Lemierre's Syndrome.

ABSTRACT: We describe a 32-year-old woman who had temperatures increasing over 7 days from l00.7 [degrees]F to 104.0[degrees]F. She had an upper respiratory infection and swelling of the left side of the neck. She was diagnosed with Lemierre's syndrome.

LEMIERRE'S SYNDROME (postanginal septicemia) was first described in 1936. The "angina" in this syndrome refers to an acute pharyngeal infection with the anaerobe Fusobacterium necrophorum. The acute pharyngitis is followed by a septic thrombophiebitis of the internal jugular vein and dissemination of the infection to multiple sites distant from the pharynx. In the preantibiotic era, Lemierre's syndrome was often fatal. [1]

CASE REPORT

A 32-year-old woman had a an upper respiratory tract infection, with temperatures increasing over 7 days from 100.7[degrees] to 104.0[degrees]F. By the fifth day, the left side of the neck was swollen. Duplex ultrasonography on day 6 showed left internal jugular vein thrombosis. The white blood cell count was 18,000/[mm.sup.3], and blood culture grew F necrophorum. After antibiotics, resection of the left internal jugular vein, and excision of several infected lymph nodes, the patient recovered uneventfully.

DISCUSSION

Lemierre's syndrome generally occurs in healthy adolescents and young adults. [2,3] The infection usually begins with a sore throat, followed by fever, septicemia, thrombosis, and metastatic abscesses. Purulent thrombophiebitis of the internal jugular vein can lead to pulmonary and other distant emboli. Septic arthritis can lead to osteomyelitis.

Fusobacterium necrophorum is a nonmotile, sporulating gram-negative anaerobe occurring in the nonnal flora of the pharynx, gastrointestinal tract, and female genital tract. It can become pathogenic, probably because of its tox ins. [4] These bacteria produce a lipopolysaccharide endotoxin with strong biologic activity, as well as a leukocidin and hemolysin, assisting in destruction of white and red blood cells.[24] Hemagglutinin production augments the fulminant nature of the disease, causing platelet aggregation and septic thrombus formation. [2]

When pharyngitis due to F necrophorum occurs, the physical proximity of the vessels in the lateral pharyngeal space permits extension from the peritonsillar space to the internal jugular vein. This usually occurs in less than a week from the development of pharyngitis. Once internal jugular vein septic thrombosis occurs, distant dissemination is common.

The first sign of Lemierre's syndrome is usually a persistent fever, followed by acute pharyngitis and then sepsis. Next, neck tenderness or swelling develops. Contrast computed tomography of the neck provides the definitive diagnosis, showing distended veins with enhancing walls, intraluminal filling defects, and swelling of adjacent soft tissues. [1,2'4] Ultrasonography can also confirm internal jugular vein thrombosis, showing localized echogenic regions within a dilated vessel. [4,5] Confirmation of Lemierre's syndrome is provided by demonstration of Enecruphorum on blood culture. [2]

Treatment of Lemierre's syndrome is high-dose parenteral antibiotics directed against anaerobes (clindamycin, metronidazole, chloramphenicol, imipenem, or cefodizime). [2] Prolonged therapy is recommended because of the endovascular nature of the infection. [2-5] Ligation or excision of the internal jugular vein is frequently required, and drainage of other abscesses may be necessary. [5]

Although rare, Lemierre's syndrome is potentially fatal. Early diagnosis and treatment are usually curative.

From the Department of Surgery, Mount Sinai Medical Center, New York, NY. (Anu Singhal is a 3rd-year medical student at Mount Sinai Medical School.)

References

(1.) Goyal M, Sharma R, Jain Y, et al: Unusual radiological manifestations of Lemierre's syndrome: a case report. Pediatr Radial 1995; 15(suppl 1):S15-S106

(2.) Stahlman GC, DeBoer DK, Green NE: Fusobacterium osteomyelitis and pyarthrosis: a classic case of Lemierre's syndrome. J Pediatr Orthop 1996; 16:529-532

(3.) Satyanarayana S, White RL: Fusobacterial infections. Dalhousie Medical Journal, May 1999. Accessed August 20, 2001. Available at http://www.medicine.dal.ca/medjourn/DMJONLIN/spring99/orig6.htm.

(4.) Golpe R, Marin B, Alonso M: Lemierre's syndrome (necro-bacillosis). Postgrad Med J 1999; 75:141-144

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

KEY POINTS

* A pharyngeal infection grew Fusobacterium necrophorum.

* Extension of the pharyngeal infection to the internal jugular vein resulted in septic thrombophiebitis.

* Contrast computed tomography confirmed the diagnosis.

* Specific antibiotic therapy and excision of the internal jugular vein is necessary.