Lemierre's syndrome

Lemierre's syndrome is characterised by internal jugular vein thrombophlebitis and septic pulmonary embolisation as a complication of pharyngotonsillitis. Systemic septic emboli can occur. Fusobacterium necrophorum is the typical causative organism. Lemierre's syndrome was common in the pre-antibiotic era, with a high mortality rate. We present a recent case and a review of the literature.

A 19 year old female presented to the Emergency Department with a 3 day history of right sided throat pain and severe odynophagia. She had been unable to eat for 3 days and had night sweats and rigors. No antibiotic treatment had been commenced. She is other wise of good health and had no history of recurrent tonsillitis or diabetes mellitus.

On examination the patient looked unwell with a pyrexia of 39.4 C. The tonsils were only mildly inflamed, being slightly larger on the right. There was no exudate or free pus. The right upper neck was tender without distinct lymphadenopathy being present. The remainder of the ENT examination was unremarkable. Full blood count showed a white cell count of 6.0 x 10^sup 9^/L and a thrombocytopenia of 59 x 10^sup 9^/L. The Monospot test was negative.

The patient was admitted with a provisional diagnosis of pharyngotonsillitis and IV penicillin treatment commenced. On hospital day 3 the initial blood culture returned a gram negative rod and the antibiotic regime was changed to ticarcillin/clavulanic acid plus gentamicin on the advice of the infectious diseases department.

On the same day the patient developed sudden onset pleuritic pain and became sweaty and tachypnoeic with pyrexia returning to 39C. By this stage the initial odynophagia was much reduced. The chest X ray demonstrated left lower lobe consolidation. The diagnosis of Lemierre's syndrome was suggested by the infectious diseases unit and this was confirmed by CT scan demonstrating thrombosis of the right internal jugular vein (see figure 1) and multiple cavitating lung lesions (less than 2cm in size) associated with bibasal effusions.

The antibiotic regimen was adjusted to intravenous metronidazole and clindamycin for a duration of 10 days. The pleuritic pain settled over this time and the patient was discharged on a 2 week course of oral metronidazole.

Followup ultrasound examination at 2 weeks showed return of internal jugular patency and chest X ray showed resolution of earlier changes.

Discussion

Lemierre's syndrome (also known as postanginal septicaemia and necrobacillosis) is characterised by pharyngotonsillitis, internal jugular vein thrombophlebitis and septic embolisation most commonly affecting the lungs. It typically occurs in healthy young adults.

The syndrome is named after Lemierre (1936) who reported on a series of 20 cases of "postanginal septicaemia" in 1936. However, many authors credit Courmont and Cade (1900) with the first report of this syndrome in 1900 in the French literature. Lemierre's syndrome was a commonly recognised entity in the preantibiotic era and was known for its high mortality rate of up to 90% (Lemierre 1936). Rapidly progressive septicaemia and embolic infections of the lungs, kidneys and liver often resulted in death within 7-15 days (Weesner and Cisek 1993). It is now infrequently reported and justifiably labelled as a "forgotten disease" (Weesner and Cisek 1993, Moore-Gillon et al 1984). A review of the literature by Sinave et al (1989) identified 38 definite cases between 1974 and 1985.

The causative organism Fusobacterium necrophorum (previously known as Bacteroides funduliformis) is a strictly anaerobic gram- negative bacillus normally resident in the oropharynx. Its virulence is related to production of a lipopolysaccharide endotoxin. It is also suggested that viral pharyngitis may also play a role in the onset of the infection (Sinave et al 1989). Other causative organisms occasionally reported include Bacteroides sp, peptostreptococci and non-Group A Streptococci (Golpe et al 1999).

The great majority of cases begin as a tonsillitis or pharyngitis with occasional cases associated with mastoiditis (Stokroos et al 1999) , otitis media, parotitis, sinusitis and dental infections (Golpe et al 1999). The post-- styloid (posterior) compartment of the parapharyngeal space is involved leading to thrombophlebitis of the internal jugular vein (IJV) with resulting tenderness and swelling of the lateral superior cervical region. Vagal and accessory nerve palsy have been reported (Agarwal et al 2000) and may be due to a toxic neuritis rather than from compression.

Septic embolism from the IN accounts for pleuropulmonary infections, reported in 97% of Sinave's reported cases (Sinave et al 1989). Bilateral pleural effusions commonly require drainage, and cavitating lesions may be seen on CT scan. Sixteen percent of cases had septic arthritis, 11% had a form of bacteraemic jaundice (in the absence of actual liver abscess). Two of the 38 cases (5%) died.

Treatment is with high dose intravenous antibiotics-- penicillin, clindamycin and metronidazole and combinations thereof. Penicillin resistance due to betalactamase production is reported (Appelbaum et al 1990). There is wide variation in the duration of treatment reported (Weesner et al 1993) ranging from weeks to even months of intravenous therapy.

The role of anticoagulation is controversial and lacks supporting evidence. It may be necessary in cases of continuing septic embolisation or retrograde involvement of the dural venous sinuses but the majority of cases improve without it. Ligation or excision of the IJV is rarely reported in cases with continuing embolisation.

References

AGARWAL R., ARUNACHALAM P.S., BOSMAN D.A.(2000) Lemierre's syndrome: a complication of acute oropharyngitis. J Laryngol Otol, 114:545-7

APPELBAUM P.C., SPANGLER S.K., JACOBS M.R.(1990) Betalactamase production and susceptibilities to amoxicillin, amoxicillin-- clavulanate, ticarcillin, ticarcillin-clavulanate, cefoxitin, imipenem and metronidazole of 320 non-Bacteroides fragilis Bacteroides isolates and 129 fusobacteria from 28 US centers. Antimicrob Agents Chemother. 34:1546-50

COURMONT P., CADE A. (1900) Sur une septico-pyohemie de I'homme simulant la peste et causee par un streptobacille. Arch Med Exp Anat Pathol, 4:17-28

GOLPE R., MARIN B., ALONSO M. (1999) Lemierre's syndrome (necrobacillosis) Postgrad Med J, 75:1414

LEMIERRE A. (1936) On certain septicemias due to anaerobic organisms. Lancet. 1:701-3

MOORE-GILLON J., LEE T.H., EYKVN S.J., PHILLIPS 1. (1984) Necrobacillosis: a forgotten disease. BMJ, 288:1526-7

SINAVE C.P., HARDY G.J., FARDY P.W. (1989) The Lemierre syndrome: Suppurative thrombophlebitis of the internal jugular vein secondary to oropharyngeal infection Medicine, 68:2: 85-94

STOKROOS R.J., MANNI J.J., DE KRUUK JR., SOUDIJN E.R.(1999) Lemierre syndrome and acute mastoiditis. Arch Otol Head Neck Surg, 125:589-91

WEESNER C.L., CISEK J.E. (1993) Lemierre syndrome: the forgotten disease. Ann Emerg Med, 22:256-8

PHILIP YEUNG and PETER NOYCE

Westmead Hospital

Sydney, New South Wales

Philip Yeung, MBBS

ENT Registrar, Westmead Hospital

Sydney

Peter Noyce, MBBS, FRACS

Consultant Otolaryngologist, Westmead Hospital Sydney

Correspondence to:

Philip Yeung

4 Black St

Vaucluse, NSW 2030

02-93887981

Copyright Australian Society of Otolaryngology Head & Neck Surgery Ltd. Jul 2001
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