In the preantibiotic era, the outcome of lemierres syndrome was rapidly fatal, with progressive septicemia. Lemierres syndrome has been shown to be increasing in incidence in the past 20 years with one popular suggesting that said rise occurred from less aggressive antibacterial coverage. The profile of this syndrome declined in the mid20th century, coinciding with the introduction of antibiotics. Fontan circulation, the definitive palliative treatment for patients with complex congenital heart disease with a functional univentricular heart, complicates clinical. We summarise the epidemiology, microbiology, pathogenesis, clinical presentation, diagnosis, complications, therapy, and outcomes of lemierre s syndrome. Volume 15, issue 2, march 2014 jayten shook, do et al. The condition is characterized by a primary oropharyngeal infection with evidence of septic thrombophlebitis, exhibited by positive blood cultures, clinical or radiographic.
Examination revealed pustular exudates on the right. Our main mission in this fight is to bring awareness of lemierres syndrome to the public. The patient was given a dose of iv ampicillinsulbactam for suspected septic thrombophlebitis of the right ij vein and transferred to a. Courmont and cade reported the first case of lemierres syndrome. In the 1960s and 1970s, lemierres syndrome became a rare phenomenon with the proliferation of penicillin in the treatment of pharyngitis. Lemierre syndrome postanginal sepsis is a classic complication of infection by fusobacterium necrophorum, characterized by thrombophlebitis of the internal jugular vein and septicemia. F necrophorum is most commonly associated with lemierre s syndrome.
The syndrome is characterised by a primary oropharyngeal infection followed by metastatic spread and suppurative thrombophlebitis of the internal jugular vein. The classic lemierres syndrome refers to a septic thrombosis of the internal jugular vein, usually caused by a fusobacterium necrophorum infection starting in the oral cavity, and typically complicated by pulmonary emboli. Lemierres syndrome, necrotizing pneumonia and staphylococcal. Lemierres syndrome an overview sciencedirect topics.
Lemierres syndrome is most commonly caused by fusobacterium necrophorum. Today, the incidence of lemierres syndrome is estimated to be 1 per million annually. Patients typically present unwell, with trismus and pain behind the angle of the jaw. Currently, since imaging has a key role in the early diagnosis of this syndrome, the radiologist should be aware of and recognize its manifestations. It most often develops as a complication of a bacterial sore throat infection in young, otherwise healthy adults. Pdf lemierres syndrome is a condition characterized by thrombophlebitis of the internal jugular vein and bacteremia caused by primarily anaerobic. There is a male predominance, with 70% of patients between the ages of 16 and 25 years. Images in clinical medicine from the new england journal of medicine lemierres syndrome. A 35yearold woman presented with a threeday history of sore throat, painful swelling of the neck, fever, rigor, hemoptysis, and dyspnea.
Lemierres syndrome ls is a rare, but a lifethreatening complication of an oropharyngeal infection. Lemierre syndrome, or postanginal sepsis, was first described in the early part of this century and is characterized by pharyngitis, followed by high fever and rigors, cervical adenopathy, thrombophlebitis of the internal jugular vein, distant abscess formation, and icterus, associated with isolation of fusobacterium necropborum from blood. A case report on the recurrence of lemierres syndrome. Lemierres syndrome is a suppurative thrombophlebitis of the internal jugular vein, usually caused by direct invasion of oropharynx anaerobes, specifically fusobacterium necrophorum. Currently, lack of awareness of this condition delays appropriate treatment and worsens prognosis. Treatment involves prolonged antibiotic therapy occasionally combined with anticoagulation. Lemierres syndrome international journal of emergency. Is lemierres syndrome transmitted from person to person.
For language access assistance, contact the ncats public information officer. Unusual case of lemierres syndrome bmj case reports. Lemierre syndrome a forgotten complication of acute. The infection originates in the throat and spreads via a septic thrombophlebitis of the tonsillar vein and internal jugular vein. This syndrome consists of internal jugular vein thrombosis after a primary oropharyngeal infection with the development of distant septic emboli. Prompt clinical diagnosis and treatment with appropriate antibiotics are critical. The interval between the oropharyngeal infection and the onset of the septicaemia is usually short.
The thrombophlebitis is a serious condition and may lead to further systemic complications such as bacteria in the blood or septic emboli. Jan 08, 2019 lemierre syndrome is a rare and potentially lifethreatening complication of bacterial infections that usually affects previouslyhealthy adolescents and young adults. Lemierres syndrome the western journal of emergency. Lemierre syndrome occurs when an oropharyngeal infection is complicated by septic thrombophlebitis of the internal jugular vein ijv or one of its tributaries, leading to sepsis and metastatic infection. Lemierre s syndrome also called necrobacillosis or postanginal septicemia is an acute oropharyngeal infection with a secondary septic thrombophlebitis of the internal jugular vein ivj. Abstractlemierres syndrome is a condition characterized by thrombophlebitis of. Aug 22, 2017 the symptoms of lemierres syndrome may not appear right away after you get the infection. We present a case of a patient with lemierre s syndrome caused by fusobacterium necrophorum who developed a right frontal lobe brain abscess. A 17year old female, with no previous medical history, was admitted to the intensive care unit for septic shock, acute respiratory distress syndrome. Lemierres syndrome is a rare disease that results in an oropharyngeal infection, which precipitates an internal jugular vein thrombosis and metastatic infection. It creates a peritonsillar abscess, a blood clot, filled with bacteria near the tonsils in the jugular vein. This has been an uncommon illness in the era of antibiotic therapy, though it has been reported with increasing frequency in the past 15 years. Lemierre syndrome is a rare and potentially lifethreatening complication of bacterial infections that usually affects previouslyhealthy adolescents and young adults. Lemierre s syndrome or postanginal septicaemia necrobacillosis is caused by an acute oropharyngeal infection with secondary septic thrombophlebitis of the internal jugular vein and frequent metastatic infections.
A 70yearold female with retropharyngeal abscess developed features of lemierre syndrome. Lemierres syndrome is an uncommon, but potentially lifethreatening complication of acute pharyngotonsillitis. Lemierre syndrome genetic and rare diseases information. Lemierres syndrome due to fusobacterium necrophorum the. Lemierre syndrome is caused by oropharyngeal infections, which cause septic thrombophlebitis of the internal jugular vein and metastatic infections. Infection of the lateral pharyngeal space may result in thrombosis of the internal jugular vein, subsequent metastatic complications e. Lemierre s syndrome is a condition characterized by thrombophlebitis of the internal jugular vein and bacteremia caused by primarily anaerobic organisms, following a recent oropharyngeal infection. Lemierres syndrome ls manifests as an oropharyngeal infection of.
Many authors consider cect as the imaging study of choice due to availability and its allowance for visualization of complications and underlying infection 68. Since the advent of antibiotics, the incidence of lemierre syndrome has reduced significantly, now affecting between 0. The ensuing bacteraemia is complicated by septic emboli to a range of sites such as lung, joints. Macsweens pathology of the liver seventh edition, 2018. A 17year old female, with no previous medical history, was admitted to the intensive care unit for septic shock, acute respiratory distress syndrome and. Lemierre s syndrome refers to infectious thrombophlebitis of the internal jugular vein. Sites of metastatic infection, in order of frequency of involvement, are lung, joint, muscle, bone, liver, skin, spleen and endocardium. It most commonly develops in association with a bacterial throat infection, but it may develop in association with an infection involving the ears, salivary glands parotitis, sinuses, or teeth. Fusobacterium necrophorum is the most common pathogen isolated from the patients. Lemierres syndrome was common in the era before the advent of antibiotics. Apr 11, 2014 volume 15, issue 2, march 2014 jayten shook, do et al.
Attempts have been made to trace genetic predispositions that modify the host detection of pathogen or the resultant systemic reaction. Longterm treatment with appropriate antibiotics is the standard therapy. The ensuing bacteraemia is complicated by septic emboli to a range of sites such as lung, joints, and. Lemierre s syndrome tends to occur toward the end of winter or in early spring. Additional symptoms included sore throat, dysphagia and left otalgia. Our case report illustrates the use of ecls in staphylococcal septic shock in a child with severe lemierres syndrome and necrotizing pneumonia.
To the best of our knowledge, this is the first report of ecls support in a patient with lemierres syndrome caused by s. Although once rare, lemierres syndrome is becoming more and more common. The propagation of the septic emboli throughout the body causes a cascade of disastrous effects, most commonly lodging into the lungs and creating cavitary lesions. Lemierres syndrome is a potentially severe disease characterised by suppurative thrombophlebitis of the internal jugular vein and subsequent disseminated septic embolisation. Occasionally, other microbiota organisms are involved, including f. Patients typically present with high fevers, neck pain, and pulmonary symptoms.
Pdf the long shadow of lemierres syndrome researchgate. Lemierres syndrome, or jugular vein thrombosis jvt associated with anaerobic infection of the head and neck and frequently complicated by septic pulmonary embolism pe, has historically been described as a disease of young adults. Lemierre s syndrome is a rare disorder of young adults caused by the anaerobic bacterium, fusobacterium necrophorum and occasionally by other fusobacterium species f. The interval between the oropharyngeal infection and the onset of the septicaemia is usually. Uptodate, electronic clinical resource tool for physicians and patients that provides information on adult primary care and internal medicine, allergy and immunology, cardiovascular medicine, emergency medicine, endocrinology and diabetes, family medicine, gastroenterology and hepatology, hematology, infectious diseases, nephrology and.
Diagnosis and management of lemierres syndrome presented. Lemierre syndrome is a rare disease of the head and neck often affecting. Here we describe a case of lemierre syndrome and present imaging classic for the disease. A previously healthy 25 year old male who initially.
Lemierres syndrome a forgotten disease andre lemierre 1936 6. Lemierre syndrome in an adult patient with fontan circulation. In recent years, an increasing number of case reports of childhood lemierres syndrome have been published, focusing mostly on the clinical and. Lemierre syndrome is a potentially fatal complication of oropharyngeal infections that is characterized by jugular vein thrombosis and septic emboli. Lemierres syndrome is a severe illness caused by the anaerobic bacterium, fusobacterium necrophorum which typically occurs in healthy teenagers and young adults.
We describe an unusual case of a previously healthy 58yearold male with lemierres syndrome. Prolonged antibiotic therapy is the cornerstone of treatment, occasionally combined with anticoagulation. Lemierre syndrome, also known as lemierre s disease is a rare condition that was first described by andre lemierre. This article is from international journal of emergency medicine, volume 6. Lemierre s syndrome presents a classic clinical picture, the pathophysiology of which remains obscure. Nonetheless the identification of thrombophlebitis of the internal jugular vein is the first hard evidence to suggest lemierres syndrome in many patients 8. Children who were aged from birth to 21 years and had received a diagnosis of jvt and lemierres syndrome at the childrens hospital denver, co be. Lemierre syndrome, also known as postanginal sepsis, is an illness characterized by the development of a fusobacterial septicaemia with multiple metastatic foci following an attack of acute tonsillitis. He was admitted to the hospital and was started on symptomatic treatment for.
Oct 21, 2019 the classic lemierres syndrome refers to a septic thrombosis of the internal jugular vein, usually caused by a fusobacterium necrophorum infection starting in the oral cavity, and typically complicated by pulmonary emboli. It is widely known that lemierres syndrome may be caused by a variety of, mostly anaerobic, oral cavity flora. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. With the advent of more advanced laboratory identification methodologies, clinicians should be alert to the implications of isolating more unusual organisms. Epidemiology the incidence of lemierre syndrome has been reported between 0. The symptoms of lemierres syndrome may not appear right away after you get the infection.
Lemierres syndrome is a rare complication following an acute oropharyngeal infection. Lemierre s syndrome, which is also known as postanginal sepsis and human necrobacillosis, is a rare disease in young adults most commonly caused by f. Expand the pharyngitis paradigm for adolescents and young adults. Most cases of lemierre s syndrome start out as a typical throat infection with a sore. Parikh ma b ryan wright bs b paul holden phd c william armstrong md c felizardo. Lemierres syndrome is a condition characterized by thrombophlebitis of the internal jugular vein and bacteremia caused by primarily anaerobic organisms, following a recent oropharyngeal infection. Lemierres syndrome international journal of emergency medicine. It was a very common disease with a high mortality rate in the preantibiotic era, but the incidence and mortality rate decreased after the introduction of antibiotics.
Lemierres syndrome ls takes its name after the french bacteriologist andrealfred lemierre, who first described the syndrome in 1936. Aug 17, 2014 lemierres syndrome a forgotten disease andre lemierre1936 6. Oct 23, 20 lemierres syndrome is a condition characterized by thrombophlebitis of the internal jugular vein and bacteremia caused by primarily anaerobic organisms, following a recent oropharyngeal infection. Lemierres syndrome necrobacillosis postgraduate medical. Lemierre syndrome at dorlands medical dictionary sibai k, sarasin f 2004. However, unusual forms of the disorder have been rarely reported. It was once called the forgotten disease because of its rarity, but it may not be that uncommon after all. If you have problems viewing pdf files, download the latest version of adobe reader. A 25yearold woman presented to the emergency department ed with 4 days of progressive, leftsided neck pain and swelling.
This condition starts in your throat, so the first symptom you notice will likely be a sore throat. Lemierres syndrome the western journal of emergency medicine. The aetiological agent is typically anaerobic bacteria of the genus fusobacterium. In recent years, an increasing number of case reports of childhood lemierres syndrome have been published, focusing mostly on the clinical. It is usually caused by caused by fusobacterium necrophorum and characterized by septic thrombophlebitis of the internal jugular vein. Lemierre syndrome is a disease that presents with oropharyngeal infection.
Due to the introduction of antibiotics, ls has aptly been. Lemierre syndrome is a serious condition that associates oropharyngeal infection and thrombosis of the internal jugular vein ijv with subsequent distant septic emboli, most frequently in the lungs. Complications of lemierre syndrome are a result of septic emboli from the thrombus, systemic effects of sepsis and clot extension. Lemierres syndrome is a rare disease, caused by the anaerobic bacteria fusobacterium, most commonly f. Combinations of fever, pharyngitis, dysphagia, odynophagia, or oropharyngeal swelling are common presenting symptoms. Lemierres and lemierres like syndromes in children. Lemierres syndrome is rare but needs to be identified early in the course. Lemierre syndrome following oropharyngeal infection. People with experience in lemierres syndrome help solve this question.
Lemierres syndrome, reemergence of a forgotten disease. Jul 21, 2009 lemierre s syndrome is characterized by a history of recent oropharyngeal infection, clinical or radiological evidence of internal jugular vein thrombosis, and isolation of anaerobic pathogens, mainly fusobacterium necrophorum. In that series, another patient who died from lemierre syndrome had a high number. Lemierres syndrome has been shown to be increasing in incidence in the past 20 years with one. Lemierres syndrome ls or suppurative thrombophlebitis of the jugular vein.
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