FRCA Notes

Lemierre's Syndrome

Another rare eponymous head and neck syndrome included primarily out of interest rather than any likelihood of FRCA involvement.

Resources

  • Lemierre's syndrome (a.k.a. necrobacillosis) is a septic thrombophlebitis of the internal jugular vein
  • It is a rare sequela (<4/1,000,000) of bacterial sore throat infection
  • It is primarily a disease of the young; 70% of cases occur in patients aged 16-25yrs
  • Arises following a bacterial infection of the head and neck, most commonly pharyngitis (>85%) but also:
    • Tonsillitis
    • Glandular fever
    • Mastoiditis (3%) or otitis
    • Dental infections (2%)
    • Sinusitis

  • The most commonly implicated bacteria is the obligate anaerobe Fusobacterium necrophorum (at least 1/3rd of cases in one case series)
    • Other fusobacteria may be the causative agent e.g. Fusobacterium nucleatum
    • Other causative organisms include Staph. aureus, MRSA and Streptococci

  • The initial pharyngeal bacterial infection spreads to the parapharyngeal space, peritonsillar vasculature and ultimately the internal jugular vein
  • Formation of a septic thrombus occurs, with possible dissemination of microemboli to:
    • The lungs (most commonly)
    • The CNS
    • Intra-abdominal organs e.g. spleen, liver, kidneys
    • Joints, bones or muscles

  • Lemierre's syndrome is sometimes referred to as the 'forgotten disease' owing to its rarity and oft-generic symptoms

Local features

  • Sore throat
  • Lethargy
  • Pyrexia ± rigors
  • Neck pain and/or swelling
  • Cervical lymphadenopathy
  • Dysphagia
  • Airway features inc. trismus, difficult intubation

Effect of septic emboli

  • Respiratory: dyspnoea, cough, pleuritic chest pain, pleural effusions, haemoptysis and ARDS
  • CNS: meningitis, abscesses
  • GI: abdominal pain, diarrhoea, nausea, vomiting
  • DIC (from haemagglutinin)
  • Sepsis
  • MODS

Diagnosis

  • Bloods show a standard infective/septic picture
  • Most patients have CT-proven IJV thrombus

  • One set of diagnostic criteria include:
    • Recent pharyngeal illness +
    • Evidence of septic emboli +
    • Thrombosis of the IJV or
    • Findings of F. necrophorum in blood cultures

Antibiotics

  • Multi-agent therapy, typically a beta-lactamase-resistant beta-lactam, metronidazole ± another
  • Typically a prolonged course is required e.g. 4 - 8 weeks

Anticoagulation

  • The evidence base for this appears contentious
  • Some suggestion an inherent prothrombotic state predisposes to Lemierre's syndrome, and therefore anticoagulation is justified

Surgical intervention

  • Internal jugular vein ligation
  • Although mechanical thrombectomy has been described in other cases of septic thrombophlebitis, it hasn't been used for Lemierre's disease (as fas as I know)

  • Mortality is variably described with a modal quoted mortality of 4% (range 2 - 12%)