Lemierre Syndrome: Definition, Causes, Symptoms, Diagnosis, Treatment, Prognosis and Complications

Also known as postanginal sepsis, it is an infectious syndrome.

It is a rare and life-threatening complication of bacterial infections that generally affects healthy adolescents and young adults.

Most often, it develops in association with a bacterial throat infection, but can develop in association with an infection affecting the ears, salivary glands ( mumps ), sinuses, or teeth, or in association with an infection of the Epstein-Barr.

In people with Lemierre syndrome, the initial infection spreads to the deep spaces within the neck, leading to the formation of a transfer clot (septic thrombophlebitis).

It is sometimes made up of pus, in the internal jugular vein (the blood vessel that carries blood to the brain , face, and neck).

The infected clot then circulates in the blood (septicemia), causing the infection to also spread to the lungs (most commonly), the skeletal system, and / or other parts of the body such as the spleen, liver, kidney, the heart or the brain.

This can lead to life-threatening complications, such as respiratory distress syndrome due to a pulmonary embolism (blood clots in the lung), damage to other affected organs, and / or septic shock (in about 7 percent of cases).

Because most throat infections in young, healthy people do not cause serious health problems, diagnosis and treatment can be delayed.

Causes of Lemierre’s syndrome

Most of the time Lemierre’s syndrome is due to a complication of a bacterial throat infection, but it has also been reported to be due to infections that affect other areas of the head and neck, such as the ears, salivary glands , sinuses and teeth.

The most common culprit for Lemierre’s syndrome is Fusobacterium necrophorum. This is the main pathogen and is a pleomorphic, anaerobic gram-negative rod.

This bacteria is normally present in healthy people in various parts of the body (including the throat, digestive tract, and female genitalia).

Bacteria can cause invasive disease by releasing toxins into surrounding tissue.

It has also been speculated that in some cases, other bacteria or a virus may be responsible for the initial infection before the onset of Lemierre’s syndrome, leading to conditions that favor the growth and invasion of F. necrophorum into the surrounding tissues. .

Bacteria other than F. necrophorum that have been reported in case studies include Streptococcus species, Bacteroides species, Staphylococcus aureus, and Klebsiella pneumoniae.

The Epstein-Barr virus has also been reported in people before the onset of Lemierre’s syndrome.

Symptoms of Lemierre’s syndrome

Lemierre’s syndrome is an acute , rare and life-threatening anaerobic oropharyngeal infection , the classic presentation of human necrobacillosis.

In addition to worsening symptoms of the initial infection, symptoms at this stage of the disease typically include persistent fever and chills (rigors), as well as pain, tenderness, and swelling of the throat and neck.

The primary infection is in the head in a young, previously healthy person who subsequently develops a persistent high fever and disseminated metastatic abscesses, often including septic thrombophlebitis of the internal jugular vein.

Lemierre’s syndrome is often complicated by septic pulmonary embolism and distant metastatic infections.

Lemierre syndrome diagnosis

Lemierre syndrome can be diagnosed based on signs and symptoms, as well as a variety of blood tests and imaging studies.

Blood tests may reveal various abnormalities that suggest the diagnosis, such as a high white blood cell count, a low platelet count, or some other evidence of clotting problems and abnormal liver or kidney function.

Blood cultures should be collected and the results commonly indicate that the blood is infected with F. necrophorum bacteria, which often raises a high suspicion for Lemierre syndrome.

In some cases, blood cultures are negative due to the difficulties that can be associated with culturing anaerobic bacteria.

Imaging studies may include a chest x-ray, which can detect septic emboli, abscesses, or other pulmonary complications.

Imaging studies to specifically evaluate septic thrombosis of the internal jugular vein may include ultrasound, such as a CT scan of the neck with contrast, and magnetic resonance imaging.

Magnetic resonance venography has the highest sensitivity for detecting internal jugular thrombosis.

Lemierre syndrome treatment

Treatment recommendations for Lemierre syndrome are primarily based on clinical experience and in vitro studies and are supported by limited data from observational studies and case reports.

The main components of treatment include intravenous antibiotic therapy, therapy, and drainage at the infection sites.

A combination of antibiotics (at least one beta-lactamase resistant antibiotic and one beta-lactam antibiotic) is recommended to cover all possible responsible bacteria, as there have been reports of failure of treatment with single antibiotics (monotherapy).

Intravenous antibiotics are indicated. F. necrophorum is usually susceptible to penicillin, cephalosporins, metronidazole, clindamycin, tetracyclines, and chloramphenicol. Some strains of F. necrophorum producing beta-lactamases have been described.

Ideally, the specific antibiotics used should depend on the culture results, when available.

Antibiotic therapy is usually continued for up to 6 weeks to allow the drug to penetrate the infected clots.

The main treatment consists of intravenous antibiotic therapy for several weeks, but surgery may be necessary when there is abscess formation, shortness of breath, or severe clotting in the chest or brain.

When patients have dyspnea, stridor, or an inability to manage secretions, an artificial airway should be established.

Airway obstruction is more likely to occur in submandibular space infections.

Surgically obtained specimens should be cultured aerobically and anaerobically.

For patients with peritonsillar abscess, high-dose intravenous penicillin is the therapy of choice.

These must be incised and drained to prevent spontaneous rupture, aspiration pneumonia, airway obstruction, or dissection of infection into the lateral retropharyngeal space.

Surgical removal of infected clots is generally reserved for cases where antibiotic therapy and drainage are not effective.

About half of the cases of Ludwig’s angina in the submandibular space can be cured without surgical intervention.

Surgical treatment may also be necessary for respiratory problems due to pulmonary embolism, severe blood clotting in other parts of the body (particularly the brain), mediastinitis, or to remove dead, damaged, or infected tissues, with the use of the debridement technique. .

The role of the use of anticoagulants) for blood clots in Lemierre’s syndrome is uncertain and has been controversial.

While anticoagulation can help prevent new clots and complications associated with thrombosis, it can cause a high risk of bleeding complications and / or help spread infected material.

Lemierre’s syndrome without evidence of extensive clotting usually resolves with appropriate antibiotic treatment and does not require anticoagulation.

However, it may be recommended in severe or progressive cases with persistent sepsis, extensive clotting, clotting spreading to the cerebral sinuses, and / or when a patient does not begin to improve within 72 hours of appropriate antibiotic or surgical treatment.

Lemierre syndrome prognosis

The long-term prognosis and likelihood of survival in people with Lemierre syndrome vary depending on the progression of the syndrome, but even with proper treatment, it is fatal in some cases.

Advanced Lemierre’s syndrome is a life-threatening condition. Current mortality (death from syndrome) is estimated to be between 5% and 18%, depending on the source of the data.

However, as the mortality in the pre-antibiotic. Reportedly the 90% era, the prognosis for people with Lemierre’s syndrome has improved significantly due to advances in antibiotic therapy and high-level intensive care.

Receiving the diagnosis as quickly as possible and starting appropriate treatment increases the chance of survival.

Lemierre syndrome complications

Serious complications of Lemierre syndrome include acute respiratory distress syndrome, septic shock, pneumonia, empyema, meningitis, osteomyelitis (bone infection), brain abscess, and vocal cord paralysis.

This infection can complicate a routine case of infectious mononucleosis.