Lemierre syndrome refers to thrombophlebitis of the jugular veins with distant metastatic sepsis in the setting of initial oropharyngeal infection such as pharyngitis/tonsillitis with or without peritonsillar or retropharyngeal abscess.
Patients typically present unwell, with trismus and pain behind the angle of the jaw. Neck swelling may be evident. Bacteraemia and distal infective thromboembolism is common (lungs most commonly affected, however almost any organ may be involved 4). A significant proportion of patients (13% to 27%) will require diagnostic arthrocentesis due to symptoms of septic arthritis. Meningitis has also been shown to complicate up to 3% of cases 8.
An anaerobic Gram-negative bacillus, Fusobacterium necrophorum, is responsible for a majority of cases and gives rise to the term necrobacillosis 1. In up to one third of patients polymicrobial bacteremia is demonstrated, anaerobic streptococci and other miscellaneous gram-negative anaerobes are also found frequently 8. Reports contain methicillin-resistant Staph. aureus (MRSA) as well 9.
The growth of characteristic anaerobic bacteria from blood culture may be a key finding but takes too much time. Depicting jugular vein thrombophlebitis is often the first diagnostic clue. Contrast-enhanced CT is considered gold-standard.
A high degree of suspicion in the appropriate clinical setting is essential for diagnosis.
Ultrasound may show thrombus within the jugular vein or other neck or facial veins. The limitations of gray-scale ultrasound are well-described in literature. Imaging with colour Doppler may overcome some of these shortcomings. The underlying site of infection is frequently not depicted.
Nonetheless the identification of thrombophlebitis of the internal jugular vein is the first hard evidence to suggest Lemierre’s syndrome in many patients 8.
CT / MRI
Many authors consider CECT as the imaging study of choice due to availability and its allowance for visualization of complications and underlying infection 6-8. Both modalities may with a high grade of confidence:
- show an intraluminal filling defect in the jugular venous wall, frequently superior to ultrasound due to better assessment of deeper venous segments
- depict sites of septic emboli, most often encountered as pulmonary septic emboli (most commonly, see above), more readily visualized by CT
- depict the site of primary infection
Treatment and prognosis
If unrecognised and untreated, systemic dissemination can occur with a dismal prognosis; studies from the modern era still report mortality rates as high as 18% 8.
History and etymology
It is named after Andre Lemierre who first described it in 1936 3.
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- 9. Chanin JM, Marcos LA, Thompson BM et-al. Methicillin-resistant Staphylococcus aureus USA300 clone as a cause of Lemierre's syndrome. J. Clin. Microbiol. 2011;49 (5): 2063-6. doi:10.1128/JCM.02507-10 - Free text at pubmed - Pubmed citation