July 8, 2021
A 26-year-old woman with worsening right maxillary facial pain, swelling, fevers, and chest pain; she presented to the ED 1 day prior for a right upper lip abscess that was incised and drained.
Lemierre Syndrome
- Background:
- First highlighted in the 1930s by Andre Lemierre, Lemierre syndrome is characterized classically by thrombophlebitis of the internal jugular vein, predominantly anaerobic septicemia with distant septic emboli, and each occurring after a recent oropharyngeal infection.
- While the thrombus classically occurs in the internal jugular vein, thrombosis of an associated tributary vein can occur in isolation. Cases of diagnostic confusion have occurred when the internal jugular vein was not initially involved; this highlights the importance of awareness of uncommon variants such as isolated facial vein thrombosis.
- In this case, the patient was found to have MRSA bacteremia and after initial presentation also developed septic arthritis and intramuscular abscesses.
- Clinical Presentation:
- Patients frequently have a history of recent or concurrent pharyngitis or tonsillitis, with or without abscess.
- Patients are most commonly in late adolescence or young adulthood.
- Patients can present with high-grade fevers, rigors, and respiratory distress. Most patients will also have focal neck and/or throat pain. Neck swelling may be seen.
- Septic emboli can result in involvement of any organ, including septic arthritis, meningitis, liver infection, skin infection, and, most commonly, septic pulmonary emboli.
- Key Diagnostic Features:
- The diagnosis should be considered in patients with a combination of recent pharyngitis/tonsillitis, subsequent systemic or respiratory symptoms, fevers despite antimicrobial therapy, and septic pulmonary emboli.
- Diagnosis is aided by culture of blood or exudate. The most common pathogen is Fusobacterium necrophorum. Other agents include Bacteroides species, other Fusobacterium species, Eikenella corrodens, or polymicrobial infections.
- CT with contrast may demonstrate luminal filling defects in the internal jugular vein, but can also affect tributary veins of the face/neck, even in isolation. Chest CT shows signs of septic pulmonary emboli.
- Differential Diagnoses:
- The combination of head/neck thrombophlebitis and septic pulmonary emboli in the setting of recent head, neck, or upper respiratory tract infection is pathognomonic for Lemierre syndrome.
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Treatment:
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Treatment primarily consists of prolonged antibiotic therapy with surgical drainage/excision of associated abscesses at primary or distant infection sites.
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Escalation of medical or surgical therapy, including internal jugular vein ligation/excision or systemic anticoagulation, is controversial. It is also case-dependent and considered depending on the extent of thrombus involvement and failure of more conservative treatment.
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