Lemierre’s syndrome with muscle necrosis and chronic osteomyelitis

Abstract

Lemierre’s syndrome is a rare disorder characterized by thrombophlebitis of the internal jugular vein and is caused by gram-negative anaerobes, primarily Fusobacterium necrophorum. We report a case of a 20-year-old man who developed chronic osteomyelitis of the femur and myonecrosis of thigh muscles secondary to this syndrome. The diagnosis was made based on clinical presentation and evidence of thrombosis in the internal jugular vein on ultrasound. The patient was initially treated with antibiotics, and anticoagulants were added later due to deep vein thrombosis in the lower limb.

Lemierre’s syndrome (LS) is a rare disorder, with a reported incidence of <3.6 cases per million. It is commonly attributed to gram-negative anaerobes; the most common species implicated is Fusobacterium necrophorum.¹ The early identification of this syndrome is vital, as a delay in diagnosis and management can lead to significant morbidity and mortality.²

CASE PRESENTATION

A 20-year-old man presented to the emergency department with complaints of fever, dyspnea, and right lower extremity pain. He visited Florida, where he cut himself on a boat while in brackish water but cleaned it with antiseptic and placed a bandage over it. In the emergency department, his blood pressure was 101/45 mm Hg; pulse, 109 beats/min; temperature, 37.9°C; respiratory rate, 18 breaths/min; and oxygen saturation, 78% on room air. On physical examination, he had painful swelling of the right thigh. He was emergently intubated for airway protection. Laboratory studies revealed leukocytosis (26 × 10³/μL) and a platelet count of 17 × 10³/μL, with a creatinine level of 1.96 mg/dL and a lactate level of 6.5 mmol/L. Venous duplex scan of the lower limbs showed no thrombus. One out of two bottles of blood culture grew Fusobacterium on day 3.

The patient was placed on intravenous vancomycin (500 mg every 6 hours) and piperacillin/tazobactam (4.5 g every 8 hours) initially, which were later modified to meropenem (1 g every 8 hours), doxycycline (200 mg on day 1, then 100 mg/d), and vancomycin (500 mg every 6 hours). Moreover, he underwent incision and drainage of the right vastus intermedius muscle abscess. Computed tomography of the chest revealed markedly progressive multifocal pneumonic foci with cavitations and multiple scattered nodularities. A Doppler ultrasound for neck swelling showed a left internal jugular vein thrombus, confirming the diagnosis of LS. He progressively got better and was extubated after 1 week. Doppler ultrasound of the right leg showed deep vein thrombosis, and he was started on apixaban.

Magnetic resonance imaging of the right femur on 1-month follow-up showed acute-on-chronic osteomyelitis. He remained on broad-spectrum antibiotics for 87 days. Repeated imaging showed the disease process had decreased significantly, and the patient was discharged home. On 2-month follow-up, the patient had no complaints, and he returned to his baseline activities.

DISCUSSION

The association of osteomyelitis and LS has been shown in multiple studies,^3–9 but the involvement of long bones is less common. Vles et al reported osteomyelitis and pyomyositis in the thigh secondary to Fusobacterium infection. In contrast to our patient, soft tissue involvement primarily included the biceps femoris, vastus medialis, and adductors.⁶ Lee et al and Stahlman et al reported similar cases of osteomyelitis in the fibula.^7,8 Murray and Lieberman also published a case of osteomyelitis involving the tibia with underlying sickle cell disease.⁹ In contrast to these cases, our patient had chronic osteomyelitis of the femur with no predisposing illness. Table 1 summarizes the findings of case reports highlighting Fusobacterium infection in long bones.^6–9

Table 1.

Cases of cured osteomyelitis of long bones caused by Fusobacterium

Author, year	Symptoms	Organism	Management	Weeks of therapy
Vles et al, 20176	Fever with rigors, low back pain radiating to right thigh with pain on palpation	Fusobacterium spp.	IV cloxacillin and ceftriaxone ⇒ clindamycin	Unknown
Lee et al, 20127	Fever with pain and swelling in right lower leg	F. nucleatum and Actinomyces	Ampicillin-sulbactam (IV) and amoxicillin-clavulanate (PO)	11
Murray & Lieberman, 20029	Left leg pain, swelling, redness	F. nucleatum	Nafcillin and ceftriaxone ⇒ IV clindamycin	6
Stahlman et al, 19968	Septic arthritis with bone pain	F. necrophorum	Penicillin G	6

The diagnosis of LS is made by clinical, laboratory, and radiological investigations. Determining the presence of thrombus in the vessels usually requires ultrasound and contrast-enhanced computed tomography. On ultrasound, our patient had an acute thrombus in the left internal jugular vein. In laboratory investigations, Fusobacterium can be isolated from blood or tissue cultures.¹⁰

The management of LS warrants an integrated approach from different specialties. Antibiotics are the mainstay of treatment, but there is a lack of consensus on specific agents. Ideally, antibiotic therapy should include at least one beta-lactamase inhibitor. As a combination drug, metronidazole has shown promising results in LS, but our patient had progression of myositis even on metronidazole. Different antibiotics, including beta-lactam drugs and clindamycin, can be employed as single-agent regimens. We used a combination of piperacillin/tazobactam and daptomycin to counter the infection. As an antipseudomonal drug, piperacillin/tazobactam was used for Fusobacterium. Daptomycin was added against the gram-positive bacteria, as the patient had a positive nasal swab test. The duration of antibiotic therapy is prolonged—at least 3 to 6 weeks.¹¹

LS is a rare disease usually characterized by internal jugular vein thrombosis and multisystem involvement due to septic emboli, following a recent upper respiratory infection by F. necrophorum. The vague initial clinical presentation and unawareness among health care professionals often result in misdiagnosis and delay in the management of LS, which not only increase mortality but also lead to prolonged hospital stays and unnecessary diagnostic workups. The presence of internal jugular vein thrombosis after oropharyngeal infection should raise the possibility of LS, and antibiotics should be considered without a further delay to improve the outcome.