Background The management of sepsis with no obvious focus in the critical care environment involves the acute stabilization of the patient and the sometimes more vexing task of diagnosis.
Case Description We present the case of a 16 year-old female, previously well, who presented to her community hospital with a 9-day history of fever and pharyngitis. Her symptoms progressed (despite oral antibiotics) to include malaise, abdominal pain, and eventually shortness of breath, pleurisy, and profound nausea, prompting her to seek medical attention. Upon assessment the patient was hypotensive and tachypneic with mild respiratory distress; she was in gallop rhythm and had a very tender abdomen. Her laboratory investigations revealed a slightly elevated white cell count with left shift, thrombocytopenia (platelet count 59,000), azotemia, elevated bilirubin, and metabolic acidosis. She was transported to our pediatric intensive care unit and electively intubated and ventilated. Despite broad-spectrum antibiotics and inotropic support, the patient's course was complicated by persistent fever and coagulopathy. Numerous other investigations, including head and abdominal imaging, revealed no focus of infection. Only after the eventual difficult identification of an organism was the diagnosis of “Lemierre Syndrome” made. Discovering that the cultured pathogen (fusobacterium necrophorum) was associated with septic thrombophlebitis, we identified an occlusive thrombus in the patients left internal jugular vein. With time and antibiotic therapy the patient gradually stabilized and improved.
Implications for Practice When a patient does not improve despite general empiric treatments, it is appropriate to consider rarer entities as the underlying disease, with the benefit of the experience in the literature. Our patient's course paralleled the descriptions of this rare entity found once the pathogen was identified, guiding our prognosis and management.
Conclusion Originally described in 1936, Lemierre syndrome is a rare occurrence in the post-antibiotic era, with incidence estimated at 1 in 1 million. A high-index of suspicion (once associated pathogens are cultured) is required to make the diagnosis. Controversies remain regarding the place of anticoagulation in therapy.
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