II. History

  1. Word "Croup" derived from Scottish for raven's "croak"

III. Epidemiology

  1. Incidence
    1. Overall Incidence: 6 cases per 100 children <6 years
    2. Accounts for 15% of pediatric respiratory visits to the emergency department
    3. Hospitalizations: 1-8% of US cases (20,000 per year)
    4. Intubation: 1-5% of cases hospitalized
  2. Boys affected more than girls by ratio of 1.5 to 1
  3. Outbreaks and epidemics occur in autumn to early winter
    1. Can occur year round sporadically
  4. Affects ages under 12 years
    1. Most common cause of Stridor in children 6 months to 3 years of age
    2. Typical age 6 months to 36 months of age (peaks between 12 and 24 months)
    3. Rare before 3 months of age

IV. Pathophysiology

  1. Subglottic infection
  2. Tracheal edema at the most narrow aspect of a child's airway
  3. Subglottic area is 4 mm in a typical infant (and croup may decrease this to 2 mm area)
  4. Every mm of airway area lost results in a loss of air flow volume to the power of 4 (Poiseuille's law)

V. Etiology

  1. Parainfluenza virus (50-75% of cases)
    1. Type 1 (18% of cases)
    2. Type 2
    3. Type 3 (more common in younger children)
  2. Adenovirus
  3. Respiratory Syncytial Virus (RSV)
  4. Influenza A and Influenza B
  5. Rhinovirus
  6. Enteroviruses
  7. Human Bocavirus (spring and fall)
  8. Mycoplasma pneumoniae (uncommon)

VI. Symptoms

  1. Low grade fever
    1. High grade fever suggests other diagnosis (see differential diagnosis)
  2. Prodrome of initially mild upper respiratory symptoms or Coryza (first 1-2 days)
    1. Nasal Congestion
    2. Rhinorrhea
  3. Upper respiratory symptoms rapidly develop
    1. Hoarseness
    2. Cough: "Barking" OR "seal-like"
    3. Inspiratory Stridor
    4. Expiratory Wheezing
    5. Dyspnea
  4. Symptoms worse at night
  5. Symptom duration <1 week (peaks at 2-4 days)
    1. Cough may persist up to 1 week

VII. Signs

  1. "Sound worse than they look" (Opposite of Epiglottitis)
    1. However, severe croup can cause complete airway obstruction
  2. Minimal Wheezing (Inspiratory Stridor instead)
  3. Mild to Moderate respiratory distress
    1. Nasal flaring
    2. Respiratory retractions
    3. Inspiratory Stridor

VIII. Labs

  1. Avoid labs unless diagnosis unclear
    1. Blood draws cause worsening distress and do not add to diagnosis in typical croup
  2. Complete Blood Count
    1. May show mild Leukocytosis

IX. Diagnosis

X. Differential Diagnosis (Croup is diagnosis of exclusion)

  1. See Airway Obstruction
  2. Angioedema
  3. Epiglottitis
    1. Less common in U.S. now since HaemophilusInfluenzae type B Immunization
  4. Bacterial Tracheitis
  5. Ludwig's Angina
  6. Peritonsillar Abscess
  7. Diphtheria
  8. Paraquat Poisoning (Herbicides)
  9. Foreign Body Aspiration
    1. History of Choking episode (88%)
    2. Neck XRay PA and Lateral if object is radiopaque
  10. Gastroesophageal Reflux
    1. Common cause of recurrent croup

XI. Imaging

  1. Avoid imaging (as with labs) unless diagnosis is unclear
    1. Typically worsens distress and does not add to diagnosis in typical croup
  2. Lateral Neck XRay
    1. Findings suggestive of croup
      1. "Steeple" sign on PA Neck XRay (40-50% of croup cases)
      2. Narrowing of subglottic region from mucosal edema
      3. Dilated hypopharynx (most sensitive finding)
      4. Images
        1. RadCroupMedPix1246.jpgFrom MedPix with permission.
    2. Findings suggestive of alternative diagnosis
      1. Epiglottitis: Thickened epiglottis
      2. Retropharyngeal Abscess: Widening retropharyngeal soft tissue
      3. Bacterial Tracheitis: Thickened trachea
  3. Chest XRay
    1. Does not diagnose croup (will not demonstrate steeple sign)
    2. Indicated only to evaluate differential diagnosis (e.g. Pneumonia) where the diagnosis is unclear

XII. Management: Home Therapy

  1. Maintain adequate Ambient humidity in house
  2. Cool mist may decrease subglottic edema
    1. No studies to support this
    2. May make Asthma worse due to irritation
    3. Theoretically decreases tracheal mucosal edema and secretion viscosity
    4. Options
      1. Cool-mist humidifier
      2. Cold weather
        1. Bundle child warmly
        2. Bring outside for 15 minutes
      3. Closed bathroom with cold shower mist
  3. Maintain adequate hydration
    1. Offer child favorite drink every 10 minutes
    2. Consider crushed ice drinks or other frozen treats
  4. Corticosteroids
    1. See Dexamethasone in Croup

XIII. Management: Emergency Department and Inpatient

  1. Primary tenet
    1. Do not distress a child with croup
    2. Avoid unnecessary procedures
    3. Position child as they are most comfortable
  2. Humidified Oxygen (cool mist)
    1. Indicated for Hypoxia or moderate to severe respiratory distress
    2. Blow-by oxygen is preferred to avoid distressing child
    3. Do not use heated humidification due to risk of burns
  3. Corticosteroids
    1. See Dexamethasone in Croup (includes Nebulized Budesonide in Croup)
    2. Single dose lasts 60-72 hours and should cover the entire croup episode (typically 2-5 days)
    3. Most important single treatment in croup
      1. Has decreased croup mortality 200% from before 1990 to now (from 0.5% to 0.03%)
    4. Dexamethasone 0.15 to 0.6 mg/kg orally (maximum 10 mg)
      1. Most emergency providers give the 0.6 mg/kg dose
      2. Onset of action in 6 hours and effect lasts for 72 hours
      3. Oral is preferred over parenteral dosing
      4. Dexamethasone is preferred over Budesonide
  4. Nebulized racemic epinephrine
    1. Indicated in moderate to severe emergency department cases with signs of respiratory distress
      1. Alpha-agonist effect Vasoconstricts, decreasing mucosal edema
      2. Beta agonsit effect increases smooth muscle relaxation as well as thinning tracheal secretions
    2. Nebulizer Dose
      1. Racemic Epinephrine 0.05 ml/kg (maximum 0.5 ml) of 2.25% in 2 ml saline via nebulizer or
      2. Standard L-Epinephrine 0.5 ml/kg (maximum 5 ml) of 1:1,000 in 2 ml saline via nebulizer
        1. As effective as Racemic Epinephrine and widely available in all ERs without special ordering
    3. Effect onset within 30 minutes and lasts up to 2 hours (some effects may persist up to 4 hours)
    4. Observe at least 2 hours after administration (some recommend 3 hours)
      1. If no recurrent Stridor, may discharge home after 2-3 hours
        1. Most croup decompensations will occur 1 to 1.5 hours after nebulized Epinephrine
      2. If Stridor recurs may give one additional Epinephrine neb and observe for additional 2-3 hours
        1. By the end of the observation period after second Epinephrine neb, Dexamethasone may be taking effect
        2. If no recurrent Stridor after 2-3 hours from second neb, may discharge home
      3. Admit patient if recurrent Stridor after second Epinephrine neb (some admit if Stridor after first Epinephrine neb)
  5. Antibiotics
    1. Not indicated unless concurrent bacterial infection
  6. Helium added to Oxygen (Heliox)
    1. Appears effective in small trials and my prevent intubation in borderline patients
      1. Vorwerk (2010) Cochrane Database Syst Rev (2): CD006822
    2. Requires mask delivery which may upset child and result in airway closure
    3. Consider with double set-up with Anesthesia to perform gas induction if sudden decompensation occurs
  7. Intubation
    1. See protocols below
    2. Indicated less frequently now with above management
    3. Significant risk of Subglottic Stenosis
    4. Use ET Tube at least 1 size smaller than predicted

XIV. Management: Outpatient Management Indications

  1. Non-toxic appearance
  2. Well hydrated and taking oral fluids
  3. Minimal or no Stridor or retractions at rest
    1. At presentation or 3 hours after Epinephrine
  4. Reassuring respiratory Vital Signs
    1. Oxygen Saturation >94%
    2. Respiratory Rate <40 per minute
  5. Reliable parents
  6. Majority of croup patients may be discharged home
    1. However, keep a high index of suspicion for children with tenuous airways
    2. Croup can cause airway compromise that rivals Epiglottitis cases of the past

XV. Management: Inpatient Observation Indications

  1. Persistent moderate to severe symptoms despite above management
    1. Dexamethasone 0.6 mg/kg and
    2. Epinephrine nebulizer treatment with observation for 3 hours (6 hours if a second Epinephrine given)
  2. Signs of respiratory distress or respiratory failure
  3. Cyanosis
  4. Tachypnea
  5. Agitation or Fatigue
  6. Stridor severity
  7. Accessory muscle use
    1. Intercostal retractions
    2. Neck or abdominal muscle use
  8. Rising arterial PCO2

XVI. Management: Severe Croup

  1. Risk of peri-respiratory arrest
    1. Notify anesthesia (may require blow-by gas induction)
    2. Notify ENT or general surgery (may require emergent surgical airway)
    3. Notify PICU
  2. Avoid upsetting child
    1. May delay Dexamethasone until airway less tenuous (consider budesonide neb)
    2. Place in position of comfort sitting in parent's lap
  3. Non-invasive strategies
    1. Administer Epinephrine neb by blow-by
    2. Consider High Flow Oxygen via large bore nasal canula (with prewarmed air blended with compressed oxygen)
    3. Consider Bipap
      1. May be tolerated with Ketamine at dissociative dose (if IV Access available)
  4. Peri-respiratory arrest emergent airway management
    1. See Modified Delayed Sequence Intubation below
    2. Gas Induction or Ketamine IV if available
    3. Video Laryngoscopy (e.g. Glidescope)
      1. Load elastic bougie in mouth ready to pass through cords
  5. References
    1. Orman and Sloas in Majoewsky (2013) EM:Rap 13(2): 4-7

XVII. Management: Modified Delayed Sequence Intubation

  1. Ketamine for Sedation
  2. Pre-oxygenate (and expel carbon dioxide)
    1. High Flow Nasal Cannula
    2. BiPap mask attached to Ventilator set to SIMV with pressure support
      1. Set to RR 0-2 and TV 8-10 ml/kg
      2. Set Pressure support to 10-15 cmH2O and PEEP 5 cmH2O
  3. Place Intubating Laryngeal Mask Airway (LMA)
    1. Provide Positive Pressure Ventilation via the LMA
  4. Intubate via pediatric bronchoscope
    1. Thread an under-sized Endotracheal Tube (ET) over pediatric bronchoscope
    2. Insert pediatric bronchoscope into the intubating LMA port
    3. Push ET Tube over the top of the bronchoscope through the Vocal Cords and remove the bronchoscope
  5. References
    1. Orman and Sloas in Majoewsky (2013) EM:Rap 13(2): 4-7

XVIII. Management: Recurrent Croup

  1. Consider esophageal reflux
  2. Consider referral to pulmonology for bronchoscopy (especially age under 3 years old)
    1. Higher Incidence of findings such as Subglottic Stenosis or cyst

Images: Related links to external sites (from Google)

Ontology: Croup (C0010380)

Definition (MSH) Inflammation involving the GLOTTIS or VOCAL CORDS and the subglottic larynx. Croup is characterized by a barking cough, HOARSENESS, and persistent inspiratory STRIDOR (a high-pitched breathing sound). It occurs chiefly in infants and children.
Concepts Disease or Syndrome (T047)
MSH D003440
ICD9 464.4
ICD10 J05.0
SnomedCT 155510007, 195702002, 71186008
English Croup, LARYNGOTRACHEOBRONCHITIS, ACUTE, CROUP, Acute laryngotracheobronchitis, Acute obstructive laryngitis [croup], croup (diagnosis), croup, Acute laryngotracheobronchitis (disorder), Croup [Disease/Finding], acute laryngotracheobronchitis, croup syndrome, croup syndromes, Croup syndrome, Croup (disorder), laryngotracheobronchitis; acute, acute; laryngotracheobronchitis, Acute Obstructive Laryngitis
French CROUP, Croup, Laryngite diphtérique
Portuguese CRUPE, Crupe
Spanish CRUP, Acute laryngotracheobronchitis, crup (trastorno), crup laríngeo, crup, síndrome crupal, Crup
Dutch kroep, acuut; laryngotracheobronchitis, laryngotracheobronchitis; acuut, Acute obstructieve laryngitis [kroep], Acute obstructieve laryngitis, Kroep, Laryngitis, acute obstructieve
Swedish Krupp
Japanese クループ, クループ, クルップ
Czech krup, Krup
Finnish Kruppi
Russian KRUP, КРУП
German Akute obstruktive Laryngitis [Krupp], KROUP, Krupp
Korean 급성 폐쇄성 후두염[크루프]
Polish Dławiec, Zapalenie krtani błonicze, Błonica krtani, Krup
Hungarian croup
Italian Croup

Ontology: Laryngotracheitis (C0023076)

Concepts Disease or Syndrome (T047)
ICD10 J04.2
SnomedCT 275494000, 55130001, 195694004
English Laryngotracheitis, NOS, Laryngotracheitis NOS, laryngotracheitis, laryngotracheitis (diagnosis), Laryngotracheitis, Laryngotracheitis (disorder), laryngitis; tracheitis, tracheitis; laryngitis, Tracheitis with laryngitis
Spanish Laryngotracheitis, Laringotraqueítis NEOM, Laringotraqueítis, laringotraqueítis (trastorno), laringotraqueítis
French Laryngotrachéite SAI, Laryngotrachéite
Dutch laryngotracheïtis NAO, laryngotracheïtis, laryngitis; tracheïtis, tracheïtis; laryngitis
Portuguese Laringotraqueíte NE, Laringo-traqueíte
German Laryngotracheitis NNB, Laryngotracheitis
Italian Laringotracheite NAS, Laringotracheite
Japanese 喉頭気管炎NOS, 喉頭気管炎, コウトウキカンエン, コウトウキカンエンNOS
Czech Laryngotracheitida, Laryngotracheitida NOS
Hungarian laryngotracheitis k.m.n., laryngotracheitis

Ontology: Laryngotracheobronchitis (C0023077)

Concepts Disease or Syndrome (T047)
SnomedCT 195938002, 85915003
English LARYNGOTRACHEOBRONCHITIS, Laryngotracheobronchitis, NOS, Tracheo-laryngeal bronchitis, Laryngotracheo bronchitis, Tracheo-laryngeal bronchitis NOS, Laryngotracheobronchitis, LTB - Laryngotracheobronchitis, Laryngotracheobronchitis (disorder), laryngotracheobronchitis
Spanish LTB - Laryngotracheobronchitis, Laryngotracheobronchitis, Bronquitis traqueolaríngea, Bronquitis traqueolaríngea NEOM, Laringotraqueobronquitis, falso crup, laringotraqueobronquitis (trastorno), laringotraqueobronquitis
French Laryngo-trachéo-bronchite, Bronchite trachéo-laryngée, Bronchite laryngotrachéite, Trachéo-laryngo-bronchite SAI
Dutch laryngotracheobronchitis, tracheolaryngeale bronchitis NAO, tracheolaryngeale bronchitis
Portuguese Bronquite traqueolaríngea, Bronquite traqueolaríngea NE, Laringotraqueobronquite, Laringo-traqueobronquite
German Laryngotracheobronchitis, laryngotracheale Bronchitis, tracheo-laryngeale Bronchitis NNB, tracheo-laryngeale Bronchitis
Italian Laringo-tracheo-bronchite NAS, Laringotracheobronchite, Laringo-tracheo-bronchite
Japanese 気管喉頭気管支炎NOS, キカンコウトウキカンシエンNOS, コウトウキカンキカンシエン, 気管喉頭気管支炎, 喉頭気管気管支炎, キカンコウトウキカンシエン
Czech Zánět hrtanu, průdušnice a bronchů, Laryngotracheobronchitida NOS, Laryngotracheobronchitida
Hungarian laryngotracheobronchitis, Tracheo-laryngealis bronchitis, Laryngotracheobtochitis, Tracheo-laryngealis bronchitis k.m.n.