lemierre syndrome is septic thrombophlebitis

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This patient has Lemierre syndrome, also termed postanginal sepsis or septic thrombophlebitis of the internal jugular vein. Proper management requires radiologic visualization of the source of the infection in the neck and antibiotics including coverage for mouth anaerobes.

Lemierre syndrome is a relatively rare infectious complication of pharyngitis, seen most often in otherwise healthy young adults. It is usually caused by species of normal mouth flora, with the most common pathogen isolated being the anaerobe Fusobacterium necrophorum. This infection usually follows a case of typical pharyngitis with a lag time of 1 to 3 weeks.  Common clinical findings are antecedent throat pain, fever, rigors, dysphagia, and trismus.  When septic embolization occurs, patients often note cough, dyspnea, pleurisy, and hemoptysis. In as many as one-half of patients, throat and neck symptoms and findings may largely resolve at the time pulmonary manifestations occur. Lung findings seen radiologically are most commonly multiple nodules that may cavitate. Empyema, lung abscesses, necrotizing mediastinitis, and pneumothoraces may also be seen. CT scan in this patient revealed abundant nodules with an area of pleural reaction correlating to his reported left-sided chest pain. After pulmonary involvement, joints are the next most common sites of metastatic infection. Less common sites of involvement are bone, liver, spleen, cardiac valves, and CNS.

Imaging of the neck allows confirmation of the diagnosis. In rare cases where infection extends into soft tissues of the neck or infection spreads to the mediastinum, surgery may be required. In this patient, involvement of the left internal jugular vein was confirmed by CT scan (Figure 2, arrow). Ultrasound has also been used to assess blood flow in the neck in this context. Empiric antibiotic therapy should be started while throat and blood cultures are pending to guide specific antibiotic treatment.  Recommended regimens include piperacillin-tazobactam; carbapenem (eg, imipenem, meropenem, or ertapenem); or ceftriaxone plus metronidazole. Ceftriaxone alone would not be sufficient because of inadequate anaerobic coverage by this agent. Most experts recommend a prolonged antibiotic course of 4 weeks, with 2 weeks of initial parenteral therapy. Blood cultures and imaging of involved sites should be used to confirm resolution. Anticoagulation is not usually recommended. 

Other infections and some malignancies may give a radiologic appearance of multiple nodules arising from hematogenous spread. Staphylococcal septic embolization can occur from endocarditis or from indwelling catheters; however, this patient did not fit this picture, and endocarditis was adequately excluded by transthoracic echocardiogram. Performing a transesophageal echocardiogram and adding vancomycin would not be warranted. On rare occasions, vancomycin will be needed for treatment of Lemierre syndrome if culture data so guides therapy. Fungal infection would be inconsistent with this patient’s history. Although thyroid malignancy can metastasize to lung(s) and manifest as multiple nodules, this patient has no risk factors for thyroid cancer, and the clinical presentation strongly suggests underlying infection. Thyroid function tests alone would not assist greatly in making a diagnosis of metastatic thyroid cancer, although thyroglobulin levels are helpful in determining if ablation therapy has been successful in the treatment of this cancer.

The patient has Lemierre syndrome, characterized by septic thrombophlebitis of the internal jugular vein. Lemierre syndrome is commonly associated with an oropharyngeal source of infection and seeding of the internal jugular vein with inflammation of surrounding tissue. This syndrome is also associated with signs of septic emboli, such as the cavitary lung nodule in this patient seen in Figure 1. Fusobacterium species are the bacteria most commonly implicated in this syndrome, but other oropharyngeal bacteria can also be causative agents; the current patient had blood cultures persistently positive for Parvimonas micra.

In addition to septic emboli in the lungs, Lemierre syndrome can also be complicated by hematogenous, arterial spread of infection to large joints, skin, skeletal muscle, liver, and kidneys. Central nervous system involvement can also occur, including meningitis, brain abscess, stroke, and dural empyema. Occasionally, retrograde venous extension of the internal jugular vein thrombus to the cerebral venous system can also occur and cause conditions such as cavernous sinus thrombosis.

Cerebral venous thrombosis most commonly manifests as a gradual onset of a diffuse headache and is more common in women, with a median age of 37 years. The most common causes of cerebral venous thrombosis include pregnancy, oral contraceptives, prothrombotic conditions, obesity, malignancy, trauma, and infection. In cavernous sinus thrombosis, a specific type of cerebral venous thrombosis, ocular signs are common, in addition to headache, including orbital pain and proptosis.

Venography of the head, either with magnetic resonance venography or CT venography, is the imaging test of choice to diagnose cerebral venous thrombosis. CT scanning cerebral perfusion studies and transcranial Doppler of the head may not reveal any abnormalities in this condition characterized by venous obstruction. Noncontrast-enhanced CT scans of the head are normal in 30% of patients, and, if abnormalities are seen, these would not necessarily be specific to cerebral venous sinus thrombosis.

The current patient underwent magnetic resonance venography that showed venous obstruction of the right cavernous sinus and right superior and inferior ophthalmic veins. Mechanical thrombectomy of the right cavernous sinus was performed with removal of purulent material (Figure 2), which on culture also grew P micra. Over the subsequent days, the patient’s proptosis and loss of visual acuity resolved.123456

A 30-year-old woman presents with 1 week of a headache and acute vision loss in her right eye. The headache has been constant over the past week and described as dull and diffuse. Over the past 2 days, she has developed slowly progressive proptosis of her right eye, with a new loss of visual acuity over the past 24 h. She has no medical history and is not taking any medications. Physical examination results are notable for proptosis of the right eye with decreased visual acuity in this eye and poor dentition.

Because of a concern for possible ischemic stroke, she was admitted to the ICU. Results of noncontrast-enhanced CT scanning of the head are normal. CT angiography of the head and neck revealed a right internal jugular vein thrombus with inflammation in the surrounding tissue and lymphadenopathy and an incidentally seen nodule in the right upper lobe of the lung. Follow-up CT scans of the chest showed multiple cavitary lung nodules.

Which of the following diagnostic tests would most likely explain the patient’s presenting signs and symptoms?

Footnotes

  1. SEEK Questionnaires ↩

  2. Armstrong AW, Spooner K, Sanders JW. Lemierre’s syndrome. Curr Infect Dis Rep. 2000;2(2):168-173. PubMed ↩

  3. Dai YL, Chen VM, Hedges TR 3rd, et al. Lemierre syndrome associated mycotic cavernous sinus thrombosis and carotid aneurysm after COVID-19. Am J Ophthalmol Case Rep. 2022;27:101642. PubMed ↩

  4. Ferro JM, Canhão P, Stam J, et al; ISCVT Investigators. Prognosis of cerebral vein and dural sinus thrombosis: results of the International Study on Cerebral Vein and Dural Sinus Thrombosis (ISCVT). Stroke. 2004;35(3):664-670. PubMed ↩

  5. Golpe R, Marín B, Alonso M. Lemierre’s syndrome (necrobacillosis). Postgrad Med J. 1999;75(881):141-144. PubMed ↩

  6. Sakaida H, Kobayashi M, Ito A, et al. Cavernous sinus thrombosis: linking a swollen red eye and headache. Lancet. 2014;384(9946):928. PubMed ↩