Lemierre's syndrome

A 20-year-old Vietnamese man was admitted to the hospital with fever and pharyngitis. He had been in excellent health until 5 days prior to admission. His illness began with a sore throat, accompanied by fever, chills, arthralgia, and headache. He was seen in the Emergency Department a day prior to admission and was treated with intramuscularly administered penicillin for presumed streptococcal pharyngitis, but symptoms persisted. The patient had been in the United States for 5 years. There was no history of recent travel or of rheumatic fever. The patient said that he had been sexually promiscuous, but denied intravenous drug use.

Physical Examination

The patient appeared ill. Vital signs included a temperature of 40[degrees]C; heart rate, 110 bpm; respiratory rate, 22 breaths per min; and BP, 90/65 mm Hg. Examination of the ears, nose, and throat showed pharyngeal erythema and bilateral exudative tonsillitis. The neck was supple with no lymphadenopathy. Cardiac exam revealed tachycardia. The lungs were clear. Examination of the abdomen, extremities, and neurologic system revealed no abnormalities.

Laboratory Examination

Laboratory values were as follows: WBC, 17,000/ [mm.sup.3] (84% polymorphonuclear leukocytes and 7% lymphocytes); hemoglobin, 12.3 g/dL; and platelet count, 21,000/[mm.sup.3]. A peripheral blood smear showed toxic granulations, hypochromia, and thrombocytopenia. Electrolyte values and results of liver and renal function tests were within normal limits. A disseminated intravascular coagulation panel showed no abnormalities. Arterial blood gas values (patient breathing room air) were pH, 7.44; [PCO.sub.2], 32 mm Hg; and [PO.sub.2], 67 mm Hg. A chest x-ray film is shown in Figure 1.

[Figure 1 ILLUSTRATION OMITTED]

What is the diagnosis?

How would you proceed with therapy?

Diagnosis: Lemierre's syndrome--postanginal sepsis due to anaerobic oropharyngeal infection or necrobacillosis with septic thrombophlebitis of the internal jugular vein and multiple pulmonary emboli.

Lemierre's syndrome is a rare form of postanginal sepsis usually caused by Fusobacterium infection. It was first described in detail by Lemierre in 1936. It is characterized by acute oropharyngeal infection, suppurative thrombophlebitis of the internal jugular vein, sepsis, and metastatic complications, the most common being septic pulmonary emboli. It may be confused with right-sided endocarditis.

Lemierre's syndrome was a fatal suppurative disease before the advent of antibiotics. Although tonsils are the usual primary source of infection, cases resulting from otitis, parotitis, and mastoiditis also have been described. ARDS has been reported as a complication of this syndrome. Metastatic complications, besides septic pulmonary emboli, include empyema, pericarditis, septic arthritis, liver abscess, and meningitis. Thrombophlebitis of the internal jugular vein, the hallmark of this syndrome, may be clinically occult or present with neck pain, swelling, and the "cord sign" (palpable induration of the internal jugular vein). Spread of infection may occur via peritonsillar veins, the jugular lymphatic system, or by direct spread. A CT scan of the neck with contrast medium is the most appropriate study, although ultrasound has been suggested.

F necrophorum is the pathogen in the majority of reported cases. It is a long, spindle-shaped, Gramnegative anaerobic bacillus. It has the ability to be an invasive pathogen in otherwise healthy subjects. Other organisms may be involved as well.

Treatment depends on the severity of infection. Intravenously administered antibiotics directed at anaerobic microbes (ie, clindamycin, penicillin, ampicillin-sulbactam, metronidazole, ticarcillin-clavulanate) are effective in patients without metastatic infections. If signs of sepsis persist with propagation of infection, ligation or excision of the internal jugular vein is required. There is no documented role for the use of anticoagulation therapy.

The present patient was initially treated with cefuroxime for tonsillitis and bacterial pneumonia but continued to have fever. On hospital day 2, the antibiotic therapy was changed to penicillin and metronidazole. Blood cultures were suggestive of anaerobic bacteria, but identification was pending. On hospital day 3, crackles were noticed at the time of lung examination, and the patient was transferred to the ICU. A chest radiograph showed bilateral nodular infiltrates of varying sizes that were consistent with septic emboli (Fig 1). Therapy with ampicillin-sulbactam was initiated. Although physical examination of the neck revealed no abnormalities, a CT scan (Fig 2) showed thrombosis of the internal jugular vein on the right side. The patient underwent an emergency ligation of the right internal jugular vein. Postoperatively, he continued to have fever despite antibiotic therapy, but gradually showed clinical improvement with resolution of symptoms and septic emboli. The platelet count improved with therapy. The organism growing in the blood cultures was identified as F necrophorum.

[Figure 1 ILLUSTRATION OMITTED]

CLINICAL PEARLS

1. Lemierre's syndrome is characterized by suppurative thrombophlebitis of the internal jugular vein associated with oropharyngeal infection, the most common being tonsillitis.

2. Usually it is caused by anaerobic infections, especially F necrophorum ("necrobacillosis").

3. It is commonly associated with septic pulmonary emboli, occasionally with distant abscesses.

4. A CT scan of the neck with contrast medium is the imaging modality of choice.

5. Treatment consists of IV administration of high-dose antibiotics that have anaerobic activity.

6. Persistent sepsis and emboli despite the use of antibiotic therapy is an indication for ligation or excision of the internal jugular vein.

SUGGESTED READINGS

Cosgrove EF, Coldny SM, Pesce RR. Adult respiratory distress syndrome as a complication of postanginal sepsis. Chest 1993; 103:1628-29

Hughes CE, Spear RK, Shinabarger CE, et al. Septic pulmonary emboli complicating mastoiditis: Lemierre's syndrome revisited. Clin Infect Dis 1994; 18:633-35

Lemierre A. On certain septicaemias due to anaerobic organisms. Lancet 1936; 1:701-03

Lustig LR, Cusick BC, Cheung SW, et al. Lemierre's syndrome: two cases of postanginal sepsis. Otolaryngol Head Neck Surg 1995; 112:767-72

Moore-Gillon J, Lee TH, Eykyn SJ, et al. Necrobacillosis: a forgotten disease. BMJ 1984; 288:1526-27

Moreno S, Altozano JG, Pinilla B, et al. Lemierre's disease: postanginal bacteremia and pulmonary involvement caused by Fusobacterium necrophorum. Rev Infect Dis 1989; 2:319-24

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

Tovi F, Fliss DM, Noyek AM. Septic internal jugular vein thrombosis, J Otolaryngol 1993; 22(6):415-20

Vogel LC, Boyer KM. Metastatic complications of Fusobacterium necrophorum sepsis. AJDC 1980; 134:356-58

Weesner CL, Cisek JE. Lemierre syndrome: the forgotten disease. Ann Emerg Med 1993; 22:256-58

(*) From the Mount Sinai Services, Elmhurst Hospital Center, Elmhurst, NY.

Manuscript received June 20, 1996; revision accepted September 17.

Reprint requests: Dr. Venkataraman, Division of Pulmonary Medicine, Mount Sinai Services--Elmhurst Hospital Center--E6-27A, 79-01 Broadway, Elmhurst, NY 11373

COPYRIGHT 1997 American College of Chest Physicians
COPYRIGHT 2004 Gale Group