SESSION TYPE: Infectious Disease Case Report Posters II
PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM
INTRODUCTION: Lemierre’s syndrome, or postanginal sepsis, begins with an oropharyngeal infection, progresses to septic thrombophlebitis of the internal jugular vein and septic metastasis. We present a young man with Lemierre’s syndrome complicated by respiratory failure.
CASE PRESENTATION: A 25-year-old healthy male presented to his PCP complaining of fevers, chills, sore throat, myalgias, and lymphadenopathy. Two days prior, he had kissed a woman with infectious mononucleosis. A work-up for EBV and streptococcal pharyngitis was negative. His symptoms progressed to nausea, emesis, cough, and weakness. While shopping a week later, EMS noted his profound weakness and brought him to his local hospital for dehydration. Blood cultures grew gram-negative rods later identified as Fusobacterium species. The patient was started on piperacillin-tazobactam and levofloxacin. A neck CT showed thrombosis involving the left internal jugular vein with extensive soft tissue changes along the left carotid sheath, contiguous with the left palatine tonsil. There was no abscess. A chest CT showed bilateral infiltrates suspicious for septic emboli. The patient was transferred to our MICU where he was emergently intubated for severe hypoxemia and worsening radiographic infiltrates. He had left neck tenderness and lymphadenopathy. The patient was treated with a regimen of piperacillin-tazobactam, clindamycin and intravenous heparin. He was extubated on ICU Day 7 and was discharged on Hospital Day 14 on amoxicillin-clavulanate for four more weeks and warfarin for three months.
DISCUSSION: This case describes Lemierre’s Syndrome that progressed to sepsis and respiratory failure. Andre Lemierre first published 20 cases in 1936, 18 patients died. The syndrome is a complication of an oropharyngeal infection, most commonly caused by Fusobacterium species. If left untreated, it can progress to internal jugular vein septic thrombophlebitis and septic pulmonary emboli. Prior to the advent of penicillin, mortality was approximately 90%. With the advent of penicillin, the incidence declined significantly to approximately 1 case per million per year, thus the syndrome became known as the “Forgotten Disease.” However, the incidence has increased over the last two decades. Mortality ranges from 5% - 15%. Treatment is directed at anaerobes, but should include coverage for potential co-infections. There are no controlled studies to support full anticoagulation, but is often recommended if response to antibiotics is poor. Abscess drainage or jugular vein ligation may be required.
CONCLUSIONS: Despite its rarity, Lemierre’s Syndrome should remain in the differential diagnosis in a young adult presenting with sore throat, fevers, and malaise.
1) Riordan T. Human infection with Fusobacterium necrophorum (Necrobacillosis) with a focus of Lemierre’s syndrome. Clin Microbiol Rev 2007; 20:622-59.
DISCLOSURE: The following authors have nothing to disclose: Susan Mucha, Nicole Secca, Kameron Ashker, Marvin Balaan, Tariq Cheema
This is an open access article which I have permission to replicate here with proper attribution. Every article I find proves my layman's theory about the history of our family blood disorder which I believe is related to Lemierre's. Consistent in all of the Lemierre's cases are the returns to the ER with the same symptoms.
Case Presentation
A middle-aged woman presented to the emergency room (ER) with complaints of severe neck pain and occipital headaches for one week, which were not relieved with analgesics. She denied fevers, sore throat, cough, shortness of breath, or any trauma to the neck. Past medical history was significant for epilepsy and prior episodes of supraventricular tachycardia (SVT). She denied smoking or illicit drug use. She had a dental scraping of her left mandibular molars two weeks prior to gingivitis. Vital signs were stable in ER. Physical exam was positive for neck tenderness and minimal restriction of neck movements. Laboratory data revealed a white blood cell (WBC) of (neutrophils 79%). Computerized tomography (CT) scan of the head and neck without contrast and lumbar puncture were done to rule out subarachnoid hemorrhage. The results did not reveal any abnormalities. Hence the patient was discharged on muscle relaxants and analgesics.
She returned to the ER in 5 days with high grade fevers, worsening neck pain and a headache. Temperature was 101°F, heart rate 162/min, respiratory rate 16/min, blood pressure 152/96 mmHg, and oxygen saturation of 92% on room air. Pharyngeal exam showed no erythema, swelling, or exudates. There was no evidence of otitis media or an active gingivitis either. There was no dental caries noted at that time, and there was no heat or cold intolerance. Percussion tenderness was not present. Neck examination showed restriction in range of movements, and a tender cord-like mass was palpable on the left side of neck. Cardiopulmonary examination revealed diffuse crackles in both lungs and no cardiac murmurs.
Laboratory data showed a WBC count of (bands 44%) and an ESR of 98 mm/hr. Complete metabolic profile (CMP), including electrolytes, renal function (BUN, creatinine), and liver enzymes (LFTs) were all within normal range. EKG showed SVT. Chest X-ray showed small bilateral pleural effusions and bilateral pulmonary infiltrates without cavitations. CT scan of the head and neck with contrast demonstrated a thrombus in the left internal jugular vein (IJV) (Figure 1) extending to left sigmoid sinus (Figure 2) and bilaterally into the cavernous sinus (Figure 3). There was diffuse edema around the soft tissues of the neck. Preliminary blood cultures grew gram-positive cocci in chains. She was started empirically on intravenous Clindamycin and Vancomycin. Unfractionated heparin was also started due to the extensive clot burden. Workup for autoimmune and hypercoagulable diseases was unrevealing for any abnormality. Autoimmune workup included anti-nuclear anti-bodies (ANA), rheumatoid factor (RF), cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA), perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA), anti-Ro/SSA and anti-La/SSB antibodies. Hypercoagulable workup included prothrombin time (PT), partial thromboplastin time (PTT), international normalized ratio (INR), factor V Leiden mutation, prothrombin gene mutation, protein C and S as well as antithrombin levels, anti-cardiolipin antibodies and lupus anticoagulant.
In summary, we present a middle-aged female who presented with severe neck pain and occipital headaches for a week who did not have fevers or a sore throat on initial presentation but did provide a history of dental work performed two weeks ago. A noncontrasted CT of the head and neck failed to reveal any pathology on admission, and she was discharged from the ER whereby she very rapidly deteriorated over the next five days and then presented with worsening fevers, headaches, neck pain, and a leukocytosis of 34,000. Five blood cultures performed over a period of six days grew Streptococcus intermedius, and a CT head and neck with contrast showed thrombosis of the jugular vein, cavernous sinuses, and left sigmoid sinus. Patient survived and recovered after eight weeks of ampicillin-sulbactam.
Lemierre’s syndrome is a rare disease, typically caused by the microorganism Fusobacterium necrophorum. Tonsillitis is the most common primary infection (87.1%) followed by mastoiditis (2.7%) and odontogenic infections (1.8%) [11, 12]. This is typically followed by invasion of the pharyngeal lateral wall and thrombophlebitis of the internal jugular vein followed by high grade bacteremia and septic seeding of vital organs, most commonly the lungs. It is quite likely that our patient developed LS secondary to the gingival scraping that she underwent two weeks before her symptoms started. S.intermedius is a rare causative organism, and only 2 case reports of LS were found with this bacterium [8, 13]. Escalona et al. [8] reported a case with LS due to S. intermedius. This patient presented with extensive mandibular swelling due to an infected molar and fevers, along with an edematous floor of the mouth on physical exam. Chemlal et al. [13], reported a patient with LS, who had a recent pharyngitis presenting with fever and lower chest pain related to multiple pulmonary abscesses. Pharyngitis is a single most common presentation of LS as mentioned by Wright et al. [14]. In contrast, our patient’s mouth examination was totally benign, and she did not have a history of sore throat in the recent past. Our patient’s benign presentation and normal oropharyngeal examination might have delayed her diagnosis. It is tempting to speculate that differences in virulence properties between S. intermedius andF. necrophorum which is the usual pathogen responsible for LS may have contributed to an atypical presentation. However, the previous 2 reported cases of S. intermedius [8, 13] did present with oropharyngeal signs. Therefore, it is important to recognize that LS can present without any signs of pharyngitis or an active dental or ear infection and hence can be missed in the early phases of infection [3].
Diagnosis using CT scan of the head and neck with IV contrast is considered superior to a neck ultrasound as it is better in locating the anatomical extension of the thrombus [4, 5]. CT scan in the absence of contrast may be of limited utility (as was the case in our patient). Blood cultures should be sent on a patient with persistent severe pharyngitis and signs of sepsis and even in patients presenting with fevers and severe neck pain. Penicillin is the drug of choice, but due to recent penicillin-resistant strains of Fusobacterium, drugs like Clindamycin or beta lactam/beta-lactamase inhibitor are preferred [7, 14, 15]. Therapy should be started as soon as the syndrome is suspected and should be continued for at least 6 weeks [14–16]. Surgical drainage of abscess and IJV ligation may be indicated for patients who fail to respond to antibiotics, as was done in the preantibiotic era, though the ligature is not frequently done now [4, 8]. Routine use of anticoagulation is controversial as there are no randomized trials, and sepsis-related thrombocytopenia is often seen in these cases [14, 16]. Anticoagulation should strongly be considered, if there is clot propagation involving the cavernous sinus or if there are septic emboli [4, 5,7]. However anticoagulation can increase the risk of bleeding and expansion of hematoma.
4. Summary
Lemierre’s syndrome usually presents in childhood but may present atypically in middle-aged people, as in our patient. It can happen after pharyngitis, otitis media, odontogenic infections, or dental procedures [4]. The number of reported cases is increasing, due to the restricted use of antibiotics for sore throat and tonsillitis [5, 7]. High grade bacteremia with Streptococcus intermedius due to septic thrombosis, without any signs of an oral or pharyngeal infection at the time of presentation, is a unique feature of this case. Further, the ability of other oral flora to be causative agents of Lemierre’s syndrome is not as well established and recognized as it is with Fusobacterium necrophorum. This suggests that a benign mouth exam should not exclude the diagnosis of LS. In light of this, we recommend that LS should be considered in the differential diagnosis in patients presenting with persistent sore throat, mastoiditis, recent history of a dental procedure, and/or signs of active gingivitis, accompanied with neck pain and swelling. Blood cultures should be obtained and CT imaging of the neck with IV contrast should be performed. This, in turn, will enable timely diagnosis and improved outcome.
1Department of Internal Medicine, Oregon Health Science University, P.O. Box BTE 119, 3181 SW Sam Jackson Park Road, Portland, OR 97239, USA 2Infectious Diseases Division, Department of Medicine, Buffalo General Hospital, University at Buffalo, 100 High Street, B-8, Buffalo, NY 14203, USA
