Lemierre’s Syndrome Presenting with Pleural Effusion
Correspondence Address :
Dr. Hrishikesh Barui,
Flat C5/6, Payamanti Housing, E.K.T.P. Phase 1, Kolkata, West Bengal, India.
Lemierre’s syndrome is a condition characterised by septic thrombophlebitis of internal jugular vein following an oropharyngeal infection along with septic embolisation to other organs mainly lungs. This report is about a 14-year-old girl who presented with complaint of high-grade fever, progressive shortness of breath and painful swelling of throat for seven days. Chest radiograph showed left hemithorax homogenous opacity with contralateral mediastinal shift. Complete haemogram and other blood investigations revealed neutrophilic leucocytosis and raised C-reactive protein. Gram stain and aerobic culture of sputum, pleural fluid and blood were inconclusive. As there was no relief of symptoms even after empirical treatment with antibiotics, Contrast Enhanced Computed Tomography (CECT) scan of neck and thorax was done, and it showed bilateral ectatic internal jugular veins with intravascular thrombus and consolidation of left lung with ipsilateral pleural effusion. Ultrasonography of neck confirmed the presence of thrombus in internal jugular vein on both sides. She was started on intravenous clindamycin and subcutaneous anticoagulants. Gradually the symptoms of the patient resolved. To conclude with, despite being called a “forgotten disease”, Lemierre’s syndrome requires strong clinical suspicion and prompt management to prevent mortality.
Internal jugular vein, Thrombus, Thrombophlebitis, Fusobacterium necrophorum
A 14-year-old girl was admitted with complaints of high-grade fever, productive cough for seven days, followed by progressive shortness of breath, and painful swelling of face and neck for three days. The girl was treated with oral amoxicillin for pharyngitis with a palpable lymph node in the right side of the neck, in the outpatient department of general medicine of the institute. There was no change in voice and no difficulty in swallowing. No history of food or drug allergy but history of recurrent upper respiratory tract infections was present.
On a general examination, there was generalised swelling of the face and neck with raised local temperature and erythema. Her temperature was 38.2°C. Other vital signs: Pulse rate 115 beats/minute, blood pressure 116/74 mmHg, respiratory rate 28 per minute, and oxygen saturation 94% in room air. On examination of the upper respiratory tract, posterior pharyngeal wall was inflammed. On chest examination, chest wall movements diminished on the left side and suprasternal suction and intercostal retraction were seen on the right side of the chest. There was diminished vocal fremitus, stony dull percussion, and absent breath sounds all over the left hemithorax. Examination of other body systems revealed no abnormalities.
Initial blood investigations disclosed neutrophilic leucocytosis and raised C-reactive protein. Chest radiography showed a left-sided homogenous opacity with a contralateral mediastinal shift (Table/Fig 1). Diagnostic thoracentesis and pleural fluid analysis revealed an exudative, lymphocyte predominant fluid with low adenosine deaminase level. Gram staining and aerobic culture of sputum, blood, throat swab, and pleural fluid were inconclusive. Sputum and pleural fluid were negative for acid fast bacilli. The CECT scan of neck and thorax revealed bilateral ectatic internal jugular veins with intravascular thrombus and consolidation of the left lung with ipsilateral pleural effusion (Table/Fig 2). Further, ultrasonography of the neck also confirmed the presence of thrombi in the bilateral internal jugular vein. Additionally, cytological examination of ultrasound-guided fine-needle aspirate from the right jugular lymph node was suggestive of acute suppurative lymphadenitis (Table/Fig 3). From clinical history and investigations, it was diagnosed as a case of septic thrombophlebitis of internal jugular vein or Lemierre’s syndrome.
Considering the possibility of anaerobic sepsis, intravenous clindamycin was initiated. As D-dimer level was 10.93 μg/mL (ref: 0.05-6.5 μg/mL), subcutaneous low molecular weight heparin (1 mg/kg body weight) was started. Therapeutic thoracentesis was done and 1.2 litres of pleural fluid was removed. Gradually, the girl improved clinically. Laboratory parameters also improved after seven days of antimicrobial therapy (Table/Fig 4). Radiological improvement was observed on chest x-ray and repeated ultrasonography of neck which revealed dissolved thrombus in jugular veins. She was discharged with an advice of oral amoxycillin and clavulanic acid combination (625 mg thrice daily) and oral clindamycin (300 mg thrice daily) for six weeks. On follow-up after six weeks, the patient was doing well.
Lemierre’s syndrome, also called as necrobacillosis or post anginal sepsis is an uncommon potentially fatal complication of oropharyngeal infection (1). Courmont first noticed this entity in the early 1900s. Andre Lemierre established a link between anaerobic sepsis and oropharyngeal infection in 1936 by reporting 20 cases, of which 18 patients died (1). It has been coined as the “forgotten disease” because it is rarely encountered by physicians in routine clinical practice (2).
Fusobacterium species, mainly Fusobacterium necrophorum have been implicated in causing this syndrome (3). However, the cause may be polymicrobial and other microbes such as Streptococcus, Peptostreptococcus, Bacteroides may be isolated from some patients (3). The bacilli have a propensity to affect young adults mostly in the 2nd decade of life (3). In this case, the age of the girl was 14 years. The syndrome was frequently encountered before the arrival of antibiotics and at present it has an incidence of 3.6 cases per 1 million per year (4). The infection spreads from several sites in the upper respiratory tract such as tonsils, peritonsillar tissue, teeth, and sinuses (3). The index patient had symptoms such as fever, cough, dyspnoea, and face and neck swelling, which are similar to previous studies (5),(6). Metastatic emboli are typical sequelae of this syndrome and lungs is the most common site of lodgement of septic emboli, reported in 97% of cases, followed by major joints (7). Since most of the cases present with a metastatic lung infection, a chest x-ray is usual among the first-line investigations (3). Chest x-ray in the index patient had left-sided homogenous opacity with ipsilateral pleural effusion, which was similar to the findings of Silva DR et al., (8).
The diagnosis of Lemierre’s syndrome depends mainly on three salient features: oropharyngeal sepsis, internal jugular vein thrombophlebitis, and metastatic infection (9). To establish the diagnosis, the growth of anaerobic bacteria on culture is essential (4). However, the study by Johannesen and Bodtger U reported that cultures may not ascertain a specific microorganism due to various factors such as prior treatment with antibiotics (10). So, negative blood cultures may not rule out the possibility of this syndrome, as in index case where culture reports were inconclusive (10). To identify the thrombophlebitis of the internal jugular vein and metastatic infection, CECT scan is the investigation of choice, although duplex ultrasonography and Magnetic Resonance Imaging (MRI) are also useful (9). In this patient, a CECT scan of the neck and thorax was employed to detect the bilateral internal jugular vein thrombus and lung consolidation with ipsilateral pleural effusion as a consequence of metastatic infection to the lungs by septic emboli. Ultrasonography was done to confirm the thrombus and its extension.
Prolonged antibiotic therapy is the cornerstone of therapy in Lemierre’s syndrome (11). This patient was treated with intravenous piperacillin-tazobactam initially and after confirming the diagnosis, antibiotic was changed to intravenous clindamycin in order to cover anaerobes (12). There is ambiguity regarding anticoagulation in the treatment of Lemierre’s syndrome. Few published series mentioned that 21-30% of patients received anticoagulation (3),(12). In lieu of clinical condition of index patient, anticoagulation with low molecular weight heparin was undertaken. If thrombus doesn’t dissolve despite pertinent medical management, ligation or excision of the internal jugular vein, a procedure often performed in the preantibiotic era, could be considered (12).
Considerable clinical suspicion of Lemierre’s syndrome is necessary in patients of internal jugular vein thrombophlebitis with the background of an oropharyngeal infection. Prompt and belligerent management is necessary to avert morbidity and mortality.
Date of Submission: Mar 01, 2022
Date of Peer Review: Mar 29, 2022
Date of Acceptance: Apr 14, 2022
Date of Publishing: Jun 01, 2022
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• Was informed consent obtained from the subjects involved in the study? Yes
• For any images presented appropriate consent has been obtained from the subjects. Yes
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