Lemierre syndrome (also known as postanginal septicemia) refers to thrombophlebitis of the internal jugular vein(s) with distant metastatic sepsis in the setting of initial oropharyngeal infection such as pharyngitis/tonsillitis with or without peritonsillar or retropharyngeal abscess.
Since the advent of antibiotics, the incidence of Lemierre syndrome has reduced significantly, now affecting between 0.6-2.3 per million people. There is a male predominance, with 70% of patients between the ages of 16 and 25 years.
Patients typically present unwell, with trismus and pain behind the angle of the jaw. Neck swelling may be evident. Bacteremia and distal infective thromboembolism are 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 (80%) 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 internal jugular vein thrombophlebitis is often the first diagnostic clue. Contrast-enhanced CT is considered gold-standard. Cases with isolated thrombophlebitis of tributaries of the internal jugular vein, e.g. common facial vein, have been described 2.
A high degree of suspicion in the appropriate clinical setting is essential for diagnosis.
Ultrasound may show hyperechoic thrombus within the internal jugular vein or other neck or facial veins. Spectral Doppler of a patent vessel may show loss of respiratory phasicity and cardiac pulsatility. This indicates the presence of a more proximal thrombus not accessible sonographically. The limitations of grey-scale ultrasound are well-described in literature. Imaging with color 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.
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. Treatment is usually with anticoagulation, intravenous antibiotics and potentially surgically drainage for non-resolving abscesses.
History and etymology
It is named after Andre Lemierre (1875–1956) 11, a French bacteriologist who described the typical features of the disease in 1936 3. However Courmont and Cade first described the condition in 1900 as "human necrobacillosis" 11.
- isolated oropharyngeal infection
- isolated jugular vein thrombosis
- cervical lymphadenitis
- pneumonia or metastatic disease
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