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Infectious Diseases in Clinical Practice:
doi: 10.1097/IPC.0b013e31802dc515
Review Articles

Lemierre Syndrome: Forgotten but Not Extinct

Dalal, Aman MD; Acharji, Subasit MBBS; Gehman, Michael DO

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Guthrie Healthcare System, Sayre, PA.

Address correspondence and reprint requests to Aman Dalal, MD, 1 Guthrie Sq, Sayre, PA 18840. E-mail: amandalal@hotmail.com.

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Abstract

Lemierre syndrome is an uncommon but potentially lethal complication of oropharyngeal infections. Early recognition and aggressive antibiotic therapy are crucial elements in reducing mortality from this condition. Of late, its incidence seems to be increasing; hence, clinicians need to be aware of this syndrome. Here, we report the case of a 19-year-old man who presented with fever and respiratory distress 2 weeks after dental extraction that was concordant with that of Lemierre syndrome.

In the preantibiotic era, infectious diseases were often associated with fatal complications. In 1936, Lemierre described a syndrome characterized by disseminated abscesses and thrombophlebitis of the internal jugular vein after an infection of the oropharynx. We describe the case of a 19-year-old man who presented to an academic center with fever and septic pulmonary emboli related to Lemierre syndrome.

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CASE REPORT

A 19-year-old man was evaluated at local emergency department for pharyngitis, for which the results of the rapid streptococcal screen, throat culture, and Monospot tests were negative. He was diagnosed as having upper respiratory tract infection and was discharged on erythromycin. He had dental extraction 2 weeks before the onset of his symptoms. His pharyngitis improved for the next 2 days, but he developed chills and experienced lethargy. He was reevaluated at the same emergency department; at this time, physical examination revealed bilateral crackles, prompting a chest radiograph which showed significant bilateral infiltrates, more pronounced on the right side, which prompted referral to our institution for further management.

He presented to our facility with acute respiratory distress, high-grade fever with chills, and pleuritic chest pain. Upon examination, he was febrile at 104°F and tachypneic at 32 breaths per minute, with severe tachycardia at 152 beats per minute; blood pressure was 90/66 mm Hg. He was hypoxic even on high-flow supplemental oxygen using a nonrebreather mask. Physical examination was remarkable for bilateral diffuse crackles. There was no pharyngeal erythema, exudates, or rash. Blood work revealed white blood cell count of 18.6 × 109/L, with significant bandemia. Repeat chest radiograph showed worsening of infiltrates. After blood cultures were drawn, the patient's respiratory status worsened further, and he was intubated. He was started on intravenous ceftriaxone 1 g every 24 hours and azithromycin 500 mg every 24 hours to cover the possibility of community-acquired pneumonia. He subsequently became hypotensive, requiring vasopressor support.

Blood cultures were reported to be positive for gram-positive rods on the next day; hence, antibiotics were changed to moxifloxacin 400 mg every 24 hours and clindamycin 600 mg every 6 hours. Despite the change in the antibiotics, patient's respiratory status deteriorated. Chest radiograph revealed left pneumothorax requiring chest tube placement. Computed tomography (CT) of the thorax done on the same day showed additional findings including multiple bilateral cavitary lesions. The CT of the neck and soft tissue revealed right internal jugular vein thrombosis. Duplex ultrasound of the left lower extremity did not show any evidence of deep venous thrombosis (Fig. 1).

Figure 1
Figure 1
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The patient showed improvement with these intravenous antibiotics. After 4 days, his blood culture isolate was identified as Fusobacterium necrophorum, confirming our diagnosis of Lemierre syndrome. The antibiotics were continued for a total of 42 days, and the patient had a slow but complete recovery.

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DISCUSSION

Patients with Lemierre syndrome commonly present with sepsis after apparent resolution of an acute pharyngitis.1 Although pharyngitis (often viral) is the most common antecedent oropharyngeal infection,2 other infections such as peritonsillar abscesses and otitis media have also been implicated. The key factor in the progression of the disease from an oropharyngeal infection to systemic septic emboli is the invasion of deep neck tissues by anaerobic oral pathogens.1,3,4,5 A resultant septic thrombophlebitis of the internal jugular vein acts as a seeding point for septic thromboemboli. The most common sites of metastasis are the lungs.1,2,3,5 However, meningitis; osteomyelitis; septic arthritis of the ankle, hips, and shoulders; and abscesses of the spleen, paravertebral muscles, thigh, and skin have been reported, suggesting aggressive systemic dissemination.6 Rarely, this syndrome is associated with headache,4 carotid thrombosis,5 and mediastinitis secondary to extension along the carotid sheath.7

Lemierre syndrome is diagnosed by the finding of a characteristic oropharyngeal infection, bacteremia confirmed by at least 1 positive blood culture, clinical or radiographic evidence of internal jugular thrombophlebitis, and demonstrated septic emboli.6 The characteristic pathogen is F. necrophorum, an oropharyngeal anaerobe. With enriched anaerobic media, cultures are typically positive in 1 to 3 days.8 In the largest published series to date, Sinave et al6 identified 36 patients who met strict diagnostic criteria for Lemierre syndrome and found 22 (61%) with definite F. necrophorum bacteremia, 8 (22%) with probable F. necrophorum bacteremia, and 6 (17%) with other organisms, including Streptococcus viridans, Bacteroides clostridiiformis, Peptostreptococcus magnus, Bacteroides species, Peptostreptococcus species, Fusobacterium naviforme, and Eikenella corrodens. It is possible that these cases reflect difficulty growing this strict anaerobe; it is also possible that multiple pathogens may be responsible for Lemierre syndrome. In fact, one third of the patients with Lemierre syndrome have a polymicrobial bacteremia.9 Without early detection and treatment, morbidity and mortality rates are high. Although magnetic resonance imaging has been used, recent literature suggests that color Doppler ultrasonography and high-resolution CT play the most important roles in identifying jugular venous thrombosis and pulmonary metastases, respectively.10 Early in the disease process, computed tomographic studies of the neck may be false negative.11 Serial imaging studies may be necessary, and ultrasound is now recommended as the initial modality to evaluate for internal jugular thrombosis.12,13

Early diagnosis and treatment are traditionally limited to a high clinical index of suspicion, early empirical antibiotic administration, and subsequent imaging; however, clinicians skilled in ultrasound evaluation may be able to improve diagnostic performance and rapidly confirm Lemierre syndrome by identifying internal jugular thrombosis on rapid emergency department scans.14 Initial treatment involves high-dose intravenous penicillin and metronidazole or intravenous clindamycin monotherapy, with conversion to 2 to 6 weeks of oral therapy after the patient's clinical condition has stabilized.8 Heparin is given only in cases where retrograde propagation of thrombosis to the cavernous sinus has occurred because heparin may actually disseminate the infection.15 Standard supportive care for respiratory and hemodynamic compromise is important. Although almost universally fatal in Lemierre's era,1 earlier detection through diagnostic imaging, aggressive use of intravenous antibiotics, and improved critical care modalities have reduced the mortality of this syndrome to approximately 8%.16 There are some suggestions16-18 that the incidence of Lemierre syndrome is increasing because clinicians are becoming less liberal in their use of antibiotics for upper respiratory tract infections. If this is true, prudent clinicians should be increasingly alert for signs of jugular thrombosis and metastatic septic disease after acute oropharyngeal infections.

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CONCLUSIONS

Lemierre syndrome, although uncommon, may sometimes occur as a fatal complication of oropharyngeal infections. Early recognition of this condition and aggressive antibiotic therapy are pivotal in reducing mortality from this potentially lethal syndrome.

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REFERENCES

3. Vandenberg SJ, Hartig GK. Lemierre's syndrome. Otolaryngol Head Neck Surg. 1998;119:516-518.

4. Gwaltney JM Jr, Bisno AL. Pharyngitis. In: Mandell GL, Bennett JE, Dolin R, eds. Principles and Practice of Infectious Diseases. 5th ed. Philadelphia, PA: Churchill Livingston; 2000:658.

1. Lemierre A. On certain septicemias due to anaerobic organisms. Lancet. 1936;1:701-703.

5. Tan NC, Tan DY, Tan LC. An unusual headache: Lemierre's syndrome. J Neurol. 2003;250:245-246.

6. Maalikjy Akkawi N, Borroni B, Magoni M, et al. Lemierre's syndrome complicated by carotid thrombosis. Neurol Sci. 2001;22:403-404.

2. Sinave CP, Hardy GJ, Fardy PW. The Lemierre syndrome: suppurative thrombophlebitis of the internal jugular vein secondary to oropharyngeal infection. Medicine. 1989;68:85-94.

7. McLean AS, Tyler K. Cardiac tamponade in a postpartum woman with Lemierre's syndrome. Anaesth Intensive Care. 1998;26:582-583.

8. Hagelskjaer Kristnesen L, Prag J. Human necrobacillosis, with emphasis on Lemierre's syndrome. Clin Infect Dis. 2000;31:524-532.

9. Hagelskjaer LH, Prag J, Malczynski J, et al. Incidence and clinical epidemiology in necrobacillosis, including Lemierre's syndrome, in Denmark 1990-1995. Eur J Clin Microbiol Infect Dis. 1998;17:561-565.

10. Gudinchet F, Maeder P, Neveceral P, et al. Lemierre's syndrome in children: high-resolution CT and color Doppler sonography patterns. Chest. 1997;112:271-273.

11. Fiesseler FW. Pharyngitis followed by hypoxia and sepsis: Lemierre syndrome. Am J Emerg Med. 2001;19:320-322.

12. De Lima JE Jr, Levin M. Lemierre's syndrome: post-anginal septicemia. Pediatr Radiol. 2003;33:281-283.

13. Albertyn LE, Alcock MK. Diagnosis of internal jugular vein thrombosis. Radiology. 1987;162:505-508.

14. Lustig LR, Cusick BC, Cheung SW, et al. Lemierre's syndrome: two cases of postanginal sepsis. Otolaryngol Head Neck Surg. 1995;112:767-772.

15. Lee BK, Lopez F, Genovese M, et al. Lemierre's syndrome. South Med J. 1997;90:640-643.

16. Smith SA. Respiratory failure as a complication of pharyngitis: Lemierre's syndrome. Pediatr Emerg Care. 1999;15:402-403.

17. Moore BA, Deckle C, Werkhaven J, et al. Bilateral Lemierre's syndrome: a case report and literature review. Ear Nose Throat J. 2002;81:234-236, 238-240, 242.

18. Jane R. Critical care nurses be aware: Lemierre's syndrome is on the rise. Aust Crit Care. 16:2003;126-132.

© 2007 Lippincott Williams & Wilkins, Inc.