Lemierre's Disease: Not so Forgotten!

Abstract

INTRODUCTION: Lemierre's disease is a complication of oropharygeal infection leading to septic thrombophlebitis of the internal jugular vein. We present a case of Lemierre's disease in a patient presenting without classic history or examination findings. CASE PRESENTATION: A 27 year old Caucasian male presented with headache, fever, chills, nausea, photophobia and night sweats for six days. He reported throbbing frontal headache 9/10 in intensity. He reported intermittent fever to 102degreeF without diurnal variation. Past medical and surgical histories were unremarkable. He served as an army tank driver who returned from Afghanistan 8 months before presentation and was poorly compliant with malaria prophylaxis. He smokes 10 cigarettes daily and denied alcohol or drug use. Upon admission, he was afebrile, tachycardic with 100% O2 saturation on room air. Physical examination was unremarkable except for photophobia and a palpable spleen tip. Laboratory revealed a normal WBC count with elevated neutrophils and banded cells, and thrombocytopenia (32,000 /mm3). Serum chemistries were unremarkable. Chest X-ray and head CT were negative, and CT of the abdomen and pelvis showed splenomegaly. Thin and thick smears for malaria and lumbar puncture were negative. He was started on broad spectrum antibiotics. After two days, he required transfer to the intensive care unit for respiratory distress where he was intubated. Repeat chest X-ray showed diffuse bilateral infiltrates suggestive of developing ARDS, and admission blood cultures were then positive for gram negative bacteria. Transthoracic echocardiogram did not show evidence of endocarditis. He became hypotensive and an ultrasound was performed to assist placement of a central venous catheter in the right internal jugular vein. Ultrasound showed non-compressible right internal jugular vein suggestive of deep vein thrombosis. CT neck confirmed thrombosis running through the entire length of the right internal jugular vein, consistent with the diagnosis of Lemierre's disease. Chest CT showed diffuse infiltrates with areas of cavitation, consistent with multiple septic emboli. The gram negative bacteria were identified as Fusobacterium Necrophorum and treatment with piperacillin-tazobactam was continued. He improved clinically, was successfully extubated, and subsequently discharged home on oral amoxcillin-clavulanate for four weeks. Thrombocytopenia had resolved at the time of discharge. DISCUSSION: Lemierre's disease, also known as post anginal sepsis, or the "forgotten disease ", is a rare complication of acute oropharyngeal infection resulting in septic thrombophlebitis of the internal jugular vein with septicemia and metastatic infections. The incidence varies between 1-14 per million cases per year. It is typically seen in young, immunocompetent adults, with a mean age at diagnosis being 20 years. Typical course has three phases. Phase one is the initial infection, which most commonly involves palatine tonsils. Phase two involves lymphangitic spread to the lateral pharyngeal space including thrombophlebitis of internal jugular vein. Phase three involves seeding of septic thromboemboli to various organs, with pleuropulmonary involvement being the most common. Classic Lemierre's disease is caused by a mono-infection with fusobacterium species. Physical signs include a tender palpable neck mass with cervical lymphadenopathy. Our patient's presentation was atypical in that there was neither antecedent throat infection nor typical physical findings of Lemierre's disease. Diagnosis is established by doppler ultrasound or CT with contrast. Due to increasing beta lactam resistance, empiric therapy should include beta lactamase resistant antibiotics. Typical duration of therapy is 4 -6 weeks. Anticoagulation and surgery remain controversial but may be considered if there is documented evidence of clot extension despite adequate antibiotic therapy. CONCLUSIONS: Lemierre's disease should be suspected in a young person with antecedent pharyngitis, septic pulmonary emboli and persis ent fevers despite antibiotic therapy.