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Author: Lonnie King, MD, Consulting Staff, Department of Emergency Medicine, Children's Healthcare of Atlanta at Scottish Rite

Lonnie King is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, and American College of Emergency Physicians

Editors: Jerry Balentine, DO, Professor of Emergency Medicine, New York College of Osteopathic Medicine; Senior Vice President, Chief Medical Officer, Medical Director, Attending Physician in Department of Emergency Medicine, Saint Barnabas Hospital; Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc; Wayne Wolfram, MD, MPH, Clinical Associate Professor, Departments of Pediatrics, Children's Hospital and University of Cincinnati; John Halamka, MD, Chief Information Officer, CareGroup Healthcare System, Assistant Professor of Medicine, Department of Emergency Medicine, Beth Israel Deaconess Medical Center; Assistant Professor of Medicine, Harvard Medical School; Richard G Bachur, MD, Assistant Professor of Pediatrics, Harvard Medical School; Associate Chief and Fellowship Director, Attending Physician, Division of Emergency Medicine, Children's Hospital of Boston

Author and Editor Disclosure

Synonyms and related keywords: croup, laryngotracheobronchitis, viral infection of the upper respiratory tract, airway obstruction, parainfluenza type 1, parainfluenza type 2, parainfluenza type 3, upper respiratory infection, URI, paramyxovirus, influenza virus type A, respiratory syncytial virus, RSV, adenovirus, rhinovirus, enterovirus, coxsackievirus, enteric cytopathogenic human orphan virus, ECHO virus, reovirus, measles virus, barking cough, viral infection

Background

Laryngotracheobronchitis (ie, croup) is a viral infection of the upper respiratory tract that causes varying degrees of airway obstruction.

The disease is most often self-limited, but it occasionally is severe and, rarely, fatal. A barking cough, stridor, and fever are characteristic, and it is the most common cause of stridor in children. With aggressive ED treatment, very few cases require admission.

Pathophysiology

This is a disease that mainly affects children. A prodrome of several days of fever and symptoms of mild upper respiratory infection are common.

As the infection extends to the proximal trachea, diffuse inflammation with exudate and edema of the subglottic area causes narrowing of the airway. The cricoid ring of the trachea (in the immediate subglottic area) is the narrowest portion of the airway in a child. A small amount of edema in this region can cause significant airway obstruction. (Remember that the resistance to flow through a tube is inversely proportional to the fourth power of the radius.) Air flowing through this narrowed subglottic area causes stridor. The uncomplicated disease usually wanes in 3-5 days but may persist for as many as 10 days.

Frequency

United States

Laryngotracheobronchitis has a peak incidence of 5 cases per 100 children per year during the second year of life. It is the most common form of airway obstruction or stridor in children aged 6 months to 6 years.

Mortality/Morbidity

The vast majority of children with croup do well. Morbidity is unusual, and mortality is rare.

Sex

Prevalence is higher in males than in females, with a male-to-female ratio of nearly 2:1.

Age

Illness is most common in children aged 3 months to 3 years.

  • The mean age of onset is 18 months.
  • Laryngotracheobronchitis is uncommon in persons older than 6 years.



History

  • The patient usually has a few days of a mild upper respiratory illness with low-grade fever, runny nose, and mild cough.
  • Typically, between 6 pm and 6 am, the child develops stridor (mainly inspiratory), hoarseness, and a brassy seal-like barking cough.
  • Parents may report worsening symptoms on the second night of the illness.
  • The child is fatigued.

Physical

The physical examination may range from totally unremarkable on presentation to severe respiratory distress.

  • Restless (common); prefers sitting upright in a parent's lap
  • Appears nontoxic (common)
  • Normal voice or laryngitis
  • Mild fever
  • Tachycardia
  • Tachypnea
  • Varying stridor, predominantly inspiratory
  • Absence of drooling
  • Retractions of the accessory chest muscles
  • No change in stridor with positioning
  • Nontender larynx
  • Worsening disease and possible impending respiratory failure may be indicated by the following:
    • Change in mental status (eg, fatigue, listlessness, restlessness)
    • Increased retractions
    • Decreased breath sounds with decreasing stridor
    • Pallor
    • Cyanosis

Causes

Croup is most commonly caused by parainfluenza type 1, although parainfluenza type 2 and type 3 also may cause disease. Other etiologies are as follows:

  • Paramyxovirus
  • Influenza virus type A
  • Respiratory syncytial virus (RSV)
  • Adenovirus
  • Rhinovirus
  • Enterovirus
  • Coxsackievirus
  • Enteric cytopathogenic human orphan virus (ECHO virus)
  • Reovirus
  • Measles virus



Diphtheria
Foreign Bodies, Gastrointestinal
Foreign Bodies, Trachea
Pediatrics, Epiglottitis
Pediatrics, Foreign Body Ingestion

Other Problems to be Considered

Subglottic stenosis
Retropharyngeal abscess
Subglottic hemangioma



Lab Studies

  • Laboratory testing usually is not needed in well-hydrated patients.
  • If laboratory tests are needed, they should be deferred while the patient is in distress.
  • Approximately 80% of admitted patients are hypoxic.
    • A pulse oximetry measurement is indicated in all but the mildest cases.
    • In the usual case, hypoxia is caused by pulmonary involvement.
    • With severe airway obstruction, respiratory failure may occur.
  • Complete blood count
    • Leukopenia in early stage of illness
    • Leukocytosis in later stage of patients with severe disease

Imaging Studies

  • Imaging tests are not required in mild cases with typical history that respond appropriately to treatment.
  • An anteroposterior (AP) soft tissue neck radiograph may show subglottic narrowing.
    • The usual squared-shoulder appearance of the subglottic area is replaced by cone shaped narrowing just distal to the vocal cords. This is called the steeple or pencil-point sign.
    • Monitor patients during imaging because progression of airway obstruction may be rapid.

Other Tests

  • Rapid antigen tests are available in some centers but usually are not needed.

Procedures

  • Direct laryngoscopy if the child in not in acute distress
  • Fiberoptic laryngoscopy
  • Bronchoscopy



Prehospital Care

  • Try to avoid actions that may agitate the child in distress and increase the work of breathing.
  • Transport the child in a parent's lap and give oxygen as tolerated, usually via a "blow by" technique.

Emergency Department Care

Goals of emergency department care are to reduce any respiratory distress, monitor for worsening condition, and consider, or evaluate for, other etiologies of stridor.

  • Make the child as comfortable as possible.
  • Avoid agitating the child with unnecessary procedures and examinations.
  • Humidified air or mist therapy may be used, but both have unproven efficacy.
  • Provide oxygen (humidified) to all hypoxic patients.
  • L-epinephrine (1:1000) is as effective as racemic epinephrine. Epinephrine therapy does not indicate the need for admission.
  • Dexamethasone has been shown to reduce symptoms in patients with moderate-to-severe croup. (0.6 mg/kg IM, not to exceed 10 mg)
  • Nebulized budesonide (2 mg) has been shown in several studies to be equivalent to oral dexamethasone. Inhaled Decadron also is used when budesonide is unavailable.

Consultations

Consultation with ORL and anesthesia prior to RSI may be necessary if patient is exhibiting rapid deterioration that might suggest an alternative diagnosis.



The goal of pharmacotherapy is to reduce morbidity and prevent complications.

Drug Category: Adrenergic agonist

Alpha-agonist effects include increased peripheral vascular resistance, reversed peripheral vasodilatation, systemic hypotension, and vascular permeability. Beta-agonist effects include bronchodilatation, chronotropic cardiac activity, and positive inotropic effects.

Drug NameEpinephrine, racemic (microNefrin)
DescriptionInhalation of racemic epinephrine is the cornerstone of symptomatic relief during exacerbations of croup. Alpha-receptor stimulation causes mucosal vasoconstriction, leading to decreased edema of the subglottic region of the larynx. Beta2-receptor stimulation may provide additional benefit by causing bronchial smooth muscle relaxation.
Pediatric DoseRacemic epinephrine: 0.25-0.5 mL of 2.25% solution via nebulizer (diluted in 3 mL of isotonic sodium chloride solution or sterile water); may be repeated 3 times
ContraindicationsDocumented hypersensitivity; cardiac arrhythmias, obstructed ventricular outflow, or angle-closure glaucoma; local anesthesia in areas such as fingers or toes because vasoconstriction may produce sloughing of tissue; do not use during labor (may delay second stage of labor)
InteractionsIncreases toxicity of beta- and alpha-blocking agents and that of halogenated inhalational anesthetics
PregnancyC - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
PrecautionsCaution or discontinue if heart rate >200; short duration of action and relapse may occur; caution in elderly persons, prostatic hypertrophy, hypertension, cardiovascular disease, patients with diabetes mellitus, hyperthyroidism, and cerebrovascular insufficiency; rapid IV infusions may cause death from cerebrovascular hemorrhage or cardiac arrhythmias

Drug Category: Corticosteroids

Steroids are used to decrease subglottic edema by suppressing local inflammatory process. The effectiveness of steroids in croup has been much debated, and, although no clear-cut information proves that steroids are beneficial, meta-analysis has shown that they decrease symptoms within 24 hours and may reduce the need for endotracheal intubations.

Drug NameDexamethasone (Decadron)
DescriptionDrug of choice. Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reducing capillary permeability.
Pediatric Dose0.6 mg/kg PO/IM once; some repeat the dose in 6 h
ContraindicationsDocumented hypersensitivity; active bacterial or fungal infection
InteractionsEffects decrease with coadministration of barbiturates, phenytoin, and rifampin; decreases effect of salicylates and vaccines used for immunization
PregnancyC - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
PrecautionsIncreases risk of multiple complications, including severe infections; monitor adrenal insufficiency when tapering drug; abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections are possible complications of glucocorticoid use

Drug NamePrednisone (Deltasone)
DescriptionMay decrease inflammation by reversing increased capillary permeability and suppressing PMN activity.
Pediatric Dose1-2 mg/kg/d PO qd or divided bid for 5 d
ContraindicationsDocumented hypersensitivity; viral infection, peptic ulcer disease, hepatic dysfunction, connective tissue infections, and fungal or tubercular skin infections; GI disease
InteractionsCoadministration with estrogens may decrease clearance; concurrent use with digoxin, may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics
PregnancyB - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
PrecautionsAbrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use

Drug NameBudesonide (Pulmicort Respules inhalation suspension)
DescriptionHas been shown in several studies to be equivalent to oral dexamethasone.
Pediatric Dose2 mg (2 mL of suspension) via nebulizer
ContraindicationsDocumented hypersensitivity; active bacterial or fungal infection
InteractionsNone reported
PregnancyC - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
PrecautionsProlonged use may increase systemic absorption of corticosteroids and may cause Cushing syndrome, reversible HPA axis suppression, hyperglycemia, and glycosuria



Further Inpatient Care

  • Perform frequent or continuous monitoring of respiratory status.
  • Obtain a continuous pulse oximetry measurement.
  • Provide a humidified environment, which may include use of the following:
    • Mist therapy
    • A croup tent (still used but generally not recommended unless no alternative therapy is available)
    • A vaporizer at bedside
  • Give intravenous fluids to dehydrated children not tolerating oral fluids.

Further Outpatient Care

  • Parents must provide a humidified environment at night, which may include use of the following:
    • A cool mist vaporizer
    • A steamed bathroom environment, which may relieve mild exacerbation
    • Exposure to cool night air, which may relieve mild exacerbation, especially if a spasmodic component is present
  • Tobacco smoke and other irritants should not be allowed to come in contact with the child.

In/Out Patient Meds

  • Inhalation of racemic epinephrine is the cornerstone of symptomatic relief during exacerbations of croup.
  • Some authorities recommend a repeat dose of dexamethasone in 6 hours.

Complications

  • Intubation (required in as many as 2% of patients)
  • Subglottic stenosis in intubated patients
  • Bacterial tracheitis
  • Cardiopulmonary arrest
  • Pneumonia

Prognosis

  • Recovery is usually complete.

Patient Education

  • For excellent patient education resources, visit eMedicine's Lung and Airway Center. Also, see eMedicine's patient education article Croup.



Medical/Legal Pitfalls

  • Rebound stridor after epinephrine therapy has been described, but it appears to be less of a problem if corticosteroid therapy is initiated early in the ED course.
  • Failure to treat patients with steroids can be a pitfall.
  • Always consider other causes of stridor, such as foreign bodies, bacterial tracheitis, and epiglottitis.
  • Failure to observe patients for an adequate period before ED discharge and failure to document satisfactory pulse oximetry.



Media file 1:  Child with croup. Note the steeple or pencil sign of the proximal trachea evident on this anteroposterior film. Courtesy of Dr. Kelly Marshall, CHOA at Scottish Rite.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  X-RAY



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Pediatrics, Croup or Laryngotracheobronchitis excerpt

Article Last Updated: Oct 1, 2007