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Lemierre’s Syndrome

  • Marios Stavrakas
  • Petros D. Karkos
  • Christos D. Karkos
Chapter

Abstract

Lemierre’s syndrome is characterized by a history of recent oropharyngeal infection and clinical or radiological evidence of internal jugular vein thrombosis, usually with septic pulmonary emboli. It is almost always caused by Fusobacterium necrophorum, although rarely other organisms have been reported as etiologies. It represents a rare diagnosis and was believed to be a “forgotten disease” for many years. Lemierre’s syndrome mainly affects previously healthy young patients (teenagers and young adults). The infection has a mortality rate of 5%. It is essential for the clinician to be aware of its existence and manifestations, as early diagnosis and appropriate treatment reduce morbidity and mortality. This chapter reviews the clinical presentation, diagnosis, and treatment of Lemierre’s syndrome.

Keywords

Lemierre’s syndrome Fusobacterium necrophorum Septic pulmonary emboli 

References

  1. 1.
    Courmont P, Cade A. Sur une septico-pyohemie de l’homme simulant la peste et causee par un streptobacille anaerobie. Arch Med Exp Anat Pathol. 1900;12:393–418.Google Scholar
  2. 2.
    Lemierre A. On certain septicaemias due to anaerobic organisms. Lancet. 1936;227:701–3.CrossRefGoogle Scholar
  3. 3.
    Karkos PD, Asrani S, Karkos CD, Leong SC, Theochari EG, Alexopoulou TD, et al. Lemierre’s syndrome: a systematic review. Laryngoscope. 2009;119:1552–9.CrossRefPubMedGoogle Scholar
  4. 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;81:458–65.CrossRefGoogle Scholar
  5. 5.
    Riordan T, Wilson M. Lemierre’s syndrome: more than a historical curiosa. Postgrad Med J. 2004;80:328–34.CrossRefPubMedPubMedCentralGoogle Scholar
  6. 6.
    Bartlett JG, Finegold SM. Anaerobic pleuropulmonary infections. Medicine (Baltimore). 1972;51:413–50.CrossRefGoogle Scholar
  7. 7.
    Eilbert W, Singla N. Lemierre’s syndrome. Int J Emerg Med. 2013;6:40.CrossRefPubMedPubMedCentralGoogle Scholar
  8. 8.
    Hagelskjaer LH, Prag J, Malczynski J, Kristensen JH. Incidence and clinical epidemiology of necrobacillosis, including Lemierre’s syndrome, in Denmark 1990–1995. Eur J Clin Microbiol Infect Dis. 1998;17:561–5.CrossRefPubMedGoogle Scholar
  9. 9.
    Vogel LC, Boyer KM. Metastatic complications of Fusobacterium necrophorum sepsis: two cases of Lemierre’s postanginal septicemia. Am J Dis Child. 1980;134:356–8.CrossRefPubMedGoogle Scholar
  10. 10.
    Armstrong AW, Spooner K, Sanders JW. Lemierre’s syndrome. Curr Infect Dis Rep. 2000;2:168–73.CrossRefPubMedGoogle Scholar
  11. 11.
    Stokroos RJ, Manni JJ, de Kruijk JR, Soudijn ER. Lemierre syndrome and acute mastoiditis. Arch Otolaryngol Head Neck Surg. 1999;125:589–91.CrossRefPubMedGoogle Scholar
  12. 12.
    Sinave CP, Hardy GJ, Fardy PW. The Lemierre syndrome: suppurative thrombophlebitis of the internal jugular vein secondary to oropharyngeal infection. Medicine (Baltimore). 1989;68:85–94.CrossRefGoogle Scholar
  13. 13.
    Roberts DS. The pathogenic synergy of Fusiformis necrophorus and Corynebacterium pyogenes. I. Influence of the leucocidal exotoxin of F. necrophorus. Br J Exp Pathol. 1967;48:665.PubMedPubMedCentralGoogle Scholar
  14. 14.
    Leugers CM, Clover R. Lemierre syndrome: postanginal sepsis. J Am Board Fam Pract Am Board Family Med. 1995;8:384–91.Google Scholar
  15. 15.
    Tadepalli S, Stewart GC, Nagaraja TG, Narayanan SK. Human Fusobacterium necrophorum strains have a leukotoxin gene and exhibit leukotoxic activity. J Med Microbiol. 2008;57:225–31.CrossRefPubMedGoogle Scholar
  16. 16.
    Riordan T. Human infection with Fusobacterium necrophorum (Necrobacillosis), with a focus on Lemierre’s syndrome. Clin Microbiol Rev. 2007;20:622–59.CrossRefPubMedPubMedCentralGoogle Scholar
  17. 17.
    Lustig LR, Cusick BC, Cheung SW, Lee KC. Lemierre’s syndrome: two cases of postanginal sepsis. Otolaryngol Neck Surg. 1995;112(6):767–72.CrossRefGoogle Scholar
  18. 18.
    Forrester LJ, Campbell BJ, Berg JN, Barrett JT. Aggregation of platelets by Fusobacterium necrophorum. J Clin Microbiol. 1985;22:245–9.PubMedPubMedCentralGoogle Scholar
  19. 19.
    Kanoe M, Yamanaka M, Inoue M. Effects of Fusobacterium necrophorum on the mesenteric microcirculation of guinea pigs. Med Microbiol Immunol. 1989;178:99–104.CrossRefPubMedPubMedCentralGoogle Scholar
  20. 20.
    Hall C. Sepsis following pharyngeal infections. Ann Otol Rhinol Laryngol. 1939;48:905–25.CrossRefGoogle Scholar
  21. 21.
    Cosgrove EF, Colodny SM, Pesce RR. Adult respiratory distress syndrome as a complication of postanginal sepsis. Chest. 1993;103:1628–9.CrossRefPubMedGoogle Scholar
  22. 22.
    Møller K, Dreijer B. Post-anginal sepsis (Lemierre’s disease): a persistent challenge. Presentation of 4 cases. Scand J Infect Dis. 1997;29:191–4.CrossRefPubMedGoogle Scholar
  23. 23.
    Wolf RFE, Konings JG, Prins TR, Weits J. Fusobacterium pyomyositis of the shoulder after tonsillitis: report of a case of Lemierre’s syndrome. Acta Orthop Scand. 1991;62(6):595.CrossRefPubMedGoogle Scholar
  24. 24.
    Jones TH, Bergvall V, Bradshaw JP. Carotid artery stenoses and thrombosis secondary to cavernous sinus thromboses in Fusobacterium necrophorum meningitis. Postgrad Med J. 1990;66:747–50.CrossRefPubMedPubMedCentralGoogle Scholar
  25. 25.
    Agarwal R, Arunachalam PS, Bosman DA. Lemierre’s syndrome: a complication of acute oropharyngitis. J Laryngol Otol. 2000;114:545–7.CrossRefPubMedGoogle Scholar
  26. 26.
    Epstein M, Pearson AD, Hudson SJ, Bray R, Taylor M, Beesley J. Necrobacillosis with pancytopenia. Arch Dis Child. 1992;67:958–9.CrossRefPubMedPubMedCentralGoogle Scholar
  27. 27.
    Eykyn SJ. Necrobacillosis. Scand J Infect Dis Suppl. 1988;62:41–6.Google Scholar
  28. 28.
    Carrie S, Fenton PA. Necrobacillosis—an unusual case of pharyngotonsillitis. J Laryngol Otol. 1994;108:1097–8.PubMedGoogle Scholar
  29. 29.
    Chand DH, Brady RC, Bissler JJ. Hemolytic uremic syndrome in an adolescent with Fusobacterium necrophorum bacteremia. Am J Kidney Dis. 2001;37:e22–1.CrossRefPubMedGoogle Scholar
  30. 30.
    Ellis GR, Gozzard DI, Looker DN, Green GJ. Postanginal septicaemia (Lemmiere’s disease) complicated by haemophagocytosis. J Infect. 1998;36:340–1.CrossRefPubMedGoogle Scholar
  31. 31.
    Potter MN, Drysdale HC, Price PA, Buck AC. Disseminated intravascular coagulation with Fusobacterium necrophorum septicaemia. Postgrad Med J. 1988;64:155–6.CrossRefPubMedPubMedCentralGoogle Scholar
  32. 32.
    Wright WF, Shiner CN, Ribes JA. Lemierre syndrome. South Med J. 2012;105:283–8.CrossRefPubMedGoogle Scholar
  33. 33.
    Alvarez A, Schreiber JR. Lemierre’s syndrome in adolescent children—anaerobic sepsis with internal jugular vein thrombophlebitis following pharyngitis. Pediatrics. 1995;96:354–9.PubMedGoogle Scholar
  34. 34.
    Gudinchet F, Maeder P, Neveceral P, Schnyder P. Lemierre’s syndrome in children: high-resolution CT and color Doppler sonography patterns. Chest. 1997;112:271–3.CrossRefPubMedPubMedCentralGoogle Scholar
  35. 35.
    Carlson ER, Bergamo DF, Coccia CT. Lemierre’s syndrome: two cases of a forgotten disease. J Oral Maxillofac Surg. 1994;52:74–8.CrossRefPubMedPubMedCentralGoogle Scholar
  36. 36.
    Gong J, Garcia J. Lemierre’s syndrome. Eur Radiol. 1999;9:672–4.CrossRefPubMedGoogle Scholar
  37. 37.
    Holzmann D, Huisman TAGM, Linder TE. Lateral dural sinus thrombosis in childhood. Laryngoscope. 1999;109:645–51.CrossRefPubMedGoogle Scholar
  38. 38.
    Albertyn LE, Alcock MK. Diagnosis of internal jugular vein thrombosis. Radiology. 1987;162:505–8.CrossRefPubMedGoogle Scholar
  39. 39.
    Karkos PD, Karkanevatos A, Panagea S, Dingle A, Davies JE. Lemierre’s syndrome: how a sore throat can end in disaster. Eur J Emerg Med. 2004;11:228–30.CrossRefPubMedPubMedCentralGoogle Scholar
  40. 40.
    Screaton NJ, Ravenel JG, Lehner PJ, Heitzman ER, Flower CDR. Lemierre syndrome: forgotten but not extinct—report of four cases. Radiology. 1999;213:369–74.CrossRefPubMedGoogle Scholar
  41. 41.
    Golpe R, Marin B, Alonso M. Lemierre’s syndrome (necrobacillosis). Postgrad Med J. 1999;75:141–4.CrossRefPubMedPubMedCentralGoogle Scholar
  42. 42.
    Mane S, Torres M, Buges J, Rivas A, Bruno C, Rodriguez E, et al. Scintigraphic demonstration of jugular obstruction in a case of Lemierre syndrome. Clin Nucl Med. 1992;17:233–5.CrossRefPubMedGoogle Scholar
  43. 43.
    Appelbaum PC, Spangler SK, Jacobs MR. Beta-lactamase production and susceptibilities to amoxicillin, amoxicillin-clavulanate, ticarcillin, ticarcillin-clavulanate, cefoxitin, imipenem, and metronidazole of 320 non-Bacteroides fragilis Bacteroides isolates and 129 fusobacteria from 28 US center. Antimicrob Agents Chemother. 1990;34:1546–50.CrossRefPubMedPubMedCentralGoogle Scholar
  44. 44.
    Agrafiotis M, Moulara E, Chloros D, Tsara V. Lemierre syndrome and the role of modern antibiotics and therapeutic anticoagulation in its treatment. Am J Emerg Med. 2015;33:733.e3–4.CrossRefGoogle Scholar
  45. 45.
    Cupit-Link MC, Nageswara Rao A, Warad DM, Rodriguez V. Lemierre syndrome: a retrospective study of the role of anticoagulation and thrombosis outcomes. Acta Haematol. 2016;137:59–65.CrossRefPubMedGoogle Scholar
  46. 46.
    Venkataraman MT, Policar M. Fever, sore throat, and pulmonary infiltrates in a 20-year-old man. Chest. 1997;112:268–70.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Marios Stavrakas
    • 1
  • Petros D. Karkos
    • 2
  • Christos D. Karkos
    • 3
  1. 1.Department of OtolaryngologyDerriford HospitalPlymouthUnited Kingdom
  2. 2.Department of OtolaryngologyAHEPA University HospitalThessalonikiGreece
  3. 3.5th Department of Surgery, Medical School, Hippocratio HospitalAristotle University of ThessalonikiThessalonikiGreece

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