Lemierre Syndrome

Lemierre syndrome is septic thrombophlebitis of the internal jugular vein following oropharyngeal infection, typically caused by Fusobacterium necrophorum, with metastatic septic emboli.

• Incidence is about 1 in 1 million per year; typically healthy adolescents and young adults, after tonsillitis or pharyngitis (or odontogenic infection and mastoiditis).
• Other organisms: Streptococcus species, Bacteroides, Eikenella, and Staphylococcus aureus.
• Mortality 5 to 10% in the modern era.
• Called the "forgotten disease" since the antibiotic era.

• Sore throat followed within 1 to 3 weeks by high fever, rigors, neck pain, swelling and tenderness along the sternocleidomastoid.
• Septic emboli to the lungs (cavitating nodules, empyema, pneumothorax), joints (septic arthritis), liver, spleen, bones, and brain (abscess, meningitis).
• Trismus, cranial nerve palsies, and cavernous sinus thrombosis in contiguous spread.
• Sepsis, jaundice, and DIC.

• Blood cultures with anaerobic bottles (growth may take 3 to 5 days; alert the laboratory).
• Contrast enhanced CT neck (thrombosis of the internal jugular vein with perivenous inflammation) or Doppler ultrasound.
• CT chest for septic emboli and CT head/MRI for intracranial complications.
• Blood tests: leucocytosis, raised CRP and procalcitonin, abnormal liver tests, thrombocytopenia.
• Exclude infectious mononucleosis, deep neck abscess, endocarditis, peritonsillar abscess, and TB.

• Prolonged IV antibiotics (3 to 6 weeks): metronidazole 500 mg IV every 8 hours plus ceftriaxone 2 g IV daily, or piperacillin tazobactam 4.5 g IV every 6 to 8 hours, or meropenem 1 g every 8 hours; clindamycin 600 to 900 mg every 8 hours as an alternative; penicillin monotherapy is unreliable due to beta lactamase production.
• Drainage of abscesses (neck, lung, empyema, joint) and surgical source control (tonsillectomy, dental extraction).
• Anticoagulation: controversial; consider heparin then warfarin (3 months) for extension of thrombus, cavernous sinus thrombosis, or persistent sepsis despite antibiotics; not routinely indicated.
• ICU support for sepsis; internal jugular vein ligation or excision is reserved for refractory cases.
• Follow up imaging for resolution of thrombosis and pulmonary lesions.