Lemierre Syndrome: Clinical Update and Protocol for a Systematic Review and Individual Patient Data Meta-analysis

Abstract

Lemierre syndrome usually affects otherwise healthy adolescents or young adults and occurs at an overall rate of 1 to 10 cases per million person-years with an estimated fatality rate of 4 to 9%. Diagnostic criteria remain debated and include acute neck/head bacterial infection (often tonsillitis caused by anaerobes at high potential for sepsis and vascular invasion, notably Fusobacterium necrophorum) complicated by local vein thrombosis, usually involving the internal jugular vein, and systemic septic embolism. Medical treatment is based on antibiotic therapy with anaerobic coverage, anticoagulant drugs and supportive care in case of sepsis. Surgical procedures can be required, including drainage of the abscesses, tissue debridement and jugular vein ligation. Evidence for clinical management is extremely poor in the absence of any adequately sized study with clinical outcomes. In this article, we illustrate two cases of Lemierre syndrome not caused by Fusobacterium necrophorum and provide a clinically oriented discussion on the main issues on epidemiology, pathophysiology and management strategies of this disorder. Finally, we summarize the study protocol of a proposed systematic review and individual patient data meta-analysis of the literature. Our ongoing work aims to investigate the risk of new thromboembolic events, major bleeding or death in patients diagnosed with Lemierre syndrome, and to better elucidate the role of anticoagulant therapy in this setting. This effort represents the starting point for an evidence-based treatment of Lemierre syndrome built on multinational interdisciplinary collaborative studies.

Rare and life-threatening complication after an attempted lower third molar extraction: Lemierre syndrome

“…4 The use of anticoagulants, particularly as low-molecular-weight heparin, is still under debate. 4,5 To date, only one group is working on a systematic review and meta-analysis to clarify the treatment of the complication of Lemierre's syndrome. 5 Since the condition appears to be underreported and not properly highlighted, an eventual systematic review and meta-analysis of the occurrence of the Lemierre's syndrome is highly recommended.…”

Sore Throat, Fever, Septic Emboli, and Acute Respiratory Distress Syndrome: A Case of Lemierre Syndrome

“…89% of patients are between the ages of 10-35 years [4]. Despite its rarity, clinical experience suggests that its incidence may be on the rise, possibly secondary to antibiotic resistance, changes in prescription patterns, or the increased efficiency of diagnostic technologies [5].…”

“…Prompt institution of anticoagulation may prevent thrombotic extension, embolization, and new thromboembolic events but may also expose patients to bleeding complications. It is also unclear whether anticoagulation may facilitate spread of septic material or result in a hemorrhagic transformation of embolic lesions [5]. When deciding on anticoagulation, the medical team must carefully consider the patients characteristics, severity of disease, bleeding risk, and risk of new thromboembolic complications as well as the drugs pharmacokinetics [5].…”

Lemierre syndrome: Current evidence and rationale of the Bacteria-Associated Thrombosis, Thrombophlebitis and LEmierre syndrome (BATTLE) registry

“…The early notion that the typical primary infection was a tonsillitis and the thrombus was usually located in the internal jugular vein initially suggested that bacteria spread through the tonsillar and peritonsillar veins [6]. As it became clear that other head or neck infections can cause the syndrome and that other vessels, including the external jugular vein or the facial vein, may be involved, other pathways were postulated, including direct extension through the fascial plane between the tonsils and the parapharyngeal space and lymphatic spread [7]. All reports from the twentieth century confirmed the syndrome's specific association with Fusobacterium necrophorum.…”

“…is not considered to be mandatory: this organism is difficult to isolate, cultures may be negative due to empiric antibiotic therapy, and other bacteria seem able to cause Lemierre syndrome [5,17]. In a recent analysis of 712 cases reported in the scientific and grey literature since 2000, which were identified using the commonly applied broad definition [7], the cases satisfying the "typical" definition (oropharyngeal infection and isolation of Fusobacterium spp.) only represented approximately half of the observed spectrum of the syndrome; Fig.…”