The Johns Hopkins Microbiology Newsletter

DEPARTMENT OF PATHOLOGY The Johns Hopkins Medical Institutions

Vol. 17, No. 44

THE JOHNS HOPKINS MICROBIOLOGY NEWSLETTER

Monday, November 30, 1998

Provided by Leslie Edwards Reger, Division of Outbreak Investigation, Maryland Department of Health and Mental Hygiene.

Outbreaks reported to DHMH from 11/23 - 11/25: 2 foodborne gastroenteritis in food service facilities

The Johns Hopkins Hospital. Information provided by Dr. Ann Smith, Department of Pathology.

Case Report: A 21 year old male college student came to the student health clinic complaining of a three day history of sore throat, fevers, muscle aches and fatigue. He has no significant past medical history or drug allergies. He denied drug and alcohol use and has no HIV risk factors. He was treated symptomatically for the presumptive diagnosis of "influenza" and sent home. Reportedly his symptoms did not improve and three days later he developed a swollen and painful ankle and medicated himself with indomethacin for "gout". One day later he was brought to the ED because of severe chest pain. On arrival he was in respiratory distress requiring intubation. On physical examination the patient had a temperature of 39.5^oC, blood pressure of 90/40 Hgmm, heart rate of 100 bpm, and an oxygen saturation of 93% on 80% FiO2. The patient had cervical lymphadenopathy, oropharyngeal erythema, and bilateral rales on pulmonary exam. The right ankle and knee were swollen, warm and tender. A chest x-ray revealed diffuse infiltrates with bilateral pleural effusions. An MRI of the head and neck demonstrated a thrombus in the right jugular vein. Labs were sent and the patient's white blood cell count was 19K (15% bands). The right ankle was tapped to reveal purulent fluid; cultures were sent to microbiology. The patient was started on Unasyn and gentamicin and admitted to the intensive care unit after fluid resuscitation.

Diagnosis: Postanginal sepsis syndrome (Lemierre's Syndrome) secondary to Fusobacterium necrophorum pharyngotonsillitis. F. necrophorum is a highly virulent, non-motile gram negative bacillus with highly variable morphology. The organism is an obligatory anaerobe and part of the normal flora found in the mouth, genital, gastrointestinal, and upper respiratory tracts. The identification of the organism is based on colony morphology, motility, biochemical characteristics, and analysis of metabolic end products by gas liquid chromatography (GLC). F necrophorum has been recently separated into two subspecies: subspecies necrophorum contains the lipase-positive, hemagglutinin-producing biovar A, and subspecies funduliforme contains the lipase-negative, non-hemagglutinin-producing biovar B.

Discussion: Lemierre's Syndrome usually occurs after an acute oropharyngeal infection often times with secondary anaerobic septic thrombophlebitis of the internal jugular vein and/or tonsillar veins complicated by multiple metastatic infections (most frequently in the lungs, pleural space, liver, and large joints) related to bacteremia. The most frequently isolated organism is F. Necrophorum which usually occurs as a single agent, however, other causative organisms including F. nucleatum, Bacterioides species (other than fragilis), peptostreptococcus, Eikenella corrodens have been reported. Although there were several cases reported earlier, Lemierre was the first to write a comprehensive article and review of 20 cases on the subject of "postangina septicemia" which appeared in Lancet in 1936. The syndrome was reported much more frequently in the preantibiotic era at which time it resulted in fulminant anaerobic sepsis and rapid death in 90% of patients. The decline in incidence is thought to be secondary to the widespread use of antibiotics in patients suffering from acute oropharyngeal infections. However, since the 1970's there have been at least 50 cases reported in the literature emphasizing the fact that clinicians need to be familiar with the syndrome in order not to let it go unrecognized.

The affected patient population of Lemierre's Syndrome is previously healthy teenagers and young adults. Pleuropulmonary disease is seen in 97% of patients with chest xrays revealing nodular infiltrates and pleural effusions suggestive of pulmonary septic emboli. Clinical presentation typically includes dyspnea, pleuritic chest pain, hemoptysis, as well as septic arthritis, hypotension, and occasionally DIC. After pleuropulmonary "metastasis", the most common distant infection is septic arthritis. Swelling and lateral neck tenderness parallel to the sternocleidomastoid or at the angle of the jaw is common and is the result of extension of infection from the oropharynx to the lateral pharyngeal space and internal jugular vein through lymphatic or contiguous spread. Septic internal jugular thrombophlebitis results in high grade bacteremia, pulmonary septic emboli, and other metastatic foci.

Treatment: Penicillin has been an effective treatment in the past but clinical failures have been reported. With the increase in reported beta-lactamase producing F. necrophorum, most recommend adding metronidazole or clindamycin to penicillin therapy. Treatment for a prolonged duration (6 weeks) is typical and aggressive search for "metastatic" foci is also recommended with subsequent surgical drainage of purulent collections as necessary.

References:

Lemierre A. On certain septicaemias due to anaerobic organisms. Lancet. 230:701-703, 1936.
Stallworth JR, et al. Lemierre's syndrome: New insights into an old disease. Clin Pediatr. 36: 715-718, 1997.