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.
Critical Care
Compendium
Leave a Reply