High risk and low prevalence diseases: Lemierre's syndrome.

INTRODUCTION: Lemierre's syndrome is a serious condition that carries with it a high rate of morbidity and even mortality. OBJECTIVE: This review highlights the pearls and pitfalls of Lemierre's syndrome, including presentation, diagnosis, and management in the emergency department (ED) based on current evidence. DISCUSSION: Lemierre's syndrome is a condition marked by septic thrombophlebitis of the internal jugular vein (IJV), with the classic triad of pharyngotonsillitis, IJV thrombosis, and septic emboli resulting in metastatic abscess. It typically begins as pharyngitis, often caused by Fusobacterium necrophorum. Patients most commonly present with fever, recently diagnosed pharyngitis, and neck pain or swelling. Septic emboli may affect multiple organ systems, most commonly the pulmonary system. The disease should be considered in patients with prolonged symptoms of pharyngitis, pharyngitis that improves but then worsens, critically ill patients with pharyngitis, patients with pharyngitis and infection at a secondary site, and neck signs/symptoms. Diagnosis includes throat and blood cultures, as well as imaging to include computed tomography of the neck and chest with intravenous contrast. Additional imaging of other areas should be performed as clinically indicated. Initial management includes hemodynamic stabilization with intravenous fluids and vasopressors as needed, as well as broad-spectrum antibiotics. Anticoagulation for the primary thrombus and possible septic emboli is controversial and should be considered in a multidisciplinary approach with admission. CONCLUSIONS: An understanding of Lemierre's syndrome can assist emergency clinicians in diagnosing and managing this potentially deadly disease.

Lemierre's syndrome in adulthood, a case report and systematic review.

Introduction: Lemierre's syndrome is a septic thromboembolic complication of an oropharyngeal or neck infection, primarily caused by Fusobacterium species. Although it usually affects young healthy patients, some case reports describe this syndrome in older population.Methods: A case report and a systematic review of the literature were conducted to investigate the late onset of Lemierre's syndrome. Forty-one articles were selected for the qualitative analysis, 39 for the quantitative analysis.Results: The average age of the study population was 52 years old. Diabetes mellitus and upper gastro-intestinal malignancy, common comorbidities in the study population, might play a role in the development of late-onset Lemierre's syndrome. Empiric antibiotic treatment should cover Fusobacterium and Streptococcus species both, which may cooperate to induce purulent disease. Reported unfavourable outcome was more than expected.Conclusion: Lemierre's syndrome in adulthood may differ from the usual version. This disease may further pass unrecognized, if presented out of the expected age range. Nevertheless, early diagnosis and prompt treatment are a requisite to prevent morbidity and mortality, which may be higher in this older population.

Lemierre's syndrome in the pediatric population: Trends in disease presentation and management in literature.

OBJECTIVE: This study aims to examine literature on Lemierre's Syndrome (LS) in the pediatric population over time in order to describe patterns in disease progression, management, and prognosis. In addition, this study assesses specific rate of literature output and the location of research over the past 10 years. METHODS: A literature review was conducted through two databases, PubMed.gov and PMC. A search was conducted using the keywords "Lemierre syndrome" and "postanginal sepsis." Literature was primarily reviewed for demographic, radiographic, and clinical data. Articles were included in the study if they were published in English and within the last 10 years. All types of research studies were recorded, however primary data collection came from case reports and series. Publications were grouped into two time periods: 2009-2013 and 2014-2019, allowing for comparison of various characteristics between these two groups. RESULTS: A total of 124 research studies on LS met inclusion criteria and were reviewed. Of these, 98 case reports (79.0%) were examined. Disease Characteristics: Fusobacterium necrophorum was the most common precipitating pathogen isolated from cultures (66.2%). The main primary treatment modalities used were antimicrobials, surgery, anticoagulation, or a combination of these treatments. A total of 63.9% of the case reports indicated use of anticoagulation at some point during treatment. Publication Trends: The number of published studies has not significantly changed in the last decade, with a non-statistically significant decline of 5.6%, when comparing 2014-2019 to 2009-2013 (p = 0.21). Case reports/series were the most common study design (82.2% vs 69.5%) and level of evidence for published studies continued to be stable (level 4-5) through the years (86.9%). The number of publications within an international journal vs US based journal has also remained steady during both time periods (p = 0.698). CONCLUSION: LS is an uncommon condition but one that is important for physicians to be aware of in the pediatric population. Treatment regimens including antibiotics and anticoagulation have remained stable through the past 10 years, however the efficacy of anticoagulation in treating LS continues to be debated. Though LS is considered a severe illness with potentially life threatening complications, publications on this topic, in pediatrics specifically, have decreased within the past five years.

Thrombosis of the internal jugular vein in the ENT-department - Prevalence, causes and therapy: A retrospective analysis.

OBJECTIVE: Less than 5% of deep vein thrombosis is due to thrombosis of the internal jugular vein. Genetic, malignant or inflammatory underlying diseases as well as insertion of venous catheters can be responsible for this pathology. Due to its rare occurrence, it is difficult to find systematic research about thrombosis of the internal jugular vein. METHODS: We performed a systematic analysis of present patient data from our ENT department with the electronic patient record considering the period from 2012-2017. Search terms were "thrombosis" and "jugular internal vein". We identified 41 patients with the requested diagnosis and performed further analysis of the cases. Internal jugular vein thrombosis was diagnosed in all patients using Duplex sonography and/or CT/MR angiography. RESULTS: Paraneoplastic thrombosis was found in 22/41 patients (54%), in 15 of the 22 (68%), the tumor was located in the ENT region. Two out of seven (29%) of the patients with tumor entities outside the head and neck region had thrombosis of the internal jugular vein as the first symptom of the disease. Another 14/41 patients (34%) had underlying inflammatory diseases - mostly streptococci-associated - for example a cervical abscess. In two patients, insertion of a central-venous catheter was causal, in three patients we could not find any reason for the development of thrombosis. CONCLUSION: To diagnose the rare and often asymptomatic thrombosis of the internal jugular vein, ultrasound of the cervical region should always include vascular imaging. Thrombosis of the internal jugular vein results mostly paraneoplastic or due to inflammation/abscess. It can be the first symptom of a malignant primary disease and always requires detailed diagnostic clarification. LEVEL OF EVIDENCE: 4.

Septic thrombosis of the internal jugular vein: Lemierre's syndrome revisited.

OBJECTIVES/HYPOTHESIS: Study of the clinical evolution of a primary ear, nose, and throat infection complicated by septic thrombophlebitis of the internal jugular vein. STUDY DESIGN: Retrospective case-control study. PATIENTS AND METHODS: From 1998 to 2010, 23 patients at our institution were diagnosed with a septic thrombosis of the internal jugular vein. Diagnostics included microbiologic analysis and imaging such as computed tomography, magnetic resonance imaging, and ultrasound. Therapy included broad-spectrum antibiotics, surgery of the primary infectious lesion, and postoperative anticoagulation. The patients were retrospectively analyzed. RESULTS: The primary infection sites were found in the middle ear (11), oropharynx (8), sinus (3), and oral cavity (1). Fourteen patients needed intensive care unit treatment for a mean duration of 6 days. Seven patients were intubated, and two developed severe acute respiratory distress syndrome. An oropharynx primary infection site was most prone to a prolonged clinical evolution. Anticoagulation therapy was given in 90% of patients. All 23 patients survived the disseminated infection without consecutive systemic morbidity. CONCLUSION: In the pre-antibiotic time, septic internal jugular vein thrombophlebitis was a highly fatal condition with a mortality rate of 90%. Modern imaging techniques allow early and often incidental diagnosis of this clinically hidden complication. Anticoagulation, intensive antibiotic therapy assisted by surgery of the primary infection site, and intensive supportive care can reach remission rates of 100%.

Lemierre syndrome.

Lemierre's syndrome is an uncommon complication of pharyngitis in the United States and caused most commonly by the bacterium Fusobacterium necrophorum. The syndrome is characterized by a history of recent pharyngitis followed by ipsilateral internal jugular vein thrombosis and metastatic pulmonary abscesses and is a disease for which patients will seek medical care and advice. As most patients are admitted to the hospital under internal medicine, practitioners should be familiar with the usual signs and symptoms of Lemierre's syndrome along with its diagnosis and treatment. Controversy involves the choice and duration of antimicrobial therapy used for treatment and anticoagulation therapy for internal jugular vein thrombosis. As the diagnosis and management of this syndrome has generated controversy, an updated review of the literature and treatment recommendations may be helpful for providing optimal care for patients with this often unrecognized and confusing infection.