CASE PRESENTATIONS OF THE BROWN UNIVERSITY DEPARTMENT OF MEDICINE
Acknowledgements: James Atkinson, MD, Achal Dhupa, MD, Eric J. Gartman, MD, Douglas W. Martin, MD
Chief Complaint: "I feel crummy."
History Of Present Illness: This 24-year-old woman was in her usual state of health until 10 days prior to admission when she developed a sore throat and anterior neck swelling. She recently babysat for her young nephews, who had been ill with fever and cough. She had no other significant symptoms, and the sore throat and swelling spontaneously resolved during the course of the next week. However, over the following 3 days she became increasingly fatigued, lightheaded upon standing, and developed a throbbing sensation at the back of her head and neck. She noted subjective fever and chills, as well as decreased appetite with early satiety. She also felt short of breath and as though she was breathing quickly. The patient's mother commented that her eyes looked yellow. She presented to the Miriam Hospital Emergency Department for evaluation.
Review of Symptoms: No cough. No headache or neck stiffness. No nausea, vomiting, or abdominal pain. No change in stool color. No dysuria.
Prior Medical History: Obesity. Medications: None.
Allergies: No known drug allergies.
Social History: Lived with her mother. Patient was not employed. Denied tobacco and drug use. Rare alcohol consumption. Not sexually active for more than 1 year.
Family History: Mother with hypertension, sister with Type II diabetes.
Temp= 36.3C BP =111/50 HR = 125 RR = 22 SaO2 = 97% room air
General: Morbidly obese young adult female, comfortable at rest.
HEENT: Icteric sclerae, conjunctivae pink. Oropharynx moist, erythematous, but no lesions or exudates.
Neck: Mild tenderness to palpation of lateral right neck, but no palpable lymphadenopathy.
CVS: Tachycardie but regular. No murmurs, rubs, or gallops.
Lungs: Clear to auscultation bilaterally, no wheezes or rales.
Abdomen: Obese. Positive bowel sounds, soft, nontender, nondistended. No organomegaly.
Extremities: No axillary or groin lymphadenopathy. 2+ pulses, no cyanosis, clubbing, or edema.
Skin: Hyperpigmentation of axillary skin, no rashes or lesions.
Neurologic: Alert and oriented. Cranial nerves grossly intact. Nonfocal.
WBC count: 30,600 per mL
Hemoglobin: 9.5 g/dL
Platelet count: 143,000 per mL
Mean corpuscular volume: 73.9 FL
Morphology: 2+ microcytosis
Differential: 79% neutrophils, 17% band forms, 4% lymphs, + toxic granules
Sodium: 129 mmol/L
Potassium: 5.2 mmol/L
Chloride: 92 mmol/L
Bicarbonate: 23 mmol/L
BUN: 13 mg/dL
Creatinine: 1.2 mg/dL
Glucose: 143 mg/dL
ALT: 150 IU/L
Total bilirubin: $.5 mg/dL
Direct bilirubin: 3.8 mg/dL
Alkaline phosphacase: 164 IU/L
Total protein: 6.3 g/dL
Amylase: 28 U/L
Lipase: 34 IU/L
Iron: 29 µ/dL
Total iron binding capacity: 269 µ/dL
Ferritin: 466 ng/mL
Leukocyte esterase: 25/µL
Mono spot: negative
Rapid strep: negative
CXR: Multiple bilateral pulmonary parenchymal nodules
EKG: Sinus tachycardia, rate 135
Hospital Course: The patient received 2 liters normal saline in the emergency department without improvement in her tachycardia, and she was admitted to the medicine service. A computed tomography (CT) scan of the chest was negative for pulmonary embolism but consistent with septic emboli. Ceftazidine was started empirically. Blood cultures obtained at admission were positive for Gram negative rods within 24 hours. She became febrile to 38.9C the morning after admission.
Infectious disease and pulmonary consults were obtained. Metronidazole and gentamicin were added. Given the antecedent pharyngitis and presence of Gram negative rods, a diagnosis of septic thrombophlebitis of the internal jugular vein was entertained. The patient became increasingly tachycardic and tachypneic. CT angiography was negative for pulmonary embolism in the large vessels, but the study was limited secondary to the patient's body habitus. The lung fields demonstrated a ground glass appearance suggestive of pulmonary edema or acute respiratory distress syndrome.
On hospital day 3, the patient's hemoglobin dropped to 8.2, and transfusion was ordered in an attempt to relieve her tachycardia and tachypnea. During infusion of the second unit of packed red blood cells, the patient became tachypneic to the 50s with significantly increased work of breathing, and was mtubated. A blood gas was not obtained. Workup for transfusion reaction was negative.
She was transferred to the Intensive Care Unit, where she required 48 hours of pressor support and was started on stress-dose steroids. Transfusion reaction workup was negative. The Gram negative rods were ultimately identified as Fusobacterium species, and antibiotic coverage was changed to ampicillin/ sulbactam. Transesophageal echo showed no vegetations.
Nonocclusive thrombosis of the right internal jugular vein was confirmed by ultrasound, and heparin therapy was initiated. No abscess was seen by CT. Surgery consultation was obtained, but invasive intervention was not deemed necessary. Right upper quadrant ultrasound and HIDA scan to evaluate abnormal liver enzymes showed only diffuse gallbladder wall thickening and delayed filling. Hepatitis serologies were negative. Liver enzymes returned to normal without intervention. Serial chest x-rays showed gradual resolution of pulmonary edema and airspace disease. Her hemoglobin reached a nadir of 6.6 g/dL without evidence of bleeding or hemolysis, and was attributed to dilutional effects of volume resuscitation superimposed on an underlying anemia. She received 3 units packed red blood cells without incident.
The patient was extubated on hospital day 7 and returned to the medicine service on day 8. She improved and was discharged to home on hospital day 15 on oral metronidazole and warfarin. She was seen in primary care followup 2 weeks after discharge and continued to do well.
1. What is the natural history of Lemierre syndrome?
Lemierre syndrome-septic thrombophlebitis of the internal jugular vein-is typically caused by Fuso-bacterium necrophorum, a Gram-negative rod that is part of the normal flora of the mouth, female genital tract, and gastrointestinal tract. The organism rarely causes disease in healthy subjects unless there is a disturbance in normal mucosal barriers. It occurs most commonly in older teens and young adults.
A primary infection, usually pharyngitis or tonsillitis, permits local bacterial invasion of the lateral phatyngeal space with a secondary thrombophlebitis of the internal jugular vein approximately 1 week later. Initial symptoms may be subtle, such as mild pharyngeal erythema, or be limited to a sore throat. Fever may occur at any time during the course. Neck swelling and tenderness along the sternocleidomastoid muscle are the most common physical findings, appearing in just over half of cases in a series of 109 patients by Chirinos et al.
Metastatic complications are common, with the lungs the most frequent site of septic emboli (nearly 80% of cases). Pleural effusion is also common, reported in 43%. Respiratory failure can occur. The joints (hips, shoulders, and knees) are the next most common target. Splenomegaly and hepatomegaly appear in approximately 15%, and mild hyperbilirubinemia with modest elevations in liver enzymes was reported in a third of cases.
In the preantibiotic era, Lemierre syndrome carried a high mortality rate (18 of 20 patients in Lemierre's original series). Early treatment with antibiotics greatly reduces mortality and limits metastatic complications. Sepsis and death still occur, especially with a delay in diagnosis and antibiotic treatment.
Historically, Lemierre syndrome was well known to physicians. Lemierre himself wrote that the constellation of sore throat, fever, and septic emboli of the lungs or joints "constitute a syndrome so characteristic that mistake is almost impossible." The introduction of penicillin vastly reduced its incidence, but some theorize that current trends toward more judicious prescribing of antibiotics may lead to an increase in cases.
2. What is the management of Lemierre syndrome?
Antibiotic coverage (typically metronidazole or clindamycin) is paramount, and may be required for 3 to 6 weeks to eradicate the infection. The internal jugular vein can be evaluated by ultrasound, CT, or MRI, but CT may be the preferred initial study for its ability to delineate local anatomy and detect abscess.
Anticoagulation is controversial because restoration of blood flow may allow extension of the infection. Some authors recommend anticoagulation only if the cavernous sinus is threatened. Ligation or excision of the affected vein - the mainstay of treatment in the preantibiotic area - may still have a role in patients with uncontrolled sepsis and extension of thrombophlebitis despite appropriate medical treatment.
Chirinos JA, et al. The evolution of Lemierre syndrome. Med 2002: 81;458-65.
Kristcnsen LH, Prag J. Human necrobacillosis, with emphasis on Lemierre's syndrome. Clin Infec Dis 2000;31:524-32.
Marek M, et al. A case of Lemierre's syndrome. Eur J Emer Med 2003; 10:139-42.
MARY HOHENHAUS, MD
Mary Hohenhaus, MD
Copyright Rhode Island Medical Society Feb 2005
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