Diagnosis of Stridor in Children - American Family Physician http://www.aafp.org/afp/1999/1115/p2289.

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Diagnosis of Stridor in Children

ALEXANDER K.C. LEUNG, M.B.B.S., and HELEN CHO, M.D., University of Calgary and the Asian Medical Centre, Alberta, Canada
Am Fam Physician. 1999 Nov 15;60(8):2289-2296.

Stridor is a sign of upper airway obstruction. In children, laryngomalacia is the most common cause of chronic stridor, while croup is the most common
cause of acute stridor. Generally, an inspiratory stridor suggests airway obstruction above the glottis while an expiratory stridor is indicative of obstruction
in the lower trachea. A biphasic stridor suggests a glottic or subglottic lesion. Laryngeal lesions often result in voice changes. A child with extrinsic airway
obstruction usually hyperextends the neck. The airway should be established immediately in children with severe respiratory distress. Treatment of stridor
should be directed at the underlying cause.

The word stridor is derived from the Latin word stridulus, which means creaking, whistling or grating. Stridor is a harsh, vibratory sound of variable pitch caused by
partial obstruction of the respiratory passages that results in turbulent airflow through the airway. Although stridor may be the result of a relatively benign process, it may
also be the first sign of a serious and even life-threatening disorder. Stridor is a distressing symptom to its victims and their parents, and presents a diagnostic challenge
to physicians. As such, stridor demands immediate attention and thorough evaluation to uncover the precise underlying cause. This article reviews the etiology of stridor
in children and suggests an approach to evaluating and managing the problem.

Etiology and Clinical Manifestations

Causes of stridor in children according to the site of obstruction are listed in Table 1.

View/Print Table

TABLE 1
Causes of Stridor in Children According to Site of Obstruction

Nose and pharynx

Choanal atresia

Lingual thyroid or thyroglossal cyst

Macroglossia

Micrognathia

Hypertrophic tonsils/adenoids

Retropharyngeal or peritonsillar abscess

Larynx

Laryngomalacia

Laryngeal web, cyst or laryngocele

Laryngotracheobronchitis (viral croup)

Acute spasmodic laryngitis (spasmodic croup)

Epiglottitis

Vocal cord paralysis

Laryngotracheal stenosis

Intubation

Foreign body

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Cystic hygroma

Subglottic hemangioma

Laryngeal papilloma

Angioneurotic edema

Laryngospasm (hypocalcemic tetany)

Psychogenic stridor

Trachea

Tracheomalacia

Bacterial tracheitis

External compression

NOSE AND PHARYNX

Choanal Atresia. Choanal atresia, although rare, is the most common congenital anomaly of the nose. Choanal atresia results from a persistence of the bucconasal
membrane in the posterior nares at the posterior margin of the hard palate. Unilateral choanal atresia does not usually cause any clinical problem unless the
contralateral side is obstructed, for example, as a result of an upper respiratory tract infection. Bilateral choanal atresia is a life-threatening condition and a well-
recognized cause of airway obstruction and respiratory distress in the newborn.

Lingual Thyroid or Thyroglossal Cyst. A lingual thyroid is due to a failure of descent of the thyroid gland. A thyroglossal cyst arises from remnants of the thyroglossal
duct.

Clinically, a thyroglossal cyst presents as a smooth, discrete mass in the midline of the neck anywhere from just above the hyoid bone to the thyroid gland. Typically, the
mass moves upward when the tongue is protruded. A lingual thyroid or thyroglossal cyst, if large enough, may cause airway obstruction and stridor.

Macroglossia. Macroglossia may result from Beckwith-Wiedemann syndrome, Down syndrome, glycogen storage disease and congenital hypothyroidism. The tongue
may be large enough to obstruct the hypopharynx.

Micrognathia. Obstruction of the oropharynx may result from micrognathia by posterior displacement of the tongue. The obstruction typically worsens in the supine
position as gravity pulls the tongue farther back.1 Micrognathia is a classic feature of Pierre-Robin syndrome (Figure 1), Treacher Collins syndrome and Hallermann-
Streiff syndrome.

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FIGURE 1.

A newborn infant with Pierre-Robin syndrome. Note the micrognathia.

Hypertrophic Tonsils/Adenoids. Tonsillar or adenoidal tissue may be so large that the supraglottic airway becomes obstructed. Characteristically, the stridor is most
noticeable during sleep.

Retropharyngeal or Peritonsillar Abscess. A retropharyngeal or peritonsillar abscess may cause stridor as edema of the hypopharynx develops. Although both may
present with fever, drooling and dysphagia, the child with a peritonsillar abscess may have difficulty opening the mouth (trismus) because of spasm of the pterygoid
muscles, while the child with a retropharyngeal abscess often keeps the neck hyperextended.

LARYNX
Laryngomalacia. Laryngomalacia is the most common cause of chronic stridor in children younger than two years. It has a male-to-female ratio of approximately 2:1.1
The condition is due to an intrinsic defect or delayed maturation of supporting structures of the larynx. The airway is partially obstructed during inspiration by the
prolapse of the flaccid epiglottis, arytenoids and aryepiglottic folds. The inspiratory stridor is usually worse when the child is in a supine position, when crying or agitated,
or when an upper respiratory tract infection occurs.2

Laryngeal Web, Cyst or Laryngocele. A laryngeal web results from a failure of the embryonic airway to recanalize.3 Most laryngeal webs occur at the level of the vocal
cords and are anterior in location.3 A laryngeal cyst typically occurs in the aryepiglottic fold or in the epiglottis. A laryngeal cyst usually contains mucus from minor

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salivary glands. A laryngocele arises as a dilatation of the saccule of the laryngeal ventricle. A laryngeal web, cyst or laryngocele may present with stridor, usually at
birth or soon after.

Laryngotracheobronchitis (Viral Croup). The most common cause of acute stridor in childhood is laryngotracheobronchitis, or viral croup. The condition is caused most
commonly by parainfluenza virus, but it can also be caused by influenza virus types A or B, respiratory syncytial virus and rhinoviruses.4 Croup usually occurs in
children six months to six years of age, with a peak incidence in the second year of life. The male-to-female ratio is approximately 3:2.4 Croup is usually preceded by an
upper respiratory tract infection of several days' duration. A low-grade fever, barking cough, inspiratory stridor and hoarseness then develop. Symptoms are
characteristically worse at night and are aggravated by agitation and crying.

Acute Spasmodic Laryngitis (Spasmodic Croup). Spasmodic croup may mimic laryngotracheobronchitis, except that it is usually not preceded by an upper respiratory
tract infection, and it often occurs with a sudden onset at night. Allergy, psychologic factors and gastroesophageal reflux may trigger spasmodic croup. Spasmodic croup
may be recurrent.

Epiglottitis. In children, epiglottitis is almost always caused by Haemophilus influenzae type b.5 In recent years, the occurrence of epiglottitis has been reduced
dramatically by the widespread use of the H. influenzae type b vaccine.5 Epiglottitis usually occurs in children two to seven years of age, with a peak incidence in three-
year-olds.6

The male-to-female ratio is approximately 3:2. The disease is characterized by an abrupt onset of high fever, toxicity, agitation, stridor, dyspnea, muffled voice,
dysphagia and drooling. The older child may prefer to sit leaning forward with the mouth open and the tongue somewhat protruding. There is no spontaneous cough. An
edematous, cherry red epiglottis, visualized in a controlled environment, is the hallmark of epiglottitis.

Vocal Cord Paralysis. Unilateral vocal cord paralysis occurs more often on the left side because of the longer course of the recurrent laryngeal nerve, which makes it
more vulnerable to injury. Unilateral dysfunction may result from birth trauma, trauma during thoracic surgery or compression by mediastinal masses of cardiac,
pulmonary, esophageal, thyroid or lymphoid origin. Bilateral vocal cord paralysis is more commonly associated with central nervous system problems including perinatal
asphyxia, cerebral hemorrhage, hydrocephalus, bulbar injury and Arnold-Chiari malformation.1 The vocal cords may also be injured by direct trauma from endotracheal
intubation attempts or during deep airway suction. In vocal cord paralysis, the stridor is typically biphasic. In unilateral vocal cord paralysis, the infant's cry is weak and
feeble; however, there is usually no respiratory distress. In bilateral vocal cord paralysis, the voice is usually of good quality, but there is marked respiratory distress.3

Laryngotracheal Stenosis. Laryngotracheal stenosis is a congenital or acquired narrowing of the airway representing a continuum of disease that may affect the glottis,
subglottis and trachea.7 The term subglottic stenosis was often used incorrectly in the past.7 The most common cause of acquired laryngotracheal stenosis is
endotracheal intubation, especially in low-birth-weight infants who require prolonged intubation and ventilation. Other causes include blunt trauma to the neck, high
tracheotomy, cricothyrotomy, external compression of the airway and gastroesophageal reflux.7

Intubation. Intubation may result in vocal cord paralysis, laryngotracheal stenosis, subglottic edema and laryngospasm. Any of the above, alone or in combination, may
lead to airway obstruction and stridor.

Foreign Body. Foreign body aspiration is a common cause of acute stridor. The peak incidence is between one and two years of age.

The foreign body is usually food. A history of aspiration or choking can be obtained in 90 percent of cases. The most common symptoms of laryngotracheal foreign
bodies are cough, stridor and dyspnea, whereas those of bronchial foreign bodies are cough, decreased breath sounds, wheezing and dysphagia. Stridor may occur
because of direct compression of the trachea by large objects lodged in the postcricoid region, paraesophageal inflammation, abscess formation or direct extension of
the inflammatory process into the trachea by ulceration and fistula formation.

Cystic Hygroma. A cystic hygroma is a collection of lymphatic sacs that contain clear, colorless lymph. The lesion is congenital and probably represents a cluster of
lymph channels that failed to connect into the normal lymphatic pathway. It commonly occurs in the neck area (Figure 2). The tumor, as it grows, may cause tracheal
compression and stridor.

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FIGURE 2.

A two-year-old child with a cystic hygroma on the left side of the neck.

Subglottic Hemangioma. A subglottic hemangioma occurs more commonly in girls, with a female-to-male ratio of 2:1.8 The lesion is usually submucosal. No color
change or, at most, a slight bluish discoloration is evident. It is frequently associated with hemangiomas elsewhere on the body. The stridor is biphasic and exaggerated
by crying or straining as the lesion tends to become engorged.8

Laryngeal Papilloma. This is the most common laryngeal neoplasm in children and usually results from vertical transmission of human papillomavirus at birth. Usually
multiple, papillomas most commonly occur in the vocal cords and ventricular bands but can involve any part of the larynx. They are most common in children between
two and four years of age. The usual presenting symptom is hoarseness, but some patients have stridor and other signs of laryngeal obstruction.

Angioneurotic Edema. Angioneurotic edema may result in acute swelling of the upper airway with resultant stridor and dyspnea. Swelling of the face, tongue or pharynx

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may also be present.

Laryngospasm (Hypocalcemic Tetany). Hypocalcemia may rarely cause laryngospasm and stridor.9 Other features include irritability, tremors, twitchings and carpopedal
spasm.

Psychogenic Stridor. Stridor may be caused by emotional stress or it may be a manifestation of a conversion disorder. Vocal cord malfunction associated with emotional
stress may result in inspiratory or expiratory stridor.10

Characteristically, the onset of stridor is sudden but without the expected amount of distress. The neck is often held in a flexed position rather than in an extended
position.

TRACHEA
Tracheomalacia. Tracheomalacia is characterized by abnormal tracheal collapse secondary to inadequate cartilaginous and myoelastic elements supporting the trachea.
Tracheal narrowing occurs with expiration and causes stridor.11 The stridor may not be present at birth but appears insidiously after the first weeks of life. The stridor is
usually aggravated by respiratory tract infections and agitation.11

Bacterial Tracheitis. Bacterial tracheitis is usually caused by Staphylococcus aureus, although it can also be caused by H. influenzae type b and Moraxella catarrhalis.
Most patients are younger than three years of age. Bacterial tracheitis usually follows an upper respiratory tract infection. The patient then becomes seriously ill with
high fever, toxicity and respiratory distress.

External Compression. Tracheal compression may result from vascular anomalies such as double aortic arch, right aortic arch with left ligamentum arteriosum,
anomalous innominate artery, anomalous left common carotid artery, anomalous left pulmonary artery or aberrant subclavian artery. The child may prefer to keep the
neck hyperextended. The stridor resulting from tracheal compression is often aggravated by feeding.

The trachea may also be compressed by a mediastinal cyst, teratoma, lymphoma or lymphadenopathy.

Clinical Evaluation
A thorough history (Table 2) and physical examination (Table 3) are important in the evaluation of children with stridor. Figure 3 presents an algorithm helpful in
evaluating stridor in children.

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TABLE 2
Historical Information in the Evaluation of Stridor in Children

HISTORICAL DATA POSSIBLE ETIOLOGY

Age of onset

Birth Vocal cord paralysis, congenital lesions such as choanal atresia, laryngeal web and vascular ring

4 to 6 weeks Laryngomalacia

1 to 4 years Croup, epiglottitis, foreign body aspiration

Chronicity

Acute onset Foreign body aspiration, infections such as croup and epiglottitis

Long duration Structural lesion such as laryngomalacia, laryngeal web or larynogotracheal stenosis

Precipitating factors

Worsening with straining or crying Laryngomalacia, subglottic hemangioma

Worsening in a supine position Laryngomalacia, tracheomalacia, macroglossia, micrognathia

Worsening at night Viral or spasmodic croup

Worsening with feeding Tracheoesophageal fistula, tracheomalacia, neurologic disorder, vascular compression

Antecedent upper respiratory tract infection Croup, bacterial tracheitis

Choking Foreign body aspiration, tracheoesophageal fistula

Associated symptoms

Barking cough Croup

Brassy cough Tracheal lesion

Drooling Epiglottitis, foreign body in esophagus, retropharyngeal or peritonsillar abscess

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HISTORICAL DATA POSSIBLE ETIOLOGY

Weak cry Laryngeal anomaly or neuromuscular disorder

Muffled cry Supraglottic lesion

Hoarseness Croup, vocal cord paralysis

Snoring Adenoidal or tonsillar hypertrophy

Dysphagia Supraglottic lesion

Past health

Endotracheal intubation Vocal cord paralysis, laryngotracheal stenosis

Birth trauma, perinatal asphyxia, cardiac problem Vocal cord paralysis

Atopy Angioneurotic edema, spasmodic croup

Family history

Down syndrome Down syndrome

Hypothyroidism Hypothyroidism

Psychosocial history

Psychosocial stress Psychogenic stridor

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TABLE 3
Physical Examination Findings in the Evaluation of Stridor in Children
PHYSICAL FINDINGS POSSIBLE ETIOLOGY

General

Cyanosis Cardiac disorder, hypoventilation with hypoxia

Fever Underlying infection

Toxicity Epiglottitis

Tachycardia Cardiac failure

Bradycardia Hypothyroidism

Quality of stridor

Inspiratory stridor Obstruction above glottis

Expiratory stridor Obstruction at or below lower trachea

Biphasic stridor Glottic or subglottic lesion12

Position of the child

Hyperextension of the neck Extrinsic obstruction at or above larynx13

Leaning over, drooling Epiglottitis

Lessening of stridor in prone position Laryngomalacia

Chest findings

Prolonged inspiratory phase Laryngeal obstruction

Prolonged expiratory phase Tracheal obstruction

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PHYSICAL FINDINGS POSSIBLE ETIOLOGY

Unilateral decreased air entry Foreign body in ipsilateral bronchus

Associated signs

Arrhythmias, significant heart murmurs, abnormal heart sounds Structural heart disease

Cutaneous hemangiomas Subglottic hemangioma

Peripheral neuropathy Vocal cord paralysis

Urticaria/angioneurotic edema Angioneurotic edema

Information from references 12 and 13.

View/Print Figure

Stridor in Children

FIGURE 3.

Algorithm for evaluating stridor in children based on clinical findings.

Adapted with permission from Handler SD. Stridor. In: Fleisher GR, Ludwig S, eds. Textbook of pediatric emergency medicine. Baltimore: Williams & Wilkins, 1993:4748.

Diagnostic Studies
Anteroposterior and lateral radiographic views of the neck are useful in the assessment of adenoidal and tonsillar size, epiglottic size and shape, retropharyngeal profile
and subglottic and tracheal anatomy.12 High-kilovoltage technique is preferable because it gives better visualization of the soft tissue. The lateral neck radiograph must
be taken with good extension of the neck and during inspiration so that the pharyngeal soft tissues are not mistaken for a retropharyngeal mass.13 Anteroposterior and
lateral views of the chest are useful in the detection of radio-opaque foreign body and concomitant pulmonary disease. If foreign body aspiration is suspected and the
preliminary films are negative, inspiratory and expiratory films should be obtained to look for air trapping behind the foreign body, producing a hyperlucent lung field in
the ipsilateral side and a shift of the mediastinum to the opposite side.

A barium swallow is a useful method if vascular compression or gastroesophageal reflux is suspected. Gastrografin should be used as the contrast medium if
tracheoesophageal fistula is suspected. Videofluoroscopy is useful in the diagnosis of tracheomalacia, foreign body aspiration and vocal cord dysfunction. Computed
tomographic (CT) scan and magnetic resonance imaging (MRI) may be obtained to visualize the airway and the surrounding soft tissue structures, including any
evidence of vascular compression.

Direct examination of the airway is often necessary to confirm the diagnosis and is essential in children with persistent stridor. Flexible fiberoptic bronchoscopy is widely
used in the evaluation of airways in children. However, rigid bronchoscopy performed under general anesthesia gives a better view of the airway, especially the part
below the level of the vocal cords. Rigid bronchoscopy also allows tissue biopsy and removal of foreign bodies using forceps.

A complete blood count is useful if an infection is suspected. Determination of the erythrocyte sedimentation rate is helpful in assessing for the presence of an infection.
Depending on the degree of respiratory distress, arterial blood gas determination may be necessary to assess the degree of hypoxia and ventilatory status. An
electrocardiogram and echocardiogram are indicated if significant heart murmurs are present or when structural heart disease is suspected.

Treatment
Treatment of stridor should be directed at the underlying cause.

The airway should be established immediately in children with severe respiratory distress or actual airway obstruction. This can be done by endotracheal intubation.
After adequate ventilation is achieved by intubation, tracheostomy can be performed if deemed necessary. Supportive measures may include oxygen, humidified air,

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intravenous fluids, suction and aerosol treatments with steroids and beta-adrenergic drugs.

The Authors show all author info

ALEXANDER K.C. LEUNG, M.B.B.S., is clinical associate professor of pediatrics at the University of Calgary, a pediatric consultant at the Alberta Children's Hospital
and medical director of the Asian Medical Centre, which is affiliated with the University of Calgary Medical Clinic in Alberta, Canada. He graduated from the University of
Hong Kong and completed an internship at Queen Mary Hospital, both in Hong Kong. He completed a residency in pediatrics at the University of Calgary....

REFERENCES show all references

1. Simon NP. Evaluation and management of stridor in the newborn. Clin Pediatr. 1991;30:2116....

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