Lemierre Syndrome


What is Lemierre Syndrome?

Lemierre Syndrome is life-threatening thrombophlebitis develops at the internal jugular vein due to oropharyngeal infection. At the advanced stage of this disease spread as septic emboli from the inhabitant of the mouth to various distant parts of the body.

The most commonly affected organ is lungs, but other included organs are liver, spleen, muscle, joints, skin and other soft tissue. At the initial stage, oropharyngeal infections like pharyngitis or tonsillitis along with or without retropharyngeal abscess or peritonsillar.

A verity of microorganisms are identified culprits for the oropharyngeal infection, which turns to Lemierre Syndrome, but Fusobacterium necrophorum is the main infecting agent for Lemierre Syndrome.

Advance antibiotics can control initial infections related to pharyngitis, otitis media, odontogenic infections, or dental procedures, but after a decade later the recurrence of the condition often leads to Lemierre Syndrome progression.


Therefore, this condition is also termed as It has been called the “forgotten disease.” The initial stage of this condition can develop during childhood, but may atypically progress in the adult age, after a decade later. In 1936, Lemierre Syndrome was first described by A. Lemierre and published in the Lancet1,2,3. Lemierre Syndrome Pathology

The different microorganisms, which are accountable for pathological modifications and cause Lemierre Syndrome outburst are mostly Gram-negative anaerobic bacteria. Fusobacterium necrophorum is identified micro-organism mainly involve in the development of the condition and from this, the term necrobacillosis is derived.

Polymicrobial bacteremia is a type of anaerobic streptococci and one-third of the case is progressed due to the attack of this microorganism. In some cases, other various gram-negative anaerobes are also liable for providing this condition. Methicillin-resistant Staph. aureus (MRSA) also sometimes involve in the development of the Lemierre Syndrome2,4.

Lemierre Syndrome in mouth

Symptoms

The advancement of antibiotic therapy almost control Lemierre Syndrome progression, however, the incidence rate is frequent in the last decade.

The syndrome is usually progressed in young immunocompetent healthy patients after a chronic pharyngitis, which is then progressed to septicaemia and pneumonia associated with respiratory distress. In addition, pain symptom is often present.

  • Sore throat
  • Persistent fever
  • Chills Initial stage symptoms, which mainly occur in children and adolescents
  • Acute otitis media
  • Neck pain Intermediate stage symptoms
  • Tenderness and inflammation spread to the internal jugular vein

The bellow mentioned symptoms occur at the advanced stage or in “metastasis” and provide fatal outcome:

  • Septic emboli development in the lungs, brain, abdominal organs, heart etc.
  • Prolong a productive cough and chest pain occur in case of lung involvement
  • Abdominal pain, enlargement of liver, jaundice occur in the case of liver involvement2,3,4,5.

Causes

The sign and symptomatic description of the Lemierre syndrome describe it is a slowly progressive disorder. In 90% cases, Fusobacterium necrophorum is responsible for the Lemierre syndrome, however other identified bacteria are Staphylococcus aureus, Streptococcus pyogenes, Peptostreptococcus, Porphyromonas, Proteus, Prevotella, Eikenella and Bacteroides.

At initial stage causative organism enters to the oropharynx of healthy individuals and then certain factors like co-existence of bacterial or viral pharyngitis and Epstein-Barr virus assist to invade the bacteria to the mucosa. At the initial stage, the symptoms are similar as other upper respiratory tract infections.

In the advancing stage, causative agent penetrates to adjacent blood vessels through the lymphatic system or fascial planes and cause thrombosis and consequent suppurative thrombophlebitis of the internal jugular vein, often forming para-pharyngeal swelling and a peritonsillar abscess along the way2,3,5.

Lemierre Syndrome (vein)

Diagnosis

The symptomatic analysis shows intense pleuritic chest pain, tachycardia in 80 percent patients because septic infarcts progress to appear as lung abscesses, respiratory distress. The included diagnostic tests for Lemierre syndrome are as follows:

1. Hematological analysis

The test report of CBC (Complete Blood Count) provides the information about the presence of infection in the body. A blood culture can provide the information of the causative organism.

2. Ultrasound

Ultrasound images indicate the presence of thrombus within the jugular vein.

3. CT (computed tomography )scan

The CT scan of the neck provides a confirmatory result. It provides the detail images of nodules, abscesses, cavitations and pleural effusions, which can form due to pulmonary septic emboli.

In some cases, CT scan findings reported abnormal joints and abdominal structures due to septic emboli progression. Micro-abscesses formation often involves in liver1,4,5.

Treatment

  • Intravenous antibiotic therapy for prolonged duration (usually 3 to 6 weeks) is prescribed for the internal jugular thrombophlebitis.
  • Some experts also prescribed anticoagulants, however, the efficacy is unknown.
  • Combination of antibiotic and anticoagulant is also an option to treat the condition, specifically for patients suffering from chronic thrombosis, who has higher risk of thromboembolic events
  • If necessary, surgical drainage along with intravenous administration of Penicillin and metronidazole can be intravenously prescribed
  • Surgical intervention only recommended for patients who fail to response antibiotic treatment1,2,4,5.

Lemierre Syndrome Treatment

Life Expectancy

The life expectancy of Lemierre syndrome depends on diagnosis and initiation of the treatment. Fast diagnosis and the immediate start of the appropriate antibiotic therapy and abscess drainage can increase the life expectancy. However, almost 4 to 12% death rate occurs with prompt appropriate therapy5.

References

  1. A.Prof Frank Gaillard; Lemierre syndrome; Radiopaedia; Retrieve from https://radiopaedia.org/articles/lemierre-syndrome
  2. Lemierre syndrome; Genetic and Rare Diseases Information Center; Retrieve from https://rarediseases.info.nih.gov/diseases/6882/lemierre-syndrome
  3. Medical Definition of Lemierre syndrome; MedicineNet.com; Retrieve from http://www.medicinenet.com/script/main/art.asp?articlekey=21380
  4. Andreas V. Hadjinicolaou, Yiannis Philippou; Lemierre’s Syndrome: A Neglected Disease with Classical Features; Case Reports in Medicine. Volume 2015 (2015), Article ID 846715, 4 pages. http://dx.doi.org/10.1155/2015/846715. Retrieve from https://www.hindawi.com/journals/crim/2015/846715/
  5. Shalini Gupta, Shehzad S. Merchant; Lemierre’s Syndrome: Rare, but Life Threatening—A Case Report with Streptococcus intermedius; Case Reports in Medicine Volume 2012 (2012), Article ID 624065, 4 pages http://dx.doi.org/10.1155/2012/624065. Retrieve from https://www.hindawi.com/journals/crim/2012/624065/

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