Croup


Article Author:
Omeed Sizar


Article Editor:
Barbara Carr


Editors In Chief:
Chaddie Doerr


Managing Editors:
Avais Raja
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Khalid Alsayouri
Trevor Nezwek
Radia Jamil
Patrick Le
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Hassam Zulfiqar
Hussain Sajjad
Steve Bhimji
Muhammad Hashmi
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Sarosh Vaqar
Mark Pellegrini
James Hughes
Beata Beatty
Beenish Sohail
Nazia Sadiq
Hajira Basit
Phillip Hynes


Updated:
6/4/2019 2:26:07 PM

Introduction

Laryngotracheitis, laryngotracheobronchitis, and laryngotracheobronchopneumonitis are all included in the spectrum of croup. Croup is a common respiratory illness of the trachea, larynx, and bronchi that can lead to inspiratory stridor and barking cough. The parainfluenza virus typically causes croup, but a bacterial infection can also cause it. Croup is primarily a clinical diagnosis. Potentially life-threatening conditions such as epiglottitis or a foreign body in the airway must be ruled out first. Corticosteroids should be administered to all patients with croup and epinephrine is reserved in those with moderate to severe croup.

Etiology

Etiology is most commonly viral with some cases caused by bacteria. 

Viral

  • Parainfluenza virus most commonly causes viral croup or acute laryngotracheitis, primarily types 1 and 2.
  • Other causes include influenza A and B, measles, adenovirus, and respiratory syncytial virus (RSV).
  • Spasmodic croup is caused by viruses that also cause acute laryngotracheitis, but lack signs of infection.

Bacterial

  • Bacterial croup is divided into laryngeal diphtheria, bacterial tracheitis, laryngotracheobronchitis, and laryngotracheobronchopneumonitis.
  • Laryngeal diphtheria is caused by Corynebacterium diphtheriae. Bacterial tracheitis, laryngotracheobronchitis, and laryngotracheobronchopneumonitis typically begin as viral infections which worsen due to secondary bacterial growth.
  • The common bacterial causes are Staphylococcus aureus, Streptococcus pneumoniae, Hemophilus influenzae, and Moraxella catarrhalis.

Epidemiology

Annually in the United States, croup accounts for 7% of hospitalizations in children younger than 5 years of age.[1] Croup affects about 3% of children per year, typically between the ages of 6 months and 3 years.[2] Parainfluenza virus accounts for more than 75% of croup infections. It is more common in boys than girls with a 1.5:1 ratio. Approximately 85% of cases are defined as mild croup, and less than 1% are considered severe croup.

Pathophysiology

Croup causes swelling of the larynx, trachea, and large bronchi due to infiltration of white blood cells. Swelling results in partial airway obstruction which, when significant, results in dramatically increased work of breathing, and the characteristic turbulent, noisy airflow known as stridor.

History and Physical

Croup is characterized by a "seal-like barking" cough, stridor, hoarseness, and difficulty breathing which typically becomes worse at night. Agitation worsens the stridor, and it can be heard at rest. Other symptoms include fever and dyspnea, but the absence of fever should not reduce suspicion for croup. Respiratory rate and heart rate may also be increased with a normal respiratory rate being between 20 to 30 breaths per minute. Visual inspection of nasal flaring, retraction, and rarely cyanosis increases suspicion for croup.

Typical Presentation

  • One to 2 days of upper respiratory infection (URI) followed by barking cough and stridor
  • Low-grade fever
  • No drooling or dysphagia
  • Duration is 3 to 7 days with the most severe symptoms on days 3 or 4

Evaluation

The most commonly used system for classifying the severity of croup is the Westley Score ranging from 0 to 17 points divided by five factors: stridor, retractions, cyanosis, level of consciousness, and air entry.

  • Inspiratory Stridor: 0 (None); 1 (When agitated); 2 (At rest)
  • Retractions: 0 (None); 1 (Mild); 2 (Moderate); 3 (Severe)
  • Air Entry: 0 (Normal); 1 (Decreased); 2 (Markedly decreased)
  • Cyanosis: 0 (None); 4 (When crying); 5 (At Rest)
  • Level of consciousness: 0 (Alert); 5 (Disoriented)

Westley score less than or equal to 2 indicates mild croup.

Westley score between 3 to 5 indicates moderate croup.

Westley score between 6 to 11 indicates severe croup and a score greater than 12 indicates impending respiratory failure.

More than 85% of children present with mild disease; severe croup is rare (less than 1%).

Croup is typically a clinical diagnosis based on signs and symptoms.

  • Consider nasal washings for influenza, RSV, and parainfluenza serologies.
  • Rule out other obstructive conditions, such as epiglottitis, an airway foreign body, subglottic stenosis, angioedema, retropharyngeal abscess, and bacterial tracheitis.
  • A frontal X-ray of the neck may be considered but is not routinely performed. It may show a characteristic narrowing of the trachea in 50% of cases, known as the steeple sign, because of the subglottic stenosis which resembles a steeple.
  • Blood tests and viral culture are advised against, as they may cause unnecessary agitation and lead to further airway swelling and obstruction.
  • Viral cultures, via nasopharyngeal aspiration, can confirm the cause but are usually restricted to research settings.
  • Consider primary or secondary bacterial etiology if a patient is not responding to standard treatments.

Treatment / Management

Treatment depends on the severity based on the Westley croup score. Children with mild croup defined as Westley croup score less than 2 are given a single dose dexamethasone. Children with moderate to severe croup defined as a Westley croup score greater than 3 are given nebulized epinephrine in addition to dexamethasone.[3] Patients with diminished oxygen saturation should receive supplemental oxygen. Moderate to severe cases require up to 4 hours of observation and if the symptoms do not improve admission is required.

Steroids

  • Corticosteroids, such as dexamethasone, results in faster resolution of symptoms, decreased return to medical care, and decreased length of stay.[4]
  • Dexamethasone is superior to budesonide for improving symptom scores, but there is no difference in readmission rates.
  • Dexamethasone at a dose of 0.15 mg/kg, 0.3 mg/kg, and 0.6 mg/kg all appear to be equally effective, 0.6 mg/kg is the most commonly used.

Epinephrine

  • For moderate to severe cases, nebulized racemic epinephrine has been found to improve symptom scores at 30 minutes, but the benefits may wear off after 2 hours. Current recommendations advocate for a prolonged period of observation in patients receiving racemic epinephrine. If symptoms do not worsen after 4 hours of observation, consider discharge home with close follow-up.
  • 0.5 mL per kg of L-epinephrine 1:1000 via nebulizer was more effective than racemic epinephrine at two hours because of its longer effects.[5]

Oxygen

  • Deliver oxygen by "blow-by" administration as it causes less agitation than the use of a mask or nasal cannula.

Intubation

  • Approximately 0.2% of children require endotracheal intubation for respiratory support.
  • Use the tube that is a one-half size smaller than normal for age/size of the patient to account for airway narrowing due to swelling and inflammation.

Hot Steam

  • Studies have not demonstrated a significant improvement with the administration of inhaled hot steam or humidified air.

Cough Medicine

  • Cough medicines, which usually contain dextromethorphan or guaifenesin, are discouraged.

Heliox

  • Little evidence supports the routine use of heliox in the treatment of croup.

Antibiotics

  • Croup is most commonly a viral disease. Antibiotics are reserved for cases when primary or secondary bacterial infection is suspected.
  • In cases of secondary bacterial infection, vancomycin and cefotaxime are recommended.
  • In severe cases associated with influenza A or B, antiviral neuraminidase inhibitors may be used.

Differential Diagnosis

Differential diagnosis includes bacterial tracheitis, epiglottitis, foreign body aspiration, hemangioma, peritonsillar abscess, neoplasm, retropharyngeal abscess, and smoke inhalation. It is extremely important to distinguish croup with epiglottitis because of the rapid deterioration in patients with epiglottitis. A cough is highly sensitive and specific for croup, whereas drooling is highly sensitive and specific for epiglottis.[6] Other symptoms to watch for in children with epiglottitis include acute onset dysphagia, odynophagia, high fever, and muffled voice. Children with peritonsillar abscess can have a sore throat, fever, and the classic "hot potato" voice. Children with retropharyngeal abscess can also have a fever, drooling, dysphagia, odynophagia but also have neck pain with a bulging posterior pharyngeal wall on neck radiography.

Pearls and Other Issues

Croup is a self-limited disease with most cases resolving within a few days. Uncommon complications may include bacterial tracheitis, pneumonia, pulmonary edema, and rarely, death.

Immunization against influenza and diphtheria may reduce the incidence of croup.

Disposition

Discharge 

  • Three hours since last nebulized racemic epinephrine
  • Able to tolerate oral fluids
  • Nontoxic appearance
  • Reliable parents and a good understanding of return precautions
  • Close follow-up for moderate or severe cases deemed appropriate for discharge

Admit

  • Persistent respiratory signs and symptoms after two or more treatments with epinephrine
  • Worsening symptoms 
  • Consider admission or longer observation periods for repeat visits

Enhancing Healthcare Team Outcomes

Croup patients are often seen by the primary care provider, nurse practitioner or the emergency department. It is important to understand that the disorder is self-limited with supportive care measures in the majority of patients. A small number of patients may benefit from pharmacological therapy. However, a community based randomized trial of children with mild to moderate croup found no difference in symptom scores between three daily doses of prednisolone 2 mg/kg and a single dose of dexamethasone 0.6 mg/kg.[7] Patients who remain symptomatic and have a recurrence of symptoms should be admitted to determine the cause. For most patients, the prognosis is excellent. An interprofessional team of nurses and clinicians working together to evaluate and treat the patient as well as educate the patients on aftercare will provide the best results. (Level V)


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Croup - Questions

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Which condition is treated with racemic epinephrine?



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A mother brings her infant to the emergency department because he has difficulty breathing and has a barking cough. The infant may have an infection caused by which of the following microorganisms?



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Laryngotracheobronchitis most commonly is caused by which of the following organisms?



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What does the steeple sign on anterior/posterior neck radiograph suggest?



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A 2-year-old girl has inspiratory stridor and a barky cough. Subglottic narrowing is seen on the anteroposterior radiograph of the neck. What is the most probable diagnosis?



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An infant presents to the emergency department with mild croup and a barky cough but no distress. Physical examination does not identify stridor. What is the most appropriate next step?



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Which of the following factors is not used for croup severity scoring?



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A young infant is suspected to have croup. On examination, he has stridor at rest and mild retractions. He is not agitated. What is the severity of croup?



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A child has mild croup. Which of the following findings is unlikely in this patient?



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What is the most common etiology of acute stridor in children?



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Which of the following viruses does not cause croup?



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Which statement is true for croup?



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An 18-month-old presents with stridor and a barky cough following an upper respiratory tract infection. Which of the following is most likely the responsible organism?



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Which of the following viruses is the most common cause of croup?



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Which of the following is a typical presentation of croup?



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Which of the following would be the most appropriate treatment of croup in a hospitalized patient?



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Which of the following would be of most concern in a patient with viral croup?



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A patient is brought in by a parent with a 2-day history of hoarse voice, sore throat, fever, and a severe, barking cough. Which of the following is most likely?



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A 22-month-old presents to the emergency department with tachypnea, tachycardia, nasal flaring, retractions, and inspiratory stridor. A barking cough that worsens at night is reported by the caregiver. The child is lying listlessly in the caregiver's arms. The patient's capillary refill is greater than 2 seconds, respiratory rate 58, pulse oximetry 93% on room air, and heart rate 168 beats/min by the pulse oximeter with a good waveform. The caregiver also relates the child has had a low-grade fever and a recent rhinovirus diagnosis from their provider in the office yesterday. Based on this data, what interventions or orders expected? Select all that apply.



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A child has been admitted with croup. The causative organism in most cases include which of the following? Select all that apply.



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Which of the following is true regarding croup? Select all that apply.



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A young child is treated for croup in the emergency department, and the mother would like to know what she can do at home to prevent the worsening of symptoms. What are the appropriate instructions to reinforce with the mother during the discharge teaching? Select all that apply.



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Croup - References

References

Weinberg GA,Hall CB,Iwane MK,Poehling KA,Edwards KM,Griffin MR,Staat MA,Curns AT,Erdman DD,Szilagyi PG, Parainfluenza virus infection of young children: estimates of the population-based burden of hospitalization. The Journal of pediatrics. 2009 May     [PubMed]
Johnson DW, Croup. BMJ clinical evidence. 2014 Sep 29     [PubMed]
Garbutt JM,Conlon B,Sterkel R,Baty J,Schechtman KB,Mandrell K,Leege E,Gentry S,Stunk RC, The comparative effectiveness of prednisolone and dexamethasone for children with croup: a community-based randomized trial. Clinical pediatrics. 2013 Nov     [PubMed]
Tibballs J,Watson T, Symptoms and signs differentiating croup and epiglottitis. Journal of paediatrics and child health. 2011 Mar     [PubMed]
Bjornson CL,Johnson DW, Croup in children. CMAJ : Canadian Medical Association journal = journal de l'Association medicale canadienne. 2013 Oct 15     [PubMed]
Bjornson CL,Klassen TP,Williamson J,Brant R,Mitton C,Plint A,Bulloch B,Evered L,Johnson DW, A randomized trial of a single dose of oral dexamethasone for mild croup. The New England journal of medicine. 2004 Sep 23     [PubMed]
Eghbali A,Sabbagh A,Bagheri B,Taherahmadi H,Kahbazi M, Efficacy of nebulized L-epinephrine for treatment of croup: a randomized, double-blind study. Fundamental     [PubMed]

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