Lemierre syndrome

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.