Lemierre's syndrome

Lemierre's syndrome is an anaerobic sepsis occurring after oropharyngeal infection in healthy teenagers and young adults. We report two cases of adolescent girls suffering from Lemierre's syndrome studied with cervical color Doppler ultrasonography (CDUS), cervicothoracic helical CT, and high-resolution CT (HRCT) scanning. In both patients, HRCT allowed a good depiction of multiple cavitated pulmonary nodules of various sizes suggestive of this entity and was able to detect small or peripheral nodules. CDUS helped to pinpoint the extent of thromboses of the internal jugular vein demonstrated by CT. CDUS and HRCT should be performed as early as possible to confirm and treat this life-threatening condition. (CHEST 1997; 112:271-73)

Key words: adolescent; color Doppler ultrasonography; high-resolution computed tomography; Lemierre's syndrome

Abbreviations: CDUS=color Doppler ultrasonography; HRCT= high-resolution CT

Lemierre's syndrome is an uncommon life-threatening anaerobic sepsis occurring after oropharyngeal infection characterized by septic thrombophlebitis of the ipsilateral internal jugular vein with subsequent systemic dissemination. The lungs are the most common metastatic target, but other sites of septic dissemination, such as the bone, joints, the liver, the peritoneum, and the kidneys, have been reported in medical literature.[1] We report two cases of teenagers who experienced jugular thrombophlebitis and pulmonary dissemination secondary to Fusobacterium sepsis.

CASE REPORTS

Two girls, 15 and 17 years old, were admitted to the hospital with fever, pain, and swelling of the ipsilateral laterocervical region following pharyngotonsillar inflammation and moderate dyspnea. Laboratory analysis on admission showed a nonspecific inflammatory reaction with leukocytosis, elevated C-reactive protein and erythrocyte sedimentation rate. Blood cultures were positive for Fusobacterium necrophorum in one patient and weakly positive in the other who received broad-spectrum anti-biotherapy before results of blood cultures were obtained. Throat cultures were negative for beta-hemolytic streptococci in both cases. The chest x-ray films taken on admission showed ill-defined nodular densities in the left upper lobe in one patient and bilateral foci of bronchopneumonia in the other patient. Follow-up chest x-ray films disclosed later bilateral partially cavitated nodules in both patients, suggestive of septic emboli. Cervical (Fig 1) and thoracic (Fig 2) helical CT scans were obtained after intravenous administration of contrast medium, followed by 1-mm high-resolution CT (HRCT) thoracic sections at 10-mm intervals. For both patients, HRCT sections gave a precise depiction of multiple bilateral nodules 3 to 10 mm in diameter, the largest being cavitated. Color Doppler ultrasonography (CDUS) of the neck (Fig 3) displayed thrombophlebitis of an internal jugular vein on the right side for the first patient and on the left side for the second one. Both patients were successfully treated with ceftriaxone disodium (Rocephin) intravenously for a period of 3 weeks followed by therapy with amoxicillin with clavulanic acid (Augmentin) orally for another 3 weeks.

[Figure 1-3 ILLUSTRATION OMITTED]

DISCUSSION

Lemierre's syndrome is a tonsillitis or peritonsillar abscess followed, when untreated, by a rapidly progressing septicemia and death.[1] Multisystem involvement of Lemierre's syndrome may suggest meningitis, septic arthritis, and intra-abdominal sepsis. Swelling of the submaxillary glands associated with tenderness of the sternocleidomastoid muscle is caused by thrombophlebitis of an internal jugular vein due to the anaerobic, nonmotile Gram-negative bacillus F necrophorum. The most frequent routes of infection occur through the upper respiratory and genital tracts.[2]

Because the number of patients reported severely declined with the use of the antibiotics, Lemierre's syndrome is denoted as a "forgotten disease." This entity also has been frequently overlooked, and only 39 patients were reported between 1974 and 1987.[3] Five additional reports have been published during the last 6 years.[4-8] Lemierre's syndrome has been reported more frequently in teenagers and young adults but has been observed in infants and children.[7,8]

Chest x-ray films may show diffuse ill-defined infiltrates that appear as round opacities of the lung. These opacities are classically related to a branching vessel and may cavitate. The differential diagnosis of infectious metastatic lesions to the lung is large and includes Wegener's granulomatosis, reactivated tuberculosis, metastases, bronchioalveolar carcinoma, rheumatoid nodules, aspergillosis, and histiocytosis. Involvement of the pleural space, pneumatoceles, and ARDS related to septicemia also have been described.[3]

Septic pulmonary emboli cause a wide spectrum of radiographic abnormalities, ranging from wedge-shaped peripheral densities abutting the pleura to nodular and cavitary lesions. The relationship of the cavitary nodule and the distal end of pulmonary vessels is better seen in 7-or 10-mm helical CT sections, a longer portion of the vessel being within a thick rather than a millimetric section. HRCT sections are a helpful complement to thicker 7- or 10-mm helical CT sections since they characterize the nodules abutting the pleura and the cavitation, as in our patients.

Septic emboli originate from the oropharynx, and the infection extends to the internal parapharyngeal space and internal jugular vein. Therefore, the presence of an internal jugular vein thrombosis, associated with septic emboli in the lung, suggests the diagnosis of Lemierre's syndrome. Although jugular vein thrombosis has been diagnosed by phlebography or scintigraphy, enhanced CT scans and sonography of the neck are presently the imaging modalities of choice.[9] Delineation of additional pathologic features of the neck, such as internal jugular thrombosis or abscess, is another advantage of enhanced CT;[8] however, in our patients, CDUS allowed a clearer demonstration of the intravascular thrombus in a nonionizing way.

Lemierre's syndrome, although rarely reported, remains a viable diagnosis today. Physicians must be aware of it as a possibility for diagnosis. CDUS and HRCT should be performed as early as possible to confirm and treat this life-threatening condition.

REFERENCES

[1] Lemierre A. On certain septicemias due to anaerobic organisms. Lancet 1936; 40:701-03

[2] Gorbach SL, Bartlett JG. Anaerobic infections (second of three parts). N Engl J Med 1974; 290:1237-45

[3] Moreno S, Altozane JG, Pinilla B, et al. Lemierre's disease: postanginal bacteremia and puhnonary involvement caused by Fusobacterium necrophorum. Rev Infect Dis 1989; 11: 319-24

[4] Sinave CP, Hardy GJ, Fardy PW. The Lemierre syndrome: suppurative thrombophlebitis secondary to oropharyngeal infection. Medicine 1989; 25:85-94

[5] Francois P, Plosse M, Frappat P, et al. Septicemic h Fuso bacterium necrophorum chez un nourrisson. Arch Fr Pediatr 1989; 46:589-90

[6] Gonzales-Gay MA, Sanchez-Audrade A, Cereijo MJ, et al. Pyomyositis and septic arthritis from Fusobacterium nucleatum in a nonimmunocompromised adult. J Rheumatol 1993; 20:518-20

[7] Hugues CE, Spear RK, Shinabarger CE, et al. Septic pulmonary emboli complicating mastoiditis: Lemierre syndrome revisited. Clin Infect Dis 1994; 18:633-35

[8] Goyol M, Sharma R, Jain J, et al. Unusual radiological manifestations of Lemierre's syndrome: a case report. Pediatr Radiol 1995; 25:S105-06

[9] Goldhagen J, Alford BA, Prewitt LH, et al. Suppurative thrombophlebitis of the internal jugular vein: report of three cases and review of the pediatric literature. Pediatr Infect Dis J 1988; 7:410-14

(*) From the Department of Radiology, University Hospital, CHUV, Lausanne, Switzerland.

Manuscript received July 18, 1996; revision accepted December 3.

Reprint requests: Dr. Pierre Schnyder, Department of Radiology--BH07, CHUV, CH-1011 Lausanne, Switzerland

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