Lemierre's syndrome

Background

• Also known as "Lemierre's disease" and "postanginal shock including sepsis" and "human necrobacillosis"
• Is a form of thrombophlebitis and defined by a history of recent oropharyngeal infection, clinical or radiological evidence of IJ venous thrombosis and anaerobic bacteremia caused primarily by Fusobacterium necrophorum
• Usually caused by Fusobacterium necrophorum, and occasionally by other members of the genus Fusobacterium (F. nucleatum, F. mortiferum and F. varium etc.) or MRSA
• Usually affects young, healthy adults, most often developing after Strep Pharyngitis causing peritonsilar abscess
  • Anerobic Bacteria grow in the peritonsilar abscess and penetrate into the neighboring jugular vein causing thrombophlebitis and bacteremia and septic emboli and occassionally pneumonia and sepsis

Epidemiology

• Very rare, incidence rate of 0.8 cases per million in the general population
• When diagnosed, mortality is 4.6%

Clinical Features

Example of an intracranial complication: paresis of the abducens nerve (demonstrated in the right eye when looking to the right).

• Persistent sore throat, fever, and general weakness
• 2 days - 2 weeks after initial symptoms:
  • Lethargy, fevers, lymphadenopathy, painful neck
  • Often abdominal pain, diarrhea, nausea and vomiting
  • May lead to:
    • Pneumonia
    • Septic Arthritis
    • Meningitis
    • Sepsis
    • Intracranial complications (6%)

Differential Diagnosis

• Q fever
• Tuberculosis
• Pneumonia
• Viral pharyngitis
• Mononucleosis-like illnesses (EBV, CMV, acute HIV)
• Peritonsillar abscess vs. Retropharyngeal abscess vs. Ludwig's angina
• Mumps
• Sjögren Syndrome
• Heerfordt's syndrome (small percentage of sarcoidosis)

Acute Sore Throat

Bacterial infections

• Streptococcal pharyngitis (Strep Throat)
• Neisseria gonorrhoeae
• Diphtheria (C. diptheriae)
• Bacterial Tracheitis

Viral infections

• Infectious mononucleosis (EBV)
  • Patients with peritonsillar abscess have a 20% incidence of mononucleosis
• Laryngitis
• Acute Bronchitis
• Rhinovirus
• Coronavirus
• Adenovirus
• Herpesvirus
• Influenza virus
• Coxsackie virus
  • Herpangina
  • Hand-foot-and-mouth disease
• HIV (Acute Retroviral Syndrome)

Noninfectious

• Stevens-Johnson Syndrome
• Pemphigus
• Angioedema

Other

• Deep neck space infection
  • Retropharyngeal abscess
  • Ludwig's angina
  • Parapharyngeal space infection
• Peritonsillar Abscess (PTA)
• Epiglottitis
• Kawasaki disease
• Penetrating injury
• Caustic ingestion
• Lemierre's syndrome
• Peritonsillar cellulitis
• Lymphoma
• Internal carotid artery aneurysm
• Oral Thrush
• Parotitis
• Post-tonsillectomy hemorrhage
• Vincent's angina
• Acute necrotizing ulcerative gingivitis

Evaluation

POCUS shows IJ thrombus

CT of the neck showing partial thrombosis of the right internal jugular vein (black arrow) and complete thrombosis of the right external jugular vein (white arrow).

Workup

• CTA of neck
• Point of care ultrasound may reveal IJ thrombus
• Blood Cultures

Evaluation

Diagnostic criteria:

• History of oropharynx pain within last 4 wks
• Evidence of IJV thrombophlebitis/carotid sheath
• Isolation of F. necrophorum from blood
• Evidence of metastatic infection in another site (lungs)

Management

• Antibiotics (specifically anaerobic coverage of F. necrophorum PLUS strep, AND bacteroides)
  • Unasyn, Zosyn, or carbapenem for at least 3-4 wks
• Drainage of abscess
• Consider ligation of the internal jugular vein where antibiotic can not penetrate.
• No evidence for or against anticoagulation

Disposition

• Admit

See Also

• Peritonsillar abscess
• Sepsis