Lemierre Syndrome

A special thank you to Dr. Paul Bunce who did an amazing job and received an unprecedented ovation with morning report today.  Also, thank you to Team 1, Dr. Lindsay Melvin and Dr. Anthony Lott who presented a fascinating case!

Today we discussed a case of sepsis secondary to an acute oropharyngeal infection cause by the organism Fusobacterium necrophorum.

This prompted a discussion of the main syndrome associated with Fusobacterium necrophorum,   Lemierre Syndrome.

Some key learning points:

1.) Pharyngitis (sore throat) is the key presenting symptom - (80 - 87%) of cases
2.) There can be metastatic lesions (septic emboli) in the lung - 80% of cases
3.) Metronidazole or Clindamycin are the key antibiotics to cover Fusobacterium necrophorum
4.) Anticoagulation is controversial and is not the standard of care currently, but a consultation with hematology may be warranted.
5.) Early surgical intervention may be needed if the patient does not respond or improve with initial therapy.

Lemierre Syndrome:
The other name given to this condition is postanginal septicemia, which involves septic thrombophlebitis of the internal jugular vein, secondary to an acute oropharyngeal infection.  The syndrome was first characterized by Andre Lemierre (1875-1956) in 1936, who reported on dozens of cases from his own clinical experience.  He was a physician, and professor of microbiology and infectious diseases.  He published a description of 20 cases in The Lancet describing the clinical syndrome.

The key clinical features of this syndrome are that the initial source is usually within the pharynx (87% of cases), and IJV thrombus is present in approximately 70% of cases.  It can also cause metastatic lesions in the lung (80%) and joints (16% of cases).

The key symptoms and signs are sore throat (80%), fever (80%), and swollen or tender neck (52%).  Most of the diagnosis are made via cultures (70%), or radiology (IJ thrombus) - 15%.

The main laboratory findings are leukocytosis (75%) and thrombocytopenia (24%), and hyper bilirubinemia (32% of cases).

From: The Evolution of Lemierre Syndrome. Chirinos, et. al. Medicine. 2002

Most cases of Lemierre syndrome occur in younger patients (usually 16- 25 years of age).

The usual infectious agent in Lemierre syndrome is Fusobacterium necrophorum (82% of cases).  Other bacteria that can cause this syndrome include Bacteroides sp., and Group B and C Streptoccus.

This bacteria is a normal inhabitant of the oral cavity, and is a strict anaerobe, non-motile gram negative rod.  It has a characteristic appearance on gram stain (filaments, short rods, and coccoid elements).

Management:

Supportive Care:
A concern in any patient presenting with an oropharyngeal abscess is potential airway compromise, so stridor or any signs of respiratory distress should prompt a clinician to notify the ICU.  These patients can also become very sick, and may require ionotropic support.

Antimicrobials:
The initial management to a patient with suspected Lemierre syndrome includes antimicrobials with anaerobic coverage.  Either Metronidazole or Clindamycin are appropriate, with possibly additional coverage in the empiric setting for a polymicrobial infection.

Examples of empiric coverage: 
Cefazolin or Penicillin  G + Flagyl or Clindamycin

Vanco + Flagyl (if MRSA is suspected)

If the patient is septic, the "big guns" (Pip/Tazo, Meropenem) also provide excellent empiric coverage for this syndrome (gram positives, gram negatives, and anaerobic coverage).

Imaging:
Diagnostic Imaging: CT, MRI, or U/S to assess the neck and pharyngeal space, as well as imaging of the vessels is important.  Practically on call overnight a CT scan with contrast would be the imaging modality of choice.

Anticoagulation:   
The role of anticoagulation for an IJ thrombus in a patient with Lemierre syndrome is controversial. The outcome of most patients is very good (mortality is 6.4% in one case series).  There are no RCT's to clarify this question, but a discussion with Infectious Diseases and Hematology should be a part of the initial management plan.

Surgical Debridement / Source Control:  
If the patient has uncontrolled sepsis, or a more complication soft tissue infection (abscess, etc.)  Than a consultation with a surgical specialist is also warranted.

Reference:
The Evolution of Lemierre Syndrome.  Chirinos, J.A. et al. 2002. Medicine. 81: 458-65.

Lemierre Syndrome. Wright, W.F. et al. 2012. Southern Medical Journal. volume 105. Number 5.