Pulmonary Diseases
Katherine Rivera-Spoljaric
Leonard B. Bacharier
CROUP (VIRAL LARYNGOTRACHEOBRONCHITIS)
Definition and Epidemiology
Croup, or viral laryngotracheobronchitis, is an acute inflammation of the entire airway, mainly in the glottic and subglottic areas, resulting in airway narrowing, obstruction, and voice loss. Therefore, it has generally been described as a triad of hoarse voice, harsh barking cough, and inspiratory stridor.
Typically, the condition affects younger children (6-36 months), with a peak incidence at 2 years of age. It is the most common cause of acute upper airway obstruction in young children; ˜3% of children experience an episode before 6 years of age.
Seasonal outbreaks have been described in the fall and winter, although it may occur year-round in some areas.
Males are more commonly affected than are females.
Etiology and Pathophysiology
Viral infection is the predominant etiology; parainfluenza virus (types 1, 2, and 3) is the most common agent. Other common viral agents are respiratory syncytial virus (RSV) and influenza virus, with less commonly encountered viruses including adenovirus, rhinovirus, enterovirus, and measles virus.
Mycoplasma pneumoniae is one of the few nonviral microorganisms that has been reported as an etiologic agent.
In children, the larynx is very narrow and is composed by the rigid ring of the cricoid cartilage; therefore, a viral infection causing inflammation of this area leads to airway edema and subsequent obstruction. This obstruction results in the classic symptoms of stridor and cough.
Clinical Presentation
Croup usually presents initially with a coryzal prodrome (1-4 days).
Common symptoms include clear rhinorrhea, low-grade temperature, and mild tachypnea followed by barking cough, hoarseness, and stridor.
Obstructive symptoms occur most commonly at night.
Severity of airway narrowing may be determined by the presence of stridor at rest, tachypnea, retractions, tracheal tug, cyanosis, and pallor as well as decreased breath sounds, which indicate critical narrowing.
Diagnosis
The diagnosis is clinical.
Radiography of the neck is not necessary but may show the typical “steeple sign” or subglottic narrowing. Radiographic appearance does not correlate with disease severity.
Radiographs should be obtained if there is concern about the diagnosis, and they may distinguish croup from other causes of upper airway obstruction such as epiglottitis.
Oxygen saturations and arterial blood gases should be obtained if hypoxemia, which may be indicated by restlessness, altered mental status, and/or cyanosis, is a concern.
The differential diagnosis includes epiglottitis (but the patient is usually toxic appearing), spasmodic croup (no viral prodrome and mostly in atopic children), bacterial tracheitis, laryngitis, foreign body, and laryngospasm.
Treatment
A few clinical scoring systems that guide assessment and management have been described in the literature. The most commonly used is the Westley score system, which is described below:
Scores are given based on the presence of stridor (none 0, when agitated 1, at rest 2), retractions (none 0, mild 1, moderate 2, severe 3), level of air entry (normal 0, decreased 1, markedly decreased 2), cyanosis in room air (none 0, with agitation 4, at rest 5), and level of consciousness (normal 0, disoriented 5).
Mild croup is described as scores 1-2, moderate croup as scores 3-8, and severe croup as scores >8, with consideration of pharmacologic therapy and hospitalization in moderate and severe cases.
In general, patients without signs of severe airway narrowing or stridor at rest may be managed on an outpatient basis after appropriate observation. Parents should be reassured and instructed about signs of worsening respiratory distress.
General supportive measures such as increased fluid intake, decreased handling, and careful observation are usually recommended.
Management strategies may include use of cool mist vaporizer, cold air exposure when riding in a motor vehicle, and use of steam inhalation, although these methods are anecdotal and have not proved beneficial in reducing symptom scores during several studies.
For children with evidence of stridor at rest and/or signs of moderate to severe airway compromise, pharmacologic therapy is indicated.
Nebulized racemic epinephrine acts by reducing vascular permeability of the airway epithelium, therefore diminishing airway edema and improving airway caliber by decreasing resistance to airflow.
It should be administered at doses of 0.25-0.5 mL along with humidified oxygen as needed. If no response is elicited after the first treatment, the dose may be repeated.
The patient may return to pretreatment state 30-60 minutes after a dose, and therefore, he or she should be observed for at least 2-3 hours after administration due to “rebound phenomenon.”
Systemic corticosteroids are effective in reducing symptoms within 6 hours and for at least 12 hours after initial treatment.
Dexamethasone 0.6 mg/kg/dose IM, IV, PO single dose is the glucocorticoid most commonly used, but prednisolone 1-2 mg/kg/dose PO for 3 days is an alternative.
Studies have shown that high-dose nebulized budesonide (2 mg) is superior to placebo and as effective as dexamethasone in reducing symptom scores, but the cost-benefit ratio limits its use.
EPIGLOTTITIS
Definition and Epidemiology
Epiglottitis represents a true pediatric emergency with acute infectious supraglottic obstruction that may rapidly lead to life-threatening airway obstruction.
It affects children of all ages, with a peak around 3-6 years of age, although its incidence has declined significantly since Haemophilus influenzae type B immunization was introduced in 1998.
Etiology and Pathophysiology
H. influenzae type B is the most common cause in children, although its prevalence has markedly decreased in the postvaccine era. Other agents include group A Streptococcus, H. influenzae (types A, F, and nontypeable), Staphylococcus aureus, Candida albicans, and Streptococcus pneumoniae.
Direct invasion by the inciting agent causes inflammation of the epiglottis, aryepiglottic folds, ventricular bands, and arytenoids. Subsequently, there is accumulation of inflammatory cells and edema fluid where the stratified squamous epithelium is loosely adherent to the anterior surface and the superior third of the posterior portion of the epiglottis.
Diffuse infiltration with polymorphonuclear leukocytes, hemorrhage, edema, and fibrin deposition occurs. Microabscesses may form. As the edema increases, the epiglottis curls posteriorly and inferiorly. This causes airway obstruction.
Inspiration tends to draw the inflamed supraglottic ring into the laryngeal inlet.
Clinical Presentation
Epiglottitis is a rapidly progressing illness in previously healthy individuals. Patients are usually anxious and toxic appearing and assume the classic “tripod position” (forward-leaning posture with bracing arms and extension of the neck that allows for maximal air entry).
Other symptoms typically present are high fever, muffled or absent voice (“hot potato”), sore throat, drooling, inspiratory stridor, dysphagia, protruded jaw, and extended neck.
Diagnosis
Presumptive diagnosis should be made on clinical grounds.
If patient is in little distress, and the diagnosis is unclear, a lateral neck radiograph may be obtained, which shows the classical thumbprint sign that represents a swollen epiglottis and aryepiglottic folds. Radiographs can be normal in 20% of the patients.
The definitive diagnosis requires direct visualization of a red swollen epiglottis under laryngoscopy, but this examination should be attempted only in a controlled setting in collaboration with an anesthesiologist and an otolaryngologist.
The differential diagnosis includes foreign body aspiration, anaphylactic reaction, angioedema, caustic ingestion, thermal injury, inhalation injury, and laryngotracheobronchial and retropharyngeal infection.
Treatment
Airway stabilization and maintenance must be performed quickly and early in the course.
Oxygen should be administered at the minimal sign of distress.
Stimulation and patient disturbance should be minimized to avoid complete obstruction.
An artificial airway should be available next to the patient, and it should always be ready for use.
After appropriate management of the airway has been established, empiric intravenous (IV) antibiotic therapy against β-lactamase-producing pathogens should be initiated promptly. For severely ill patients, consider combination therapy with an antistaphylococcal agent active against MRSA.
The use of IV glucocorticosteroids is controversial and not shown to be beneficial in the initial management; however, doses are frequently administered for the management of airway inflammation especially on patients that have had difficulty with extubation.
BACTERIAL TRACHEITIS
Definition and Epidemiology
This acute bacterial infection of the trachea often also involves the larynx and bronchi. It has been called bacterial laryngotracheobronchitis and pseudomembranous croup.
A cause of acute airway obstruction, this condition may potentially be life threatening.
Most patients are <3 years of age (usually 3 months to 2 years), although older children may be affected. There are no clear sex differences in incidence or severity.
There seems to be no seasonal preferences.
Etiology and Pathophysiology
The most common cause is S. aureus, but other encountered agents are S. pneumoniae, S. pyogenes, Moraxella catarrhalis, and H. influenzae. Anaerobic organisms have also been reported.
Invasion of opportunistic bacterial organisms, often following an upper airway viral infection, causes subglottic edema with ulcerations, copious and purulent secretions, and pseudomembrane formation.
Clinical Presentation
The typical presentation involves a history of an upper respiratory infection (URI) for ˜3 days characterized by a low-grade fever and a “brassy” cough. The illness then evolves rapidly with high fever and signs of airway obstruction, including stridor, cough, drooling, and supine positioning (preference to lie flat).
Patients generally appear toxic.
There is also evidence of purulent airway secretions.
Diagnosis
Diagnosis is clinical with classical signs of epiglottitis and croup absent. Direct visualization of the trachea via laryngoscopy demonstrates thick, abundant, and purulent secretions.
The differential diagnosis includes epiglottitis (although no dysphagia, and patient may lie flat), croup (although voice is normal and there is a lack of a barky cough), and laryngeal and retropharyngeal abscess.
Treatment
Management of the airway is critical with intubation, and assisted ventilation should be strongly considered.
There is no proven role for bronchodilators or corticosteroids.
Antimicrobial therapy should be immediately instituted. Choice of therapy includes broad-spectrum antibiotics with antistaphylococcal activity.
FOREIGN BODY ASPIRATION
Definition and Epidemiology
This accidental ingestion occurs commonly in children <5 years of age but has been described at any age.
Younger children are typically at higher risk because of oral exploration and immaturity of their swallowing functions.
This situation may be life threatening; it is the leading cause of accidental death by ingestion in younger children.
Etiology and Pathophysiology
Ingested food and toy parts are aspirated into the airways, causing choking.
A foreign body can be localized in the larynx, trachea, or bronchi.
Impaction of the larynx is particularly dangerous, although most particles travel well into the airways and lodge in the intrathoracic area.
The foreign particle provokes localized airway inflammation with mucosal edema, inflammation, and development of granulation tissue. Atelectasis of the area involved and empyema may occur.
Clinical Presentation