Lemierre’s Syndrome

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]

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

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

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

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]])
  • 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

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]])

Treatment

Antibiotics

Agents

  • β-Lactamase Resistant β-Lactam
  • Vancomycin (see xxxx, [[xxxx]]): may be added to cover skin flora for cases with catheter associated jugular vein suppurative thrombophlebitis
  • Other Agents
  • 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

Internal Jugular Vein Ligation

  • Indications
    • Refractory Septic Emboli

Prognosis

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

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]