Lemierre’s syndrome (LS) is a severe illness caused by the anaerobic bacterium, Fusobacterium necrophorum. It originates in the oropharynx and spreads via septic thrombophlebitis of the tonsillar and internal jugular veins (IJV) to sites such as the lung, joints and bones.
An 18-year-old woman with no past medical history presented to our Emergency Department (ED) with a 3-day history of fever, pleuritic chest pain and dyspnea. She had presented twice to the ED over the past 2 weeks, with complaints of sore throat, neck pain and myalgias and was discharged home with the presumed diagnosis of a viral illness. Vital signs on admission were temperature 98.8F, blood pressure 92/50, pulse 122/min, respiratory rate 22–36/min and pulse-oximetry 84% on room air. Physical exam findings included bilateral tonsillar swelling, tender cervical lymphadenopathy and bibasilar crackles. Admission labs revealed white blood cell count 42,000/cu.mm with 25% bands, blood urea nitrogen level 34mg/dl and creatinine 4.3mg/dL. Chest radiograph was consistent with a multi-lobar pneumonia. Chest CT scan revealed bibasilar consolidation with moderate right pleural effusion and cavitating peripheral opacities suspicious for septic emboli. She was subsequently intubated in the ED due to worsening hypoxemia and admitted to the ICU. She was started on ampicillin/sulbactam, vancomycin, and vasopressors for septic shock. Bedside transthoracic echo showed no valvular vegetations. Thoracentesis of the right pleural effusion was consistent with an empyema thus a pig-tail chest tube was placed. Admission blood cultures grew Gram-negative bacilli hence piperacillin/tazobactam was started. Subsequent chest radiographs and persistently high oxygen requirements were consistent with ARDS. The patient proceeded to develop a right-sided pneumothorax. Later that day, a left-sided pneumothorax ensued requiring bilateral tube thoracostomy. Continuous renal replacement therapy (RRT) was initiated on day 4 for acute renal failure (ARF). Blood cultures revealed Fusobacterium necrophorum and antibiotics were changed to meropenem and metronidazole. CT scan and ultrasound of the IJV were both negative for thromboses. Her renal function improved and pneumothoraces resolved. The patient was extubated after 2 weeks and discharged home after a 1-month hospitalization.
Lemierre’s syndrome is characterized by oropharyngeal infection, Fusobacterium septicemia, findings of metastatic infection, and occasionally evidence of IJV thrombophlebitis. It classically affects healthy young adults, with a male preponderance. It was described in 1936 by Andre Lemierre who reviewed 20 cases in young adults, of which only 2 survived. It was a common disease with high mortality in the pre-antibiotic era. There is evidence of resurgence in recent years, possibly associated with reduced use of antibiotics. LS specifically relates to Fusobacterium infection arising in the oropharynx. It may include pharyngeal inflammation with exudative tonsillitis, peritonsillar or parapharyngeal abscesses. Patients often have dysphagia, neck pain and tender cervical lymphadenopathy. Septicemia usually occurs 4–5 days after the onset of pharyngitis. Lung lesions are present in 80% of cases. Cavitations, pleural effusions, and empyema can be present. Fewer than 10% of cases require mechanical ventilation. ARDS, septic shock and acute renal failure requiring RRT are unusual. Delayed antibiotic treatment may explain the severe clinical presentation of our patient. The mainstay of therapy is long-term antibiotics and drainage of collections. A carbapenem or a penicillin/β-lactamase inhibitor combination, plus metronidazole are the antibiotics of choice. Diagnostic methods to detect IJV thrombophlebitis include CT, MRI, and ultrasound. The role of anticoagulants is controversial.
LS is a rare, life-threatening illness which can lead to septic shock and multi-organ failure if it is not recognized early. LS should be suspected in young patients with recent pharyngitis and clinical evidence of septic emboli.
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