A 43-year-old woman presented with right lateral neck swelling for three days. She said she had a mild sore throat for two weeks, which was treated empirically with antibiotics by her primary care physician.
Her oropharynx was clear, but she had an obvious nonpulsatile 3“×4”-inch mass on her right lateral neck. This a CT scan of her neck. What is the diagnosis? See p. 24.
Diagnosis: Lemierre's Syndrome
Lemierre's syndrome, also known as human necrobacillosis and anaerobic postanginal sepsis (angina from the Latin angor, or sore throat), is an uncommon condition in which colonization from an oropharyngeal source results in thrombophlebitis of the internal jugular vein, bacteremia with confirmation on at least one distant metastases. (Medicine 1989;68:85.) Occurring most commonly in previously healthy adults, the condition was first described in the 1900s but fully characterized by Andre Lemierre in 1936. (Clin Microbiol Rev 2007;20:622.)
The most common pathogenic etiology is Fusobacterium necrophorum, but streptococcus species, staphylococci, Eikenella corrodens, Peptostreptococcus species, Bacteroides species, and other Fusobacterium species have been reported. (J Clin Microbiol 2003;41:3445.) Fusobacterium are particularly virulent organisms and create photolytic enzymes that destroy local tissue. (Br J Biomed Sci 2000;57:156.) The spread of infection is thought to occur by direct extension through the soft tissue (into the ipsilateral lateral pharyngeal space), transmission via oropharyngeal venous circulation, and lymphatic spread via perivascular inflammation and extension. (Otolaryngol Head Neck Surg 1995;112:767.)
Classic presentation of Lemierre's syndrome involves peritonsillar disease, internal jugular vein thrombophlebitis, metastatic pulmonary lesions, and isolation of Fusobacterium from a sterile body site, most commonly the blood. (South Med J 2012;105:283.) Most cases present with pharyngitis (87%), but findings of exudative tonsillitis, grey pseudomembranes, and oral ulcers have been reported. (Postgrad Med J 1999;75:141; JAMA 1982;248:1348; Medicine 2002;81:458.) Less common sources of primary infection include mastoiditis, otitis media, sinusitis, dental infections, and parotitis. Thrombophlebitis on initial evaluation ranges from 30 to 70 percent. (Postgrad Med J 2004;80:328; Medicine 2002;81:458.)
Patients may have a tender, enlarged swelling at the anterolateral neck with painful swelling of the glands below the maxillary angle consistent with thrombophlebitis of the jugular (internal or external) veins. Less commonly, patients may present with sepsis or in septic shock as the result of secondary infectious metastasis following resolution of the primary oropharyngeal infection (approximately one week). Secondary infection is most common in the pulmonary system, but can cause liver or spleen abscesses, septic arthritis (most commonly the hip; Medicine 1989;68:85), osteomyelitis, meningitis, or cellulitis.
Patients with secondary pulmonary infection may present with fever, rigors, dyspnea, pleuritic chest pain, or hemoptysis. Few patients present with diffuse abdominal pain, which is thought to be caused by pelvic vein septic thrombus or microabscess in the hepatobiliary system, which results in hyperbilirubinemia, transaminitis (50% of cases), and sclera icterus (bacteremic jaundice). (J Clin Microbiol 2003;41:3445; Postgrad Med J 2004;80:328.) The differential diagnosis includes other infectious etiologies of bacteremia, other deep space neck infections, endocarditis, and aspiration pneumonia depending on the presentation.
Vascular ultrasound or contrast CT is helpful to diagnose an acute vascular thrombus. The ideal diagnostic imaging modality for distant lesions depends on the anatomic location.
If left untreated, Lemierre's syndrome can result in local erosion through the vascular wall or disseminated infection, DIC, and septic shock. The mortality rate of Lemierre's was reported to be as high as 90 percent before the advent of antibiotics, but is now approximately 20 percent or less. (Laryngoscope 2009;119:1552.) Metronidazole is the drug of choice against Fusobacterium, but mixed pathogens are not uncommon so gram-positive coverage with a beta-lactam agent is recommended. (J Clin Microbiol 2003;41:3445.) Prolonged antibiotic therapy is recommended to eradicate conformed Lemierre's infections. Optimal duration has yet to be defined, but three to six weeks is generally recommended to eliminate the infectious component of the associated fibrin clots, with relapses documented for treatments less than two weeks. (Clin Microbiol Rev 2007;20:622.)
Therapeutic anticoagulation of a thrombus associated with Lemierre's syndrome is controversial, predominantly because the condition is rare and no good blinded control studies have been performed. The risks and benefits must be weighed against the potential complications of incompletely treating septic thrombosis. (Ann Otol Rhinol Laryngol 2008;117:679.) Surgical drainage of focal infected fluid collections may have a role as well. (Am J Otolaryngol 2010;31:38.)
This patient had obliteration of the right internal jugular vein noted to be highly suspicious for thrombophlebitis Lemierre's syndrome on CT scan. She was admitted for intravenous antibiotic treatment. She returned three days after discharge with recurrent neck swelling, and was taken to the operating room for incision and drainage of a deep space neck abscess. She was discharged on three weeks of antibiotics, and has not returned to the ED.
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