CASE REPORT


https://doi.org/10.5005/jp-journals-10001-1543
International Journal of Head and Neck Surgery
Volume 14 | Issue 1 | Year 2023

Lemierre’s Syndrome: A Retrospective Case Series


Shiv Kumar1, Nishant Singh Gill2https://orcid.org/0000-0002-2725-6175, Resuciannie3

1Department of ENT (Head and Neck Surgery), Government Medical College, Kota, Rajasthan, India

2Department of Otorhinolaryngology (Head and Neck Surgery), Government Medical College, Kota, Rajasthan, India

3Government Medical College, Kota, Rajasthan, India

Corresponding Author: Nishant Singh Gill, Department of Otorhinolaryngology (Head and Neck Surgery), Government Medical College, Kota, Rajasthan, India, Phone: +91 9413725577, e-mail: nishantsky@gmail.com

Received on: 02 January 2022; Accepted on: 17 November 2022; Published on: 21 February 2023

ABSTRACT

Introduction: Lemierre’s syndrome is a rare and less commonly occurring clinical condition that generally relates to the septic thrombophlebitis of the internal jugular vein. Common bacteria causing infection are Streptococci followed by Staphylococci and Klebsiella. The major proportion of cases that come to light are due to deep neck space infections that ultimately leads to thrombotic involvement of IJV, accounts for majority of the cases. The other causes include complications of chronic suppurative otitis media and thrombosis occurring in deep veins. Since the internal jugular vein is involved the infection can also undergo hematogenous spread. The infection generally spreads to spleen, liver, kidney, heart and brain. Lemierre’s syndrome can be diagnosed on the basis of clinical symptoms, multitude of blood series and imaging. Since it is an infectious condition the treatment involves systemic antibiotic therapy and early administration of broad spectrum antibiotics in high dose become necessary for prevention of complications and systemic spread of infection. We are presenting a case series of 5 cases over a period of six years from November 2015 to November 2021.

Materials and methods: This case series focused on the cases of Lemierre’s syndrome that presented in the MBS Hospital, Kota over a course of 2 years from November 2019 to November 2021. The cases that were encountered were admitted, diagnosed and treated uneventfully. The investigations that the patients underwent were: blood cultures, chest skiagrams, contrast-enhanced computed tomography (CECT), skiagrams of mastoid bone, throat cultures and thorough clinical and ENT examination.

Results: The observation and analysis of all the 5 recorded cases reveals that 2 cases of Lemierre’s syndrome were having deep neck space infection as a causative factor in development of the disease and the other 2 cases were due to complications of chronic suppurative otitis media and the last remaining case had deep venous thrombosis as a causative factor for involvement of IJV and development of Lemierre’s syndrome. Early administration of broad spectrum antibiotics is absolutely necessary on suspicion of Lemierre’s syndrome so as to prevent adverse clinical outcome.

How to cite this article: Kumar S, Gill NS, Resuciannie. Lemierre’s Syndrome: A Retrospective Case Series. Int J Head Neck Surg 2023;14(1):6-8.

Source of support: Nil

Conflict of interest: None

Keywords: Hematogenous spread, Internal jugular vein, Internal jugular vein thrombosis, Neck infection, Neck swelling, Septicemia, Tenderness, Thrombophlebitis.

INTRODUCTION

In 1936, André Lemierre described a syndrome with post-anginal septicemia, which was complicated with thrombosis of the IJV and the presence of distant septic emboli in the patient’s bloodstream.1,2 It has gained a special status due to few cases coming to light and as a “forgotten” and a condition which is often missed in diagnosis resulting in various complications and poor patient outcomes and sometimes may result in mortality.3-5 André Lemierre explained that septic emboli reaching IJV could originate from many sites such as nasopharynx, oral cavity, ear having otitis media, mastoiditis, uterus having purulent endometritis, and appendicitis.4 The involvement of IJV provides a pathway for the spread of infection through bloodstream. The symptoms are tenderness in neck region, pain, fever with chills and rigors, and erythema in pharyngeal and peritonsillar region on oropharyngeal examination. Since the IJV is involved, the infection can potentially undergo hematogenous spread. The infection generally spreads to spleen, liver, kidney, heart, and brain.6,7 Lemierre’s syndrome can be diagnosed based on clinical symptoms, multitude of blood series, and imaging. Since it is an infectious condition, the treatment involves systemic antibiotic therapy, and early administration of broad-spectrum antibiotics in high dose becomes necessary for prevention of complications and systemic spread of infection.8,9

EPIDEMIOLOGY

Lemierre’s syndrome is generally seen in young adults.10 According to a study carried out in Denmark, there was an annual incidence of 3–6 cases of Lemierre’s syndrome per million people from 1998 to 2001, with a significantly higher annual incidence of 14.4 cases per million people with age-group of 14–24 years.10,11

Clinical Features

In most of the cases, oropharynx is the primary site of infection and exudative tonsillitis may be present in many cases, and sometimes just mild hyperemia and ulcers in the oropharynx and peritonsillar area are noted in some cases. The general signs are tenderness and hyperemia over the angle of jaw and pain with neck movement, which is sometimes associated with trismus. The time period between the onset of symptoms and development of septicemia and bacteremia may be a week or sometimes even less in some cases. When the bacteria enter the bloodstream, the most common sites of septic emboli are within the lungs and it is often a source of diagnostic dilemma as the presence of lung emboli moves clinician toward deep vein thrombosis (DVT).12 After that, the presence of infiltrative exudates on skiagrams is a common finding and development of lung abscess and pleural effusion ensues.13,14 Pneumatoceles and pneumothorax have also been reported. Hepatomegaly and splenomegaly are common, but splenic and hepatic abscess is rare.15 Hospital stays of these patients are quite long, ranging from a few days to a few weeks.

Diagnosis

Since the symptoms lie in a common spectrum, a high degree of clinical suspicion is often required to diagnose this clinical entity. In early course of disease, high-grade fever may be the only presenting symptom. The ear, nose, and throat (ENT) exam might not reveal any significant diagnostic findings13 and the diagnosis is not suspected until the microbiologic culture reports are available. Any suspicion of IJV thrombosis must be objectively confirmed and the clinician must try to rule out the condition once suspected. The contrast-enhanced computed tomography (CECT) scanning is a very useful investigation in this regard. It can help in diagnosis by showing intraluminal filling defects and low-grade selling of adjacent tissues.12 Ultrasonography (USG) is less expensive and will reveal echogenic regions of thrombosis and irregular filling defects, it is advantageous in being less expensive and invasive than a contrast-enhanced radionucleotide technetium venography scan.16,17

MATERIALS AND METHODS

This case series focuses on the cases of Lemierre’s syndrome that presented in the MBS Hospital, Kota over a course of 2 years from November 2019 to 2021.18 The cases that were encountered were admitted, diagnosed, and treated uneventfully. The investigations that the patients underwent were: blood cultures, chest skiagrams, CECT, skiagrams of mastoid bone, throat cultures, and thorough clinical and ENT examination.

INFERENCES

A total of five cases were studied and after reviewing the investigations the inferences were drawn that two of the cases presented as a complication of deep neck space infection (Fig. 1) and the infection spreading ultimately leading to spread to the IJV and development of the Lemierre’s syndrome (Fig. 2). Two cases were due to a complication of chronic suppurative otitis media (CSOM). The patients presented with chronic ear discharge and hearing loss. One case was of a 55-year-old female with a complication of DVT who had a postoperative case of prosthetic knee replacement. Four of these patients presented with chief complaints of neck rigidity and tenderness near the angle of jaw, making it the most commonly presenting complaint after fever, which was exhibited by all cases. CECT neck proved to be diagnostic as it showed peritonsillar swelling and intraluminal filling defects in the IJV in all the studied cases. USG showed echogenicity in almost all the cases but did not show any definite filling defects specifically pointing toward thrombosis. Blood cultures revealed Streptococci, Klebsiella, and Staphylococcusepidermidis in two cases.

Fig. 1: Neck swelling due to deep neck space infection in a patient with Lemierre’s syndrome (Source: Shivkumar, Department of Otorhinolaryngology, Kota)

Figs 2A and B: Subpart label shows IJV thrombosis visible in a patient with Lemierre’s syndrome (Source: Shivkumar, Department of Otorhinolaryngology, Kota)

Chest skiagrams of patients did not reveal any abnormalities except in that of the 55-year-old obese female having DVT due to an inactive lifestyle. Skiagrams of patients did not reveal any significant abnormalities except in those patients having a history of CSOM. The skiagrams revealed sclerosed mastoid air cells and fluid levels suggesting mastoiditis and presence of purulent material. One patient who had a deep neck space infection also had a sclerosed mastoid air cell cavity on the contralateral side of IJV involvement but had a normal ear exam making the infectious etiologies of neck space infections and oropharynx the major culprits of the IJV thrombosis and development of Lemierre’s syndrome and complications of an unsafe ear and venous thrombosis other rare causes of IJV thrombosis.

CONCLUSION

The observation and analysis of all five recorded cases reveal that two cases of Lemierre’s syndrome had deep neck space infection as a causative factor in the development of the disease, and the other two cases were due to complications of chronic suppurative otitis. Media and the last remaining case had deep venous thrombosis as a causative factor for the involvement of IJV and the development of Lemierre’s syndrome. Early administration of broad-spectrum antibiotics is absolutely necessary on suspicion of Lemierre’s syndrome so as to prevent adverse clinical outcomes.

ORCID

Nishant Singh Gill https://orcid.org/0000-0002-2725-6175

REFERENCES

1. Lemierre A. On certain septicemias due to anaerobic organisms. Lancet 1936;227(5874):701–703. DOI: 10.1016/S0140-6736(00)57035-4

2. Osowicki J, Kapur S, Phuong LK, et al. The long shadow of Lemierre’s syndrome. J Infect 2017;74(1):S47–S53. DOI: 10.1016/S0163-4453(17)30191-3

3. Chen FL, Jean SS, Ou TY, et al. Pulmonary empyema caused by co-infections of mycoplasma pneumoniae and Fusobacterium necrophorum: a rare case of Lemierre syndrome. J Microbiol Immunol Infect 2017;50(4):552–554. DOI: 10.1016/j.jmii.2016.11.007

4. Lee WS, Wang FD, Shieh YH, et al. Lemierre syndrome complicating multiple brain abscesses caused by extended-spectrum β-lactamase-producing klebsiella pneumonia cured by fosfomycin and meropenem combination therapy. J Microbiol Immunol Infect 2012;45(1):72–74. DOI: 10.1016/j.jmii.2011.09.012

5. Lin HY, Liao KH, Jean SS, et al. Lemierre syndrome with cervical spondylodiscitis and epidural abscess associated with direct injection of heroin into the jugular vein. J Microbiol Immunol Infect 2015;48(2):238–239. DOI: 10.1016/j.jmii.2013.11.008

6. Srivali N, Ungprasert P, Kittanamongkolchai W, et al. Lemierre’s syndrome: an often missed life threatening infection. Indian J Crit Care Med 2014;18(3):170–172. DOI: 10.4103/0972-5229.128708

7. Gupta N, Kralovic SM, McGraw D. Lemierre syndrome: not so forgotten! Am J Crit Care 2014;23(2):176–179. DOI: 10.4037/ajcc2014975

8. Lee WS, Jean SS, Chen FL, et al. Lemierre’s syndrome: a forgotten and re-emerging infection. J Microbiol Immunol Infect 2020;53(4):513–517. DOI: 10.1016/j.jmii.2020.03.027

9. Brook Itzhak. Fusobacterial head and neck infections in children. Int J Pediatr Otorhinolaryngol 2015;79(7):953–958. DOI: 10.1016/j.ijporl.2015.04.045

10. Hagelskjaer Kristensen L, Prag J. Lemierre’s syndrome and other disseminated Fusobacterium necrophorum infections in Denmark: a prospective epidemiological and clinical survey. Eur J Clin Microbiol Infect Dis 2008;27(9):779–789. DOI: 10.1007/s10096-008-0496-4

11. Ramirez S, Hild TG, Rudolph CN, et al. Increased diagnosis of Lemierre syndrome and other Fusobacterium necrophorum infections at a children’s hospital. Pediatrics 2003;112(5):e380–e383. DOI: 10.1542/peds.112.5.e380

12. Lustig LR, Cusick BC, Cheung SW, et al. Lemierre’s syndrome: two cases of postanginal sepsis. Otolaryngol Head Neck Surg 1995;112(6):767–772. DOI: 10.1016/S0194-59989570192-3

13. Weesner CL, Cisek JE. Lemierre syndrome: the forgotten disease. Ann Emerg Med 1993;22(2):256–258. DOI: 10.1016/s0196-0644(05)80216-1

14. Seidenfeld SM, Sutker WL, Luby JP. Fusobacterium necrophorum septicemia following oropharyngeal infection. JAMA 1982;248(11):1348–1350. DOI: 10.1001/jama.1982.03330110044024

15. Kern W, Dolderer M, Krieger D, et al. Lemierre’s syndrome with splenic abscesses. Dtsch Med Wochenschr 1992;117(40):1513–1517. DOI: 10.1055/s-2008-1062472

16. Celikel TH, Muthuswamy PP. Septic pulmonary emboli secondary to internal jugular vein phlebitis (postanginal sepsis) caused by Eikenella corrodens. Am Rev Respir Dis 1984;130(3):510–513. DOI: 10.1164/arrd.1984.130.3.510

17. Mañé S, Torres M, Bugés J, et al. Scintigraphic demonstration of jugular obstruction in a case of Lemierre syndrome. Clin Nucl Med 1992;17(3):233–235. DOI: 10.1097/00003072-199203000-00017

18. Carlson ER, Bergamo DF, Coccia CT. Lemierre’s syndrome: two cases of a forgotten disease. J Oral Maxillofac Surg 1994;52(1):74–78. DOI: 10.1016/0278-2391(94)90019-1

________________________
© The Author(s). 2023 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (https://creativecommons.org/licenses/by-nc/4.0/), which permits unrestricted use, distribution, and non-commercial reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.