Chapter 30-7: Lemierre Syndrome

TEXTBOOK PRESENTATION

Lemierre syndrome is a septic thrombophlebitis of the internal jugular vein. Typically, presenting symptoms and signs include high fevers, rigors, respiratory distress, and neck or throat pain. Examination of the oropharynx may reveal ulceration, pseudomembrane, or erythema. Tenderness and swelling may be observed overlying the jugular vein. In some cases, abnormal physical findings may be absent.

DISEASE HIGHLIGHTS

1. Lemierre syndrome is a rare (3.6 cases/million/year) but potentially life-threatening cause of sore throat.

2. Approximately 81% of cases are caused by the anaerobic gram-negative rod F necrophorum.

3. Although widespread use of antibiotics in the 1940s led to a dramatic decline in its incidence, there recently has been an increase.

4. F necrophorum is now recognized as an endemic cause of pharyngitis in adolescents and young adults. The relationship between F necrophorum pharyngitis and Lemierre syndrome is not well understood.

5. Symptoms

   1. The most common symptoms of Lemierre syndrome are nonspecific (such as sore throat, neck tenderness or swelling or both, and fever).

   2. Some findings seen in Lemierre syndrome that are not commonly seen in pharyngitis are dyspnea (23.8%), pleuritic chest pain (31.1%), abdominal pain (13.7%), and trismus (9.1%).

6. Septic emboli can arise from the septic thrombophlebitis. Potential complications include:

   1. Lung lesions

      1. Septic emboli

      2. Infiltrates

      3. Lung abscess

      4. Pleural effusion

      5. Empyema

   2. Bone and joint

      1. Septic arthritis

      2. Osteomyelitis

   3. Liver abscess

   4. Central nervous system complications

      1. Brain abscess

      2. Meningitis

      3. Cavernous sinus thrombosis

7. The symptoms of septic thrombophlebitis and septic emboli can mask the initial oropharyngeal symptoms.

   In Lemierre syndrome, symptoms of thrombophlebitis and septic emboli can mask the initial oropharyngeal symptoms. The diagnosis should be considered in those patients with septic findings consistent with septic emboli.

EVIDENCE-BASED DIAGNOSIS

1. A high clinical suspicion is necessary to make the diagnosis of Lemierre syndrome, since the signs and symptoms are often nonspecific.

2. Lemierre syndrome should be considered strongly in patients with chills, high fever, and unilateral neck swelling. Patients may also have antecedent pharyngitis, septic pulmonary emboli, and persistent fevers despite antimicrobial therapy.

3. It may take 5–8 days to isolate F necrophorum.

4. The following criteria are accepted as strong evidence for the presence of Lemierre syndrome:

   1. Anaerobic primary infection of the oropharynx

   2. Subsequent septicemia (with at least 1 positive blood culture)

   3. Metastatic infection of 1 or more distant site

   4. Thrombophlebitis of the internal jugular vein

5. CT scan of neck with contrast is the best diagnostic modality.

TREATMENT

1. Antibiotics

   1. F necrophorum is usually susceptible to beta-lactamase resistant beta-lactam antibiotics, clindamycin, metronidazole, and chloramphenicol.

   2. There is variable response to second- and third-generation cephalosporins.

2. Surgical therapy may be needed for patients with abscesses or those who do not respond to antibiotic therapy.

3. The role of anticoagulation in treatment of Lemierre syndrome is controversial. Due to low incidence of Lemierre syndrome, the risks and benefits of anticoagulation have not been addressed in controlled studies.