Lemierre Syndrome

Lemierre syndrome (or suppurative thrombophlebitis of the internal jugular vein) was originally described as a complication of postanginal sepsis (56-60). Lemierre, in 1936, wrote a comprehensive article on the subject and called this syndrome "postanginal septicemia" (59).

This syndrome is a rare but severe life-threatening complication of oral infections, particularly those resulting in lateral pharyngeal space infection. It is characterized as thrombosis and suppurative thrombophlebitis of the internal jugular vein that is associated with spread of septic emboli to the lungs and other sites. Before the availability of antimicrobial agents, death was the common result, unless patients were treated with surgical ligation of the vein (57,58).

Fusobacterium is the predominant genus and Fusobacterium necrophorum is the most prevalent species. Other Fusobacteria include F. nucleatum, Fusobacterium gonidiaforum and Fusobacterium varium. Other isolates recovered alone or in combination include pigmented Prevotella, Bacteroides and Peptostreptococcus spp. (61-63).

The source of the infection is pharyngitis, exudative tonsillitis, peritonsillar abscess or oral procedure (i.e., tonsillectomy), which precedes the onset of septicemia. The initiating event is generally a localized infection in an area drained by the large cervical veins. Thereafter, the infection quickly progresses to cause a pathognomic triad of findings: (i) local symptoms of neck pain, torticollis, trismus, dysphagia or dysarthria ascribable to involvement of the hypoglossal, glossopharyngeal, vagus or accessory nerves; (ii) development of thrombophlebitis; (iii) embolic infection of the lungs, viscera, joints or brain, or direct extension of the infection to the internal ear, middle ear or mastoid. Death can occur as a result of the erosion of a blood vessel wall with rupture into the mediastinum, ear, or crania vault (60).

Most patients with Lemierre's syndrome are older than 10 years (62). The patients look toxic and manifest fever, sore throat, cough neck, pain, dyspnea, and arthralgia. Palpable jugular arch can be detected in about 20% of patients. Swelling and tenderness at the angle of the jaw and along the sternocleidomastoid muscle with signs of severe sepsis along with evidence of pleuropulmonary emboli, is very suggestive of thrombophlebitis of the internal jugular vein (61).

Pulmonary emboli are found in most untreated patients, as most present with pleuritic pain. Empyema is however rare. Seeding of other body sites occurs, mostly to the joints. Other potential sites that are involved are the liver causing "bacteremic jaundice" (64). Chest x-ray is indicated.

High resolution ultrasonography can confirm the diagnosis of suppurative thrombophlebitis (65). CT can also demonstrate intravascular thrombus; however, it is more expensive, produces higher morbidity because of intravascular contrast agents and is probably less sensitive than high resolution ultrasonography for identifying small mural thrombi (65-67). Radionuclide gallium scans can localize the source of the original infection in the internal jugular vein (68). However, inability to document a thrombus should not delay initiation of appropriate antibiotic therapy for anaerobic sepsis.

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