Rapidly progressing incomplete Lemierre syndrome

TM Marques, A Almeida, L Cruz, T Ferreira 
 Internal Medicine Department, Hospital Santa Marta, Centro Hospitalar de Lisboa Central, Lisboa, Portugal

Correspondence Address:
T M Marques
Internal Medicine Department, Hospital Santa Marta, Centro Hospitalar de Lisboa Central, Lisboa
Portugal

Full Text



We report the case of a healthy 23-year-old man living in Belgium who presented to an emergency department in his home country with chest pain, which had been worsening over the previous 2 days and described as pleuritic. Mild symptom relief was experienced with analgesics. He travelled to Portugal the day after treatment and came to our emergency department with odynophagia and hematemesis. Physical examination revealed pale and sweaty skin, fever of 38.0°C, low blood pressure (106/54 mm Hg), and tachycardia (106 bpm). There was tonsillar exudate and enlarged cervical lymph nodes. A nasogastric tube was placed eliciting hematic content. Laboratory findings included normocytic anemia (Hb 11.6 g/L, VGM 85.2 fL), neutrophilic leukocytosis (leukocytes 19,970, neutrophils 18,570/μL), elevated C-reactive protein (408.6 mg/L), acute kidney injury (urea 92 mg/dL, creatinine 3.14 mg/dL), and raised liver enzymes (AST 76 U/L, ALT 66 U/L, GGT 150 U/L), bilirubin (total 1.28 mg/dL, direct 1.08 mg/dL), and prothrombin time (17.0”). Arterial blood gas analysis (with 1 L/min oxygen supplementation) revealed slight hypoxemia (69 mmHg) and lactate elevation (1.4 mmol/L). Blood tests for HIV and Epstein-Barr virus were negative. Neck and chest computed tomography (CT) showed bilateral tonsillar hypertrophy, diffuse thickening of the right masticator and pterygoid muscles, homolateral enlarged lymph nodes, lung consolidations, multiple bilateral nodules, some with a halo of ground glass opacity (halo sign); and a small left pleural effusion [Figure 1]. The simple absence of jugular vein thrombosis led to the exclusion of Lemierre syndrome. Multiorgan failure due to septic shock developed, and the patient was admitted to the intensive care unit. Blood cultures were sent. Besides fluid and antibiotic therapy with amoxicillin-clavulanate, vasopressors were started in order to maintain appropriate perfusion and high-flow oxygen therapy in order to correct his hypoxemia. The patient's case was compounded by melena, worsening anemia, and thrombocytopenia (nadir hemoglobin 5.9 g/L and platelet count 20,000/μL), requiring red blood cell and platelet transfusions. The microbiology results showed Fusobacterium necrophorum in blood cultures, which could also support the diagnosis of Lemierre syndrome, and prompted the addition of metronidazole to the antimicrobial therapy regimen. On day 4 of admission, a neck ultrasound ruled out thrombosis of the internal jugular veins, as well as the CT on Day 6, which showed improvement regarding the parapharyngeal space. A chest CT showed enlargement of the multiple bilateral condensations, cavitations, and new bilateral pleural effusions, which were larger and loculated on the left [Figure 2]. A left thoracic drain was inserted to drain the effusion. His recovery from multiorgan failure was gradual, allowing for transfer to the Internal Medicine ward on day 12, where antimicrobial therapy was continued and physical rehabilitation was started. The chest drain was removed after eight days, allowing him to walk around the ward unassisted. His blood reports showed slow but steady normalization of all parameters. He was later transferred to a medical facility in his home country.{Figure 1}{Figure 2}

The lack of literature supporting variants of the classic Lemierre syndrome[1] causes unrecognition of most cases where one of the clinical hallmarks is absent, leading to late diagnosis and suboptimal therapeutic approaches.

Most cases, like the one we present, are caused by Gram-negative Fusobacterium species, affecting young male adults[2] and usually one week after the onset of the primary infection, thrombosis of the internal jugular vein is seen, and septicemia and septic embolization occurs.[3] Many complications arise from this phenomenon, mainly pulmonary involvement with dyspnea, sharp pleuritic pain, cavitations, and empyema. To date, two cases have been described as Lemierre syndrome without internal jugular vein thrombosis,[4],[5] with the proposed name incomplete Lemierre syndrome. In addition to the fact that the present case did not present with internal jugular vein thrombosis, he experienced a clinical course that was unique. His first symptoms included pleuritic chest pain—probably due to early septic embolization later described on CT scan––instead of oropharyngeal complaints. Furthermore, the evolution of symptoms from chest pain to septic shock in only three days was faster than usually described. The present patient's quickly aggravating status may be explained by early septic embolization, which, along with the bilateral pleural effusions, led to respiratory insufficiency.

Such cases, even when they present with some clinical and chronological variations, should raise the suspicion of incomplete or Lemierre-like syndromes, thus enabling prompt early lifesaving measures.

Declaration of patient consent

The authors certify that appropriate patient consent was obtained.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References