Abstract
Background
Lemierre syndrome is a rare disease of the head and neck often affecting adolescents and young adults. Classically, infection begins in the oropharynx with thrombosis of the tonsillar veins followed by involvement of the parapharyngeal space and the internal jugular vein. Septicemia and pulmonary lesions develop as infection spreads via septic emboli. Although a rare entity in modern times, Lemierre syndrome remains a disease of considerable morbidity and potential mortality.
Methods
This was a retrospective review of 3 cases and associated literature.
Results
A common 1- to 2-week history of fever, sore throat, neck pain, and fatigue was observed in all patients. Patient 1 developed right facial swelling, neck tenderness, trismus, and tonsillar exudate. Patient 2 displayed right tonsillar erythema and enlargement with right neck tenderness. Patient 3 revealed bilateral tonsillar enlargement with exudate and left neck tenderness. Subsequent studies included blood cultures and computed tomography, after which empiric antibiotic therapy was started. Patient 1 underwent drainage of a right peritonsillar abscess, right pressure equalization tube placement, and ligation of the right external jugular vein. He subsequently developed subdural empyemas, cavernous sinus thrombosis, and carotid artery narrowing and required 9 weeks of antibiotic therapy. Patients 2 and 3 developed pulmonary lesions and received 6 weeks of antibiotic therapy. Timing was crucial in all cases.
Conclusions
Lemierre syndrome is a rare but severe opportunistic infection with poor prognostic outcomes if left untreated. Early diagnosis and treatment is essential. Aggressive antibiotic therapy coupled with surgical intervention, when necessary, provides excellent outcomes.
1
Introduction
Lemierre syndrome, also known as postanginal septicemia or human necrobacillosis, is a rare disease of the head and neck that often affects healthy adolescents and young adults. Initially described in 1900 by Courmont and Cade and again in 1918 by Schottmuller , it was not until 1936 when the French microbiologist Dr Andre Lemierre best characterized the disease process that now bears his name . In a case series of 20 patients, Dr Lemierre described the development of septic thrombophlebitis of the tonsillar and peritonsillar veins secondary to pharyngotonsillitis or peritonsillar abscess formation. The rapidly progressive thrombophlebitis would then spread to involve the internal jugular and facial veins with the subsequent development of metastatic emboli to the respiratory tract and ultimately the remaining end points of circulation . During the pre-antibiotic era, Lemierre syndrome was a frequent complication of head and neck infections in which nearly all patients died of overwhelming sepsis within 7 to 14 days .
Since the advent of antimicrobial therapy, and routine use of penicillin in the treatment of oropharyngeal infections, the incidence of Lemierre syndrome has been in steady decline. Because of the infrequency of this once common clinical disease, initial patient evaluations may not include this syndrome as a potential diagnosis . In an effort to emphasize the importance of early diagnosis and treatment of this once “forgotten disease,” we present 3 pediatric patients with Lemierre syndrome and provide a comprehensive review of current literature.
3
Results
A common 1- to 2-week history of fever, sore throat, neck pain, and fatigue was observed in all patients. Patient 1 developed right facial swelling, neck tenderness, trismus, and tonsillar exudate. Patient 2 displayed right tonsillar erythema and enlargement with right neck tenderness. Patient 3 revealed bilateral tonsillar enlargement with exudate and left neck tenderness. Subsequent studies included blood cultures and computed tomography, after which empiric antibiotic therapy was started. Patient 1 underwent drainage of a right peritonsillar abscess, right pressure equalization tube placement, and ligation of the right external jugular vein. He subsequently developed subdural empyemas, cavernous sinus thrombosis, and carotid artery narrowing, and required 9 weeks of antibiotic therapy. Patients 2 and 3 developed pulmonary lesions and received 12 and 6 weeks of antibiotic therapy, respectively. Timing was crucial in all cases.
3.1
Case 1
A 16-year-old adolescent boy was evaluated with a 1-week history of fever, sore throat, right neck pain, decreased appetite, right facial swelling, and right eye ptosis. On admission, the patient was found to have a white blood cell (WBC) count of 22.3, hemoglobin level of 14, hematocrit of 39, platelet value of 10, and a C-reactive protein of higher than 20. The patient was empirically started on clindamycin, ceftriaxone, and azithromycin after obtaining blood cultures. Computed tomography of the head and neck was performed revealing a right peritonsillar and parapharyngeal abscess, right mastoiditis, and the suspicion of thrombosis of the right internal jugular vein. This was confirmed with ultrasound ( Fig. 1 ). Computed tomographic (CT) angiogram of the neck was performed to define the limits of the thrombosis and evaluate the arterial system. With thrombosis limited to right internal and external jugular veins, the patient was taken to the operating theater; and a right tonsillectomy, drainage of the right parapharyngeal abscess, and placement of a right pressure equalization tube were performed. Cultures were sent for Gram stain, anaerobic, aerobic, acid fast bacteria, and fungal evaluation. Lovenox (Sanofi-Aventis, Bridgewater, NJ) was started postoperatively at 50-mg dosing.
During the patient’s hospital course, he developed septic emboli to the lungs, narrowing of the right internal carotid artery (ICA) cavernous segment, and a subdural empyema ( Fig. 2 ). At this time, blood cultures were found to be positive for Fusobacterium necrophorum , and antibiotic therapy was modified to ceftriaxone and Flagyl (Pfizer, New York, NY). With the patient developing meningeal symptoms, a lumbar tap was performed with cerebral spinal fluid revealing a WBC count of 65. Vancomycin was added to the antibiotic regimen. Serial magnetic resonance imaging (MRI) studies were performed revealing the stabilization of the subdural collection and cavernous ICA narrowing. Conservative therapy was once again modified to ceftriaxone, Flagyl, and penicillin G after a repeat cerebrospinal fluid study revealed increased protein and a WBC count of 85. During the remainder of the patient’s hospitalization, serial MRI studies revealed gradual resolution of his cerebral, neck, and mastoid disease. However, marked narrowing of the cavernous ICA persisted. The patient was discharged on IV antibiotic of ceftriaxone and penicillin G for a period of 5 weeks.
Fourteen months later the patient presented with a 2-day history of left sore throat, fever, and neck pain. The patient immediately underwent CT evaluation of the head, neck, and chest, which revealed thrombosis of the left internal jugular vein ( Fig. 3 ). The patient was empirically started on ceftriaxone, Flagyl, penicillin G, and Lovenox while waiting for the results of blood cultures. Once again the cultures were positive for F necrophorum . The patient was discharged after 4 days of hospitalization and underwent an additional 4 weeks of antimicrobial therapy.
3.2
Case 2
A previously healthy 10-year-old boy presented to her primary care provider with a 3-day history of fever, otalgia, otorrhea, and scleral icterus. Because of the severity of the patient’s symptoms, she was sent to the emergency department with concerns of sepsis vs meningitis. On initial evaluation, the patient was found to be lethargic with a temperature of 103.6°F. The remaining vital signs were stable. Initial laboratory studies included a complete blood count, chem 7, blood cultures, and a liver panel. The patient was found to be neutropenic with a count of 3, thrombocytopenic with platelets count of 56, as well as multiple abnormalities in her liver panel with a total bilirubin of 5.3, direct bilirubin of 3.9, aspartate aminotransferase of 69, alanine aminotransferase of 45, and a gamma-glutamyl transpeptidase at 147. Physical examination revealed jaundice, left ear otorrhea, 3+ right tonsil, and diffuse right cervical lymphadenopathy.
An extensive workup was performed including cerebrospinal fluid studies and culture, monospot testing, hepatitis panel, urinalysis and culture, varicella reactive protein, cytomegalovirus, Epstein-Barr panel, and abdominal ultrasound. Computed tomography of the head, neck, and chest were also obtained and revealed opacification of bilateral mastoid processes, bilateral enlarged cervical lymph nodes, and multiple pulmonary nodules ( Fig. 4 ). The patient’s empiric antibiotic therapy was changed from ceftriaxone to meropenem at this time. Subsequent Doppler ultrasound was negative for internal jugular vein thrombosis.
Despite the absence of jugular thromboses on CT and ultrasound, it was felt that this patient most likely represented a case of Lemierre syndrome. This was later confirmed by the initial blood cultures obtained in the emergency department, which grew F necrophorum . The remaining blood cultures on postadmission days 3, 4, and 6 were negative. The patient clinically improved while on meropenem and was ultimately discharged with a total of 6 weeks of amoxicillin antibiotic therapy.
3.3
Case 3
A 17-year-old adolescent boy with no significant medical history was admitted with a 1-week history of fevers, chills, night sweats, sore throat, decreased appetite, diffuse body aches, and unintentional weight loss. He further complained of a 3-day history of nausea, vomiting, productive cough, and minor swelling of the left knee. On initial evaluation, standard laboratory tests and blood cultures were obtained, and the patient received cefotaxime, vancomycin, and IV hydration. White blood cell count was 25.2, hemoglobin level 15, hematocrit 45, and platelets 55. Conventional chest radiographs revealed a round opacity of the right lower lung field with cavitation. Subsequent CT imaging of the chest revealed bilateral cavitary nodules, bilateral pulmonary effusions, atelectasis in addition to mediastinal and axillary lymphadenopathy ( Fig. 5 ). The patient was placed in isolation out of concern for tuberculosis, started on Levaquin (Ortho-McNeil Pharmaceutical, Raritan, NJ), and scheduled to undergo an echocardiogram as part of initial evaluation for septic emboli. The patient continued to develop high spiking fevers and began to complain of left-sided neck pain and greater left knee pain. Computed tomography of the neck revealed lymphadenitis and thrombosis of the left internal jugular vein, which was confirmed with ultrasound ( Fig. 6 ). Clinical findings during therapeutic arthrotomy confirmed septic arthritis of the left knee. The initial blood cultures were positive for F necrophorum . The patient was started on anticoagulation therapy and received antibiotic therapy for a period of 6 weeks.
