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Genetic and Rare Diseases Information Center (GARD)

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Lemierre syndrome


Other Names for this Disease
  • Necrobacillosis
  • Oropharyngeal infection leading to secondary septic thrombophlebitis of the internal jugular vein
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Your Question

My daughter has been diagnosed with Lemierre syndrome. Please provide me with information.

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is Lemierre syndrome?

Lemierre syndrome is a rare disease that is typically characterized by the following four findings: (1) pharyngitis or tonsillitis; followed by (2) oropharyngeal infection with Fusobacterium necrophorum, a bacteria that is normally present in the pharynx, gastrointestinal tract, and genital tract of humans; leading to (3) internal jugular vein thrombosis; and subsequent (4) septic emboli (pus-containing tissue that migrates from its original location in the body to the lungs or other parts of the body). Antibiotics are usually the primary treatment.[1]
Last updated: 11/12/2008

What causes Lemierre syndrome?

In about 90% of cases, Lemierre syndrome is caused by Fusobacterium necrophorum; however, the syndrome has also been reported with other bacteria, including Stapylococcus aureus and Streptococcus pyogenes.[1]
Last updated: 11/12/2008

What are the symptoms reported in children who have Lemierre syndrome?

In children and adolescents, Lemierre syndrome usually begins with a severe sore throat, persistent fever, and possibly chills. Some cases begin with acute otitis media. As the syndrome progresses, there is neck pain and tender swelling along the internal jugular vein. If undiagnosed, the next stage is the "metastasis" of septic emboli to the lungs, abdominal organs, brain or heart. Lung involvement typically results in a productive cough (a cough that brings up mucus or phlegm) and chest pain. Girls may report abdominal pain and have enlargement of the liver (hepatomegaly) and jaundice, all of which indicate involvement of the liver.[1]
Last updated: 2/17/2009

How is Lemierre syndrome diagnosed?

After performing blood cultures and complete blood counts, contrast computed tomography (CT) of the neck provides the definitive diagnosis. Ultrasound can also confirm internal jugular vein thrombosis.[1]
Last updated: 11/12/2008

How is Lemierre syndrome treated?

Most cases of internal jugular thrombophlebitis can be managed medically without the need for surgery of the infected vein. Prolonged courses of intravenous antibiotics (3 to 6 weeks) is usually required. Anticoagulants have sometimes been used, but efficacy is unconfirmed. Surgery of the internal jugular vein may be required only in the rare patient who fails to respond to antibiotic treatment alone.[1]
Last updated: 11/12/2008

Why do several recent reports indicate a rise in the number of cases of Lemierre syndrome?

Possible explanations for the reported rise might be: (1) a decrease in antibiotic prescription, (2) more selective use of antibiotics, and/or (3) prescription of antibiotics that are not as effective against an infection with Fusobacterium necrophorum.[2]
Last updated: 11/12/2008

How can I find additional information and supportive resources on Lemierre syndrome?

To locate information and supportive resources on Lemierre syndrome, click here.
Last updated: 11/12/2008

References
  • Schwartz RH. Chapter 30: Infections Related to the Upper and Middle Airways. In: Long SS, Pickering LK, Prober CG. Long: Principles and Practice of Pediatric Infectious Diseases, 3rd ed. PA: Churchill Livingstone, An Imprint of Elsevier; 2008;
  • Aspesberro F, Siebler T, Van Nieuwenhuyse JP, Panosetti E, Berthet F. Lemierre syndrome in a 5-month-old male infant: Case report and review of the pediatric literature. Pediatr Crit Care Med. 2008;