High risk and low prevalence diseases: Lemierre's syndrome

Lemierre's syndrome is a serious condition that carries with it a high rate of morbidity and even mortality.

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.

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.

An understanding of Lemierre's syndrome can assist emergency clinicians in diagnosing and managing this potentially deadly disease.