Case Study: Sore Throat
The following case study was used by James P. Keating, MD, MSc, medical director, St. Louis Children’s Hospital Diagnostic Center, and his co-editor, Andrew J. White, MD, division director of pediatric rheumatology/immunology, as part of the “Patient of the Week” (POW) series. Many of the POW case studies cover uncommon illnesses, or common illnesses with unusual symptoms that can be overlooked. If you would like to be added to the POW e-mail distribution list, send an e-mail to firstname.lastname@example.org or email@example.com.
EU: S. O’Connor, J. Gunn
ENT: J. Lieu
ID: R. Orscheln
PICU: C. Capers, A. Cargill
AU: C. Sontheimer
This 19-year-old female came with CC: malaise, sore throat x 10 days.
10 days PTA: Onset of fever, malaise, myalgia and sore throat. At her school health clinic,monospot test was positive, and she was discharged with symptomatic care for infectious mononucleosis. She felt worse, developed fever to 100.9 and began having headache.
3 days PTA: OSH ER 3 days prior. Monospot again positive. Rx: NS bolus, odansetron (Zofran), famotidine (Pepcid), and dexamethasone (Decadron).Blood cultures were obtained. She was discharged. She continued to have temperatures up to 103.5.
1 day PTA: Developed left eye swelling, proptosis, ptosis and neck pain. Her blood grew three different bacteria, and she was instructed to come to the SLCH ED.
ROS: Rash on trunk early in course now resolved.
PMH: Abscess on leg in 12/2010 treated with oral antibiotics.
PE: VS T 38.6, HR 120, RR 22, BP 117/71. Ill appearing, sleepy but arousable. Impaired lateral gaze in left eye. Left-sided ptosis. Fundi normal. Trismus present. Tonsils large, red with no exudate. Neck tenderness to palpation with cervical lymphadenopathy L>R. Neck: full ROM. Lungs clear to auscultation. Tachycardic, regular rhythm, no murmurs. Abdomen soft, NTND. No organomegaly. Extremities warm, well-perfused. Neurological: Normal strength, sensation and DTRs.
Blood Culture (2/5): Fusobacterium necrophorum, arcanobacterium haemolyticum, beta-hemolytic Group C strep.
Blood Culture (2/8): Strep anginosus (2/2 bottles), Fusobacterium necrophorum (1/2), arcanobacterium haemolyticum (1/2)
Maxillofacial CT (2/8): Pansinusitis with extension of infection into the left medial and lateral pterygoid muscles, with formation of tiny abscesses.
Brain MRI (2/8): Pansinusitis. Masticator space edema and enhancement of pterygoids, masseter and left temporalis muscles. Dural thickening and enhancement in anterior left temporal lobe, which likely represents intracranial extension of inflammatory process.
Brain MRI (2/12): Cavernous sinus thrombosis. Interval development of an early subacute left hypothalamic ischemic infarct and left masticator space abscess.
Brain MRI (2/14): New thrombophlebitis of the right internal jugular vein with occlusion
Chest CT angio (2/18): Multiple, predominantly peripheral pulmonary nodules likely septic emboli.
- Lemierre’s syndrome with pulmonary septic emboli
- Polymicrobial bacteremia
- Cavernous sinus thrombosis
- Thalamic/hypothalamic infarction
- EBV mononucleosis
Hospital Course: The patient was treated initially with vancomycin and meropenem. On HD 2, endoscopic sinus drainage. On HD 4,drainage of the new pterygoid space abscess. On HD 5, anticoagulation with heparin and lovenox on HD 8.Imaging on HD 5 showed new thrombophlebitis of the right internal jugular, which had improved by HD 9.On HD 10, she had repeat sinus drainage. Vancomycin was stopped on HD 7.She defervesced and was discharged on parenteral meropenem on HD 13.
Discussion: Lemierre’s syndrome is a septic thrombophlebitis of the internal jugular vein with resulting septic emboli. Infection weakens natural barrier and allows mouth bacteria to invade the parapharyngeal space and the internal jugular vein. The most common preceding infection is tonsillitis with possible peritonsillar abscess (87 percent).The remainder of causes are precipitated by parotitis, sinusitis, mastoiditis, otitis media and odontogenic infections. The preceding infection is usually bacterial, though EBV mononucleosis has been reported as well. The reported incidence of Lemierre’s syndrome is increasing, possibly related to decreased antibiotic usage in acute tonsillitis and pharyngitis.
Fusobacterium necrophorum is the most commonly isolated agent in Lemierre’s syndrome, present in 82 percent of cases, both as a single and multiorganism disease. F necrophorumproduces multiple virulence factors likely involved in pathogenesis through increased platelet aggregation and increasing coagulation. Patients have no predisposing hypercoagulability. A small study in children showed all patients had laboratory findings of hypercoagulability during infection that almost all resolved after infection. Our patient tested positive for lupus anticoagulant, and she had elevated factor VIII activity and decreased antithrombin III.
Most common clinical symptoms include sore throat (82 percent), fever (82 percent) and a tender or swollen neck (52 percent). Many patients are seen several times, usually in ED, before the nature of the process is recognized. Emboli spread to the lungs in 85 percent. Other common sites of embolism are large joints and the liver. Meningitis and brain emboli also have been reported.
The mainstay of therapy is an extended course of antibiotics of four weeks or more. Empiric therapy should also cover anaerobics. The role of anticoagulation is unclear in Lemierre’s syndrome. The extent of the thrombus and evidence of propagation should be considered in the decision. In our patient, anticoagulation was started because of the concomitant sinus venous thrombosis.
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