A unique case of Lemierre’s syndrome status post blunt cervical trauma

Introduction/background

Andre Lemierre first described this rare but potentially fatal condition in 1936. ¹ It is a rare but potentially fatal condition usually affecting young, healthy adults with an incidence of approximately 3.6 per million per year. ² The clinical course is usually characterized by acute tonsillopharyngitis, bacteremia, internal jugular vein (IJV) thrombosis, and septic embolization with the most common clinical presentation starting with sore throat and fever, followed by neck pain and mass/swelling.

Lemierre’s syndrome (LS), as stated above, usually develops as a complication of oropharyngeal infection, which progresses to a peritonsillar abscess. About one-third of the patients either have a polymicrobial bacteremia with anaerobic streptococci and other Gram-negative anaerobes.^3,4 The most common causative microbiologic agent is Fusobacterium necrophorum, ¹ which is a nonmotile sporulating Gram-negative anaerobe that is a part of the normal oral flora. Fusobacterium necrophorum produces lipopolysaccharide endotoxin, leucocidin, and hemolysin, which lead to white and red blood cell destruction causing hemagglutinin production. This cascade augments the fulminant nature of this pathologic process causing further platelet aggregation and septic thrombus formation. ⁵

As the major vessels of the neck are in close proximity to the lateral pharyngeal space, this allows for extension from the peritonsillar space to the IJV via local invasion, either from tonsillar veins or via lymphatic channels, causing infection and thrombosis. ⁶ If left unchecked, the bacterial infection then may spread hematologically, leading to further systemic complications such as meningitis (3%), disseminated intravascular coagulation (DIC) (3–9%), jaundice (11–49%), and septic emboli with metastatic abscesses in lungs (79–100%), joints causing osteomyelitis or septic arthritis (13–27%), central nervous system (CNS), liver, heart (pericardium or endocardium), spleen, and kidneys. These sequelae cause significant morbidity leading to prolonged hospitalization and mortality rates up to 5–18%. ⁷

Definitive diagnosis may be obtained using contrast-enhanced computerized tomography (CT), which may confirm the presence of dilated veins with contrast enhancing walls, filling defects within the lumen, associated abscesses, and edema of surrounding soft tissues.^4,7 Ultrasound (US) can be an adjunct to identify IJV thrombosis with localized echogenic regions within a dilated vessel. ⁴ Staples of therapy involve targeted antimicrobials and surgical intervention involving ligation or excision of IJV with drainage and/or debridement in those who fail to respond to antibiotics. ⁸

As stated above, most cases are secondary to oropharyngeal infections. There have been some case reports of penetrating trauma to the neck that have been associated with this etiology. ⁹ However, literature review has yielded no other cases secondary to blunt neck trauma in the abscess of concomitant oropharyngeal injury. The purpose of this case report is to shed light on this unique cause of Lemierre’s syndrome, so as to raise the index of suspicion for clinicians evaluating patients with recent blunt cervical trauma.

Clinical presentation

We present a case of a 25-year-old male who developed LS secondary to blunt injury from seatbelt after a motor vehicle collision (MVC). This was a restrained driver who presented with left neck and shoulder pain with a 6-cm superficial abrasion to the left neck from the seatbelt. The patient was initially evaluated by the Emergency Room physicians and was discharged later that day without a surgical/trauma team evaluation.

The patient returned to the hospital two days later with vague abdominal pain. His initial evaluation including chemistry, complete blood count, vitals, and abdominal CT were negative. At that time, a set of blood cultures were sent. He was sent home later that day. The patient was called at home one day later as preliminary blood cultures were positive for Gram positive cocci and Gram negative anaerobes. On arrival to the ED, he endorsed persistent left neck pain, fever, malaise, anorexia, and odynophagia. He was found to be febrile to 104 F, tachycardic to 130 beats per minute, and was diaphoretic. Physical exam was notable for left neck abrasion with induration and tenderness. Laboratory analysis yielded a white blood cell count of 16.7 × 10³/mm³, platelet count of 38,000/µL, and international normalized ratio (INR) of 2.6.

A contrast-enhanced CT scan of the neck revealed extensive occlusive left IJV thrombosis with irregular multilocular fluid collections concerning for abscesses (most prominent of which measured 2.9 × 2.3 × 4.5 cm), and extensive superficial and deep left anterior neck stranding underlying the sternocleidomastoid muscle (SCM) all of which concerning for septic thrombophlebitis (Figure 1). Venous duplex showed a partial thrombosis of the left IJV.

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Figure 1.

Sagittal image (panel a), axial image (panel b) of the contrast-enhanced computed tomography of soft tissues of the neck, and 3D reconstruction (panel c) showing filling defect throughout the left internal jugular vein (IJV).

Patient was admitted to the intensive care unit and started on vancomycin, aztreonam, and flagyl due to penicillin/cephalosporin allergy. He was transitioned to levofloxacin when final cultures revealed Fusobacterium necrophorum as well as microaerophilic strep species and peptostreptococcus.

The patient had no history of anticoagulant use and anticoagulation was not initiated in this case secondary to severely diminished platelets and elevated INR.

Over the next few hours, the patient continued to decompensate, developing severe sepsis complicated by disseminated intravascular coagulation (DIC). Decision was made at that time to proceed with operative exploration via a left neck exploration with en bloc resection of the left IJV, drainage of abscesses deep to the SCM, and washout/debridement of necrotic tissue. Direct laryngoscopy at the time of surgery revealed no injury to the aerodigestive tract. Wound cultures were consistent with preoperative blood cultures and grew pan sensitive Fusobacterium necrophorum, as well as staph epidermidis and Methicillin-Resistant Staph Aureus (MRSA).

Post-operative course was further complicated by hypotension requiring vasopressor support and acute respiratory distress syndrome (ARDS) requiring prolonged intubation. Spiral CT angiography of the chest was negative for pulmonary embolism (PE). He underwent two subsequent wound washouts without any evidence of residual infection prior to closure. He improved dramatically thereafter and was discharged to his home on post-operative day 13 on a course of antibiotics and aspirin.

Discussion

Lemierre’s syndrome is a clinical diagnosis. The classic syndrome is characterized typically in three stages; starting with oropharyngeal or tonsillar infection, followed by extension of infection and inflammation to the pharyngeal and retropharyngeal space, and lastly precipitating septic IJV thrombophlebitis which may lead to further systemic complications.

Duplex ultrasonography, contrast-enhanced CT, and magnetic resonance imaging along with laboratory testing including blood cultures (usually growing Fusobacterium necrophorum) aid in diagnosis. Targeted antimicrobials are the mainstay of treatment, as Fusobacterium necrophorum is usually pan-sensitive. However, most patients are initially treated with broad-spectrum intravenous antibiotics due to the severity of presenting illness and concern for polymicrobial infection. Duration of treatment has not been established although two to six-week course is recommended.^9–11

However, failure to respond to conservative antibiotic therapy warrants surgical intervention involving ligation or excision of IJV and drainage and debridement. Those who develop parapharyngeal or peritonsillar abscess, empyema, septic arthritis, or other abscesses or cavitations should undergo drainage. Use of anticoagulation remains controversial as there are no randomized trials, but is strongly recommended if there is clot progression or evidence of septic emboli. ¹²

Diagnosis of this syndrome should be entertained in patients with a combination of neck swelling, respiratory symptoms, and signs of toxicity in the setting of recent or concomitant oropharyngeal infection such as pharyngitis/tonsillitis with or without the presence of peritonsillar or retropharyngeal abscess. With an increasing number of cases reported in the recent literature, either due to reporting bias versus antibiotic resistance or prescribing patterns, a high index of suspicion is required for prompt diagnosis and treatment. In this case, an early surgical/trauma team evaluation may have helped as the seatbelt sign would have raised suspicion for blunt cerebrovascular injury and ultrasound or contrast-enhanced CT scan would have been obtained.

Early and effective antibiotics therapy may prevent the development of full spectrum of the syndrome and its associated complication. Bukari et al. ¹³ report a case of a five-year-old boy who developed LS secondary to pencil tip injury to the pharynx resulting in inoculation of oral microflora into deeper oropharyngeal tissues. Here, we present a case of LS caused by blunt injury from seatbelt post MVC without any evidence of oropharyngeal injury. To our knowledge, this is the first reported case of LS secondary to blunt trauma. We postulate that external trauma to the neck from the seatbelt likely caused microscopic injury to oropharyngeal tissues resulting in IJV injury compromising the integrity of the vessel leading to thrombosis and infection.