Severe neck pain in a very sick patient
Mrs. J was 67 years old, with chief complaints of fever and neck pain. On questioning, she said that these symptoms had begun about two weeks earlier, preceded by a mild sore throat that lasted two or three days. She denied cough, shortness of breath, or dysphagia and had no complaints regarding her teeth or gums. The symptom bothering her most was the neck pain and swelling.
I was surprised to learn that during the entire two weeks of illness, Mrs. J had not sought medical advice and was self-treating with ibuprofen. Previously in excellent health, she had been hospitalized only once, 10 years earlier, to have a hysterectomy for vaginal bleeding. She was not on any regular medications. Though her husband was a chain smoker, she had never smoked and drank only on social occasions. She had retired after working for a multinational company for more than 30 years.
Mrs. J looked very sick. Her temperature was 104°F, pulse 130 beats per minute, and blood pressure 140/95 mm Hg. Several anterior cervical and submandibular lymph nodes on the right side of her neck were enlarged and painful. There was no lymphadenopathy on the left side. Her throat was slightly congested, and her mouth was dry, with no evidence of dental abscess. Heart and lung examinations were normal. On her abdomen, there was a well-healed scar.
Laboratory results showed a WBC count of 20,000/mL, with 90 percent neutrophils; a hemoglobin of 12 g/dL; and hematocrit of 35 percent. Serum electrolytes were normal. A chest x-ray was unremarkable. She had been started on IV clindamycin 600 mg every eight hours, a recommendation with which I agreed.
The next day, the ear, nose, and throat consultant recommended a contrast-enhanced CT scan of Mrs. J's neck. The scan showed a thrombosed right internal jugular vein.
The patient's history, physical, and radiographic findings were consistent with jugular vein septic thrombophlebitis, or Lemierre's syndrome. The syndrome is usually caused by a fusobacterium; however, other anaerobic bacteria can also be causative agents. Symptoms include fever; neck pain; trismus; torticollis; and dysphagia or dysarthria, ascribable to involvement of hypoglossal, vagus, or accessory nerves.
Most Lemierre's syndrome occurs in healthy young adults as a predilection with an unknown cause. A few cases have been reported as complications of acute infectious mononucleosis in patients who are taking prednisone. The initiating event is usually a localized infection in an area drained by large cervical veins, followed by development of thrombophlebitis.
In some patients, the primary focus of infection, however, may not be identified. Jugular vein septic thrombophlebitis is sometimes accompanied by metastatic spread of septic thrombi to the lungs, causing cavitating lesions, and to other organs. Chest radiography may lead to the diagnosis of pneumonia and infective endocarditis. High-resolution ultrasound examination or contrast-enhanced CT scan of the neck that demonstrates thrombosis of the jugular vein is necessary to make the diagnosis. Ultrasound is cheaper, safer, easier to perform, and probably more sensitive than CT scan. Radionuclide scans with gallium have also been used to localize the infection to the internal jugular vein. In a minority of patients, the thrombosed vein may be palpable during the physical examination.
Fusobacteria are gram-negative anaerobic rods that normally inhabit the mouth, alimentary canal, and female genital tract. Though they are the second most frequently isolated anaerobic bacteria in blood cultures, the reason for the high prevalence of these organisms as the causative agents of jugular vein septic thrombophlebitis is not known. The production of heparinase and other proteolytic enzymes by these bacteria probably aids in the invasion of regional veins.
Lemierre's syndrome can be treated by a variety of antibiotics with activity against fusobacteria and other anaerobes. Penicillins, tetracyclines, and clindamycin are all suitable choices. Cephalosporins, commonly used to treat a variety of infections, are not effective. Surgical excision of the jugular vein is rarely required today. Heparin is sometimes used in the early management of Lemierre's syndrome and may lead to faster resolution of symptoms.
Mrs. J did well on clindamycin and defervesced on the third hospital day. On the fourth hospital day, her blood culture grew fusobacteria and Streptococcus intermedius. Isolation of the latter bacterium was unusual and not previously reported. She was discharged on a 10-day course of ampicillin/clavulanic acid.
Dr. Vaghjimal is currently in private practice at Medical Associates of West Alabama in Tuscaloosa.
Lemierre A. On certain septicemias due to anaerobic organisms. Lancet 2:701, 1936.
Goldhagen J, Alford BA, Prewitt LH, et al. Suppurative thrombophlebitis of the internal jugular vein: report of three cases and review of pediatric literature. Pediatr Infect Dis J 7:410, 1988.
Gradon JD. Space-occupying and life-threatening infections of the head, neck, and thorax. Infect Dis Clin North Am 10:857, 1996.