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 ]
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]])
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
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
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 ]
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