A healthy 37 year old female presented with sore throat, swollen neck glands, and high fevers for 3 days. The patient was first evaluated in the emergency department. A monospot and rapid strep test were performed and they were negative. A throat culture was also obtained and was later negative for beta hemolytic strep. Patient was discharged home with a diagnosis of a viral pharyngitis. The patient however came back to the emergency department a week later with persistent pharyngitis, enlarging tender neck glands, recurrent fevers, rigors, and now shortness of breath on exertion.
Initial vital signs were significant for a fever of 38.2 Celsius and a tachycardia of 108. Her physical exam revealed a right sided 4 cm tender submandibular soft tissue swelling accompanied by a palpable cord-like induration that extended to the angle of the mandible. Right sided tonsillar swelling was also noted without any exudates. Her lungs were clear. No murmur detected. No Roth spots, splinter hemorrhages, or any type of skin rash were seen.
Work up demonstrated a white blood cell count of 12.6. CT neck showed soft tissue swelling and phlegmonous changes of the right palatine tonsillar region without abscess formation. There was also evidence of a luminal filling defect within the right internal jugular vein concerning for a thrombus (figure 1). CT chest also revealed multiple bilateral pulmonary nodules, some of which were cavitating, concerning for septic emboli (figure 2). Serological EBV was positive for both IgM and IgG. Blood cultures eventually grew Streptococcus Milleri. The diagnosis of Lemierre’s syndrome was made based on her constellation of symptoms as well as microbiological and radiological findings.
Discussion:
Infamously labelled as the “forgotten disease”, Lemierre’s syndrome or septic thrombophlebitis of the internal jugular vein is a very rare but potentially fatal disease if unrecognized. The disease typically begins as an oropharyngeal infection from where oral commensal bacteria invade the lateral pharyngeal space including the internal jugular veins. This results in the formation of a septic thrombus which can disseminate and shower emboli into the lungs and other systemic organs. Although the illness is characteristically associated with hematological dissemination of Fusobacterium Necrophorum, there have been rare case reports of Streptococcus, Bacteroides, Peptostreptococcus, and even Staphylococcus species involvement also. Given our patient had a prodrome of pharyngitis with a positive EBV IgM, we postulated that she had acute infectious mononucleosis which resulted in a superimposed bacterial infection by an oral Streptococcal organism. Our patient was initiated on broad spectrum antibiotics on admission but they were de-escalated to IV ceftriaxone following the sensitivities of Streptococcus Milleri. The management of Lemierre’s syndrome involves early recognition and imaging, empirical intravenous antibiotics, and occasionally surgical drainage of any abscesses. Anticoagulation to target the venous thrombus is controversial with few guidelines to support its additional use to conventional treatment.
Conclusions:
In the face of unresolving pharyngitis, fevers, and a swollen/tender neck, clinicians should have a high suspicion of Lemierre’s syndrome followed by a prompt investigation. If diagnosed early, patients have a very good prognosis with limited complications.
