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Home | CCC | Lemierre syndrome

Lemierre syndrome

by Dr Chris Nickson, last update June 2, 2019

OVERVIEW

  • Lemierre syndrome is thrombophlebitis of the internal jugular (IJ) vein and bacteraemia caused by primarily anaerobic organisms, following a recent oropharyngeal infection
  • a ‘forgotten’ disease first described by Andre Lemierre in 1936, Lemierre syndrome has increased in incidence since the 1990s
  • mostly affects children, adolescents and young adults
  • delayed diagnosis is common

CAUSE

Infection due to:

  • fusobacterium species, especially fusobacterium necrophorum most commonly (an anaerobic GNB, a part of normal oral flora)
  • One third have a  polymicrobial bacteraemia (typically anaerobic streptococci and other miscellaneous gram-negative anaerobes)
  • other gram positive causes have been reported (e.g. S.aureus)

PATHOPHYSIOLOGY

Primary infection is followed by local invasion of the lateral pharyngeal space then septic thrombophlebitis of the IJ vein

  • primary source of infection is commonly the palatine tonsils and peritonsillar tissue
  • other sources include the lungs, middle ear, mastoid, teeth and sinuses
  • may follow on from a viral (e.g. EBV) or bacterial pharyngitis of another cause

Metastatic infections following the IJ thrombophlebitis occur in >2/3 of cases

  • lungs, joints, liver, muscle, pericardium, brain and skin

Complications

  • Thrombosis may propagate from the IJ vein inferiorly into the subclavian vein or superiorly into the cavernous, sigmoid or transverse sinuses
  • metastatic infection
  • DIC (~5%)
  • meningitis (3%)
  • septic shock
  • death (near 100% mortality in the pre-antibiotic era)

CLINICAL FEATURES

Clinical manifestations vary according to the presence of metastatic complications

  • Fever (but may appear otherwise well)
  • Pharyngitis/peritonsillar abscess
  • Anterior cervical lymphadenopathy
  • Neck mass/tenderness (~50%)
  • Trismus
  • Cranial nerve 10, 11, 12 palsies
  • Septic arthritis (most commonly hip or knee)
  • Jaundice/hepatomegaly
  • Shock

INVESTIGATIONS

Bedside

  • glucose +/- VBG

Laboratory

  • FBC, UEC, LFTs
  • Septic screen including blood cultures
  • Joint fluid aspirates if appropriate

Imaging

  • CXR — metastatic infection of the lungs is common: nodules, cavities, effusions
  • CT Neck with contrast — IJV thrombophlebitis and/or neck mass/ collections

MANAGEMENT

Resuscitation

  • address life threats such as airway compromise, respiratory failure and septic shock

Specific therapy

  • antibiotics
    • if severely unwell: piperacillin+tazobactam 4/0.5 g (child: 100+12.5 mg/kg up to 4/0.5 g) IV q8h
    • if systemically unwell: benzylpenicillin 1.2 g (child: 50 mg/kg up to 1.2 g) IV q6h AND metronidazole 500 mg (child: 12.5 mg/kg up to 500 mg) IV q12h
    • if systemically well: amoxicillin/ clavulanate or clindamycin
    • typically 2-6 weeks duration
  • anticoagulation
    • controversial (administered in about 1/4 to 1/3 of case reports)
    • some recommend if IJ thrombophlebitis leads to venous sinus thrombosis or failure to improve with antibiotics

Supportive care and monitoring


References and Links

LITFL

  • André-Alfred Lemierre (1875 – 1956)
  • Lemierre A. On certain septicemias due to anaerobic organisms. Lancet. 1936; 227(5874): 701–3 
  • ENT Equivocation 002 – Just a Sore Throat

Journal articles

  • Eilbert W, Singla N. Lemierre’s syndrome. Int J Emerg Med. 2013 Oct 23;6(1):40. doi: 10.1186/1865-1380-6-40. PubMed PMID: 24152679; PubMed Central PMCID: PMC4015694.
  • Riordan T. Human infection with Fusobacterium necrophorum (Necrobacillosis), with a focus on Lemierre’s syndrome. Clin Microbiol Rev. 2007 Oct;20(4):622-59. Review. PubMed PMID: 17934077; PubMed Central PMCID: PMC2176048.

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About Dr Chris Nickson

An oslerphile emergency physician and intensivist suffering from a bad case of knowledge dipsosis. Key areas of interest include: the ED-ICU interface, toxicology, simulation and the free open-access meducation (FOAM) revolution. @Twitter | INTENSIVE| SMACC

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