Epidemiology

History

• Lemierre’s Syndrome was First Described in a Case Series (20 Patients) by Lemierre in 1936: 18 patients in the series died
  • Mortality was >50% in the Pre-Antibiotic Era (Prior to the 1950’s)
• Lemierre’s Syndrome Became Far Less Prevalent in the 1950’s-1960’s
• Recent Resurgence of Reporting of Lemierre’s Syndrome Cases in the 1990’s-2000’s: unclear if this reflects a true increase in disease incidence

Demographics

• Mean Age: 22 y/o (Laryngoscope, 2009) [MEDLINE]
  • Age Range: 2 mo-78 y/o
• Male:Female Ratio: 1:1 (Laryngoscope, 2009) [MEDLINE]

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Microbiology

• Fusobacterium Species (see Fusobacterium, [[Fusobacterium]])
  • Anaerobic Gram-Negative Rods, Which are Typically a Non-Pathogenic Organism in the Oral Flora
  • Fusobacterium is Often Mistaken for Bacteroides Species
  • Fusobacterium is the Most virulent and Common Etiologic Pathogen in Lemierre’s Syndrome *
  • Species
    • Fusobacterium Nucleatum
    • Fusobacterium Necrophorum: most common organism (Laryngoscope, 2009) [MEDLINE]
• Bacteroides Species (see Bacteroides, [[Bacteroides]])
• Peptostreptococcus Species (see Peptostreptococcus, [[Peptostreptococcus]])
• Eikenella Corrodens (see Eikenella Corrodens, [[Eikenella Corrodens]])
• Streptococcus Pyogenes (see Streptococcus Pyogenes, [[Streptococcus Pyogenes]]): rare etiology
• Salmonella (see Salmonella, [[Salmonella]]): rare etiology

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Physiology

Jugular Vein Suppurative Thrombophlebitis

• Source of Infection (Laryngoscope, 2009) [MEDLINE]
  • Tonsil: 37 % of cases
    • Lemierre’s Syndrome Occurs 4-5 days After Onset of the Tonsillitis Episode
  • Pharynx (Upper Respiratory Tract) (see Pharyngitis, [[Pharyngitis]]): 30% of cases
    • Lemierre’s Syndrome Occurs 4-5 days After Onset of the Pharyngitis Episode
  • Chest (Lower Respiratory Tract): 25% of cases
  • Middle Ear/Mastoid: 2% of cases
  • Larynx: 2% of cases
  • Dental Infection (see Dental Abscess, [[Dental Abscess]]): 1% of cases
  • Paranasal Sinuses (see Acute Rhinosinusitis, [[Acute Rhinosinusitis]]): 1% of cases
  • Orbit: 1% of cases
  • Metastatic Disease: 0.5% of cases
  • Gastrointestinal: 0.4% of cases
  • Lip Piercing: 0.1% of cases
  • Infectious Mononucleosis (see Infectious Mononucleosis, [[Infectious Mononucleosis]])
• Septic Embolism with Metastatic Abscesses
  • Lung
  • Pleura
  • Bone
  • Liver
  • Spleen
• Retrograde Extension to Cavernous Sinus
  • May Result in Cavernous Sinus Thrombosis or Meningitis

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Diagnosis

Chest X-Ray (see Chest X-Ray, [[Chest X-Ray]])

• Findings
  • Pleural Effusion (see xxxx, [[xxxx]])
  • Septic Embolic Infiltrates (see Lung Nodule or Mass, [[Lung Nodule or Mass]])

Chest CT (see Chest Computed Tomography, [[Chest Computed Tomography]])

• Findings
  • Pleural Effusion (see xxxx, [[xxxx]])
  • Septic Embolic Infiltrates (see Lung Nodule or Mass, [[Lung Nodule or Mass]])

Neck CT with Intravenous Contrast (see Neck Computed Tomography, [[Neck Computed Tomography]])

• Diagnostic
• Findings
  • Internal Jugular Vein Thrombosis

Blood Culture (see Blood Culture, [[Blood Culture]])

• May Take 2-7 Days for Fusobacterium Necrophorum to Grow

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Clinical Manifestations

Cardiovascular Manifestations

• Tamponade (see Tamponade, [[Tamponade]])
  • Epidemiology: 7% of cases (Laryngoscope, 2009) [MEDLINE]

Gastrointestinal Manifestations

• Hepatic Infarct (see xxxx, [[xxxx]])
  • Epidemiology: overall, hepatic manifestations (including infarct and abscess) occur in 6% of cases (Laryngoscope, 2009) [MEDLINE]
• Jaundice/Elevated LFT’s (see Elevated Liver Function Tests, [[Elevated Liver Function Tests]])
  • Epidemiology: LFT’s are abnormal in 50% of cases
• Pyogenic Liver Abscess (see xxxx, [[xxxx]])
  • Epidemiology: overall, hepatic manifestations (including infarct and abscess) occur in 6% of cases (Laryngoscope, 2009) [MEDLINE]
• Other Gastrointestinal Symptoms
  • Epidemiology: 1% of cases (Laryngoscope, 2009) [MEDLINE]

Hematologic Manifestations

• Splenic Infarct (see xxxx, [[xxxx]])
  • Epidemiology: overall, splenic manifestations (including infarct and abscess) occur in 6% of cases (Laryngoscope, 2009) [MEDLINE]

Neurologic Manifestations

• Brain Abscess (see Brain Abscess, [[Brain Abscess]])
  • Epidemiology: overall, brain manifestations (including brain abscess, meningitis, epidural/subdural abscess, cavernous/sigmoid/transverse/lateral sinus thrombosis, and stroke) occur in 30% of cases (Laryngoscope, 2009) [MEDLINE]
• Cavernous/Sigmoid/Transverse/Lateral Sinus Thrombosis (see Cerebral Venous Thrombosis, [[Cerebral Venous Thrombosis]])
  • Epidemiology: overall, brain manifestations (including brain abscess, meningitis, epidural/subdural abscess, cavernous/sigmoid/transverse/lateral sinus thrombosis, and stroke) occur in 30% of cases (Laryngoscope, 2009) [MEDLINE]
• Epidural/Subdural Abscess (see Intracranial Epidural Abscess, [[Intracranial Epidural Abscess]] and Intracranial Subdural Abscess, [[Intracranial Subdural Abscess]])
  • Epidemiology: overall, brain manifestations (including brain abscess, meningitis, epidural/subdural abscess, cavernous/sigmoid/transverse/lateral sinus thrombosis, and stroke) occur in 30% of cases (Laryngoscope, 2009) [MEDLINE]
• Ischemic Cerebrovascular Accident (CVA) (see Ischemic Cerebrovascular Accident, [[Ischemic Cerebrovascular Accident]])
  • Epidemiology: overall, brain manifestations (including brain abscess, meningitis, epidural/subdural abscess, cavernous/sigmoid/transverse/lateral sinus thrombosis, and stroke) occur in 30% of cases (Laryngoscope, 2009) [MEDLINE]
• Cranial Nerve 11 and 12 Palsies
  • Epidemiology: 3% of cases (Laryngoscope, 2009) [MEDLINE]
• Meningitis (see Meningitis, [[Meningitis]]): may occur in cases with retrograde extension to cavernous sinus
  • Epidemiology: overall, brain manifestations (including brain abscess, meningitis, epidural/subdural abscess, cavernous/sigmoid/transverse/lateral sinus thrombosis, and stroke) occur in 30% of cases (Laryngoscope, 2009) [MEDLINE]

Ophthalmologic Manifestations

• Uveitis (see Uveitis, [[Uveitis]])
  • Epidemiology: overall, ophthalmologic manifestations (including uveitis, vitreous hemorrhage, retrobulbar mass, and cranial nerve 6 palsy) occur in 5% of cases (Laryngoscope, 2009) [MEDLINE]
• Vitreous Hemorrhage
  • Epidemiology: overall, ophthalmologic manifestations (including uveitis, vitreous hemorrhage, retrobulbar mass, and cranial nerve 6 palsy) occur in 5% of cases (Laryngoscope, 2009) [MEDLINE]

Otolaryngologic Manifestations

• Deep Neck Infection (see Deep Neck Infection, [[Deep Neck Infection]])
  • Epidemiology: 14% of cases (Laryngoscope, 2009) [MEDLINE]
  • Clinical: parapharyngeal or retropharyngeal abscess
• Dental Pain (see Dental Pain, [[Dental Pain]])
  • Epidemiology: 5% of cases (Laryngoscope, 2009) [MEDLINE]
• Internal Jugular Vein Thrombosis (see Internal Jugular Vein Thrombosis, [[Internal Jugular Vein Thrombosis]])
  • Diagnostic
    • Neck CT with Intravenous Contrast (see Neck Computed Tomography, [[Neck Computed Tomography]])
• Mastoiditis (see Mastoiditis, [[Mastoiditis]])
  • Epidemiology: 6% of cases (Laryngoscope, 2009) [MEDLINE]
• Neck Mass (see Neck Mass, [[Neck Mass]])
  • Epidemiology: 23% of cases (Laryngoscope, 2009) [MEDLINE]
• Neck Pain (see Neck Pain, [[Neck Pain]])
  • Epidemiology: 20% of cases (Laryngoscope, 2009) [MEDLINE]
• Otalgia/Otorrhea (see Otalgia, [[Otalgia]] and Otorrhea, [[Otorrhea]])
  • Epidemiology: 8% of cases (Laryngoscope, 2009) [MEDLINE]
• Orbital Pain (see Eye Pain, [[Eye Pain]])
  • Epidemiology: 1% of cases (Laryngoscope, 2009) [MEDLINE]
• Pharyngitis (see Pharyngitis, [[Pharyngitis]])
  • Epidemiology: 33% of cases (Laryngoscope, 2009) [MEDLINE]
  • Clinical: exam may be normal or may demonstrate ulcers or pseudomembrane
• Rhinorrhea (see Rhinorrhea, [[Rhinorrhea]])

Pulmonary Manifestation

• General Comments
  • Over 90% of Cases have Pleuropulmonary Involvement
• Mediastinitis (see Mediastinitis, [[Mediastinitis]])
  • Epidemiology: overall, pulmonary manifestations (including mediastinitis, empyema, pleural effusion, pneumothorax, and pneumonia) occur in 22% of cases (Laryngoscope, 2009) [MEDLINE]
• Pleural Effusion/Empyema (see Pleural Effusion-Exudate, [[Pleural Effusion-Exudate]] and xxxx, [[xxxx]])
  • Epidemiology: overall, pulmonary manifestations (including mediastinitis, empyema, pleural effusion, pneumothorax, and pneumonia) occur in 22% of cases (Laryngoscope, 2009) [MEDLINE]
• Pleuritic Chest Pain (see Chest Pain, [[Chest Pain]])
• Septic Emboli to Lungs (see Septic Embolism, [[Septic Embolism]])
  • Epidemiology: overall, pulmonary manifestations (including mediastinitis, empyema, pleural effusion, pneumothorax, and pneumonia) occur in 22% of cases (Laryngoscope, 2009) [MEDLINE]
  • Clinical
    • Cough (see Cough, [[Cough]])

Renal Manifestations

• Acute Kidney Injury (AKI) (see Acute Kidney Injury, [[Acute Kidney Injury]])

Rheumatologic Manifestations

• Myalgias (see Myalgias, [[Myalgias]])
• Osteomyelitis (see Osteomyelitis, [[Osteomyelitis]])
  • Clinical: involving the humerus, hip, clavicle, tibia, and fibula
• Septic Arthritis (see Septic Arthritis, [[Septic Arthritis]])
  • Clinical: involving the humerus, hip, clavicle, tibia, and fibula
    • Bone/Joint Pain: 8% of cases (Laryngoscope, 2009) [MEDLINE]
• Soft Tissue Abscesses (see xxxx, [[xxxx]])

Other Manifestations

• Fever (see Fever, [[Fever]])
• Sepsis (see Sepsis, [[Sepsis]])

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Treatment

Antibiotics

Agents

• β-Lactamase Resistant β-Lactam
  • Ampicillin-Sulbactam (Unasyn) (see Ampicillin-Sulbactam, [[Ampicillin-Sulbactam]]): 3 g q6hrs
  • Piperacillin-Tazobactam (Zosyn) (see Piperacillin-Tazobactam, [[Piperacillin-Tazobactam]]): 4.5 g q6hrs
  • Ticarcillin-Clavulanic Acid (Timentin) (see Ticarcillin-Clavulanic Acid, [[Ticarcillin-Clavulanic Acid]]): 3.1 g q6hrs
  • Imipenem (see Imipenem, [[Imipenem]])
  • Meropenem (see Meropenem, [[Meropenem]])
• Vancomycin (see xxxx, [[xxxx]]): may be added to cover skin flora for cases with catheter associated jugular vein suppurative thrombophlebitis
• Other Agents
  • Clindamycin (see Clindamycin, [[Clindamycin]])
  • Metronidazole (Flagyl) (see Metronidazole, [[Metronidazole]])
• Ineffective Agents
  • Gentamicin (see Gentamicin, [[Gentamicin]])
  • Macrolides (see Macrolides, [[Macrolides]])
  • Penicillin (see Penicillins, [[Penicillins]]): failure with penicillin has been reported (and β-lactamase producing Fusobacterium Necrophorum has been reported)

Duration of Therapy

• Treatment Duration Should Be at Least 4 wks (with a Minimum of 2 wks of Intravenous Therapy) and Extend Until Pulmonary Abscesses Resolve by CT Scan

Drainage of Oropharyngeal Abscesses

• If Present

Anticoagulation

• Anticoagulation Has Unclear Clinical Efficacy in Lemierre’s Syndrome with Internal Jugular Vein Thrombosis, But Without Cavernous Sinus Thrombosis
  • Some Authors Suggest Anticoagulation Only When There is Extension of Thrombus (Postgrad Med J, 1999) [MEDLINE]
• Indications
  • Cavernous Sinus Thrombosis (see Cerebral Venous Thrombosis, [[Cerebral Venous Thrombosis]])

Internal Jugular Vein Ligation

• Indications
  • Refractory Septic Emboli

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Prognosis

• Mortality Rate: 5% (Laryngoscope, 2009) [MEDLINE]

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References

• Lemierre A. On certain septicemias due to anaerobic organisms. Lancet. 1936;1:701-3
• The Lemierre syndrome: suppurative thrombophlebitis of the internal jugular vein secondary to oropharyngeal infection. Medicine 1989; 69:85-94
• Lemierre’s syndrome (necrobacillosis). Postgrad Med J. 1999;75(881):141 [MEDLINE]
• Human necrobacillosis, with emphasis on Lemierre’s syndrome. Clin Infect Dis. 2000 Aug;31(2):524-32. Epub 2000 Sep 14 [MEDLINE]
• The evolution of Lemierre syndrome: report of 2 cases and review of the literature. Medicine (Baltimore). 2002 Nov;81(6):458-65 [MEDLINE]
• Lemierre’s syndrome: A systematic review. Laryngoscope. 2009 Aug;119(8):1552-9. doi: 10.1002/lary.20542 [MEDLINE]