A prospective study in denmark detected an annual incidence of 36 cases of lemierres syndrome per million people from 1998 to 2001, with a substantially higher annual rate of 144 cases per million people aged 1424 years. Although the comparison is correct in terms of the location, bacterial characteristics, and recommended antibiotics, the pathophysiology and the importance of surgery are dramatically different. This is a rare illness in the modern era of antibiotic therapy, though it has. Lemierres syndrome due to fusobacterium necrophorum. Since the advent of antibiotics, the incidence of lemierre syndrome has reduced significantly, now affecting between 0. Atypical presentation of lemierres syndrome causing septic shock. Initially described in 1900 by courmont and cade 1 and again in 1918 by schottmuller 2, it was not until 1936 when the french microbiologist dr andre lemierre best. In recent years, an increasing number of case reports of childhood lemierres syndrome have been published. It was once called the forgotten disease because of its rarity, but it may not be that uncommon after all. In the 1960s and 1970s, lemierres syndrome became a rare phenomenon with the proliferation of penicillin in the treatment of pharyngitis. Lemierres syndrome is a rare complication following an acute oropharyngeal infection. A high index of suspicion and early recognition is important for successful management and to prevent systemic complications like multiorgan failure with extremely high morbidity, prolonged hospitalization and, not uncommonly, death. Sites of metastatic infection, in order of frequency of involvement, are lung, joint, muscle, bone, liver, skin, spleen and endocardium. Currently, since imaging has a key role in the early diagnosis of this syndrome, the radiologist should be aware of and recognize its manifestations.
Fusobacterium necrophorum is the most common organism implicated in causation of lemierre s syndrome. Lemierre syndrome is a rare but important constellation of clinical findings that classically includes four key manifestations. Patients typically present unwell, with trismus and pain behind the angle of the jaw. Today, this syndrome is typically seen in healthy adolescents. Complications of lemierre syndrome are a result of septic emboli from the thrombus, systemic effects of sepsis and clot extension. This incidence in young males over females is very slight, and the small number of samples of this condition make it hard to know for sure whether this is a real demographic difference. Lemierres syndrome results from a rare type of bacterial infection in your throat. Lemierre syndrome is a potentially fatal complication of oropharyngeal infections that is characterized by jugular vein thrombosis and septic emboli. Lemierres syndrome an overview sciencedirect topics.
The incidence of lemierre syndrome has significantly decreased and the outcomes have improved with the wide spread use of antibiotics 2. It creates a peritonsillar abscess, a blood clot, filled with bacteria near the tonsils in the jugular vein. 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. The thrombophlebitis is a serious condition and may lead to further systemic complications such as bacteria in the blood or septic emboli. Due to the introduction of antibiotics, ls has aptly been referred to as the forgotten disease. Lemierre s syndrome is diagnosed through blood tests that identify the bacteria causing the observed symptoms. Lemierre syndrome has been reported to be serious and potentially fatal in the preantibiotic era1. The ensuing bacteraemia is complicated by septic emboli to a range of sites such as lung, joints. Many cases present as benign, localized venous cords that resolve completely with minimal intervention. The ensuing bacteraemia is complicated by septic emboli to a range of sites such as lung, joints, and. There has been a significant increase in the number of documented cases over the past 10 to 20 years.
When first described in the early 1900s, lemierre syndrome was almost universally fatal. Lemierres syndrome is a suppurative thrombophlebitis of the internal jugular vein, usually caused by direct invasion of oropharynx anaerobes, specifically fusobacterium necrophorum. There is a male predominance, with 70% of patients between the ages of 16 and 25 years. A potentially lethal form of sore throat caused by the bacterium fusobacterium necrophorum, a common inhabitant of the mouth. Hyperbaric oxygen hbo2 in the treatment of lemierre syndrome. Infection of the lateral pharyngeal space may result in thrombosis of the internal jugular vein, subsequent metastatic complications e. Lemierres syndrome refers to infectious thrombophlebitis of the internal jugular vein.
A case report on the recurrence of lemierres syndrome. An unusual cause of calf abscess blood cultures and intraoperative tissue cultures grew gramnegative rods on day 2 of admission, from which fusobacterium necrophorum was isolated. An 18yearold man presented with a sore throat, fever, and pleuritic chest pain. The clinical course and severity of septic thrombophlebitis are quite variable. Lemierres syndrome presenting with neurological and. The lemierres syndrome is a rare and often underdiagnosed septic disease. Lemierres 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.
It also is called postanginal sepsis, as well as necrobacillosis. The patient was given a dose of iv ampicillinsulbactam for suspected septic thrombophlebitis of the right ij vein and transferred to a. Lemierre syndrome is an uncommon disease which commonly arise from acute bacterial oropharyngeal infection. This is in striking contrast to necrobacillosis arising outside the head and neck. Internal jugular vein thrombosis and lemierre s syndrome. 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. Lemierres syndrome international journal of emergency. Fusobacterium necrophorum is the most common pathogen isolated from the patients. This disease vanished with the advent of antibiotics but then returned decades later.
The diagnosis of lemierres syndrome was then established based on her. 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. It is commonly caused by fusobacterium necrophorum1. Incidence rates have been reported to vary between 0.
For language access assistance, contact the ncats public information officer. Lemierres syndrome is suppurative thrombophlebitis of the jugular vein. Lemierres syndrome is caused by septic thromboembolic from the jugular veins. A case of lemierres syndrometamara soh and mui hong lim lemierres syndrome. Lemierres syndrome, reemergence of a forgotten disease. 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. An appropriate antibiotic therapy is mandatory for a successful treatment of this. Lemierres syndrome almost invariably arises in patients who were previously fit. Although once rare, lemierres syndrome is becoming more and more common. Prompt clinical diagnosis and treatment with appropriate antibiotics are critical.
First described by coumont and cade in 1900, it is named for a french physician named andre lemierre after he described 20 cases in 1936. A mass in the neck may be palpable at the angle of the jaw or along the anterior margin of the. The septic emboli seed many organs, classically causing a multifocal pneumonia. Lemierre syndrome is caused by oropharyngeal infections, which cause septic thrombophlebitis of the internal jugular vein and metastatic infections. Fusobacterium species are most commonly implicated. Our main mission in this fight is to bring awareness of lemierres syndrome to the public. Lemierre syndrome postanginal sepsis is a fulminant infectious syndrome caused by acute oropharyngeal infection that is complicated by secondary septic thrombophlebitis of the internal jugular vein. Lemierre syndrome is a rare and potentially lifethreatening complication of bacterial infections that usually affects previouslyhealthy adolescents and young adults. The washington post has a medical mysteries article on nov. Lemierres syndrome international journal of emergency medicine.
Lemierre s syndrome is a rare disease that results in an oropharyngeal infection, which precipitates an internal jugular vein thrombosis and metastatic infection. It is usually seen in healthy adolescents or young adults. It is commonly described in young healthy adults with isolation of fusobacterium necrophorum. Fusobacterium necrophorum is a bacterium usually found in the oropharynx, female genital tract, and gastrointestinal tract, and is the etiologic agent of lemierre syndrome in 81% of modern reports. Group a streptococcus has mainly been observed as a polymicrobial organism in the syndrome. Most cases of lemierre s syndrome start out as a typical throat infection with a sore.
Lemierre syndrome is a rare clinical entity, with fewer than 160 cases reported since it was first described in the early 1900s. Jan 08, 2019 lemierre syndrome may be diagnosed based on signs and symptoms, as well as a variety of blood tests and imaging studies. The aetiological agent is typically anaerobic bacteria of the genus fusobacterium. Lemierre syndrome is an uncommon condition classically described in acute oropharyngeal infection with septic thrombophlebitis of the internal jugular vein and metastatic septic embolism particularly to the lungs. Lemierre syndrome infectious disease and antimicrobial. Dec 07, 2018 septic thrombophlebitis is a condition characterized by venous thrombosis, inflammation, and bacteremia. The classic cause is fusobacterium necrophorum which is normal flora. This disease was first described in 1900 by courmont and cade lemierre. Lemierres syndrome ls is a rare but fatal complication of oropharyngeal infections. Occasionally, other microbiota organisms are involved, including f. This syndrome consists of internal jugular vein thrombosis after a primary oropharyngeal infection with the development of distant septic emboli.
Lemierres syndrome is a severe illness caused by the anaerobic bacterium, fusobacterium necrophorum which typically occurs in healthy teenagers and young adults. 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. Currently the incidence of lemierres syndrome is about one in a million. Lemierre syndrome is therefore often referred to as the forgotten disease. 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 is principally a disease of healthy adolescents and young adults.
Problems occur when bacteria begin to thrive within abscesses in the throat, leading to deep infections that may reach a major blood vessel called the jugular vein. The syndrome is most commonly associated with the anaerobic gramnegative rod fusobacterium necrophorum. In hagelskjaers series of nonhead and neck cases,5 nine out of 25 had an underlying cancer, seven were alcohol or drug abusers, and. Lemierre syndrome infectious disease and antimicrobial agents. An otogenic variant of lemierre s syndrome was seen in 5 children with dissemination to nearby regions, and other variants of lemierre s syndrome, e. The relentless pain drove her to seek acupuncture treatment 3 days before admission. Patients present with ill appearance, fevers, a history of pharyngitis and neck pain. Lemierre s syndrome tends to occur toward the end of winter or in early spring.
Lemierres syndrome was common in the era before the advent of antibiotics. Ctmri many authors consider cect as the imaging study of choice due to availability and its allowance for visualization of complications and underlying infection 68. The prognosis of lemierre syndrome depends on the early. Apr 15, 2020 lemierre s syndrome is a possible complication of a severe, untreated throat or tonsil infection. The tree was inferred from 16s rrna data by the neighborjoining method. Oct 23, 20 lemierres syndrome occurs primarily in young, otherwise healthy individuals and is characterized by a history of recent oropharyngeal infection, clinical or radiological evidence of ij venous thrombosis and anaerobic bacteremia caused primarily by f. 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. Blood tests may reveal various abnormalities suggestive of the diagnosis, such as a high white blood cell count, low blood platelet count or other evidence of clotting problems, and abnormal kidney andor liver function. Lemierre syndrome a frequently overlooked infectious disease.
Lemierres syndrome is characterized by an oropharyngeal infection followed by internal jugular vein septic thrombophlebitis and metastatic emboli, most often to the lungs and joints. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. Lemierre syndrome, also known as postanginal septicemia or human necrobacillosis, is a rare disease of the head and neck that often affects healthy adolescents and young adults. Lemierres syndrome occurs most often when a bacterial throat infection progresses to the formation of a peritonsillar abscess. Shouldnt it be lemierre syndrome, since the eponym is named for the person who described it, and not for the person who had it. The syndrome is characterised by a primary oropharyngeal infection followed by metastatic spread and suppurative thrombophlebitis of the internal jugular vein. Epidemiology the incidence of lemierre syndrome has been reported between 0. Diagnosis is established with evidence of metastatic infection and internal jugular vein thrombophlebitis.
Learn about its symptoms, how its treated, and more. Lemierre syndrome is a disease that presents with oropharyngeal infection. However, there are reported cases of lemierrelike syndrome lls due to aerobes. In that series, another patient who died from lemierre syndrome had a high number. Lemierres syndrome ls is a rare, but a lifethreatening complication of an oropharyngeal infection.
It is characterized by thrombosis of internal jugular vein that rapidly progresses into sepsis and is typically caused by the anaerobe fusobacterium necrophorum. It is most commonly seen in otherwise healthy adolescents and young adults. Since this infection involves the throat and the mouth, and is frequently due to anaerobic bacteria, it may be mistaken for lemierres syndrome risoud et al. 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. Aug 17, 2014 lemierres syndrome a forgotten disease andre lemierre1936 6. Suppurative intracranial thrombophlebitis sciencedirect. Lemierres syndrome postanginal septicaemiahuman necrobacillosis was first defined and described in 1936 by lemierre, a french physician and professor in microbiology, although the first case report of human necrobacillosis was published in 1900 by courmont and cade 1,2. A high index of suspicion and early recognition is important for successful management and to prevent systemic complications like multiorgan failure with extremely high. Lemierre s syndrome is a rare disorder of young adults caused by the anaerobic bacterium, fusobacterium necrophorum and occasionally by other fusobacterium species f. Lemierre s syndrome is an often undiagnosed disease seen in previously healthy young subjects, presenting with symptoms of pharyngitis, fever and elevated markers of inflammation.
Lemierre s syndrome refers to infectious thrombophlebitis of the internal jugular vein. Several lab tests can diagnose this syndrome, including. Aug 22, 2017 lemierres syndrome is usually diagnosed after you show certain symptoms for an extended amount of time, especially a sore throat. Combinations of fever, pharyngitis, dysphagia, odynophagia, or oropharyngeal swelling are common presenting symptoms. 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. Lemierre s syndrome is an extremely rare and almost universally fatal disease characterized as thrombophlebitis of the internal jugular venous system with subsequent metastatic infection.
Lemierre syndrome following oropharyngeal infection. 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. Lemierre syndrome genetic and rare diseases information. If you have problems viewing pdf files, download the latest version of adobe reader. Lemierres syndrome resulting from streptococcal induced. The physical exam findings of lemierres syndrome are variable and depend on the extent of metastatic disease table 2. Nonetheless the identification of thrombophlebitis of the internal jugular vein is the first hard evidence to suggest lemierres syndrome in many patients 8.
For unknown reasons, lemierres syndrome is a disease that affects young adult males more than any other demographic. Patients typically present with high fevers, neck pain, and pulmonary symptoms. Currently, since imaging has a key role in the early diagnosis of this syndrome, the radiologist should. Pdf lemierres syndrome is a rare but a life threatening condition which affects young healthy individuals, was first described by dr. In people with lemierre syndrome, the initial infection spreads into tissues and deep spaces within the neck, leading to the formation of an infected blot clot septic thrombophlebitis, sometimes made up of pus, in the internal jugular vein the blood vessel that carries blood away from the brain, face, and neck. The infection originates in the throat and spreads via a septic thrombophlebitis of the tonsillar vein and internal jugular vein. Lemierre s syndrome ls is a potentially lifethreatening condition, characterized by clinical or radiologic evidence of internal jugular vein thrombosis following an oropharyngeal infection, most commonly by fusobacterium necrophorum. It most often develops as a complication of a bacterial sore throat infection in young, otherwise healthy adults. Lemierre syndrome is a lifethreatening disease characterized by infectious thrombophlebitis of the internal jugular. Lemierres syndrome occurs most often when a bacterial throat infection.
Fever is the most common physical finding reported, present in 92% to 100% of cases, followed by pharyngitis or peritonsillar abscess and neck mass 2, 7, 9, 10, 1218, 23, 24. The condition is characterized by a primary oropharyngeal infection with evidence of septic thrombophlebitis, exhibited by positive blood cultures, clinical or radiographic evidence. The interval between the oropharyngeal infection and the onset of the septicaemia is usually. Lemierre s syndrome represents occlusion of the internal jugular vein with septic pulmonary emboli and at times systemic sepsis edibam et al. Today, the incidence of lemierres syndrome is estimated to be 1 per million annually. Referral was made to the infectious disease specialist and antibiotic. Ct scans and ultrasound imaging are also often used to detect blood clots in the. Lemierres syndrome ls takes its name after the french bacteriologist andrealfred lemierre, who first described the syndrome in 1936. Lemierres syndrome associated with campylobacter rectus bac.