Respiratory

CroupDiagnosis and Treatment

a report by

M a r i o C a n c i a n i , M D , M a t t i a G u e r ra , M D , and I n g r i d To l l e r , M D
Allergology and Pulmonology Unit, Department of Pediatrics, Department of Experimental and Clinical Pathology and Medicine, University of Udine, Italy

In ancient timesas well as throughout the first third of the 20th century the term croup referred to the characteristic airway noise of patients affected by diphtheria. Nowadays, croup is a broad, clinical diagnostic term used for several respiratory illnesses that have varying degrees of inspiratory stridor, hoarse voice, and harsh, barking cough.1 These symptoms are thought to occur as a result of inflammation and edema of the upper airway, including the larynx, trachea, and bronchi (hence the term laryngotracheobronchitis), which, in the majority of cases, are triggered by recent viral infection.2 The frightening nature of croup often prompts parents and caregivers to consult a physician.

Degree of respiratory distressstridor at rest, tracheal tug, intercostal and subcostal indrawing on inspiration, tachypnea, or palpable pulsus paradoxus indicate moderate to severe croup. Cyanosis or extreme pallor indicates very severe obstruction. Oxygen desaturation, indicated by oximetry, is usually a late and unreliable sign of severity, and should never be a substitute for good clinical assessment. The loudness of the stridor is not a reliable indicator of the severity of croup. Auscultation of the chest usually reveals only transmitted upper-airway noise; breath sounds that are reduced in volume also indicate severe illness. Diagnosis The well-prepared clinician can often make a diagnosis based solely on the history and physical examination, using radiographs and laboratory examinations to aid in diagnosis when the clinical scenario is unclear. Standard work-up for clinical diagnosis includes the assessment of skin color, hydration, breath sounds, and air movement. X-ray examination is not part of the standard assessment. Only 50% of patients with croup show classic steeple signs on plain neck radiography (see Figure 1).5 Patients with atypical features in whom the diagnosis is unclear should have a different work-up to exclude other less common entities such as retropharyngeal abscess, epiglottitis, bacterial tracheitis, and foreign bodies. This work-up may include: cell blood count and blood culture; soft-tissue plain radiography of the neck; and computed tomography scan of the neck with intravenous contrast. The classical radiography signs are: the thumb sign in the epiglottitis on the lateral airway neck film, due to

X-ray examination is not part of the standard assessment. Only 50% of patients with croup show classic steeple signs on plain neck radiography.

Epidemiology and Etiology Croup is a common cause of upper-airway obstruction in young children, occurring in about 2% of pre-school-age children annually.3 It mainly affects children aged between six and 36 months, with a peak incidence at 1224 months. There is a male predominance of 3:2 and, although the disease can occur throughout the year, it predominates in the fall and winter months.1 The classification presented in Table 1 notes specific illnesses by etiology, anatomical location, and clinical characteristics. Parainfluenza virus type 1 and influenza virus A are the agents most commonly identified in cases of croup.4 Differentiating spasmodic croup from viral croup is difficult and often not useful because the treatment does not differ. Clinical Presentations Signs and symptoms of croup are presented in Table 2; they get worse at night and may peak on the second or third night. Determining the degree of airway obstruction (based primarily on the history) is the most important consideration when assessing children with croup. As airway obstruction can worsen rapidly, repeated careful clinical assessment is essential. Assessing Croup Severity 3 The following are important points in the assessment of croup severity: General appearanceagitation, restlessness, irrational behavior, hypotonia, and lethargy are clinical signs of severe obstruction.

Mario Canciani, MD, is Head of the Allergology and Pulmonology Unit in the Department of Pediatrics, Department of Experimental and Clinical Pathology and Medicine, University of Udine, Italy. He is also Professor at the University of Trieste and Udine Schools of Medicine. Professor Canciani is a Member of the Italian Pediatric Society and the European Respiratory Society, Secretary and then Chair of the Pediatric Respiratory Infectious Disease and Immunology Group, and a Member of the Website Committee and the Executive Committee of the Italian Childhood Respiratory Society.

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Respiratory

Table 1: Comparison Of Upper-airway Obstructions

Laryngotracheobronchitis (Viral Croup) Six months to five years Parainfluenza viruses Influenza A and B Adenovirus Respiratory syncytial virus Gradual Low-grade fever Non-septic Barking cough Stridor Hoarseness Spasmodic Croup Six months to three years ?Viral ?Airway reactivity Epiglottitis Two to seven years Hemophilus influenzae Staphylococcus aureus (rarely) Inhaled Foreign Body Newborn to adult Object small enough to fit in mouth or nares

Age range Etiology

Onset Clinical presentations

Sudden Afebrile Non-septic Barking cough Stridor Hoarseness

Sudden High fever Septic Non-barking cough Muffled voice Drooling Dysphagia Sitting forward with mouth open

Sudden Afebrile Respiratory distress Choking

Adapted from Custer, 1993.21

Table 2: Clinical Manifestations of Croup

1. Constitutional state (toxicity, fever, pulse rate) 2. Stridor 3. Drooling 4. Barking cough 5. Speech 6. Tachypnea 7. Tracheal tug on inspiration 8. Intercostal and subcostal indrawing on inspiration 9. Asynchrony of chest and abdominal wall movement 10. Cyanosis in air
Adapted from Custer, 1993.21

Treatment The most important aspect in the treatment of croup is airway maintenance; standard management includes mist therapy, corticosteroids, and adrenalin. Any child with croup and evidence of respiratory distress should be considered a candidate for steroid treatment.1,3 Less frequently, hospitalization and intubation are necessary. A clinical croup score (according to the Westley croup score) should be recorded before and after each treatment (see Table 3). A score of 2, if there is some accessory muscle use/recessions and stridor at rest, is considered to indicate moderate to severe airway obstruction and requires oximetry and monitoring heart rate, and powering of the treatment by oxygen. Mist Therapy Treatment with humidified air was previously widely used; theoretically, inspired air that is cooler than body temperature and less than 100% saturated with water vapor will result in mucosal cooling, vasoconstriction, and lessened edema. Although this treatment has never been scientifically validated, it is still recommended as a home treatment: parents should take the symptomatic child into the bathroom while running a hot shower and filling the room with warm water vapor.7,8 Warm steam may improve symptoms. Steroids The use of corticosteroids in patients with croup was controversial for many years but, in the last decade, has transformed the management of this illness. The results of a meta-analysis showed that steroids are effective in improving symptoms of croup within six hours, for up to 12 hours, with significant improvement in scores of croup severity, shorter hospital stays, reduced need for endotracheal intubation, and less use of adrenalin.4,9 While it seems clear that steroids provide benefits, more recent studies have tried to determine the optimal method of administration of the treatment. The effectiveness of oral or intramuscular dexamethasone (0.6mg/kg) as a treatment for patients with moderate to severe croup is well established.10,11 Doses of dexamethasone ranging from 0.15mg/kg to 0.6mg/kg have been shown to be similarly efficacious for treating moderate croup.12 Two recent studies suggested that the use of a single dose of oral dexamethasone treatment for mild croup demonstrates more

Table 3: Westley Croup Score

Stridor 0 1 2 0 1 2 3 0 1 2 0 1 2 0 5 None When agitated or at rest, audible with stethoscope At rest, audible without stethoscope None Mild Moderate Severe Normal Decreased but easily audible Markedly decreased None With agitation At rest Normal (including sleep) Altered mental state, disoriented

Retractions

Air entry

Cyanosis (sulphur dioxide <92% on air) Level of consciousness

edema of the epiglottis thickening the free edge (the posterior-anterior radiograph is usually unremarkable); and a widening of the retropharyngeal space, due to the abscess (see Figure 2). Measuring at the level of C2, the normal distance from the anterior surface of the vertebrae to the posterior border of the airway should be 7mm, regardless of the patients age. A simpler (but less precise) rule is that the soft-tissue plane should be less than half the width of the corresponding vertebral body.6

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CroupDiagnosis and Treatment

Figure 1: Classic Steeple Signs on Plain Neck Radiography

Figure 2: Retropharyngeal Abscess (arrow)

The classic steeple sign of croup as shown on posterior-anterior neck radiography, resulting in a narrowed column of subglottic air (top arrow) and an enlargement of the column (bottom arrow).

rapid symptom resolution, with important clinical and economical benefits;11,13 for these reasons, oral steroids are currently preferred in most pediatric emergency departments. Commonly used alternatives to dexamethasone are prednisone or prednisolone (12mg/kg).14 The use of nebulized budesonide (2mg) to treat patients with moderate croup has been shown to be effective.15,16 A number of trials have shown that oral and intramuscular dexamethasone and nebulized budesonide have the same effectiveness for treatment of moderate croup and the choice depends on the status of the patient, availability, and cost.17 Adrenalin A child with persisting inspiratory stridor at rest and marked chest-wall retractions should receive immediate treatment with nebulized L-adrenalin (1:1,000 dilution at a dose of 0.5ml/kg to a maximum dose of 5ml). Inhaled adrenalin has a rapid onset of action (30 minutes), has a temporary

beneficial effect on airway obstruction, and, although not a definitive treatment, may allow time for the basic pathology to resolve.18 The L-isomer of adrenalin alone is preferred to racemic adrenalin as it is safe, much less expensive, and readily available worldwide.19 The association of a nebulized steroid (beclomethasone or budesonide) improves the efficacy of L-adrenalin, since the steroid begins to work when L-adrenalin decreases.20 Common adverse effects of L-adrenalin include tachycardia and hypertension, so should be used with caution in patients who have heart conditions or arrhythmias. As the effect of adrenalin is brief, croup symptoms may reappear, demonstrating a rebound phenomenon; children receiving adrenalin must be observed for a minimum of four hours in the accident and emergency (A&E) department prior to discharge and should only be discharged after the clinician is convinced that the parent thoroughly understands the disease process and is able to return to the A&E department expeditiously if stridor should recur.3 Hospitalization is indicated in children with increasing or persistent respiratory distress, cyanosis, toxic-appearing, depressed sensorium, and in young infants and patients with atypical symptoms.4

1.

2. 3. 4. 5. 6. 7. 8.

9.

James D, State of the evidence for standard-of-care treatments for croup: are we where we need to be?, Paediatr Infect Dis J, 2005;24:198202. De Soto H, Epiglottitis and croup in airway obstruction in children, Anesthesiol Clin North Am , 1998;16:85368. Fitzgerald DA, Kilham HA, Croup: assessment and evidence-based management, MJA , 2003;179:3727. Knutson D, Aring A, Viral croup, Am Fam Physician , 2004;69: 53540. Malhotra A, Krilov LR, Viral croup, Pediatr Rev , 2001;22:512. Philpott CM, Selvadurai D, Banerjee AR, Pediatric retropharyngeal abscess, J Laryngol Otol , 2004;118:91926. Moore M, Little P, Humidified air inhalation for treating croup, Cochrane Database Syst Rev , 2006;3:CD002870. Scolnik D, Coates AL, Stephens D, et al., Controlled delivery of high vs low humidity vs mist therapy for croup in emergency departments: a randomized controlled trial, JAMA , 2006;296: 3934. Fitzgerald DA, The assessment and management of croup, Paediatr

Respir Rev , 2006;7:7381. 10. Donaldson D, Poleski D, Knipple E, et al., Intramuscular versus oral dexamethasone for the treatment of moderate-to-severe croup: a randomized, double-blind trial, Acad Emerg Med , 2003;10: 1621. 11. Luria JW, Gonzalez-del-Rey JA, DiGiulio GA, et al., Effectiveness of oral or nebulized dexamethasone for children with mild croup, Arch Pediatr Adolesc Med , 2001;155:134045. 12. Geelhoed GC, Macdonald WBG, Oral dexamethasone in the treatment of croup: 0.15 mg/kg versus 0.3 mg/kg versus 0.6 mg/kg, Pediatr Pulmonol , 1995;20:3628. 13. Bjornson C, Klassen T, Williamson J, et al., A randomized trial of a single dose of oral dexamethasone for mild croup, N Engl J Med , 2004;351:130613. 14. Tibballs J, Shann FA, Landau LI, Placebo-controlled trial of prednisolone in children intubated for croup, Lancet , 1992;340:7458. 15. Klassen TP, Feldman ME, Watters LK, et al., Nebulized budesonide for children with mild-to-moderate croup, N Engl J Med , 1994;331:

2859. 16. Fitzgerald DA, Mellis CM, Johnson M, et al., Nebulized budesonide is as effective as nebulized adrenaline in moderately severe croup, Pediatrics , 1996;97:7225. 17. Johnson DW, Jacobson S, Edney PC, et al., A comparison of nebulized budesonide, intramuscular dexamethasone, and placebo for moderately severe croup, N Engl J Med , 1998;20:5535. 18. Wright RB, Pomerantz WJ, Luria JW, New approaches to respiratory infections in children. Bronchiolitis and croup, Emerg Med Clin North Am , 2002;20:93114. 19. Waisman Y, Klein BL, Boenning DA, et al., Prospective randomized double-blind study comparing L-adrenalin and racemic adrenalin aerosol in the treatment of laryngotracheitis (croup), Pediatrics , 1992;89:3026. 20. Canciani M, Marchi AG, Efficacy of L-epinephrine and beclomethasone aerosol in croup, Eur Resp J , 1994;7:379. 21. Custer JR, Croup and related disorders, Pediatr Rev , 1993;14:1929.

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