Lemierre ’ s syndrome : A forgotten entity with a very rare presentation

Lemierre’s syndrome is a potentially lethal condition, which originates as a complication of an oropharyngeal infection which causes suppuration of lateral pharyngeal space, bacteremia, and thrombophlebitis of internal jugular vein (IJV) leading to distant septic emboli. This is a forgotten disease in modern era of antibiotic therapy though it has been Sailesh Kumar Bansiwal1, Prabal Rajvanshi2, Harshita Sharma1, Rajesh Manocha2 Affiliations: 1Postgraduate Student, Department of Medicine, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India; 2Professor, Department of Medicine, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India. Corresponding Author: Sailesh Kumar Bansiwal, Office of the Head of the Department, Department of Medicine, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India, New Delhi, 110029; Email: kb22sunny@ gmail.com Received: 10 July 2017 Accepted: 26 July 2017 Published: 01 October 2017 reported with increasing frequency in 21st century. We present a case of 34-year-old female who presented with fever, headache, neck pain, odynophagia, and hemoptysis, found to have thrombosis of IJV and Internal Carotid Artery (ICA) on imaging which responded to antibiotics and anticoagulant therapy. A high index of suspicion is necessary for diagnosis and early treatment is life saving.


INTRODUCTION
Lemierre's syndrome, also known as postanginal septicemia or necrobacillosis was first reported in 1890.It was named after André Lemierre who explained the disease in 1936 [1].It is usually caused by gram negative anaerobic bacillus Fusobacterium necrophorum [2].However Peptostreptococcus, Staphylococcus, Streptococcus and Proteus have also been isolated.The disease progresses in several steps following a primary infection, which is usually pharyngitis (87.1%).Further invasion of deep neck spaces occurs, leading to internal jugular vein (IJV) thrombophlebitis (71.5%), and infective metastasis to distant sites most commonly to lungs (79.8%) [3].Imaging and other investigations helps in the diagnosis.Surgery is seldom required as prolonged IV antibiotics have been documented as the mainstay of treatment.

CASE REPORT
A 34-year-old housewife presented with a short history of fever, and headache for 20 days; odynophagia, neck pain, right sided facial swelling for 10 days; and hemoptysis for five days.The patient denied any history www.ijcasereportsandimages.com
On examination, the patient was ill looking, tachypneic, and febrile to touch.However, her vitals were normal with pulse rate 104/min, and blood pressure 130/80.General examination showed right sided facial swelling and tenderness along the right sided sternocleidomastoid.Oral examination revealed inflammation of right tonsillar fossa.In the systemic examination, there were fine infraaxillary crepitations in bilateral lungs; and neurological examination showed terminal neck rigidity; while the examination of other systems was unrewarding (Figure 1).
Ultrasonography of abdomen and echocardiography were normal.However, electrocardiography showed sinus tachycardia.The chest radiograph showed bilateral patchy shadows with mild bilateral pleural effusion (Figure 2).
Magnetic resonance imaging scan of head and neck showed right peritonsillar abscess, thrombosis of bilateral proximal IJV along with the thrombosis of right sigmoid sinus and left retromandibular vein.It also suggested bilateral ethmoid and sphenoid sinusitis/ abscess, with extension of abscess to bilateral masticator space caudally and cranially to dura leading to subdural abscess and leptomeningitis.
A computed tomography angiography chest (Figure 3) and neck (Figure 4) was done which revealed thrombosed left internal carotid artery (ICA), partially thrombosed left IJV, sphenoid sinusitis and cavitation in bilateral lung fields suggestive of septic emboli.Color doppler bilateral neck was done to assess the extension of thrombus in the vessels.Surprisingly, it showed involvement of both IJV (partially occluded) and ICA (80% occluded) of left side.
Blood (anaerobic and aerobic), urine, sputum, and throat cultures were negative as the patient had received antibiotics prior to admission in our hospital.
The patient was given intravenously ceftriaxone (1 g q12 h), vancomycin (1 g q12 h), and clindamycin (600 mg q8 h) for 4 weeks.She also received anticoagulation with LMW heparin (6 mg SC q12 h) and acenocoumarol (2 mg q24h) and INR was monitored subsequently.The patient responded to the therapy as evident on radiographic resolution of the thrombosis both in internal jugular vein and internal carotid artery.

DISCUSSION
Lemierre's syndrome, being a rare entity (incidence 1 in a million) [4] has been drawing our attention because of its increasing incidence and dreaded complications; reemergence of this disease is most likely due to the antibiotic resistance and discouragement of the use of antibiotics for sore throat.
This patient had been worked up to rule out the closely mimicking differentials which were disseminated tuberculosis, cavernous sinus thrombosis, autoimmune vasculitis and thrombophilias.
Lemierre's syndrome can have a spectrum of presentation ranging from mild pharyngitis to peritonsillar abscess.However, sinusitis, otitis media, mastoiditis, and odontogenic infections have also been described rarely.Sinusitis/sinus abscess (sphenoid and ethmoid) was probably the primary source of infection in our case (as per the history given by the patient), which involved the pharynx (peritonsillar abscess), lateral pharyngeal space, masticator space and the meninges.Meningitis in this case could be either due to direct spread from the sinus (sphenoid and ethmoid), or indirect spread through septic emboli (via ICA and IJV), or retrograde involvement of the sigmoid sinus (via IJV).There was involvement of the carotid sheath which was evident on CT angiography and color Doppler neck, leading to thrombosis of ICA and IJV.In our case, ICA thrombosis could be either due to its proximity to IJV or due to septic emboli.Though involvement of ICA is rarely reported in literature but in our case, this finding was very much evident on the imaging.Considering the endovascular nature of the disease, metastatic spread most commonly involves the lungs (79-100%) [3].However, joints, bones, skin, liver, and kidney may also be involved.
A prolonged antibiotic course (2-6 weeks) is needed in order to eradicate the microorganisms within the thrombus.The role of anticoagulation is controversial, however, some authors consider it if there is extension of thrombosis.Surgical intervention is seldom required in the present era.
Untreated cases may have mortality of up to 90% [5].The prognosis of Lemierre's syndrome is favorable if there is early diagnosis and treatment.Rare presentation of Lemierre's syndrome, ICA thrombosis along with IJV thrombosis, meningitis and sinusitis are being highlighted in our case report.

CONCLUSION
Lemierre's syndrome occurs primarily in young, otherwise healthy individuals and is characterized by a history of recent oropharyngeal infection, clinical or radiological evidence of internal jugular venous thrombosis and anaerobic bacteremia caused primarily by F. necrophorum.This is a rare illness in the modern era of antibiotic therapy, though it has been reported

Figure 1 :
Figure 1: (A) Patient on presentation with right sided facial swelling, (B) Patient after four weeks of receiving treatment, and (C) Oral examination showing right peritonsillar abscess.

Figure 2 :
Figure 2: (A) Chest X-ray at the time of admission, and (B) Chest X-ray at the time of discharge.

Figure 4 :
Figure 4: (A) Computed tomography angiography showing partial left internal jugular vein thrombosis.(B,C,D) Computed tomography angiography showing left internal carotid artery thrombosis.