Lemierre's Syndrome: Role of Anticoagulation and Thrombosis Outcomes, a Retrospective Study

Background

Lemierre’s syndrome, sometimes referred to as postanginal septicemia or human necrobacillosis, is a multisystemic infection beginning in the oropharynx and leading to thrombosis of the internal jugular vein (IJV) with septic emboli and potential thrombotic extension to the central nervous system. This disorder occurs in the setting of oropharynx infection, leading to peritonsillar abscess.  Within the abscess, anaerobic organisms penetrate the neighboring IJV resulting in an infected thrombus. Often polymicrobial, the infection is associated with the gram-negative bacillus Fusobacterium necrophorum, and if left untreated, may lead to fatal sepsis. With the advancement of antimicrobial therapies and earlier diagnosis of the disease, patient outcomes have improved.  There is no clear consensus regarding the role of anticoagulation in this disorder. Some reports are in favor of anticoagulation therapy in conjunction with systemic antibiotic therapy while others support the use of antibiotic therapy alone. The purpose of this study is to better define the role of anticoagulation in this disorder and if thromboses outcomes are improved with anticoagulation therapy.  

Methods

Pediatric and adult patients diagnosed with Lemierre’s syndrome at the Mayo Clinic (Rochester, MN) between the years 1964 and 2014 were identified. Study inclusion criteria required radiologic confirmation of jugular vein thrombosis (JVT) in the setting of oropharynx infection, clinically diagnosed blood stream or local bacterial infection and intraoperative or radiological confirmation of head and neck loculated infection, and at least 3 months follow up. Patient demographics, clinical, microbiological and radiographic features, management and thromboses outcomes were reviewed. Thrombosis outcome was defined based on radiological findings defined as: CR (complete response-resolution of thrombus), PR (partial response-improvement with decrease in size of thrombus) and NR (no response). Progression/recurrence of thrombosis and bleeding complications data were also reviewed.

Results

Twenty-four patients were identified with the diagnosis of Lemierre’s syndrome. Six patients were excluded due to lack of at least 3 months of follow up.  Of the remaining 18 (8 females; 10 males) patients included in this analyses, 7 patients received anticoagulation therapy.  Six patients were of the pediatric age group (<18 years). Median age of patients who received anticoagulation was 18.6 years [age range: 14-46.4 years].   Median age for the non-anticoagulated group was 19.2 years [age range: 16-71.8 years].

Microbiologically documented infective organisms were:  Fusobacterium necrophorum (n=10), Fusobacterium necrophorum and Arcanobacterium heamolyticum (n=1), Staphylococcus aureus (n=2), Mycoplasma pneumoniae (n=1), Streptococcus pyogenes (n=1), others (n=3). Laterality of thrombosis presentation at diagnosis:  right IJV (n=7), left IJV (n=6), bilateral IJV (n=2), left external jugular vein (n=3). Other site involvement/extension included pulmonary (n=2), cavernous sinus thrombosis (n=1), and sigmoid sinus thrombosis (n=1).

All patients received broad-spectrum antibiotics. Seven patients received ≥4 weeks anticoagulation (median: 20.6 weeks; range 4-32.9 weeks]. Choice of anticoagulants and duration of anticoagulation practices were at the discretion of the treating physicians. Anticoagulation therapy consisted of unfractionated heparin (UFH) (n=1), UFH with transition to Coumadin (n=1), low molecular weight heparin (LMH) with transition to Coumadin (n=4), LMH (n=1). All patients had an improvement in their thrombus by 3 months (non-anticoagulated patient group: CR=9 and PR=2; anticoagulated patient group:  CR=2 and PR=5). None of the patients developed recurrent thrombosis or progression during the follow up period regardless of their anticoagulation status. None of the patients who were anticoagulated developed bleeding complications.   

Conclusions

This retrospective study suggests that anticoagulation in Lemierre’s syndrome does not affect thrombosis outcomes. All patients achieved partial or complete thrombus resolution and no progression/recurrence of thrombus was noted in any patient regardless of whether anticoagulation status. Aggressive and prompt antimicrobial therapy remains the standard of care for this life-threatening disorder.