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What is Lemierre Syndrome?

Lemierre syndrome (LS), a condition normally seen in young adults, is a rare complication comprising oropharyngeal infection and thrombophlebitis of the internal jugular vein (IJV). Also known as post-anginal sepsis and necrobacillosis, Lemierre syndrome was first described by Andre Lemierre in 1936.

This condition was widespread before the invention of antibiotics and had a deadly course in a week to a fortnight. With the advent of antibiotics, the cases of Lemierre's syndrome declined.

However, it has been recorded more frequently in recent years. The success of antibiotic therapy and prompt diagnosis is essential for this syndrome's favorable prognosis. 

Causes and symptoms

Commonly caused by the bacteria F. necrophorum and Fusobacterium nucleatum, the early stages of Lemierre syndrome are often characterized by a sore throat and neck pain accompanied by persistent fever.

There have also been reports of additional organisms in other case studies, including Streptococcus species, Bacteroides species, Klebsiella pneumoniae, and others. Initially, a localized oropharyngeal infection leads to Lemierre syndrome.

In addition to direct condition, pharyngeal mucosal injury from other bacterial or viral illnesses creates an environment favorable for a fusobacterial superinfection. The lateral pharyngeal area and soft tissues of the neck are affected next.

The IJVs eventually experience venous thrombosis, which results in the development of septic thrombosis.  

Most young patients initially appear with pharyngitis, whereas the elderly group later presents with empyema or brain abscess. The various indications of the aging population's concealed illnesses or immunological responses could be to blame. Seizures and blindness are among the unusual presentations.

A study found that the primary infection site can affect the prognosis; infections in the oropharynx were linked to a higher likelihood of prolonged hospitalizations in intensive care units because of complications like respiratory issues.

Epidemiology

The prevalence of Lemierre syndrome significantly decreased once antibiotics were developed in the 1940s and used widely for streptococcal pharyngitis (strep throat). It was referred to as a "forgotten disease" in the 1980s and 1990s.

However, since the late 1990s, there has been a rise in reports of this syndrome for unknown reasons. According to estimates, it is an uncommon condition with a 1 in 100,000 global occurrence. Young, previously healthy adolescents and young adults are often affected by Lemierre syndrome.

Depending on the study, Lemierre syndrome patients range in age from 19 to 22 years old, with a 2:1 male-to-female ratio. Most patients with Lemierre syndrome are diagnosed between the ages of 10 and 35.

Case reports

Eilbert et al. reported on a 13-year-old child who had previously been in good condition but had been complaining for five days of right-sided throat soreness. The ache affected his right ear and the right side of his neck. Swallowing, as well as the neck's flexion, extension, and rotation, made it worse.

The patient had a temperature of 37.8°C when they entered the emergency room, but 1 hour later, they discovered they had a temperature of 38.1°C. He did not seem to be in pain, and an inspection of his heart, lungs, belly, and skin revealed nothing unusual.

White blood cells comprised 9,400 cells/mcL of a total blood count. From the jugular foramen to the common jugular confluence, the right IJ vein was shown to be opaque on a CT scan.

The patient received an IV dosage of ampicillin/sulbactam to treat a possible septic thrombophlebitis. The child showed no symptoms of metastatic infection during his 5-day hospital stay. On hospital days 1 and 5, two sets of blood cultures were taken, but no organisms were grown.

The patient received enoxaparin as an anticoagulation after testing for the presence of a hypercoagulable state proved negative. He was sent home to finish a six-week regimen of anticoagulation and antibiotics. One month later, the patient was doing well at the clinic visit, complaining only of a slight weight loss.

Srivali et al. described the case of a healthy young man who displayed flu-like symptoms and was initially misdiagnosed as having a viral upper respiratory tract infection. A 38.1°C temperature and an injected pharynx without any neck discomfort or swelling were notable physical examination findings.

A total blood count revealed thrombocytopenia. Blood cultures were taken, and therapeutic antibiotics, including piperacillin and vancomycin, were administered. The next day, F. necrophorum was detected in his blood cultures, which reduced the options for his antibiotic therapy. Antibiotics and intravenous fluids had a positive effect on him.

Intensivists should be highly suspicious of this diagnosis in young adults who are otherwise healthy. A previous pharyngitis history or signs of septic emboli, particularly to the lung, could give more information to the diagnosis.

Diagnosis and treatment

The main clinical components of the diagnosis are the examination of the systemic inflammatory response syndrome (SIRS) criteria, abnormal liver function tests, thrombocytopenia, and Fusobacterium species detection.

Since the lungs are the most typical site of metastatic infection, a chest radiograph should be included in imaging. Magnetic resonance imaging (MRI), ultrasound, and CT of the neck with contrast may also be performed to test for septic thrombosis of the IJV.

Antibiotic medication is the cornerstone of treatment. When available, susceptibility and culture results data should be used to customize antibiotic treatment. Other choices for patients with severe clinical beta-lactam allergy include clindamycin or metronidazole.

Most patients continue their antibiotic therapy for six weeks to obtain adequate penetration into fibrin clots. In situations of metastasis, pulmonary thrombosis-related respiratory discomfort, and abscess formation, surgical treatment may be required.

To manage infection, surgical incision and abscess draining may be necessary at the afflicted locations.

There is debate concerning anticoagulation treatment for Lemierre syndrome. Simple LS that does not show signs of a significant clot burden can be treated with the right antibiotics and supportive therapy without anticoagulation.

For patients, the best anticoagulant medication has not been determined by randomized clinical trials. According to anecdotal accounts of LS, anticoagulation helps to reduce septic embolic episodes brought on by IJV thrombosis.


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