LEMIERRE ’ S SYNDROME – A Rare Fusobacterial Complication

Corresponding Author: Dr. Abhishek PG2, Department of Medicine, Saraswathi Institute of Medical Sciences, Hapur, UP, India Email-abhishek9july.ag@gmail.com Abstract: Lemierre’s syndrome is a rare complication following an acute episode of upper respiratory tract infection. Dr. André Lemierre, a French bacteriologist in 1936, who first published 20 cases in lancet out of which only 2 survived. The causative agent is typically Fusobacterium. The number of cases of Lemierre’s syndrome subsequently declined with the introduction of antibiotics (1940) and widespread use of antibiotics to treat tonsillitis. With the increase of antibiotic resistance and a greater reluctance to prescribe antibiotics for minor conditions such as tonsillitis and pharyngitis, there are now concerns developing about the re-emergence of the condition. This increasing prevalence along with unfamiliarity of clinicians with the classical features of this syndrome may result in the misdiagnosis or delay in diagnosis of this potentially fatal illness. If left untreated, the mortality rate is over 90%. We report a case of 48-year-old male diagnosed with this condition and successfully treated, any delay in the diagnosis could have been fatal.


Introduction
The incidence of Lemierre syndrome has been estimated at between 0.6 and 2.3 per million.Fusobacterium necrophorum which is a obligate anaerobe and a gut commensal has been described as the most common etiological agent, with positive cultures in 81.7% of patients (Chirinos et al., 2002), occasionally F. nucleatum, F. mortiferum and F. varium can also leads to this complication.Brazier (2006;Huggan and Murdoch, 2008;Kristensen and Prag, 2000;Kushawaha et al., 2009).The fusobacterium is considered as the normal commensal of oral cavity and gi tract, the pathogenesis of the syndrome is unknown but some theories believe that Invasion of viral e.g., ebstein barr virus or other bacterial infection in oropharynx can facilitate the invasion of fusobacterium through mucosa, with the formation of a peritonsillar abscess.When the abscess wall ruptures internally, the bacteria can spread through the soft tissue and infects the nearby structures, which can lead to involvement of internal jugular vein (Brazier, 2006;Huggan and Murdoch, 2008;Kushawaha et al., 2009).As a consequence of this bacteraemia it can lead to thrombus formation in internal jugular vein and this also acts a source of metastatic septic emboli to distant organs (Lemierre, 1936;Huggan and Murdoch, 2008;Forrester et al., 1985).In addition to internal jugular vein it can also involve some branches of external jugular vein (Morris et al., 2006).Lemierre's syndrome remains a rare condition, with one retrospective study from Denmark estimated an incidence of around 1 case per 1000000 (Dagan and Powell, 1987).

Case Report
About 48-year-old male presented with fever for 7 days, Cough since last 7 days and neck swelling/pain from last 2 days along with headache and vomiting.Fever was intermittent, cough was productive with blood, neck swelling was acute in onset and progressive.He was treated for fever by general physician with antibiotics and analgesics.On examination there was swelling of right side of neck (Fig. 1) (black arrow), his temperature was 4°C, His chest exam showed bilateral bibasilar crackles.Oral examination demonstrated erythema of the pharyngeal mucosa and tonsils.The neck was tender to palpation but brudinsky and kernig sign were absent.Cervical lymphadenopathy along with dilatation of the internal jugular vein.was started with amoxicillin(1g) tds and oral metronidazole (400 mg) tds.He was also started on warfarin(5mg) od and dalteparin at the time of admission.The patient was asymptomatic on discharge, he continued antibiotics till 12 days after discharge and he continued to take warfarin for 90 days.Patient attended follow up after eight weeks, he was asymptomatic and the neck swelling was resolved.

Discussion
Following a more acute presentation and appropriate evidence from computed tomography, we diagnosed Lemierre's syndrome.Detection of a Fusobacterium spp.from blood culture will provide the confirmation to diagnosis (Brazier, 2006;and Singla, 2013).However, culturing may take upto few days and prior treatment with antibiotics will decrease chance of detection of the bacteria in culture as was in this case (Eilbert and Singla, 2013;Riordan and Wilson, 2004).F. necrophorum and F. nucleatum are discriminated from other species by their abilities to grow in 20% bile, produce indole, display lipase activity and form gas in glucose agar (Hagelskjaer and Prag, 2000;Sinave et al., 1989).Furthermore, F. necrophorum have unique property of lactate fermentation to propionate and haemolysis (Sinave et al., 1989).It can classically present as fever reaching 39-41 degree Celsius, which may or may not be accompanied by rigors (Riordan and Wilson, 2004).Sore throat usually occurs few days before the septicaemia, it can be with no findings in oropharynx or with exudative tonsillitis (Riordan and Wilson, 2004).Patients may also exhibit an induration of the internal jugular vein, slightly inferior to the sternocleidomastoid muscle's anterior border.The infection can be metastatic leading to osteomyelitis, pulmonary involvement, pericarditis, arthritis, meningitis, hepatic abscess (Sinave et al., 1989).Septic arthritis which can occur in 13-27% of cases, typically affecting the hip joint and osteomyelitis in 3% (Riordan and Wilson, 2004).Hepatomegaly due to liver involvement abdominal pain is common (Alherabi, 2009) Contrast enhanced CT is most specific for the diagnosis (Kushawaha et al., 2009).CT scan is always better than ultrasonography (Kushawaha et al., 2009).
Antibiotic coverage to include anaerobic organism should be started as soon as diagnosis is suspected and should last from 3 to 6 weeks.We used amoxicillin along with metronidazole in this case however, according to Katrine M Johannesen carbapenem and piperacillin/tazobactam were commonly used (Johannesen et al., 2016).Surgical exploration may be indicated.
Use of anticoagulant is controversial, according to Amaro et al., routine anticoagulation is not advisable, because of risk of hematogenic dissemination of the infection and should be reserved for the cases in which there is retrograde progression of thrombus in the direction of cavernous sinus.

Conclusion
Through this case report we want to discuss this rare outcome of a common infection, so that the diagnosis should not be delayed and infection like tonsillitis should not be under treated.Primary prevention of this syndrome should be kept in mind while dealing with tonsillitis due to its high mortality.We also wanted to discuss the controversial role of anticoagulants through this case report.