Approach to the management of croup

Author Charles R Woods, MD, MS Section Editor Ralph D Feigin, MD Ellen M Friedman, MD Deputy Editors Mary M Torchia, MD

Last literature review version 16.1: January 2008 | This Topic Last Updated: February 1, 2008 (More)

INTRODUCTION Croup (laryngotracheitis) is a respiratory illness characterized by inspiratory stridor, barking cough, and hoarseness. It typically occurs in children 3 months to 3 years of age and is caused by parainfluenza virus. (See "Clinical features, evaluation, and diagnosis of croup").

The treatment of croup has changed significantly since the 1980s. Corticosteroids and nebulized epinephrine have become the cornerstones of therapy. Substantial clinical evidence supports the efficacy of these interventions [1-5] . The impact also is evident in the decrease in annual hospital admissions for croup in children in the United States between 19791982 and 1994-1997 (from 2.8 to 2.1 per 1000 for children <1 year and from 1.8 to 1.2 per 1000 children for children 1 to 4 years) [6] .

The approach to the management of croup will be discussed below. The clinical features and evaluation of croup and the evidence supporting the use of the

pharmacologic and supportive interventions included below are discussed separately. (See "Clinical features, evaluation, and diagnosis of croup" and see "Pharmacologic and supportive interventions for croup").

OVERVIEW The treatment of croup and the setting in which the child is initially evaluated depend upon the severity of symptoms and the presence of risk factors for rapid progression. There is no definitive treatment for the viruses that cause croup. Pharmacologic therapy is directed toward decreasing airway edema and supportive care is directed toward the provision of respiratory support and the maintenance of hydration. (See "Pharmacologic and supportive interventions for croup").

Most children with croup who seek medical attention have a mild, self-limited illness and can be successfully managed as outpatients. The clinician must be able to identify children with mild symptoms, who can be safely managed at home and those with moderate to severe croup or rapidly progressing symptoms who require full evaluation and possible treatment in the office or emergency department setting.

Severity assessment The severity of croup is often determined by the clinical scoring systems. Although there are a number of validated croup scoring systems, the Westley croup score [1] has been the most extensively studied; it is described below. No matter which system is used to assess severity, the presence of chest wall retractions and stridor at rest are the two most critical clinical features.

Westley croup score The elements of the Westley croup score describe key features of the physical examination [1] . Each element is assigned a score, as illustrated below: 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

The total score ranges from 0 to 17. Mild croup is defined by a Westley croup score of 2. Typically these children have a barking cough, hoarse cry, but no stridor at rest. Children with mild croup may have stridor when upset or crying

(ie, agitated) and either none, or only mild chest wall/subcostal retractions [7,8] . Moderate croup is defined by a Westley croup score of 3 to 7. Children with moderate croup have stridor at rest, at least mild retractions, and may have other symptoms or signs of respiratory distress, but little or no agitation [7,8] . Severe croup is defined by a Westley croup score of 8. Children with severe croup have significant stridor at rest, although stridor may decrease with worsening upper airway obstruction and decreased air entry [7,8] . Retractions are severe (including indrawing of the sternum) and the child may appear anxious, agitated, or fatigued. Prompt recognition and treatment of children with severe croup are paramount.

Respiratory failure Croup occasionally results in significant upper airway obstruction with impending respiratory failure, heralded by the following signs [7,9] : Fatigue and listlessness Marked retractions (although retractions may decrease with increased obstruction and decreased air entry) Decreased or absent breath sounds Depressed level of consciousness Tachycardia out of proportion to fever Cyanosis or pallor

PHONE TRIAGE The first contact with the health-care system regarding a child with symptoms of croup may occur by phone. When assessing patients by phone, the health-care provider must distinguish children who need immediate medical attention or further evaluation from those who can be managed at home. Children who need further evaluation include those who have: Stridor at rest An abnormal airway (eg, subglottic narrowing from care in the neonatal intensive care unit) Previous episodes of moderate to severe croup Medical conditions that predispose to respiratory failure (eg, neuromuscular disorders) Rapid progression of symptoms (ie, symptoms of upper airway obstruction after less than 12 hours of illness) Inability to tolerate oral fluids Parental concern that cannot be relieved by reassurance Prolonged symptoms (more than three to seven days) or an atypical course (perhaps indicating an alternative diagnosis) (See "Clinical features, evaluation, and diagnosis of croup", section on Differential diagnosis)

Patients who are assessed by phone to have mild symptoms and none of the above indications for further evaluation can be managed at home. (See "Home treatment" below).

MILD CROUP Children with mild symptoms, defined by a Westley croup score of 2, should be treated symptomatically with humidity, fever reduction, and oral fluids. Many such children can be managed by phone, provided that none of the criteria for further evaluation described above are present.

Home treatment The caregivers of children with mild croup who are managed at home should be instructed in provision of supportive care including mist, antipyretics, and encouragement of fluid intake.

In acute situations and for short periods of time, caregivers may try sitting with the child in a bathroom filled with steam generated by running hot water from the shower to improve symptoms. This may help reassure parents that "something" is being done to reverse the symptoms, and anecdotal evidence supports some value of this measure.

Exposure to cold night air also may lessen symptoms of mild croup, although this has never been systematically studied. If parents or caregivers wish to use humidifiers at home, only those that produce mist at room temperature should be used to avoid the risk of burns from steam or the heating element.

Patients who are managed at home should receive a follow-up phone call; caregivers should receive instructions regarding indications to seek medical attention, including [7] : Difficulty breathing Pallor or cyanosis Severe coughing spells Drooling or difficulty swallowing Fatigue Worsening course Fever (>38.5C) Prolonged symptoms (longer than seven days) Stridor at rest Suprasternal retractions

Caregivers also should be provided with some guidance regarding when it is safe for them to drive the child to the emergency department and when to call for emergency medical services. Emergency medical services should provide transportation for children who are severely agitated, cyanotic, struggling to breathe, or lethargic [7] .

Outpatient treatment Children who are seen in the office or emergency department with mild croup may require little or no therapy, or may have improvement with humidified air. Randomized controlled trials have

demonstrated that treatment with a single dose of oral dexamethasone (0.15 to 0.6 mg/kg, maximum dose 10 mg) may reduce the need for reevaluation, shorten the course, improve duration of the child's sleep, and reduce parental stress [10,11] .

We suggest that children with mild croup who are seen in the outpatient setting be treated with a single dose of oral dexamethasone (0.6 mg/kg). Treatment of such children in the late morning or early afternoon hours may prevent worsening of symptoms as evening approaches. However, anticipatory guidance about potential worsening and when to seek care or return for followup also is reasonable. (See "Pharmacologic and supportive interventions for croup", section on Dexamethasone).

Children with mild croup who are tolerating fluids and have not received nebulized epinephrine can be sent home after specific follow-up (which may occur by phone) has been arranged and the caregiver has received instructions regarding home care and indications to seek medical attention as described above. (See "Home treatment" above).

MODERATE TO SEVERE CROUP Children with moderate croup (Westley croup score 3 to 7, stridor and retractions at rest without agitation) should be evaluated in the emergency department or office (provided the office is equipped to handle acute upper airway obstruction). Children with severe croup (Westley croup score 8, stridor and retractions at rest with agitation, lethargy, or cyanosis, marked sternal wall indrawing) should be evaluated in the emergency department. Such children require aggressive therapy, monitoring, and supportive care.

Supportive care Supportive care for children with moderate/severe croup includes administration of humidified air or humidified oxygen as indicated for hypoxemia (oxygen saturation <92 percent in room air) or respiratory distress. (See "Pharmacologic and supportive interventions for croup").

The child with severe croup must be approached cautiously, as any increase in anxiety may worsen airway obstruction. The parent or caregiver should be instructed to hold and comfort the child and to administer humidified oxygen. Nebulized epinephrine should be added as quickly as possible, as described

below. In the meantime, health-care providers should continuously observe the child and be prepared to provide bag mask ventilation and advanced airway techniques if the condition worsens. (See "Advanced airway management in children").

Monitoring Monitoring should include pulse oximetry and close observation of respiratory status including level of consciousness, stridor, cyanosis, air entry, and retractions. Trends in ventilation can be monitored noninvasively with capnography if capnography is available and the child will tolerate the nasal prongs [12] .

Fluids Administration of intravenous fluids may be necessary in some children. Fever and tachypnea may increase fluid requirements and respiratory difficulty may prevent the child from achieving adequate oral intake. (See "Maintenance fluid therapy in children").

Intubation Endotracheal intubation is required in less than 1 percent of those who are seen in the emergency department and 2 to 6 percent of those who are hospitalized [13-16] . The need for intubation should be anticipated in children with progressive respiratory failure so that the procedure can be performed in as controlled a setting as possible. A tracheal tube that is 0.5 to 1 mm smaller than would typically be used may be required. (See "Respiratory failure" above and see "Advanced airway management in children", section on Size of endotracheal tube).

Pharmacotherapy The benefits of corticosteroids and nebulized epinephrine for moderate to severe croup have been demonstrated in meta-analysis and randomized controlled trials, respectively [1,17-19] . Specific pharmacologic intervention depends upon the severity of symptoms: For children with mild stridor at rest and mild retractions, we recommend administration of dexamethasone (0.6 mg/kg, maximum of 10 mg), by the least invasive route possible: oral if oral intake is tolerated, intravenous if IV access has been established, IM if oral intake is not tolerated and IV access has not been established. The oral preparation of dexamethasone (1 mg per mL) has a foul taste. The intravenous preparation is more concentrated (4 mg per mL) and can be given orally mixed with syrup [7,20-22] . Nebulized budesonide (as described below) is another option, particularly for children who are vomiting. (See "Pharmacologic and supportive interventions for croup", section on

Corticosteroids). For children with moderate stridor at rest and moderate retractions, or more severe symptoms, we recommend nebulized epinephrine in addition to dexamethasone:

- Racemic epinephrine is administered as 0.05 mL/kg per dose (maximum of 0.5 mL) of a 2.25 percent solution diluted to 3 mL total volume with normal saline. It is given via nebulizer over 15 minutes.

- L-epinephrine is administered as 0.5 mL/kg per dose (maximum of 5 mL) of a 1:1000 dilution. It is given via nebulizer over 15 minutes.

Nebulized epinephrine can be repeated every 15 to 20 minutes. The administration of three or more doses within a two- to three-hour time period should prompt initiation of close cardiac monitoring if this is not already underway. (See "Pharmacologic and supportive interventions for croup", section on Nebulized epinephrine).

Children who receive nebulized epinephrine should also receive dexamethasone by the least invasive route that can be accomplished, as described above. (See "Pharmacologic and supportive interventions for croup", section on Corticosteroids).

Although it is not routinely indicated in the treatment of croup, nebulized budesonide (2 mg [2 mL solution] via nebulizer) may provide an alternative to IM or IV dexamethasone for children with vomiting or severe respiratory distress [7] . In children with severe respiratory distress, budesonide may be mixed with epinephrine and administered simultaneously [7] . (See "Pharmacologic and supportive interventions for croup", section on Budesonide).

Observation Children with moderate/severe croup should be observed after pharmacologic intervention. During the observation period, children should be encouraged to drink. Children who have received nebulized epinephrine and dexamethasone with good response should be observed for at least three to four hours [23-26] . Croup symptoms usually improve within 30 minutes of administration of nebulized epinephrine [27,28] , but may return to baseline as

the effects of epinephrine wear off (usually by two hours). Children who received dexamethasone and remain symptomatic should be observed for at least four hours before deciding whether they require hospital admission (as the effect of dexamethasone may not be apparent for several hours) [7] .

Discharge to home Many children with moderate/severe croup have symptomatic improvement after treatment with nebulized epinephrine and/or corticosteroids.

After three to four hours of observation, children who remain comfortable may be discharged home if they meet the following criteria [23-26] : No stridor at rest Normal pulse oximetry Good air exchange Normal color Normal level of conscious Demonstrated ability to tolerate fluids by mouth Caregivers understand the indications for return to care and would be able to return if necessary

Before discharge, follow-up with the primary care provider should be arranged within the next 24 hours. Instructions regarding home treatment should be provided. (See "Home treatment" above).

About 5 percent of children well enough for discharge from the emergency department after receiving corticosteroids and nebulized epinephrine treatments may be expected to return for care. Relapse within 24 hours is unlikely in those who have minimal symptoms at the time of discharge [29] .

Hospitalization

Indications Children with moderate/severe croup whose condition worsens or fails to improve as expected after treatment with nebulized epinephrine and corticosteroids should be admitted to the hospital for repeated doses of nebulized epinephrine, observation, and supportive care. Poor response to nebulized epinephrine in conjunction with high fever and toxic appearance should prompt consideration of bacterial tracheitis [7] . (See "Clinical features, evaluation, and diagnosis of croup", section on Differential diagnosis).

Additional factors that influence the decision regarding admission include [7,30] : Need for supplemental oxygen Moderate retractions and tachypnea, indicating increased work of breathing, which may lead to respiratory fatigue and failure Degree of response to initial therapies "Toxicity" or clinical picture suggesting serious secondary bacterial infection Poor oral intake and degree of dehydration Young age, particularly younger than 6 months Ability of the family to comprehend the instructions regarding recognition of features that indicate the need to return for care Ability of the family to return for care (eg, distance from home to care site, weather/travel conditions) Recurrent visits to the ED within 24 hours

Interventions Children who are admitted to the hospital should continue to be monitored for heart rate and oxygen saturation and to receive humidified oxygen as necessary. Capnography, if it is available, is a useful technique for monitoring ventilation if the child will tolerate nasal prongs. If the child is unable to tolerate oral intake, maintenance intravenous fluids should be administered.

Pharmacologic interventions for hospitalized patients may include nebulized epinephrine for persisting severe respiratory distress. Nebulized epinephrine can be repeated every 15 to 20 minutes, as described above. (See "Pharmacotherapy" above).

However, children who require repeated doses of epinephrine (eg, three or more doses within two to three hours, or ongoing administration more frequently than every one to two hours) should be admitted/transferred to an intensive care unit or other setting where appropriately close monitoring can be accomplished.

Repeat doses of corticosteroids are not necessary on a routine basis, and may have adverse effects. Moderate to severe symptoms that persist for more than a few days should prompt investigation for other causes of airway obstruction. (See "Clinical features, evaluation, and diagnosis of croup", section on Differential diagnosis).

Infection control Children who are admitted to the hospital with croup should be managed with contact precautions (ie, gown and gloves for contact), particularly if parainfluenza or respiratory syncytial virus is the suspected etiology. If influenza is suspected, droplet isolation measures (ie, respiratory mask within three feet) also should be followed. (See "General principles of infection control").

Discharge criteria Children who require hospital admission may be discharged when they meet the following criteria: No stridor at rest Normal pulse oximetry Good air exchange Normal color Normal level of conscious Demonstrated ability to tolerate fluids by mouth

FOLLOW-UP Any patient who was admitted to the hospital, received nebulized epinephrine, or had a prolonged outpatient visit should have followup scheduled with the primary care provider within 24 hours or as soon as can be arranged. Although some children may continue to have mild to moderate symptoms at the time of follow-up, there are no studies that support the routine use of corticosteroid therapy beyond 24 hours.

Follow-up should continue until the child's symptoms have begun to resolve. The child who does not improve as expected (over the course of approximately seven days) may have an underlying airway abnormality or may be developing a complication of croup. Further evaluation, particularly with a radiograph of the soft tissues of the neck, or consultation with otolaryngology, may be warranted. (See "Clinical features, evaluation, and diagnosis of croup", section on Differential diagnosis).

PROGNOSIS Symptoms of croup resolve in most children within three days, but may persist for up to one week. Fewer than 5 percent of children with croup require hospital admission [31] , and among those, 1 to 6 percent require intubation [13-16,32] . Mortality is rare, occurring in <0.5 percent of intubated children [33] .

Complications Complications of croup are uncommon. Children with moderate to severe croup are at risk for hypoxemia (oxygen saturation <92 percent in room air) and respiratory failure. Other complications include pulmonary edema, pneumothorax, and pneumomediastinum [34] . These

complications can be anticipated and managed by aggressive monitoring and intervention in the medical setting. Out-of-hospital cardiac arrest and death also have been reported [35] .

Secondary bacterial infections may arise from croup. Bacterial tracheitis, bronchopneumonia, and pneumonia occur in a small number of patients [9,14,36-38] . In most instances, the child has been relatively stable or beginning to improve after several days of illness, but then suddenly worsens, with higher or recurrent fever, increased (and potentially productive) cough, and/or respiratory distress. (See "Clinical features and diagnosis of communityacquired pneumonia in children").

INFORMATION FOR PATIENTS Educational materials on this topic are available for patients. (See "Patient information: Croup in infants and children"). We encourage you to print or e-mail this topic review, or to refer parents to our public web site, www.uptodate.com/patients, which includes this and other topics.

SUMMARY AND RECOMMENDATIONS Most children with croup who seek medical attention have a mild, self-limited illness and can be successfully managed as outpatients. (See "Overview" above). Children with croup who should be seen in the office or emergency department include those who have stridor at rest, an abnormal airway, previous episodes of moderate to severe croup, underlying conditions that may predispose to respiratory failure, rapid progression of symptoms, inability to tolerate fluids, prolonged symptoms, or an atypical course. (See "Phone triage" above). Children with mild symptoms can be managed at home. Families should be instructed in provision of supportive care and indications to seek medical attention. (See "Home treatment" above). We suggest that a single dose of oral dexamethasone (0.6 mg/kg) be used when electing to treat children with mild croup who are seen in the outpatient setting (Grade 2A). (See "Outpatient treatment" above and see "Pharmacologic and supportive interventions for croup", section on Dexamethasone). Children with moderate croup should be evaluated in the office or emergency department and those with severe croup should be evaluated in the emergency department. Children with severe croup must be approached cautiously, as any increase in anxiety may worsen airway obstruction. (See "Moderate to severe croup" above). Supportive care for the child with moderate or severe croup includes administration of humidified air or oxygen as indicated by hypoxemia and/or respiratory distress, provision of intravenous fluids, and monitoring for worsening respiratory distress. (See

"Supportive care" above and see "Pharmacologic and supportive interventions for croup", section on Mist therapy). We recommend that children with moderate to severe croup who have moderate stridor at rest, moderate retractions, and/or more severe symptoms be treated with nebulized epinephrine (Grade 1A) in addition to dexamethasone. (See "Pharmacotherapy" above and see "Pharmacologic and supportive interventions for croup", section on Nebulized epinephrine).

- Racemic epinephrine is administered as 0.05 mL/kg per dose (maximum of 0.5 mL) of a 2.25 percent solution diluted to 3 mL total volume with normal saline. It is given via nebulizer over 15 minutes.

- L-epinephrine is administered as 0.5 mL/kg per dose (maximum of 5 mL) of a 1:1000 dilution. It is given via nebulizer over 15 minutes.

Nebulized epinephrine can be repeated every 15 to 20 minutes. The administration of three or more doses within a two- to three-hour time period should prompt initiation of close cardiac monitoring if this is not already underway. We recommend that children with moderate to severe croup be treated with dexamethasone (0.6 mg/kg, maximum of 10 mg), by the least invasive route (Grade 1A). (See "Pharmacotherapy" above and see "Pharmacologic and supportive interventions for croup", section on Corticosteroids). Children with moderate to severe croup should be observed for three to four hours after intervention. Those who improve may be discharged home. (See "Discharge to home" above). Children with moderate to severe croup whose condition worsens or fails to improve as expected after treatment with nebulized epinephrine and corticosteroids should be admitted to the hospital. (See "Hospitalization" above). We suggest not using repeated doses of corticosteroids. (Grade 2C). (See "Hospitalization" above and see "Pharmacologic and supportive interventions for croup", section on Repeated dosing). Other causes of upper airway obstruction should be investigated in children who have moderate to severe symptoms that persist for more than a few days. (See "Clinical features, evaluation, and diagnosis of croup", section on Differential diagnosis). Children who received nebulized epinephrine, had a prolonged outpatient visit, or were admitted to the hospital should have followup scheduled with the primary care provider within 24 hours of discharge or as soon as follow-up can be arranged. (See "Follow-up" above). Most children with croup recover uneventfully. (See "Prognosis" above).

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