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Correspondence to: Alfredo De Giorgi, MD, Department of Medical Sciences, Clinica Medica Unit, School of Medicine, University of Ferrara, University Hospital of Ferrara, Via Aldo Moro 8, 44121 Ferrara, Italy. ti.orebil@oderflaigroiged
Telephone: +39-0532-237071 Fax: +39-0532-236816
Lemierre’s syndrome (LS) is an uncommon condition with oropharyngeal infections, internal jugular vein thrombosis, and systemic metastatic septic embolization as the main features. Fusobacterium species, a group of strictly anaerobic Gram negative rod shaped bacteria, are advocated to be the main pathogen involved. We report a case of LS complicated by pulmonary embolism and pulmonary septic emboli that mimicked a neoplastic lung condition. A Medline search revealed 173 case reports of LS associated with internal jugular vein thrombosis that documented the type of microorganism. Data confirmed high prevalence in young males with Gram negative infections (83.2%). Pulmonary embolism was reported in 8.7% of cases mainly described in subjects with Gram positive infections (OR = 9.786; 95%CI: 2.577-37.168, P = 0.001), independently of age and gender. Only four fatal cases were reported. LS is an uncommon condition that could be complicated by pulmonary embolism, especially in subjects with Gram positive infections.
Core tip: We report a case of Lemierre’s syndrome (LS) complicated by pulmonary embolism (PE) that mimicked a neoplastic lung condition. The case was related to previously reported cases in Medline that documented the type of microorganism. We associated PE with LS due to Gram positive infections.
Lemierre’s syndrome (LS) is an uncommon condition characterized mainly by oropharyngeal infections complicated with internal jugular vein (IJV) thrombosis and subsequently metastatic infections secondary to septic emboli. This syndrome was first reported by Andrè Lemierre in 1936 in a personal experience describing 20 patients.
Primary sites of infection in these patients are the tonsils (palatine tonsils or peritonsillar tissue), pharynx and lower respiratory tract. Fusobacterium represents the most common micro-organism related to this syndrome (about 90% of cases). Fusobacterium spp. are strictly anaerobic Gram-negative rod shaped bacteria, mainly isolated from the oral cavity. The mechanisms underlying virulent clinical conditions are not known, and Fusobacterium is considered a rare cause of head and neck infections.
After local proliferation, neck infection is associated with IJV thrombosis and then hematogenous spread to other peripheral organs could happen such as the lung, joints, soft tissue, abdominal parenchyma, and central nervous system.
We report a case of LS complicated by pulmonary embolism and pulmonary septic emboli after IJV thrombosis.
A 53-year-old man presented to emergency department because of a history of occipital headache, malaise, hacking cough, chest pain exacerbated by inspiration, and fever for one month. He had a history of smoking, hypertension, hyperuricemia, and gastro-esophageal reflux. His general practitioner treated him unsuccessfully with clarithromycin and ceftriaxone. Blood chemistry panel showed increasing inflammatory indexes, such as white blood cells (WBC) 16.560/mm3, C-reactive protein (CRP) 13.60 mg/dL, and erythrocyte sedimentation rate (ESR) 70 mm. Chest X-ray did not show parenchymal lesions, and either spinal column X-ray or encephalic nuclear magnetic resonance (NMR) was unremarkable.
On admission, the physical examination was unremarkable except that pharyngeal and tonsil hyperemia was detected. He was diagnosed with chronic tonsillitis by an otorhinolaryngologist. Pharyngeal packing with cultural exam identified saprophytic flora. Levofloxacin and nebulizer therapies were prescribed.
Further laboratory tests showed WBC = 11.070/mm3, CRP = 3.70 mg/dL, ESR = 53 mm, fibrinogen = 706 mg/dL, and D-dimer = 773 ng/mL. Immunoglobulin-A was 559 mg/dL. Chest X-ray showed parenchymal and pulmonary consolidation associated with pleural effusion. Bronchoscopy with broncho-alveolar lavage (BAL) including microbiology and cytology was negative.
A chest computed tomography (CT) scan showed left pleural effusion, contralateral sub-pleural fibrosis and, above all, an important ovular lesion at the level of medial right lobe measuring 25.7 mm with central cavitation. Further three lesions of 5-6 mm at the superior right lobe, and enlargement of pulmonary hilar lymph nodes were evident (the largest was 13.4 mm). Since these images were suggestive of pulmonary neoplastic lesions (Figure (Figure1A),1A), a brain CT scan was planned. The latter detected a deficit of right sigmoid sinus and bulb of jugular vein filling, which were suggestive of thrombosis of the right jugular vein (Figure (Figure2A).2A). Doppler ultrasonography of upper and lower limbs and echocardiography were negative. A further careful re-evaluation of chest CT supported the hypothesis of septic pulmonary outbreaks, and filling defect in the upper and middle branches of the right pulmonary artery suggested pulmonary embolism (Figure (Figure1B).1B). A diagnosis of LS associated with IJV thrombosis secondary to tonsillitis and pulmonary emboli was made, and low molecular weight heparin (LMWH) was added to levofloxacin. Eleven days later, the patient was discharged in good general conditions. One month after discharge, a cerebral magnetic resonance angiogram (MRA) showed the complete re-canalization of the IJV (Figure (Figure2B2B).
LS is an oropharyngeal infection complicated with IJV thrombosis and subsequently metastatic infections due to septic emboli. LS represent an uncommon condition, and its prevalence is 0.6-2.3 cases per million population. Mortality rate is 4%-18%. LS incidence is higher in people aged 14-24, and its annual rate is 14.4 cases per million people per year. Mean age of patients is reported to be 18-20 years[6,7]. Male patients seen to be at higher risk, especially in autumn and winter.
The most common etiology of LS is infection due to Fusobacterium necrophorum, an anaerobic, non-motile, filamentous and non-spore forming Gram negative rod, which is described in 80% of cases. Several other organisms have been reported, isolated as single pathogen (5% of cases) or in association with Fusobacterium necrophorum (10.1%), such as many bacteria of Bacteroides family, Group B and C Streptococcus, Streptococcus oralis, Staphylococcus epidermidis, Klebsiella pneumoniae, Enterococcus sp., Proteus mirabilis, Eubacterium sp., Eikenella corrodens, lactobacilli and Candida sp. On the other hand, culture results are negative in 12% of cases.
The main site of infection is palatine tonsils (87.1% of cases) and it could lead to exudative tonsillitis and peritonsillar tissue ulcer. However, it has been reported that only hyperemia or grey pseudo-membrane could be detected. Moreover, odontogenic infections, mastoiditis, parotitis, sinusitis, otitis, and skin or subcutaneous tissue infection of the head or neck may represent the primary infection site. Finally, the disease could happen even if the appearance of the pharynx was not remarkable[5-7].
Pulmonary embolism is not frequently described in LS. Lesions of the lungs are due to haematogenous spread of bacteria from the IJV, and necrotic cavitary lesions, inﬁltrates, pleural effusions or empyema, abscesses, pneumo-thoraces, or necrotising mediastinitis have been reported.
We performed a Medline literature search to identify papers reporting cases with LS associated with IJV thrombosis. The following search terms were used: “Lemierre syndrome” in combination with “internal jugular vein thrombosis” and “vein thrombosis”. We found that isolation of microorganism was available in 173 cases (Table (Table1).1). LS was described more frequently in males (61.3%), aged 25.5 ± 14 years. Gram negative bacteria (84.3%), particularly Fusobacterium spp (76.3%), were related to it. Multiple microorganisms were reported in 8.7% of cases. Complications such as IJV thrombosis, arterial thrombosis, and pulmonary embolism were reported in 71.7%, 2.9% and 8.7% of cases, respectively. Only four fatal cases (2.3%) were described. Univariate analysis (Table (Table2)2) showed that pulmonary embolism was more frequent in patients with Gram positive bacteria. This finding was further confirmed by multivariate analysis and we calculated an odd ratio of 9.786 (95%CI: 2.577-37.168, P = 0.001). The relationship was independent from age, gender, and site of thrombosis.
In conclusion, LS is a rare condition that can mimic a neoplastic disease. However, the careful evaluation of clinical evolution should suggest a correct diagnosis. Moreover, the presence of pulmonary embolism represents a serious complication, and should be suspected when infection is due to Gram positive bacteria.
We are indebted to Mrs. Francesca Molinari and Mrs. Cristina Rinaldi, from the University of Ferrara Library Staff, and to Dr. Donato Bragatto, Dr. Claudia Righini, Mrs. Manuela Zappaterra, from the Health Science Library of the Azienda Ospedaliera-Universitaria of Ferrara, for their valuable and precious collaboration.
A 53-year-old man with a history of smoking, hypertension, hyperuricemia, and gastro-esophageal reflux presented with occipital headache, malaise, hacking cough, chest pain exacerbated by inspiration, and fever.
Physical examination showed only pharyngeal and tonsil hyperemia related to chronic tonsillitis in the patient with a history of gastro-esophageal reflux.
Pulmonary infection with slow resolution, pulmonary abscess, and pulmonary neoplasia.
Laboratory work-up showed increased white blood cells, C-reactive protein, and erythrocyte sedimentation rate.
Chest X-ray was negative for parenchymal lesions at first, but then it showed parenchymal and pulmonary consolidation associated with pleural effusion confirmed by a computed tomography scan. Moreover, filling defect in the upper and middle branches of the right pulmonary artery suggestive of pulmonary embolism was detected. A brain computed tomography scan excluded parenchymal lesions, but a deficit of the right sigmoid sinus and bulb of jugular vein filling suggestive of thrombosis of right jugular vein were shown.
Tonsillitis related to Fusobacterium infection complicated with internal jugular vein thrombosis and pulmonary embolism.
The patient was treated with levofloxacin and low molecular weight heparin.
Lemierre’s syndrome is a rare condition characterized by oropharyngeal infection complicated by internal jugular vein thrombosis and pulmonary embolism.
Lemierre’s syndrome could mimic a neoplastic process. A careful follow-up of this condition is necessary.
The paper is well written.
Institutional review board statement: The study was reviewed and approved by the Azienda Ospedaliero-Universitaria S. Anna of Ferrara Institutional Review Board.
Informed consent statement: The patient involved in this study gave her written informed consent authorizing use and disclosure of her protected health information.
Conflict-of-interest statement: The author(s) declare no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Manuscript source: Invited manuscript
Specialty type: Medicine, research and experimental
Country of origin: Italy
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Peer-review started: August 11, 2016
First decision: September 12, 2016
Article in press: December 9, 2016
P- Reviewer: Grignola JC, Lazo-Langner A, Pereira-Vega A, Tarazov PG, Turner AM, Wang HY S- Editor: Qiu S L- Editor: Wang TQ E- Editor: Lu YJ