory viral panel), which simultaneously assess the presence of multiple agents in one
specimen (typically using PCR), are widely available [50,51]. 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 in infants and children", section on 'Laboratory confirmation')

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● Coronavirus (all types) (see "Coronaviruses", section on 'Diagnosis' and "COVID-19:

Clinical manifestations and diagnosis in children", section on 'Laboratory tests for SARS-
CoV-2')

● Adenovirus (see "Diagnosis, treatment, and prevention of adenovirus infection", section

on 'Diagnostic tests of choice for different adenovirus syndromes')

● Metapneumovirus (see "Human metapneumovirus infections", section on 'Diagnosis')

● Measles (see "Measles: Clinical manifestations, diagnosis, treatment, and prevention",

section on 'Diagnosis')

● Enteroviruses (see "Enterovirus and parechovirus infections: Clinical features,

laboratory diagnosis, treatment, and prevention", section on 'Laboratory diagnosis')

RECURRENT CROUP

A child who has 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 of 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 airway lesions, gastroesophageal reflux
or eosinophilic esophagitis, or atopic conditions [37,38,40,52-56]. (See 'Differential diagnosis'
below.)

Children who have recurrent croup should be referred to an otolaryngologist. Radiographic

evaluation, laryngoscopy, bronchoscopy, and/or esophagoscopy may be warranted. (See
'Radiographs' above and "Assessment of stridor in children".)

DIFFERENTIAL DIAGNOSIS

The differential diagnosis of croup includes other causes of stridor and/or respiratory
distress. (See "Assessment of stridor in children", section on 'Causes of stridor'.)

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 ( table 2 and table 3) [4,5]:

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● 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 usually rapid, and when accompanied by 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 [43]. The lateral
radiograph in virtually all children with epiglottitis demonstrates swelling of the
epiglottis, sometimes called the "thumb sign" ( image 2). (See "Epiglottitis
(supraglottitis): Clinical features and diagnosis".)

● Bacterial tracheitis – Bacterial tracheitis (sometimes called "bacterial croup") is an

invasive exudative bacterial infection of the soft tissues of the trachea. It may occur as a
primary infection or as a complication of viral croup. With secondary infection, patients
typically present with symptoms of viral croup and then have marked worsening with
high fevers, toxic appearance, and severe respiratory distress. The lateral radiograph in
children with bacterial tracheitis may demonstrate only nonspecific edema or
intraluminal membranes and irregularities of the tracheal wall ( image 3). (See
"Bacterial tracheitis in children: Clinical features and diagnosis".)

● Peritonsillar, parapharyngeal, or retropharyngeal abscesses – Children with deep

neck space abscesses, cellulitis of the cervical prevertebral tissues, or other painful
infections of the pharynx may present with fever, drooling, neck stiffness,
lymphadenopathy, and varying degrees of toxicity. Barking cough and stridor are
usually absent. (See "Peritonsillar cellulitis and abscess", section on 'Typical
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 and producing a barking cough
and inspiratory stridor. Ingestion of a nonobstructive but subsequently erosive foreign
bodies such as a button battery may produce stridor more remote from the time of
ingestion that persists or recurs [57]. (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 [4,5]. There may be a history of

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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. (See "Inhalation injury from heat, smoke, or chemical irritants".)

● Anomalies of the airway – Stridor can be caused by congenital or acquired anomalies

of the upper airway ( table 2 and table 3), including laryngeal webs,
laryngomalacia, congenital subglottic stenosis, subglottic hemangioma, bronchogenic
cyst, laryngeal papillomas, and vocal cord paralysis (which can be secondary to
laryngeal nerve injury from trauma or surgery or due to neurologic disease [eg, Chiari
malformation, Guillain-Barré syndrome, brain or spinal cord tumor]) [58]. Most of these
tend to have a more chronic course with absence of fever and symptoms of upper
respiratory tract illness, unless the child presents because the airway narrowing is
exacerbated by a concomitant viral infection. Subglottic hemangioma ( picture 4 and
picture 5) should be considered in any young infant who presents with a barking
cough and no other signs of a viral infection, particularly if there is a visible
hemangioma present in the beard distribution. Often, these infants will respond
temporarily to the usual treatment for croup (steroids and nebulized epinephrine);
however, the symptoms will recur within a few days of treatment completion. (See
"Assessment of stridor in children" and "Congenital anomalies of the larynx" and
"Infantile hemangiomas: Epidemiology, pathogenesis, clinical features, and
complications", section on 'Airway hemangiomas'.)

SOCIETY GUIDELINE LINKS

Links to society and government-sponsored guidelines from selected countries and regions
around the world are provided separately. (See "Society guideline links: Croup".)

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 6th
grade 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.

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Here are the patient education articles that are relevant to this topic. We encourage you to
print or email 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 education: Croup (The Basics)")

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

SUMMARY AND RECOMMENDATIONS

● Etiology – Croup is a respiratory illness characterized by inspiratory stridor, barking

cough, and hoarseness resulting from inflammation in the larynx and subglottic airway.
Croup is usually caused by viruses, most commonly parainfluenza virus type 1. Other
common causes include rhinovirus, respiratory syncytial virus (RSV), and adenoviruses.
Bacterial infection may occur secondarily. (See 'Definitions' above and 'Microbiology'
above.)

● Epidemiology – Croup is one of the most common respiratory illnesses in young

children. It occurs mostly in children ≤6 years old, with a peak incidence between six
months to three years of age. In temperate climates, most cases occur in the fall or
early winter. Host factors that may contribute to the development of croup include
functional or anatomic susceptibility to upper airway narrowing. (See 'Epidemiology'
above and 'Host factors' above.)

● Clinical presentation – Symptoms usually begin with nasal discharge, congestion, and
coryza and progress over 12 to 48 hours to include fever, hoarseness, barking cough,
and stridor. Respiratory distress increases as upper airway obstruction becomes more
severe. Croup is usually a self-limited illness, and the cough typically resolves within
three days. (See 'Clinical presentation' above.)

● Rapid assessment – The objectives of the evaluation of the child with croup include
assessment of severity ( table 1) (calculator 1) and exclusion of other causes of upper
airway obstruction. 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 'Evaluation' above
and 'Rapid assessment and initial management' above.)

● Diagnosis – Croup is diagnosed clinically, based upon the characteristic 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

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diagnosis is in question ( image 1A-B). (See 'Diagnosis' above and 'Differential

diagnosis' above.)

● Differential diagnosis – 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 severe upper airway obstruction)
and those that require specific therapy. Important considerations include acute
epiglottitis, peritonsillar and retropharyngeal abscesses, foreign body aspiration, acute
angioedema (eg, anaphylaxis), upper airway injury, and congenital abnormalities of the
upper airway ( table 2). (See 'Differential diagnosis' above.)

● Recurrent croup – Recurrent episodes of croup-like symptoms that are atypical for
simple croup (ie, severe or prolonged symptoms) or that occur outside of the typical
age range (ie, earlier than six months or beyond age five or six years) should raise
suspicion for another underlying condition (eg, congenital airway abnormalities
( table 2), gastroesophageal reflux, eosinophilic esophagitis, atopic conditions).
Children with recurrent croup should be referred to an otolaryngologist for further
evaluation. (See 'Recurrent croup' above and "Assessment of stridor in children".)

Use of UpToDate is subject to the Terms of Use.

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41. Van Bever HP, Wieringa MH, Weyler JJ, et al. Croup and recurrent croup: their association
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1999; 158:253.
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treatment of croup: a double-blind study. Am J Dis Child 1978; 132:484.

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croup in children. Pediatr Emerg Med Pract 2012; 9:1.
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Paediatr Child Health 2001; 6:347.
48. Khemani RG, Schneider JB, Morzov R, et al. Pediatric upper airway obstruction:
interobserver variability is the road to perdition. J Crit Care 2013; 28:490.
49. Yang WC, Hsu YL, Chen CY, et al. Initial radiographic tracheal ratio in predicting clinical
outcomes in croup in children. Sci Rep 2019; 9:17893.
50. McCulloh RJ, Andrea S, Reinert S, Chapin K. Potential Utility of Multiplex Amplification
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Children: A Retrospective Analysis. J Pediatric Infect Dis Soc 2014; 3:146.
51. Couturier MR, Barney T, Alger G, et al. Evaluación del panel respiratorio FilmArray® para
uso clínico en un hospital infantil grande. Anal de laboratorio J Clin 2013; 27:148.
52. Duval M, Tarasidis G, Grimmer JF, et al. Papel de la evaluación operativa de las vías
respiratorias en niños con crup recurrente: un estudio de cohorte retrospectivo. Clin
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Otol 2013; 127:494.
54. Jabbour N, Parker NP, Finkelstein M, et al. Incidencia de hallazgos endoscópicos
operatorios en crup recurrente. Cirugía de cabeza y cuello de otorrinolaringol 2011;
144:596.

55. Chun R, Preciado DA, Zalzal GH, Shah RK. Utilidad de la broncoscopia para el crup
recurrente. Ann Otol Rhinol Laryngol 2009; 118:495.

56. Hampton T, Ghazal Asswad R, Bhat J, et al. El crup recurrente es un buen indicador de
problemas subyacentes de las vías respiratorias pediátricas: un estudio de cohorte

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retrospectivo de 10 años de endoscopia de las vías respiratorias. Oído Nariz Garganta J

2023; :1455613231170087.
57. Gohil R, Culshaw J, Jackson P, Singh S. La ingestión accidental de pilas de botón se
presenta como crup. J. Laryngol Otol 2014; 128:292.
58. Gerard R, Nolent P, Lerouge-Bailhache M, et al. Cuando el estridor no es crup: reporte de
un caso. J Emerg Med 2022; 63:673.
Tema 6002 Versión 39.0

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GRÁFICOS

Traqueítis bacteriana: traqueobroncoscopia

Obsérvense las membranas mucopurulentas adherentes dentro de la tráquea.

Cortesía de Glenn C Isaacson, MD, FAAP.

Gráfico 55364 Versión 5.0

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Laringoscopia en un niño con crup.

(A) Endoscopic view of the larynx and subglottic airway seen from above the vocal cords in a child with
viral croup. The vocal cords are swollen, there is marked subglottic swelling (arrow), and the opening
of subglottic airway is narrow.

(B) Endoscopic image of a normal pediatric larynx.

VC: vocal cord.

Courtesy of Glenn C Isaacson, MD, FAAP.

Graphic 108046 Version 2.0

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Westley croup severity score

Clinical feature Assigned score

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

Total

Score total Severity Description Management

≤2 Mild Occasional barky Home treatment –

cough, no stridor at Symptomatic care
rest, mild or no including
retractions antipyretics and ora
fluids
Outpatient
treatment – Single
dose of oral
dexamethasone*
0.15 to 0.6 mg/kg
(maximum 16 mg)

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or oral prednisolone
(1 mg/kg)

3 to 7 Moderate Frequent barky cough, Single dose of oral

stridor at rest, mild to dexamethasone 0.6
moderate retractions mg/kg (maximum
16 mg)*
Nebulized
epinephrine ¶
Hospitalization is
generally not
needed but may be
warranted for
persistent or
worsening
symptoms after
treatment with
glucocorticoid and
nebulized
epinephrine

8 to 11 Severe Frequent barky cough, Single dose of

stridor at rest, marked oral/IM/IV
retractions, significant dexamethasone 0.6
distress mg/kg (maximum
16 mg)*
Repeated doses of
nebulized
epinephrine ¶ may
be needed
Inpatient admission
is generally required
unless marked
improvement occur
after treatment with
glucocorticoid and
nebulized
epinephrine

≥12 Impending respiratory Depressed level of Single dose of IM/IV

failure consciousness, stridor dexamethasone 0.6
at rest, severe mg/kg (maximum
retractions, poor air 16 mg)
entry, cyanosis or pallor Repeated doses of
nebulized
epinephrine ¶ may
be needed
Intensive care unit
admission is
generally required
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Consultation with
anesthesiologist or
ENT surgeon may
be warranted to
arrange for
intubation in a
controlled setting

IM: intramuscular; IV: intravenous; ENT: ear, nose, throat.

* The oral liquid preparation of dexamethasone (1 mg/mL) has a foul taste. Solutions for injection are
more concentrated (4 mg/mL or 10 mg/mL) and can be given orally mixed with syrup. Alternatively,
oral tablets can be crushed and mixed with syrup or pureed food.

¶ Dosing and administration are as follows:

Racemic epinephrine: 0.05 mL/kg per dose (maximum of 0.5 mL) of a 2.25% solution diluted to
3 mL total volume with normal saline (in the United States and some other countries, single-use
preservative-free bullets [ampules] of racemic epinephrine for nebulized administration are
commercially available).
L-epinephrine: 0.5 mL/kg per dose (maximum of 5 mL) of a 1 mg/mL (1:1000) preservative-free
solution (this is the parenteral preparation of epinephrine used for IM injection [eg, for
anaphylaxis]).
Administer by nebulizer over 15 minutes. Use of either product is acceptable and may be determined
by availability and institutional protocol. Nebulized epinephrine has an onset of effect within 10
minutes. Refer to UpToDate topic on management of croup for additional details.

Graphic 100744 Version 9.0

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Management of croup in outpatient and emergency department settings

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This figure summarizes our suggested approach to managing children with croup in the ED or
outpatient setting based upon the severity of symptoms. Clinical scoring systems can be used to
assess croup severity. The most commonly used scoring system is the Westley croup score, which
assesses level of consciousness, cyanosis, stridor, air entry, and retractions. This figure is intended for
use in conjunction with other UpToDate content. For additional details, including information about
the Westley croup score and a discussion of the evidence supporting the efficacy of these treatments,
refer to UpToDate topic on management of croup.

ED: emergency department; ETT: endotracheal tube; IM: intramuscular; IV: intravenous; PICU:
pediatric intensive care unit.

* Signs of impending respiratory failure include: fatigue and listlessness, profound retractions,
decreased or absent breath sounds, depressed level of consciousness, tachycardia out of proportion
to fever (note that tachycardia may also be caused by epinephrine), and/or poor color (cyanosis or
pallor).

¶ The oral liquid preparation of dexamethasone (1 mg/mL) has a foul taste. Solutions for injection are
more concentrated (4 mg/mL or 10 mg/mL) and can be given orally mixed with syrup. Alternatively,
oral tablets can be crushed and mixed with syrup or pureed food.

Δ Dosing and administration are as follows:

Racemic epinephrine: 0.05 mL/kg per dose (maximum of 0.5 mL) of a 2.25% solution diluted to
3 mL total volume with normal saline (in the United States and some other countries, single-use
preservative-free bullets (ampules) of racemic epinephrine for nebulized administration are
commercially available).
L-epinephrine: 0.5 mL/kg per dose (maximum of 5 mL) of a 1 mg/mL (1:1000) preservative-free
solution (this is the parenteral preparation of epinephrine used for IM injection [eg, for
anaphylaxis]).

Administer by nebulizer over 15 minutes. Use of either product is acceptable and may be determined
by availability and institutional protocol. Nebulized epinephrine has an onset of effect within 10
minutes. Refer to UpToDate topic on management of croup for additional details.

◊ A single dose of nebulized budesonide, if available, may provide an alternative to IM or IV

dexamethasone for children with vomiting and no IV access.

§ For children requiring inpatient admission for croup, the level of care (inpatient ward versus PICU) is
determined by croup severity. PICU care is warranted for children with any of the following:
Respiratory failure requiring endotracheal intubation
Persistent severe symptoms requiring frequent nebulized epinephrine dosing

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Underlying conditions placing the child at high risk for progressive respiratory failure (eg,
neuromuscular disease or bronchopulmonary dysplasia)

¥ Poor response to nebulized epinephrine in conjunction with high fever and toxic appearance should
prompt consideration of bacterial tracheitis. Refer to UpToDate topic on evaluation and diagnosis of
croup for a guide to the differential diagnosis.

Graphic 100747 Version 10.0

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Epiglottitis: Tripod posture

This child's "tripod" positioning (trunk leaning forward, neck hyperextended, chin thrust forward) is
caused by epiglottitis and represents the patient's attempt to maximize the patency of a significantly
obstructed upper airway. Also, note the child's toxic appearance.

Tripod positioning may also be seen in other causes of respiratory distress, such as severe asthma.

Reproduced with permission from: M Douglas Baker, MD.

Graphic 79826 Version 3.0

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Child with classic presentation of acute epiglottitis

This 4-year-old girl has epiglottitis caused by Haemophilus influenzae type b.

(A) She prefers to sit and appears anxious.

(B) The child assumes the characteristic sniffing position to maximize the patency of her airway.

Reproduced with permission from: Fleisher GR, Ludwig W, Baskin MN. Atlas of Pediatric Emergency Medicine, Lippincott
Williams & Wilkins, Philadelphia 2004. Copyright © 2004 Lippincott Williams & Wilkins.

Graphic 76538 Version 6.0

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Croup: Anteroposterior radiograph with "steeple sign"

Anteroposterior (AP) radiograph of the neck in a child with croup, demonstrating the characteristic
tapering of the upper trachea ("steeple sign"). While this finding is characteristic of croup, it can be
simulated by differing phases of respiration even in healthy children.

Courtesy of the Department of Diagnostic Imaging, Texas Children's Hospital.

Graphic 52418 Version 3.0

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Lateral neck radiograph of a child with croup

Lateral neck radiograph showing subglottic narrowing (arrow) and distended hypopharynx
(arrowheads) consistent with acute laryngotracheitis.

Courtesy of Joe Black, Diagnostic Imaging, Texas Children's Hospital.

Graphic 64727 Version 4.0

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Congenital anomalies associated with stridor

Malformation Characteristics

Nose* Nasal deformities Choanal atresia or agenesis, septum deformities, turbinate

hypertrophy, vestibular atresia, or stenosis.

Pharynx* Craniofacial Anomalies causing facial retrusion are associated with upper
anomalies airway obstruction, including Crouzon, Pierre Robin, and
Apert syndromes.

Tongue Macroglossia and glossoptosis.

Larynx Laryngomalacia Most common cause of chronic stridor in infants. Almost all
patients present by 6 weeks of age. Symptoms are more
pronounced after upper respiratory infections.

Laryngeal webs 75% located in the glottic area. Complete webs cause
respiratory distress at birth and partial webs produce stridor
weak cry, and different degrees of respiratory distress.
Associated anomalies are common.

Laryngeal cysts If located in supraglottic area, may cause respiratory distress

and stridor.

Laryngeal clefts Characterized by abnormal communication between the

larynx and pharynx, sometimes extending downward
between the trachea and esophagus. Patients may present
with aspiration, cough, swallowing difficulties, respiratory
distress, hoarse cry, or occasionally with stridor; often
associated with other congenital anomalies.

Subglottic Presents as with stridor and respiratory distress, usually

hemangioma worsening during the first few months of life. Often
associated with cutaneous hemangiomas.

Subglottic May be congenital but more often acquired secondary to

stenosis intubation. Usually located 2 to 3 mm below the glottis.

Vocal cord Idiopathic or secondary to a neurologic disorder (including

paralysis Chiari II malformation, hydrocephalus, meningomyelocele,
hypoxic cerebral palsy, and cerebral hemorrhage) [1,2] .

Trachea ¶ Tracheal stenosis Usually presents with stridor or both stridor and wheezing. I
stenosis is significant, respiratory distress occurs.

Vascular rings or 74% of vascular rings are symptomatic. The airway

slings compression usually is intrathoracic, causing expiratory
stridor. Associated anomalies are common.

Tracheomalacia Often associated with other congenital anomalies. May be

secondary to a vascular ring or cysts. Worsens with upper
respiratory infections, crying, coughing, or feeding. May
cause severe spells with cyanosis.

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Bronchi and Bronchogenic cyst May occur at any point throughout the tracheobronchial
distal airways ¶ tree. Typically present during childhood with recurrent
coughing, wheezing, or pneumonia, but may become
symptomatic during infancy or adulthood or present as an
incidental finding on chest radiographs.

* Noise generated from the nose or pharynx is typically low in pitch and is referred to as snoring or
stertor.

¶ Noise generated from the trachea, bronchi, or distal airways is mostly wheezing.

References:
1. Nisa L, Holtz F, Sandu K. Paralyzed neonatal larynx in adduction. Case series, systematic review and analysis. Int J
Pediatr Otorhinolaryngol 2013; 77:13.
2. Holinger LD, Holinger PC, Holinger PH. Etiology of bilateral abductor vocal cord paralysis: a review of 389 cases. Ann
Otol Rhinol Laryngol 1976; 85:428.

Graphic 62718 Version 8.0

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Noncongenital causes of stridor in children

Typical age of presentation

Infants
Inspirato
Cause and Preschool
School- stridor
Neonate toddlers (3 to 5 Adolescents
aged
(6 to 24 years)
months)

Acute or subacute onset

Viral croup X X +
(laryngotracheitis) ¶

Spasmodic croup X X +

Bacterial X X X +
tracheitis ¶

Epiglottitis X X X +

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Retropharyngeal X X +/–
abscess

Peritonsillar X X X +/–
abscess

Inducible laryngeal X X +
obstruction (vocal
cord dysfunction or
paradoxical vocal
cord motion)

Foreign body X X +
aspiration Δ

Anaphylaxis X X X X +

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Airway burn X X X X +

Postextubation X X X X +

Therapeutic X X
hypothermia

Chronic

Congenital X X +/–
anomalies

Vocal cord X X X X X +
paralysis ¶

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Subglottic stenosis X X X X X +

Tumor X X X X X +/–

Recurrent X X +
respiratory
papillomatosis

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Hypocalcemic X X +/–
laryngeal spasm

+: usually present; +/–: may or may not be present; PICU: pediatric intensive care unit; URI: upper
respiratory tract infection.

* Any obstructive process that leads to a fixed airway narrowing will produce both inspiratory and
expiratory noise.

¶ Onset either acute or subacute/gradual.

Δ Foreign body aspiration can occur in any age group but is most common in toddlers and preschool-
aged children.

Graphic 101526 Version 8.0

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Epiglottitis: Lateral radiograph

Lateral neck radiograph demonstrating swollen epiglottis (arrow) and aryepiglottic folds (asterisks) in
a child with epiglottitis due to Haemophilus influenzae type b. The swollen epiglottis is often called a
"thumb sign."

Courtesy of Evelyn Y Anthony, MD, Wake Forest University School of Medicine.

Graphic 67878 Version 7.0

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Bacterial tracheitis: Lateral neck radiograph

Lateral neck radiograph showing intraluminal membranes and tracheal wall irregularity consistent
with bacterial tracheitis.

Courtesy of R Paul Guillerman, MD, Department of Radiology, Baylor College of Medicine.

Graphic 80331 Version 6.0

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Subglottic hemangioma in a young child

(A) Subglottic hemangioma (arrow) seen from above the vocal cords. There is near-complete
obstruction of the subglottic airway.

(B) Normal pediatric larynx.

AC: arytenoid cartilage; AF: aryepiglottic fold; E: epiglottis; VC: vocal cord.

Panel A is courtesy of Anna Messner, MD.

Panel B is reproduced with permission from: Nagdev A. Airway, breathing, circulation: Normal airway. In: Greenberg's Text-
Atlas of Emergency Medicine, Greenberg MI, Hendrickson RG, Silverberg M, et al (Eds), Lippincott Williams & Wilkins,
Philadelphia 2005. Copyright © 2005 Lippincott Williams & Wilkins. [Link].

Graphic 108045 Version 3.0

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Endoscopic view of subglottic hemangioma

Note the red-blue sessile lesion in the posterolateral subglottis.

Courtesy of Glenn C Isaacson, MD, FAAP, FACS.

Graphic 73275 Version 1.0

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Divulgaciones del colaborador

Charles R Woods, MD, MS No hay relaciones financieras relevantes con empresas no elegibles para
revelar. Anna H Messner, MD No hay relaciones financieras relevantes con empresas no elegibles
para revelar. Sheldon L Kaplan, MD Subvención/Investigación/Apoyo a ensayos clínicos: Pfizer
[Streptococcus pneumoniae]. Otros intereses financieros: Elsevier [honorarios de libros de texto –
Enfermedades infecciosas pediátricas]; Pfizer [Honorario por una charla – PCV13]. Todas las relaciones
financieras relevantes enumeradas han sido mitigadas. Joshua Nagler, MD, MHPEd No hay
relaciones financieras relevantes con empresas no elegibles para revelar. Carrie Armsby, MD, MPH
No hay relaciones financieras relevantes con empresas no elegibles para revelar.

El grupo editorial revisa las divulgaciones de los contribuyentes para detectar conflictos de intereses.
Cuando se encuentran, estos se abordan mediante un proceso de revisión de varios niveles y mediante
requisitos de referencias que se deben proporcionar para respaldar el contenido. Se requiere que
todos los autores tengan contenido con las referencias adecuadas y deben cumplir con los estándares
de evidencia de UpToDate.

Política de conflicto de intereses

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