A case of Lemierre's syndrome with disturbed venous flow confirmed by conventional angiography
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/0028-3886.246255
Source of Support: None, Conflict of Interest: None
Lemierre first isolated Fusobacterium necrophorum, a Gram-negative anaerobic bacillus, from 20 patients with thrombophlebitis of the internal jugular vein (IJV) after oropharyngeal infection in 1936., This condition is called Lemierre's syndrome (LS) or post-anginal septicemia. Before the introduction of antibiotics, LS was relatively common. This disease is caused by an acute oropharyngeal infection in young adults or adolescents previously in good health. It leads to the development of secondary septic thrombophlebitis of IJV complicated with frequent metastatic infections., It can lead to rapid septicemia and death within 1–2 weeks. We report a case of LS where the primary lesion was otitis media and the patient developed bacterial meningitis as a septic metastasis. The significant differences in the angiographic features of IJV before and after treatment have been described.
A 56-year old woman presented to the emergency room with severe headache and fever. She had visited the Ear-Nose-Throat (ENT) outpatient clinic with left otalgia and headache 7 days earlier. She was diagnosed with chronic otitis media, and a culture study of the pus from the left ear was performed. Although otalgia subsided after treatment using ciprofloxacin, the headache worsened with nausea and vomiting. Her blood pressure was 140/80 mmHg and body temperature was 38.5°C. The initial laboratory studies revealed the following values: white blood cell count, 8,600 cells/mm3; hemoglobin levels, 12.2 g/dL; platelet count, 1.32 × 105 platelets/mm3; C-reactive protein (CRP), 24.517 mg/dL. She had no neurological deficits except neck stiffness. In the cerebrospinal fluid (CSF) study, the opening pressure was 12 cm H2O, the fluid was turbid, the leukocyte count was 3,380/μL with 96% polymorphonuclear neutrophils and 4% monocytes, the protein level was 149 mg/dL, and the glucose level was 72 mg/dL with the corresponding blood glucose level being 152 mg/dL. She was diagnosed as having bacterial meningitis and was admitted to the neurology department.
We immediately started intravenous vancomycin 500 mg four times a day, ceftriaxone 2 g two times a day, and ampicillin 2 g six times a day as empirical antibiotics. On the fourth day of hospitalization, Pseudomonas aeruginosa was confirmed in the culture study of the pus from the left ear. The headache and fever had improved but the left side of her neck started to get swollen and tender. Computed tomography (CT) of the neck showed thrombophlebitis in the IJV [Figure 1]a. At this point, the patient had bacterial meningitis combined with left IJV thrombophlebitis, probably secondary to chronic otitis media. She was tentatively diagnosed with LS. The antibiotics were changed to intravenous tazobactam/piperacillin 4.5 g four times a day to target P. aeruginosa. The C-reactive protein (CRP) decreased to 14.5 mg/dL. There was no bacterial growth in the blood culture.
Although follow-up study of the cerebrospinal fluid and inflammatory markers on the 15th day after admission showed improvement, the subsequent CT scan revealed a decreased mass effect [Figure 1]b. Anticoagulation was initiated with intravenous heparin and transfemoral cerebral angiography (TFCA) was performed to confirm the flow during the venous phase [Figure 2].
After 3 weeks of anticoagulation and 1 month of antibiotic therapy, the headache had significantly improved and there was no lasting neurologic deficit. The patient was discharged on oral levofloxacin, 750 mg a day, and warfarin, 5 mg a day, maintaining the INR (international normalized ratio) between 2 and 3. After 2 months, we confirmed on a CT scan that the thrombosis in the left IJV had significantly reduced.
After the widespread availability of antibiotics, the incidence of LS has decreased dramatically., However, reports of LS have been increasing recently. This may be due to the presence of an increased antibiotic resistance or due to changes in antibiotic prescription patterns.
The first symptom of LS is usually a sore throat followed by fever, chills, neck swelling, and tenderness. Without appropriate management, the primary infection can spread to the surrounding tissues and the posterior neurovascular compartment of the lateral pharyngeal space. Therefore, it could provoke the development of IJV thrombophlebitis and can involve the carotid artery, vagus nerve, lymph nodes, cranial nerves X–XII, or cervical sympathetic trunk, with neck tenderness and swelling. It can also cause sepsis with metastatic infection. The most common metastatic target are the lungs, followed by the joints, muscle, liver, spleen, and meninges.,, LS can be diagnosed if thrombophlebitis of the IJV is found on the CT or magnetic resonance imaging of the neck. In this case, the patient complained of left otalgia, headache, and fever. Subsequently, the left submandibular swelling and thrombophlebitis of the IJV on the neck CT scan led to the diagnosis of LS. Metastatic spread to the meninges led to bacterial meningitis.
The main treatment is the early administration of antibiotics. Although F. necrophorum is usually treatable by penicillin, the drug of choice is a non-beta-lactam antibiotic such as metronidazole or clindamycin to prevent de novo mutation of beta-lactamase. The antibiotics should be started as soon as possible to prevent progression to sepsis and diminish mortality. In previous studies, antibiotics were administered for 3–6 weeks to treat intravenous infection. However, the necessary course of antibiotics is still not definitively proven. In this case, we used intravenous (IV) piperacillin/tazobactam for 4 weeks because P. aeruginosa was isolated in the pus culture from the left ear.
Anticoagulant therapy has been tried in previous studies, but there have been no controlled studies focusing on the risks and benefits of this treatment because of the low prevalence of septic thrombosis associated with this infeciton. The role of anticoagulants is controversial. Since some studies reported the benefit of anticoagulant therapy, we used IV heparin for 4 weeks and oral warfarin for 2 months.
LS is usually caused by F. necrophorum., However, this case was suspected to be due to P. aeruginosa. The primary infection was probably otitis media, which led to IJV thrombophlebitis and meningitis after the occurrence of a metastatic infection. Cases of LS with otitis media as the primary focus are rare. As far as we know, this is the first case report that confirmed infection in the venous blood flow using TFCA in a patient suffering from LS. If a patient with infection around the oropharynx has fever, neck swelling, and tenderness, investigations using blood cultures and a neck imaging study should be carried out to rule out the presence of LS. An early administration of antibiotics is paramount and anticoagulation might be an optional treatment.
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The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
This research was supported by the Original Technology Research Program for Brain Science through the National Research Foundation of Korea (NRF) funded by the Korean government (MSIP) (no. 2014M3C7A1064752).
Conflicts of interest
There are no conflicts of interest.
[Figure 1], [Figure 2]