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    Child’s ear pain progresses to leg pain, arm pain, and fever

     

    Further Investigations

    Because of intermittent low-grade fevers (100.4°F to 101.8°F) for 5 days despite drainage of the right ankle and foot abscesses, wide-spectrum IV antibiotics, and persistent pain of right ear and neck, a computed tomography (CT)scan of the temporal bones and an MRI of the brain and neck were obtained. The CT showed right otomastoiditis (Figure 2) and thrombophlebitis of the right sigmoid sinus, jugular vein, and transverse sinus (Figures 3 and 4). Otolaryngology took the patient to the operating room for a right myringotomy with pressure equalization tube placement, right mastoidectomy, and facial nerve monitoring. To decrease the risk of idiopathic intracranial hypertension resulting from cerebral venous thrombosis, Hematology recommended starting enoxaparin.

    Diagnosis

    Because the patient had mastoiditis, thrombophlebitis of nearby vessels, evidence of embolization with a septic left elbow, and abscesses of the right ankle and foot that grew group A beta-hemolytic strep, the patient was diagnosed with Lemierre syndrome (LS), also known as postanginal sepsis or human necrobacillosis.

    More: Early exposure to infection may benefit preterm infants

    Lemierre syndrome was best characterized by the French microbiologist Dr. Andre Lemierre in 1936. This is a rare head and neck infection that most commonly affects healthy adolescents and young adults but that can occur in younger and older patients.4,5 It traditionally starts with a pharyngeal infection but can rarely be due to mastoiditis.6-9 From the mastoid air cells, the infection spreads to the local venous system including the internal jugular vein, and clot formation can occur.9The etiology of hypercoagulability in LS is not completely understood but infection seems to be a potent mediator.7Finally, there is metastatic spread most commonly to the lungs and large joints, but it can also cause abscesses in the liver, spleen, kidney, brain, heart, muscles, and soft tissues.4,6 The most common causative organism is Fusobacterium necrophorum in 82% of cases,10 but there have been 6 cases attributed to Streptococcus pyogenes, the most common group A beta-hemolytic strep, reported in the literature.9,11-15

    As in this patient, initial symptoms of LS are related to the primary infection (sore throat or ear pain) along with fever, nausea, and vomiting. A week later, patients develop symptoms of vascular involvement. Internal jugular thrombophlebitis may cause pain in the sternocleidomastoid muscle.5 Finally, approximately 2 weeks into the illness, patients develop symptoms of metastatic spread, which may be respiratory distress, joint pain, liver and renal abnormalities, or soft tissue abscesses such as in the patient in this case.5

    Treatment is usually 4 to 6 weeks of parental antibiotics and 3 months of anticoagulation therapy.7,10 Depending on the initial source of infection such as otitis media/mastoiditis, surgical intervention such as pressure equalization tube placement and mastoidectomy may be indicated. Also, surgery may be indicated to drain metastatic infection/abscesses such as this patient required.

    NEXT: Patient outcome

    Holly D Smith, MD
    Dr Smith is an assistant professor in the Department of Pediatrics, McGovern Medical School, Houston, Texas.
    Peter T Scully, MD
    Dr Scully is a second-year pediatric critical care fellow in the Department of Pediatrics, McGovern Medical School, Houston.

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