Lemierre syndrome - a rare but serious complication of angina

Lemieur syndrome, post-anginal septicemia, is characterized by the presence of 4 signs: the primary focus of infection in the oropharynx; sepsis confirmed by a positive culture; clinical or radiographic signs of thrombosis of the internal jugular vein and at least 1 metastatic lesion. The literature describes several hundred cases of the disease. Patients under 18 years of age represent less than 1/5 of the case. The incidence is 1 in 1 million people per year.

The most common Lemierre syndrome is caused by Fusobacterium necrophorum, but the etiological role of other Fusobacterium species is also possible, as well as Bacteroides , Streptococcus, Lactobacillus spp. Fusobacterium causes 10 to 17% clinically significant bacteremia in children. The invasive properties of the microorganism are due to the production of proteolytic enzymes, endotoxin, leucocidin and hemagglutinin. Damage to the mucous membrane of the oropharynx leads to tissue necrosis, while creating the anaerobic conditions necessary for the multiplication of bacteria.

The disease begins with pharyngitis or tonsillitis, which lasts from several days to 3 weeks. The anatomy of the peripharyngeal space facilitates the penetration of infection into the internal jugular vein, both directly and through the lymphogenic and hematogenous pathways. The septic embolism of the jugular vein can penetrate any organ. Long fever, pain in the lateral neck and swelling. Soon there are signs of pulmonary insufficiency or myalgia with arthralgia. Septic arthritis and osteomyelitis can also occur, but no abscesses in the soft tissues and the abdominal cavity are ever observed. There may be abdominal pain (in women), hepatomegaly, jaundice, encephalopathy, myelitis. Often the diagnosis is made late, mainly due to the rarity of this syndrome and the atypical nature of the initial period. The vigilance of the doctor in the face of this syndrome is of great importance. It should be suspected in a child or adult with fever, neck pain and swelling after suffering from pharyngitis or tonsillitis, especially if there are changes in the lungs.

Leukocytosis in children is in the range of 15 to 20,000 / mm3, combined with a large shift in formula to the left. The ESR is always increased and can reach more than 100 mm / h. From other laboratory data, there may be an increase in liver transaminases, sterile pyuria, anemia, electrolyte disturbances and impaired secretion of antidiuretic hormone, increased serum amylase. A bacteriological examination of the blood usually produces F.necrophorum. In the lungs, infiltrates, thromboembolism, effusion in the pleural cavity or empyema can be detected. But the x-ray image may be normal.

The diagnosis is confirmed by the detection of a phlebitis of the internal jugular vein. Radiography, contrast computed tomography, ultrasound examination of blood vessels or MRI are used. Less useful methods are gallium scanning, radioisotope scintigraphy. Retrograde venography, dangerous for perforation and embolism, should be used last.

The basis for the treatment of Lemierre syndrome is antibiotic therapy. Although the anaerobic oral flora is primarily sensitive to penicillin, some strains of fusobacteria are resistant due to the production of β-lactamases. Therefore, it is desirable to determine the susceptibility to antibiotics of the isolated microorganisms. Clindamycin, metronidazole, penicillin, inhibitor-resistant penicillins (amoxicillin / clavulanate), β-lactamase-resistant cephalosporins with anti-anerobic activity are recommended as empirical therapy. Chloramphenicol has already been used, but it is no longer recommended. Antibiotic therapy with the patient's response lasts 7 to 14 days parenterally, then oral antibiotic therapy for 2 to 4 weeks follows.

The progression of the syndrome is generally prolonged due to septic embolization. Heparin anticoagulant therapy is recommended, especially in cases of generalized thrombosis. This therapy reduces the duration of the disease and avoids surgery. Surgical intervention is necessary for drainage of abscesses, suppuration of the joint, pleural cavity with the ineffectiveness of antibiotic therapy. Ligation or ablation of the internal jugular vein is rarely required and is only performed if the disease progresses, despite optimal conservative treatment.

Lemierre's syndrome, with the onset of antibiotic therapy, although less frequently, continues to occur in children and adults. Due to the rarity of the disease or the unusual nature of the initial period, a delayed diagnosis is possible. The detection of phlebitis using visualization methods is of great importance. Early recognition and aggressive antibiotic therapy, supplemented by judicious use of anticoagulants and surgery, provide a favorable outcome. Lethal outcomes are currently rare.