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Referral and warning signs
When to refer
IMMEDIATE URGENT referral/transfer to hospital (H/RR) with capabilities for paediatric airway management (paediatric anaesthesia/ENT/ICU) for ALL suspected cases. This is a life-threatening emergency.
Red flags
- Rapid onset fever, sore throat, difficulty swallowing
- Drooling
- Stridor
- Tripod/Sniffing position
- Muffled voice
- Toxic appearance, anxiety, air hunger
Signs and symptoms
- Rapid onset (hours) of high fever.
- Severe sore throat.
- Dysphagia (difficulty swallowing), Odynophagia (painful swallowing).
- Drooling.
- Muffled voice ('hot potato voice').
- Inspiratory stridor.
- Respiratory distress, anxiety, air hunger.
- Characteristic 'tripod' or 'sniffing' position (sitting upright, leaning forward, neck extended, chin thrust out, mouth open).
- Patient often appears toxic, anxious, and acutely ill.
Risk factors
- Lack of Haemophilus influenzae type b (Hib) vaccination.
- Age 2-6 years (historically), but can occur at any age.
- Immunodeficiency.
Diagnosis
Clinical assessment
Clinical diagnosis based on high index of suspicion in a patient with rapid onset fever, sore throat, drooling, stridor, and characteristic posture. DO NOT attempt to visualize the epiglottis with a tongue depressor in a suspected case outside a controlled setting (e.g., operating room) - can precipitate complete airway obstruction. Lateral neck X-ray may show classic 'thumb sign' (swollen epiglottis) but should not delay management if unstable. Definitive diagnosis via direct laryngoscopy in controlled setting (OR). Differential diagnosis: Croup (usually viral, barking cough, slower onset, less toxic), Bacterial tracheitis, Retropharyngeal/Peritonsillar abscess, Foreign body aspiration, Angioedema.
Investigations
- Direct Laryngoscopy (in OR): Visualizes swollen, cherry-red epiglottis. Allows for intubation.
- Lateral Neck X-ray: May show 'thumb sign'. Perform only if stable and doesn't delay airway management.
- Blood cultures, Epiglottic cultures (after airway secured): Identify causative organism (often H. influenzae type b, also Strep. pneumoniae, Staph. aureus, Group A Strep).
Diagnostic criteria
Strong clinical suspicion is paramount. Definitive diagnosis by visualization of swollen epiglottis.
Management
Non-pharmacological management
- Airway Management is the TOP priority - Treat as Medical Emergency.
- - Keep patient (especially child) calm, allow position of comfort (often tripod). Avoid agitation.
- - Minimize procedures (e.g., IV attempts, throat exam) until airway secured.
- - Administer humidified oxygen.
- - Prepare for immediate intubation (ideally in OR by experienced personnel - anaesthesia/ENT). May require smaller ETT size. Tracheostomy equipment should be available.
- - Once airway secured, obtain cultures (blood, epiglottis).
- - Admit to ICU/HDU setting.
Pharmacological treatment
- Antibiotics: Start IV antibiotics immediately after securing airway/obtaining cultures.
- - Ceftriaxone 50-100 mg/kg/day IV (max 2-4g/day) OR Cefotaxime.
- - Chloramphenicol is an alternative if cephalosporins unavailable/contraindicated.
- - Continue IV antibiotics until clinically improved, then consider switch to oral course (e.g., Amoxicillin/Clavulanate, Cefuroxime axetil) to complete 7-10 day total course based on sensitivities.
- Corticosteroids: Role uncertain, may be considered to reduce inflammation (e.g., Dexamethasone) but airway management remains key.
- Racemic Epinephrine Nebulization: Generally NOT effective or recommended.
Monitoring
- Continuous monitoring of respiratory status, oxygen saturation, vital signs.
- Monitor for signs of airway obstruction relief after intubation/treatment.
- Monitor clinical response to antibiotics.
Complications
- Sudden complete airway obstruction
- Respiratory arrest
- Death
- Epiglottic abscess
- Pneumonia
- Meningitis, sepsis (if bacteraemia)
- Pulmonary oedema (post-obstructive)
Prevention
- Haemophilus influenzae type b (Hib) vaccination (part of routine childhood immunization schedule - Pentavalent vaccine).
- Rifampicin prophylaxis for close household/daycare contacts if index case caused by Hib (consult guidelines).
Patient education
- (Usually to parents/guardians) Explain the critical nature of the illness and the need for immediate airway management.
- Explain procedures (intubation, antibiotics).
- Discuss Hib vaccination status and importance.
- Explain potential need for contact prophylaxis.
Uganda-specific notes
Incidence drastically reduced due to Hib vaccine in Pentavalent. Still consider in unvaccinated/partially vaccinated children or adults. Requires immediate recognition and transfer to facility capable of securing airway. Avoid examining throat.
References
- Uganda Clinical Guidelines (UCG) 2023
Chapter 5, Section 5.2.4