Lemierre’s Syndrome

Introduction

  • Lemierre's syndrome is a thrombophlebitis (vein inflammation due to thrombus) of the internal jugular vein.
  • It occurs when a bacterial throat infection progresses to the formation of a peritonsillar abscess.
  • The most likely microbial offenders are normal oral flora, most often Fusobacterium necrophorum.
  • Deep in the abscess, these anaerobic bacteria flourish and may eventually penetrate the abscess wall to infect nearby structures.
  • Spread of infection to the nearby jugular vein may lead to clot formation, due to pro-thrombotic bacterial endotoxins.
  • Once this nidus of infection is established, the bacteria can spread through the bloodstream.
  • Pieces of the infected clot embolize, blocking branches of the pulmonary artery, and causing metastatic abscesses.

lemm_0.png

Illustration of bacterial passage from abscess to vasculature; internal jugular vein thrombophlebitis; and systemic septic embolus from lookfordiagnosis.com

Epidemiology

  • Lemierre’s syndrome appears to have been relatively common in the pre-antibiotic era.
  • Although it is now a rare disease with an incidence of approximately 1 per 1,000,000 persons per year, there seems to be a resurgence in the disorder.
  • One hypothesis to explain the resurgence is that primary care physicians are now heavily discouraged from prescribing antibiotics for sore throats, and that early infections which would previously have been aborted by antibiotics now progress to the full blown syndrome.

Clinical Features

  • Lemierre’s should be suspected in patients with antecedent pharyngitis, septic pulmonary emboli, and persistent fever despite antimicrobial therapy.

  • The septicemic illness typically begins 4–5 days after the onset of the sore throat, but the interval may be up to 12 days.

  • Several features are highly suggestive of Lemierre’s syndrome including:

  • Previously healthy young adult (mean age 20 years)
  • History of sore throat in the preceding week
  • High fever and rigors
  • Signs of internal jugular venous thrombosis, presenting as neck swelling or cervical spine pain
  • Dry cough
  • Pleuritic pain and possible effusion
  • Chest radiograph with nodular, cavitary lesions
  • Metastatic abscess may occur, e.g. septic arthritis

CTSvc.png

Abhishek, Agarwal, Sandeep, Singla, & Tarun, Pandey. (2013). Lemierre syndrome from a neck abscess due to methicillin-resistant Staphylococcus aureus. Brazilian Journal of Infectious Diseases, 17(4), 507-509.

Laboratory Diagnosis

  • Patients typically have a neutrophilic leukocytosis.
  • Liver function tests are abnormal in approximately 50% of patients and the C-reactive protein is invariably raised.
  • However, the key to laboratory confirmation of the diagnosis of Lemierre’s syndrome is blood or abscess culture of likely specimens.

Differential Diagnosis

The differential diagnosis of Lemierre’s includes the following conditions:

  •  Viral pharyngitis
  • Infectious mononucleosis
  •  Bacterial pneumonia
  • Aspiration pneumonia
  • Other pneumonia

Therapy

  • A key point regarding Lemierre’s syndrome is the slow response to antibiotics.
  • In three series, the median time from initiation of appropriate antibiotic therapy to resolution of fever ranged from eight to 12 days. Key features in the management of Lemierre’s are:
  • Antibiotic therapy, generally penicillin and metronidazole, for up to 1 month
  • Drainage of any accessible abscesses
  • Anticoagulation is rarely indicated

Prognosis

  • The illness varies in its severity, with some reports of patients being discharged within days while other cases are fatal.
  • In the pre-antibiotic era, the prognosis of Lemierre’s syndrome was grave. In 1936, Lemierre himself reported that 18 of 20 of his cases died. 
  • With antibiotic therapy, despite the severity of sepsis, full recovery can generally be expected, with mortality figures in different studies ranging from 0% to 18%.

References

1. Baker CC, Petersen SR, Sheldon GF. Septic phlebitis: a neglected disease. Am J Surg 1979; 138:97.

2. Khan EA, Correa AG, Baker CJ. Suppurative thrombophlebitis in children: a ten-year experience. Pediatr Infect Dis J 1997; 16:63.

3. Riordan T, Wilson M. Lemierre's syndrome: more than a historical curiosa. Postgrad Med J 2004; 80:328.

4. Chirinos JA, Lichtstein DM, Garcia J, Tamariz LJ. The evolution of Lemierre syndrome: report of 2 cases and review of the literature. Medicine (Baltimore) 2002; 81:458.

5. Alston JM. Necrobacillosis in Great Britain. BMJ1955;ii:1524–8.

6. Abhishek, Agarwal, Sandeep, Singla, & Tarun, Pandey. (2013). Lemierre syndrome from a neck abscess due to methicillin-resistant Staphylococcus aureus. Brazilian Journal of Infectious Diseases, 17(4), 507-509.

7. Lemierre A. Septicaemias and anaerobic organisms. Lancet1936;i:701–3.

8. Spelman, D. Suppurative thrombophlebitis. In: UpToDate, Post, TW (Ed), UpToDate, Waltham, MA, 2015

9. Lemierre's syndrome. (2015, May 9). In Wikipedia, The Free Encyclopedia. Retrieved 00:48, July 22, 2015