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. Lemierres syndrome, necrotizing pneumonia and staphylococcal. There is a male predominance, with 70% of patients between the ages of 16 and 25 years. 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. A 25yearold woman presented to the emergency department ed with 4 days of progressive, leftsided neck pain and swelling. Nonetheless the identification of thrombophlebitis of the internal jugular vein is the first hard evidence to suggest lemierres syndrome in many patients 8. 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.
Apr 11, 2014 volume 15, issue 2, march 2014 jayten shook, do et al. Lemierre syndrome following oropharyngeal infection. Lemierres syndrome international journal of emergency. It is usually caused by caused by fusobacterium necrophorum and characterized by septic thrombophlebitis of the internal jugular vein. 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. Patients typically present with high fevers, neck pain, and pulmonary symptoms. We present a case of a patient with lemierre s syndrome caused by fusobacterium necrophorum who developed a right frontal lobe brain abscess. The aetiological agent is typically anaerobic bacteria of the genus fusobacterium. Pdf the long shadow of lemierres syndrome researchgate. Lemierres syndrome, reemergence of a forgotten disease. Lemierres and lemierres like syndromes in children. Diagnosis and management of lemierres syndrome presented.
This syndrome consists of internal jugular vein thrombosis after a primary oropharyngeal infection with the development of distant septic emboli. Aug 17, 2014 lemierres syndrome a forgotten disease andre lemierre1936 6. The interval between the oropharyngeal infection and the onset of the septicaemia is usually short. Lemierres syndrome ls or suppurative thrombophlebitis of the jugular vein. Today, the incidence of lemierres syndrome is estimated to be 1 per million annually. Our case report illustrates the use of ecls in staphylococcal septic shock in a child with severe lemierres syndrome and necrotizing pneumonia. 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. Complications of lemierre syndrome are a result of septic emboli from the thrombus, systemic effects of sepsis and clot extension.
The ensuing bacteraemia is complicated by septic emboli to a range of sites such as lung, joints. Lemierres syndrome ls manifests as an oropharyngeal infection of. Aug 22, 2017 the symptoms of lemierres syndrome may not appear right away after you get the infection. In that series, another patient who died from lemierre syndrome had a high number. 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 is a rare disease that results in an oropharyngeal infection, which precipitates an internal jugular vein thrombosis and metastatic infection. Lemierre s syndrome is characterized by acute oropharyngeal infection with documented septicemia complicated by septic jugular thrombophlebitis and septic pulmonary.
Currently, lack of awareness of this condition delays appropriate treatment and worsens prognosis. Is lemierres syndrome transmitted from person to person. Lemierres syndrome the western journal of emergency medicine. Lemierre syndrome at dorlands medical dictionary sibai k, sarasin f 2004. 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. Lemierre s syndrome refers to infectious thrombophlebitis of the internal jugular vein.
It creates a peritonsillar abscess, a blood clot, filled with bacteria near the tonsils in the jugular vein. It is widely known that lemierres syndrome may be caused by a variety of, mostly anaerobic, oral cavity flora. 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. Parikh ma b ryan wright bs b paul holden phd c william armstrong md c felizardo. This article is from international journal of emergency medicine, volume 6. The symptoms of lemierres syndrome may not appear right away after you get the infection. People with experience in lemierres syndrome help solve this question. In recent years, an increasing number of case reports of childhood lemierres syndrome have been published, focusing mostly on the clinical. The infection originates in the throat and spreads via a septic thrombophlebitis of the tonsillar vein and internal jugular vein. The profile of this syndrome declined in the mid20th century, coinciding with the introduction of antibiotics. Combinations of fever, pharyngitis, dysphagia, odynophagia, or oropharyngeal swelling are common presenting symptoms. Fusobacterium necrophorum is the most common pathogen isolated from the patients. Sites of metastatic infection, in order of frequency of involvement, are lung, joint, muscle, bone, liver, skin, spleen and endocardium.
It most often develops as a complication of a bacterial sore throat infection in young, otherwise healthy adults. Pdf lemierres syndrome is a rare and feared complication of pharyngitis, occurring most commonly in adolescents. F necrophorum is most commonly associated with lemierre s syndrome. Epidemiology the incidence of lemierre syndrome has been reported between 0. He was admitted to the hospital and was started on symptomatic treatment for. Infection of the lateral pharyngeal space may result in thrombosis of the internal jugular vein, subsequent metastatic complications e. Lemierre syndrome is a rare and potentially lifethreatening complication of bacterial infections that usually affects previouslyhealthy adolescents and young adults. Lemierre s syndrome presents a classic clinical picture, the pathophysiology of which remains obscure. Lemierres syndrome is a suppurative thrombophlebitis of the internal jugular vein, usually caused by direct invasion of oropharynx anaerobes, specifically fusobacterium necrophorum. Images in clinical medicine from the new england journal of medicine lemierres syndrome. 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.
Lemierre syndrome a forgotten complication of acute. Lemierre syndrome is a rare disease of the head and neck often affecting. 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. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree.
In the 1960s and 1970s, lemierres syndrome became a rare phenomenon with the proliferation of penicillin in the treatment of pharyngitis. 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. The condition is characterized by a primary oropharyngeal infection with evidence of septic thrombophlebitis, exhibited by positive blood cultures, clinical or radiographic. This condition starts in your throat, so the first symptom you notice will likely be a sore throat.
Lemierre syndrome genetic and rare diseases information. Although once rare, lemierres syndrome is becoming more and more common. Lemierres syndrome international journal of emergency medicine. Lemierre syndrome, also known as lemierre s disease is a rare condition that was first described by andre lemierre. Lemierres syndrome is a potentially severe disease characterised by suppurative thrombophlebitis of the internal jugular vein and subsequent disseminated septic embolisation. Abstractlemierres syndrome is a condition characterized by thrombophlebitis of. Since the advent of antibiotics, the incidence of lemierre syndrome has reduced significantly, now affecting between 0. In recent years, an increasing number of case reports of childhood lemierres syndrome have been published, focusing mostly on the clinical and. A 70yearold female with retropharyngeal abscess developed features of lemierre syndrome. Lemierres syndrome is rare but needs to be identified early in the course. Expand the pharyngitis paradigm for adolescents and young adults. Currently, since imaging has a key role in the early diagnosis of this syndrome, the radiologist should be aware of and recognize its manifestations.
We summarise the epidemiology, microbiology, pathogenesis, clinical presentation, diagnosis, complications, therapy, and outcomes of lemierre s syndrome. Longterm treatment with appropriate antibiotics is the standard therapy. Lemierre syndrome is a disease that presents with oropharyngeal infection. If you have problems viewing pdf files, download the latest version of adobe reader. Lemierre s syndrome tends to occur toward the end of winter or in early spring. Prompt clinical diagnosis and treatment with appropriate antibiotics are critical.
Patients typically present unwell, with trismus and pain behind the angle of the jaw. Lemierres syndrome is an uncommon, but potentially lifethreatening complication of acute pharyngotonsillitis. 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. 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. Macsweens pathology of the liver seventh edition, 2018. Lemierres syndrome was common in the era before the advent of antibiotics. With the advent of more advanced laboratory identification methodologies, clinicians should be alert to the implications of isolating more unusual organisms. 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. For language access assistance, contact the ncats public information officer. Lemierres syndrome necrobacillosis postgraduate medical. 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. Fontan circulation, the definitive palliative treatment for patients with complex congenital heart disease with a functional univentricular heart, complicates clinical. Lemierre syndrome is a rare clinical entity, with fewer than 160 cases reported since it was first described in the early 1900s.
Due to the introduction of antibiotics, ls has aptly been. Unusual case of lemierres syndrome bmj case reports. It was once called the forgotten disease because of its rarity, but it may not be that uncommon after all. Additional symptoms included sore throat, dysphagia and left otalgia. Attempts have been made to trace genetic predispositions that modify the host detection of pathogen or the resultant systemic reaction. Treatment involves prolonged antibiotic therapy occasionally combined with anticoagulation. Our main mission in this fight is to bring awareness of lemierres syndrome to the public. Lemierres syndrome is a rare disease, caused by the anaerobic bacteria fusobacterium, most commonly f. Lemierres syndrome ls is a rare, but a lifethreatening complication of an oropharyngeal infection. Jan 08, 2019 lemierre syndrome is a rare and potentially lifethreatening complication of bacterial infections that usually affects previouslyhealthy adolescents and young adults. Here we describe a case of lemierre syndrome and present imaging classic for the disease. A 35yearold woman presented with a threeday history of sore throat, painful swelling of the neck, fever, rigor, hemoptysis, and dyspnea. Jul 17, 2006 lemierre s syndrome presents a classic clinical picture, the pathophysiology of which remains obscure. Volume 15, issue 2, march 2014 jayten shook, do et al.
Lemierres syndrome has been shown to be increasing in incidence in the past 20 years with one. 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. 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. Many authors consider cect as the imaging study of choice due to availability and its allowance for visualization of complications and underlying infection 68. 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. We describe an unusual case of a previously healthy 58yearold male with lemierres syndrome. Lemierre syndrome is caused by oropharyngeal infections, which cause septic thrombophlebitis of the internal jugular vein and metastatic infections. The syndrome is characterised by a primary oropharyngeal infection followed by metastatic spread and suppurative thrombophlebitis of the internal jugular vein. 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. A 17year old female, with no previous medical history, was admitted to the intensive care unit for septic shock, acute respiratory distress syndrome and. Lemierres syndrome is most commonly caused by fusobacterium necrophorum. Occasionally, other microbiota organisms are involved, including f. Lemierre syndrome postanginal sepsis is a classic complication of infection by fusobacterium necrophorum, characterized by thrombophlebitis of the internal jugular vein and septicemia.
Lemierre syndrome in an adult patient with fontan circulation. The interval between the oropharyngeal infection and the onset of the septicaemia is usually. The ensuing bacteraemia is complicated by septic emboli to a range of sites such as lung, joints, and. 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. Lemierres syndrome an overview sciencedirect topics. However, unusual forms of the disorder have been rarely reported. Lemierres syndrome due to fusobacterium necrophorum the. Lemierres syndrome the western journal of emergency. Examination revealed pustular exudates on the right. A previously healthy 25 year old male who initially. 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. The thrombophlebitis is a serious condition and may lead to further systemic complications such as bacteria in the blood or septic emboli. Lemierre s syndrome is a rare disorder of young adults caused by the anaerobic bacterium, fusobacterium necrophorum and occasionally by other fusobacterium species f.
Lemierres syndrome is a rare complication following an acute oropharyngeal infection. 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. A case report on the recurrence of lemierres syndrome. The patient was given a dose of iv ampicillinsulbactam for suspected septic thrombophlebitis of the right ij vein and transferred to a. In the preantibiotic era, the outcome of lemierres syndrome was rapidly fatal, with progressive septicemia. Pdf lemierres syndrome is a condition characterized by thrombophlebitis of the internal jugular vein and bacteremia caused by primarily anaerobic. A 17year old female, with no previous medical history, was admitted to the intensive care unit for septic shock, acute respiratory distress syndrome. Most cases of lemierre s syndrome start out as a typical throat infection with a sore.