Chest Infections NetWork Puzzler

Blood culture results were positive for Fusobacterium necrophorum, an anaerobe.

This patient had Lemierre syndrome, a suppurative infection associated with jugular vein thrombophlebitis, septic emboli to the lung, metastatic abscess formation, and bacteremia with F necrophorum. This typically occurs in young, otherwise healthy adults. It is a rare but well-described complication of tonsillitis, pharyngitis, odontogenic infections, and mastoiditis.

The syndrome was described in the early 1900s by Andre Lemierre and other reseachers. In a historic article in Lancet, Lemierre described 20 patients with pharyngitis followed by anaerobic septicemia, 18 of whom died. Numerous cases have been described since then. The organism, F necrophorum, is part of the normal flora in the oral cavity, female genital tract, and some areas of the GI tract. The organism has a number of potential virulence factors, including lipopolysaccharide. Other pathogens have been associated with Lemierre syndrome, including Eikenella corrodens (a member of the HACEK group), Streptococcus pyogenes, and Arcanobacterium haemolyticum.

Histologically, there is suppurative inflammation within the wall of the vein, with thrombus and pus in the lumen. Often, there is extension to the lateral pharyngeal space and can be life threatening with involvement of the carotid sheath.

As with this patient, Lemierre syndrome usually presents initially with an exudative pharyngitis. Symptoms may improve as the infection progresses, but edema and/or pain develops in the neck. At this point, there is the acute onset of fevers, shaking chills, and pain along the sternocleidomastoid or angle of the jaw. Septic emboli occur in the majority of patients. Other complications include septic arthritis, osteomyelitis, disseminated intravascular coagulopathy, and liver abscesses. In some patients, the presentation mimics right-sided endocarditis.

A number of life-threatening complications can be seen. Cavernous sinus thrombosis can result from retrograde extension of the suppurative process. Mediastinitis can occur as a result of direct extension along the vessels, with or without airway obstruction. Carotid artery rupture has been described, with sentinel bleeding from the ear, nose, or mouth.

Diagnosis rests on both a compatible clinical presentation and imaging. CT scans of the neck typically show soft tissue edema, inflammation, and filling defects in the vein, as with our patient.

Antibiotics should be targeted against anaerobes. A number of different empiric regimens have been proposed including ampicillin-sulbactam, imipenem, ceftriaxone plus clindamycin, and others. In this case, the patient was treated with penicillin G, 24 million units per day, because the isolate was β-lactamase-negative. In the literature, treatments have varied from 2 to 14 weeks. Patients should be followed carefully, given the wide range of complications. In some instances, ligation or excision of the infected jugular vein may be necessary if sepsis is uncontrolled.

The role of anticoagulation therapy is controversial, and there are no randomized controlled trials for an evidence-based decision. In this case, the patient was given anticoagulation therapy with IV heparin, and he did well on coumadin and IV penicillin.