EM@3AM: Lemierre’s syndrome

Author: Rachel Bridwell, MD (@rebridwell, EM Attending Physician) // Reviewed by: Alex Koyfman, MD (@EMHighAK, EM Attending Physician, UTSW / Parkland Memorial Hospital); Brit Long, MD (@long_brit, EM Attending Physician, San Antonio, TX)

Welcome to EM@3AM, an emDOCs series designed to foster your working knowledge by providing an expedited review of clinical basics. We’ll keep it short, while you keep that EM brain sharp.


A 23-year-old male presents for severe throat pain and cough. He states that his neck hurts, noticing that the left side of his neck is red and painful. Review of systems is remarkable for a recent bout of pharyngitis that initially improved.

Vital signs include BP 91/49, HR 130, T 102.2 temporal, RR 25, SpO2 91% on room air. He appears toxic.  The ENT exam reveals a midline uvula; soft mouth floor; prominent generalized cervical, submandibular, and submental swelling with corresponding lymphadenopathy; but no voice changes or difficulty tolerating secretions. His neck is red and tender, with mild swelling overlying the left side of neck and a painful tracheal rock. He is tachycardic and tachypneic, with multiple areas of wheezes and rhonchi.

What is the most likely diagnosis?


Answer: Lemierre’s Syndrome1-16

Epidemiology:

  • Suppurative thrombophlebitis of the internal jugular vein
  • Incidence of 3.6 cases per 1 million persons
    • Predominantly in 15-24 year old
  • Resurgence in Group A streptococcus negative rapid testing
  • Pathophysiology: hematogenous spread of bacteria from commonly an oropharyngeal infection via tonsillar vein vs. lymphangitis
    • Cases can occur after tonsillitis (37%), pharyngitis (30%)
    • More rare sources include:
      • Mandibular fracture
      • Bartholin gland abscess with hematogenous spread
  •  Mortality was previously 90%, but more recent rates are 5-10% with early antibiotics
  • Increased incidence with antibiotic resistance, decreased tonsillectomies, and use of rapid Strep pharyngitis testing
  • Named for Anton Lemierre who published a 20 patient case series of this disease—18 patients in that cohort died

 

Microbiology:

  • Oral flora:
    • Fusobacterium necrophorum (classically—accounts for 33%)
    • Fusobacterium nucleatum
    • Streptococcus
    • Staphylococcus
    • Klebsiella pneumoniae

 

Evaluation:

  • Assess ABCs and begin resuscitation
    • May present with toxic appearance
      • Shock is unsurprisingly associated with increased mortality
    • Up to 97% will experience a cough, which can be due to septic pulmonary emboli
    • Abdominal symptoms to include nausea, vomiting, and abdominal pain occur in 50% of patients
    • 83% of patients demonstrate fever
  • Classic triad: pharyngitis, anterior neck tenderness/swelling, and non-cavitary pulmonary infiltrates
  • Patient may be leaning forward to optimize diameter of the airway
  • Perform a complete physical examination
    • ENT: Unilateral neck pain aggravated by movement
    • Integumentary: Overlying erythema, edema, or tenderness on ipsilateral neck may be present in 52% of patients
    • Neck: Bilateral cervical lymphadenopathy (LAD) whereas mononucleosis often has unilateral cervical LAD, torticollis
    • Pulmonary: Wheezes, rhonchi, or diminished breath sounds due to septic pulmonary emboli
    • MSK: Assess for evidence of arthralgias indicating septic arthritis
    • Neuro: may demonstrate cranial nerve palsies (seen in 6% of patients)
  • Laboratory evaluation:
    • Leukocytosis with left shift may occur as well as thrombocytopenia
    • 1/3 of patients will demonstrate hyperbilirubinemia with elevation in liver associated enzymes
      • 11-49% of these patients will demonstrate jaundice
      • Splenomegaly and hepatomegaly seen in patients with large septic thromboembolic burden
  • Imaging:
    • Computed tomography (CT) neck with contrast is initial study of choice
      • Allows for imaging of clot and potential abscess formation
      • May demonstrate intraluminal filling defect, peripheral rim enhancement
      • Thrombus can measure 10-20cm and CT allows for evaluation of complete occlusion
      • Can extend to chest to visualize septic pulmonary emboli
    • Ultrasound (US)
      • POCUS allows for rapid identification, though reduced sensitivity for recently formed thrombus as well as deeper infections and areas not amenable to US (e.g., mandibular)
    • Chest plain radiograph may demonstrate septic pulmonary emboli

 

Treatment:

  • ABCs—Sit upright and resuscitate appropriately, very likely to be septic
    • Tachycardic, febrile
      • Hypoxia if septic pulmonary emboli
  •  Antibiotics: Ampicillin-sulbactam, piperacillin-tazobactam, or a carbapenem
    • Median duration of antibiotics is 4-6 weeks
  • Consider anticoagulation in consultation with hematology and infectious disease
    • No consensus statement and no randomized control trials available to assess safety, efficacy, or mortality
      • Recent meta-analysis did not demonstrate reduced mortality with anticoagulation though confounded by anticoagulation rate varied widely based on side and clot burden
    • Pulmonary system is the most common site of septic emboli
      • Other sites include liver, muscle, pericardium, brain, and skin
  • Consider surgery if abscess noted or if persistence of disease/worsening clinical picture despite maximal medical therapy
  • 3% of cases are complicated by meningitis

 

Pearls

  • The classic triad includes pharyngitis, anterior neck tenderness/swelling, and non-cavitary pulmonary infiltrates, though only 52% of patients have neck tenderness or swelling on exam
  • Diagnosis includes CT with IV contrast of the neck
  • Management includes resuscitation/stabilization and antibiotics

Additional FOAM Resources:

  1. https://emergencymedicinecases.com/em-quick-hits-september-2019/
  2. https://litfl.com/lemierres-syndrome/

 

References:

  1. Jaber TM, Saini V, Ogbebor O, et al. Lemierre’s Syndrome: A Case Series. Cureus. 2021;13(10):e18436. Published 2021 Oct 2.
  2. Karkos PD, Asrani S, Karkos CD, et al. Lemierre’s syndrome: A systematic review. Laryngoscope. 2009;119(8):1552-1559.
  3. 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.
  4. Riordan T. Human infection with Fusobacterium necrophorum (Necrobacillosis), with a focus on Lemierre’s syndrome. Clin Microbiol Rev. 2007;20(4):622-659.
  5. Eilbert W, Singla N. Lemierre’s syndrome. Int J Emerg Med. 2013;6(1):40. Published 2013 Oct 23
  6. 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(8):561-565.
  7. Leugers CM, Clover R. Lemierre syndrome: postanginal sepsis. J Am Board Fam Pract. 1995;8(5):384-391.
  8. 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(6):458-465.
  9. Thakur A, Chen W. An atypical presentation of lemierre syndrome of urogenital source. IDCases. 2021;26:e01314. Published 2021 Oct 19.
  10. Sinave CP, Hardy GJ, Fardy PW. The Lemierre syndrome: suppurative thrombophlebitis of the internal jugular vein secondary to oropharyngeal infection. Medicine (Baltimore). 1989;68(2):85-94.
  11. Valerio L, Zane F, Sacco C, et al. Patients with Lemierre syndrome have a high risk of new thromboembolic complications, clinical sequelae and death: an analysis of 712 cases. J Intern Med. 2021;289(3):325-339.
  12. Gore MR. Lemierre Syndrome: A Meta-analysis. Int Arch Otorhinolaryngol. 2020;24(3):e379-e385.
  13. Johannesen KM, Bodtger U. Lemierre’s syndrome: current perspectives on diagnosis and management. Infect Drug Resist. 2016;9:221-227. Published 2016 Sep 14.
  14. Ungprasert P, Srivali N. Diagnosis and treatment of Lemierre syndrome. Am J Emerg Med. 2015;33(9):1319.
  15. Venglarcik J. Lemierre’s syndrome. Pediatr Infect Dis J. 2003;22(10):921-923.
  16. Alherabi A. A case of Lemierre syndrome. Ann Saudi Med. 2009;29(1):58-60. doi:10.4103/0256-4947.51822

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