Septic phlebitis of the internal jugular vein, postanginal sepsis secondary to oropharyngeal infection, postanginal shock including sepsis, Lemierre's disease, human necrobacillosis
Fusobacterium necrophorum, the most common cause of Lemierre's syndrome
Lemierre's syndrome occurs most often when a bacterial (e.g., Fusobacterium necrophorum) throat infection progresses to the formation of a peritonsillar abscess. Deep in the abscess, anaerobic bacteria can flourish. When the abscess wall ruptures internally, the drainage carrying bacteria seeps through the soft tissue and infects the nearby structures. Spread of infection to the nearby internal jugular vein provides a gateway for the spread of bacteria through the bloodstream. The inflammation surrounding the vein and compression of the vein may lead to blood clot formation. Pieces of the potentially infected clot can break off and travel through the right heart into the lungs as emboli, blocking branches of the pulmonary artery that carry deoxygenated blood from the right side of the heart to the lungs.[citation needed]
Sepsis following a throat infection was first described by Hugo Schottmüller in 1918.[3] In 1936, André Lemierre published a series of 20 cases where throat infections were followed by identified anaerobic sepsis, of whom 18 died.[4]
Signs and symptoms
The signs and symptoms of Lemierre's syndrome vary, but usually start with a sore throat, fever, and general body weakness. These are followed by extreme lethargy, spiked fevers, rigors, swollen cervical lymph nodes, and a swollen, tender or painful neck. Often there is abdominal pain, diarrhea, nausea and vomiting during this phase. These signs and symptoms usually occur several days to two weeks after the initial symptoms.
Symptoms of pulmonary involvement can be shortness of breath, cough and painful breathing (pleuritic chest pain). Rarely, blood is coughed up. Painful or inflamed joints can occur when the joints are involved.[citation needed]
Lemierre's syndrome begins with an infection of the head and neck region, with most primary sources of infection in the palatine tonsils and peritonsillar tissue.[10] Usually this infection is a pharyngitis (which occurred in 87.1% of patients as reported by a literature review[6]), and can be preceded by infectious mononucleosis as reported in several cases.[10] It can also be initiated by infections of the ear, mastoid bone, sinuses, or saliva glands.[citation needed]
During the primary infection, F. necrophorum colonizes the infection site and the infection spreads to the parapharyngeal space. The bacteria then invade the peritonsillar blood vessels where they can spread to the internal jugular vein.[5] In this vein, the bacteria cause the formation of a thrombus containing these bacteria. Furthermore, the internal jugular vein becomes inflamed. This septic thrombophlebitis can give rise to septic microemboli[11] that disseminate to other parts of the body where they can form abscesses and septic infarctions. The first capillaries that the emboli encounter where they can nestle themselves are the pulmonary capillaries. As a consequence, the most frequently involved site of septic metastases are the lungs, followed by the joints (knee, hip, sternoclavicular joint, shoulder and elbow[12]). In the lungs, the bacteria cause abscesses, nodulary and cavitary lesions. Pleural effusion is often present.[6] Other sites involved in septic metastasis and abscess formation are the muscles and soft tissues, liver, spleen, kidneys and nervous system (intracranial abscesses, meningitis).[5]
Diagnosis and the imaging (and laboratory) studies to be ordered largely depend on the patient history, signs and symptoms. If a persistent sore throat with signs of sepsis are found, physicians are cautioned to screen for Lemierre's syndrome.[15]
Thrombosis of the internal jugular vein can be displayed with sonography. Thrombi that have developed recently have low echogenicity or echogenicity similar to the flowing blood, and in such cases pressure with the ultrasound probe show a non-compressible jugular vein - a sure sign of thrombosis. Also color or power Doppler ultrasound identify a low echogenicity blood clot. A CT scan or an MRI scan is more sensitive in displaying the thrombus of the intra-thoracic retrosternal veins, but are rarely needed.[citation needed]
Chest X-ray and chest CT may show pleural effusion, nodules, infiltrates, abscesses and cavitations.[citation needed]
Lemierre's syndrome is primarily treated with antibiotics given intravenously. Fusobacterium necrophorum is generally highly susceptible to beta-lactam antibiotics, metronidazole, clindamycin and third generation cephalosporins while the other fusobacteria have varying degrees of resistance to beta-lactams and clindamycin.[14] Additionally, there may exist a co-infection by another bacterium. For these reasons is often advised not to use monotherapy in treating Lemierre's syndrome. Penicillin and penicillin-derived antibiotics can thus be combined with a beta-lactamase inhibitor such as clavulanic acid or with metronidazole.[5][9] Clindamycin can be given as monotherapy.[citation needed]
If antibiotic therapy is unsuccessful, additional treatments include draining of any abscesses and ligation of the internal jugular vein where the antibiotic cannot penetrate.[6][9][16]
There is no evidence to opt for or against the use of anticoagulation therapy. The low incidence of Lemierre's syndrome has not made it possible to set up clinical trials to study the disease.[9]
Prognosis
The mortality rate was 90% prior to antibiotic therapy. In the contemporary era, a mortality of 4% has been estimated.[17] Since this disease is not well known and often remains undiagnosed, mortality might be much higher. Approximately 10% of those with the condition experience clinical sequelae, including cranial nerve palsy and orthopaedic limitations.[17]
Epidemiology
Lemierre's syndrome is currently rare, but was more common in the early 20th century before the discovery of penicillin. The reduced use of antibiotics for sore throats may have increased the risk of this disease, with 19 cases in 1997 and 34 cases in 1999 reported in the UK.[18] The estimated incidence rate is 0.8 to 3.6 cases per million in the general population, but is higher in healthy young adults. The number of cases reported is increasing; however, because of its rarity, physicians may be unaware of its existence, possibly leading to underdiagnosis.[19]
History
Sepsis following from a throat infection was described by Hugo Schottmüller in 1918.[3] In 1936, André Lemierre published a series of 20 cases where throat infections were followed by identified anaerobic sepsis, of whom 18 patients died.[4]
^Bentley TP, Brennan DF (August 2009). "Lemierre's syndrome: methicillin-resistant Staphylococcus aureus (MRSA) finds a new home". The Journal of Emergency Medicine. 37 (2): 131–134. doi:10.1016/j.jemermed.2007.07.066. PMID18280087.
^ abcdPuymirat E, Biais M, Camou F, Lefèvre J, Guisset O, Gabinski C (March 2008). "A Lemierre syndrome variant caused by Staphylococcus aureus". The American Journal of Emergency Medicine. 26 (3): 380.e5–380.e7. doi:10.1016/j.ajem.2007.05.020. PMID18358967.
^Screaton NJ, Ravenel JG, Lehner PJ, Heitzman ER, Flower CD (November 1999). "Lemierre syndrome: forgotten but not extinct--report of four cases". Radiology. 213 (2): 369–374. doi:10.1148/radiology.213.2.r99nv09369. PMID10551214. The absence of proximal thrombus at CT pulmonary angiography suggests that microemboli, rather than the macroembolic clot burden more typical of acute pulmonary embolism, are responsible for the pulmonary findings in Lemierre syndrome
^Beldman TF, Teunisse HA, Schouten TJ (November 1997). "Septic arthritis of the hip by Fusobacterium necrophorum after tonsillectomy: a form of Lemierre syndrome?". European Journal of Pediatrics. 156 (11): 856–857. doi:10.1007/s004310050730. PMID9392400. S2CID30745447.
^Kanoe M, Yamanaka M, Inoue M (1989). "Effects of Fusobacterium necrophorum on the mesenteric microcirculation of guinea pigs". Medical Microbiology and Immunology. 178 (2): 99–104. doi:10.1007/bf00203305. PMID2659950. S2CID35453227.
^Aspesberro F, Siebler T, Van Nieuwenhuyse JP, Panosetti E, Berthet F (September 2008). "Lemierre syndrome in a 5-month-old male infant: Case report and review of the pediatric literature". Pediatric Critical Care Medicine. 9 (5): e35–e37. doi:10.1097/PCC.0b013e31817319fa. PMID18779698. S2CID52858512.