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Lemierre's syndrome.

On a Monday, an 18-year-old boy was feeling ill with a sore throat that had been painful for a few days. Thinking that it was strep throat, his mother took him to an outpatient clinic. The nurse practitioner obtained vital signs and performed a quick strep test that came back negative. She told him he had a viral infection and to take ibuprofen (Motrin). On Thursday, the nurse practitioner from the clinic reported the strep culture as negative. On Friday morning, the boy continued with a very painful sore throat, with swelling noted on his left neck. His mother accompanied him to the pediatrician, who repeated the rapid strep test with negative results. The pediatrician attributed the neck swelling to a viral infection that could take 7-14 days to clear. On Monday morning, still feeling poorly, his mother took him to the local hospital's emergency department (ED) by 9:30 a.m. At this point, he had a temperature of 104 F, rigors, and disorientation. The ED physician admitted him; within the hour, a team of ear, nose, and throat surgeons and infectious disease (ID) doctors surrounded his bedside.

The ear, nose, and throat surgeon immediately drained several abscesses that were now in the back of his throat. He was admitted and put on a broad-spectrum antibiotic, pending the results of the cultures. It was not until the next day, which was Tuesday at 4:00 p.m., that the ID doctor had any information; he scribbled on a post-it note "Lemierre's Syndrome" (LS), and we were told to Google it! Two ideas occurred to the family after searching online for information about this disease: (a) it was a very dangerous condition, and (b) he needed to be admitted to a university hospital to get the best care for this uncommon illness.

We had heard of LS because of a young co-worker who had been ill with it; he had been in intensive care for months, at first mistakenly diagnosed with meningitis. Little did we know that we would be living this reality 2 years later. On transfer to the university hospital, the patient was admitted to the otorhinolaryngology unit where the doctors immediately switched his antibiotic. They were concerned with his airway, which was constricted by tire swelling, and his hypotension; he was septic.

The following days involved several blood tests, changing the intravenous (IV) antibiotics, and head and neck scans. The head and neck scans revealed a cerebral venous thrombosis, and he now had a team of neurologists involved with his care. He responded quickly to the IV antibiotics, and within 3 days, he was discharged from the hospital, 20 pounds lighter. He was sent home with an IV antibiotic for 2 weeks, to be followed by oral antibiotics for another 4 weeks. He was put on Coumadin for 6 months.

It has been 8 months since that dreaded disease came into our lives. The patient received his last scan in May 2013, which revealed a completely occluded jugular vein. The neurologist said she was not surprised by the results and that he can lead a healthy nonnal life with an occlusion. She explained that the body creates new routes to drain the blood from the head. There are no words to describe how lucky he is to have made a full recovery.

Notes of Care

Immediate care in the ED of a local hospital, where he was first admitted, included magnetic resonance imaging (MRI) of the brain; aspiration of neck and throat abscesses with cultures sent; Benadryl (diphenhydramine), Ativan (lorazepam), Zofran (ondansetron), and Tylenol (acetaminophen) with no relief of pain or discomfort; and additionally, Dilaudid (hydromorphone) for severe neck pain. An IV was started of normal saline solution 0.9%, 1000 ml q8h, and antibiotics were initiated to include Rocephin (ceftriaxone) of 2 g q12h and Flagyl (metronidazole) of 500 mg q6h.

On transfer to the large university hospital, his care included repeat MRI of the brain with contrast and continued IV fluids; nomial saline solution 0.9%, 125 ml/hr; Dilaudid (hydromoiphone) of 0.4 mg IV push q4h pm; Heparin injection of 5000 units subcutaneous q8h; Clindamycin (cleocin) IVPB of 600 mg q8h (one dose); and Dexamethasone injection of 6 mg q6h.

After 3 days, his orders were changed to include Fragmin (dalteparin) of 12,500 units subcutaneous daily, Percocet (oxycodone/acetaminophen) of 5- to 325-mg tablet q4h pm; Unasyn (ampicillin-sulbactam) IVPB of 3 g q6h, and Lovenox (enoxaparin) injection of 70 mg subcutaneous daily. He was discharged on Coumadin (warfarin) and antibiotics for 6 weeks to include Rocephin (ceftriaxone) IV of 1 g and Flagyl (metronidazole) of 500 mg for 2 weeks and then changed to Augmentin (amoxicillin/clavulanic acid) of 875-125 mg for 4 weeks. The ID monitored his laboratory work while on IV antibiotics. Neurology followed up in 1 week and managed Coumadin levels; an international normalized ratio was drawn the day before the appointment. A computed tomography scan of the veins of the head was completed 4 weeks after discharge. Percocet (oxycodone/acetaminophen) of 5-300 1-2 orally q4-6 hours prn was given for pain. Fragmin (dalteparin) of500 unit/0.2 ml subcutaneous was continued for 2 days until the Coumadin (warfarin) levels were deemed adequate and then only Coumadin of 5 mg po tab daily.

Review of the Literature

Coumont and Cade first described this disease in 1900 (O'Dwyer et al., 2011), but LS is named after French physician, Andre Lemierre, who reported 20 cases of the disease in 1936. Initially calling them "anaerobic septicemia," 18 of those cases turned out to be fatal. These events preceded the widespread use of penicillin in the 1940s. Hence, in the 1950s and 1960s, incidence of this syndrome dropped enough for it to become somewhat of a "forgotten disease."

This syndrome is also known as postanginal septicemia or necrobacillosis (Alherabi, 2009), and the number of published cases has increased recently, with six appearing between 1980 and 1990, 50 more from 1991 to 2000, and then climbing to 121 from 2001 to 2008. The reason for this increase in reporting rates may be because of an increase in incidence, clinical recognition, or emerging antibiotic resistant pathogens (Young & Steele, 2010). The recent rise in incidence may also have a relation to the renewed interest in restricting use of antibiotics in cases of pharyngitis along with a decrease in tonsillectomies. LS is more common in patients with retained tonsils (Vaid & Kornfed, 2010).

How often is the phrase "just a sore throat" used, knowing this condition is usually self-limited without serious sequelae (Centor & Samlowski, 2010). A natural history of a sore throat would include resolution of symptoms in 3-5 days. Evidence is pointing out that, when the patient's symptoms persist or worsen, the diagnosis no longer fits this category, and a more careful diagnostic and therapeutic approach is needed. The combination of recent pharyngitis, septic pulmonary emboli, and internal jugular vein thrombosis is consistent with a diagnosis of LS. This can be a life-threatening condition, even if diagnosis is made early and appropriate treatment is started (Vaid & Kornfeld, 2010).

LS is typically a disease occurring in previously healthy adolescents and young adults, with a higher incidence in men. It initially presents with a sore throat or neck pain but can present with a variety of nonspecific symptoms such as rigor, shaking chills, fever, night sweats, unilateral neck swelling, otalgia, otorrhea, dental ache, orbital pain, bone/joint pain, limb weakness, or gastrointestinal symptoms. Bacterium of the Streptococcus species most often causes this primary pharyngitis, which then leads to peritonsillar cellulitis and/or abscess. Anaerobic species multiply within this abscess penetrating into the nearby internal or, less commonly, external jugular vein. One to two weeks after initial infection, a septic thrombus forms within the jugular vein--this is known as the cord sign (Vaid & Kornfeld, 2010)--along with bacteremia leading to high fever, lethargy, or shock. The infected emboli from this thrombus may also disseminate seeding into the lungs, joints, liver, kidneys, spleen, or meninges.

Systemic complications often lead to multiorgan failure with extremely high morbidity. Systemic complications include (a) deep neck space infections; (b) septic arthritis/osteomyelitis of the humerus, hip, clavicle, tibia, and fibula; (c) brain manifestations to include meningitis, epidural/subdural abscess, sinus thrombosis, and stroke; (d) lung manifestations to include mediastinitis, pulmonary embolism, empyema, hydrothorax/pneumothorax, and pneumonia; (e) eye manifestations to include uveitis, vitreous hemorrhage, retrobulbar mass, and cranial nerve VI palsy; (f) liver/spleen infarct or abscess; and (g) lower cranial nerve, XI-XII, palsies (Karkos et al., 2010).

Fusobacterium is an anaerobic, nonspore forming gram-negative bacilli. Two species exist within the genus Fusobacterium, F. necrophorum, and F. nucleatum. F. nucleatum is the cause of most reported human bacteremias, but F. necrophorum accounts for more than half of the cases of LS. F. necrophorum exists normally in the pharynx, gastrointestinal tract, and female genital tract. It has the potential to become highly vimlent because of toxin production. It is not clear if the Fusobacterium species directly causes the sore throat or is a bystander that takes advantage of the favorable anaerobic environment created via endotoxins and exotoxins. Trauma or coinfection of the oral mucosa with bacteria or virus may also play a role in the development of LS.

General Diagnosis

The key to diagnosis of LS is a high clinical suspicion because physical examination might not be helpful in recognizing the disease. During the early stages of the disease, persistently high fevers can be the only clue (Kuppali, Livorsi, Talati, & Osbom, 2012). Steps to diagnosis include the following:

1. Isolation of the causative organism, usually from blood cultures. Fusobacterium is difficult to grow on routine throat swab; blood cultures grow the organism, but the identification is slow (up to 7 days).

2. Demonstration of internal jugular vein thrombosis utilizing ultrasound, computed tomography, or MRI scan.

3. Chest x-ray may reveal infiltrates, nodules, effusions, cavitations, or abscesses because of septic emboli.

4. Laboratory studies include neutrophilic leukocytosis, elevated erythrocyte sedimentation rate, and elevated C-reactive protein.

5. Abnormal liver and renal function tests secondary to septic emboli disseminated to the liver or kidneys.

General Treatment

1. High-dose parenteral antibiotics. Most literature suggests a carbapenem, a penicillin/beta-lactamase inhibitor combination, or metronidazole. Metronidazole is the preferred antibiotic because of its activity against all Fusobacterium species, good penetration into tissues, bactericidal activity, low minimum inhibitory concentration, and ability to achieve high concentration in cerebrospinal fluid if meningitis occurs (Vaid & Kornfeld, 2010). Six weeks of antibiotics are given for adequate penetration into protective fibrin clots.

2. Anticoagulation therapy with heparin with large thrombus burden, transitioning to outpatient Coumadin for 3 months. Anticoagulation is used generally when there is a poor clinical response following about 2-3 days of antibiotic therapy or when thromboses occur in the cavernous sinus (Vaid & Kornfeld, 2010).

3. Surgical intervention for deep tissue abscesses or if mediastinitis develops.

4. Internal jugular ligation or excision in extreme cases of thrombosis.

5. There is some evidence supporting beneficial use of hyperbaric oxygen (Alherabi, 2009).

Despite diagnosis, morbidity and mortality are significant with LS. Hospital admission usually occurs with care in the intensive care unit, and the average stay is 21 days. A mortality rate of 5% is reported (Young & Steele, 2010). Throughout decades of a nursing career, LS had been an unknown diagnosis. In the last 2 years, however, in our local area, two families had children with cases of LS. Information about LS should be added to the nursing literature in case nurses encounter this in their patient care.

References

Alherabi, A. (2009). A case of Lemierre Syndrome. Annals of Saadi Medicine, 29(1), 58-60. doi: 10.4103/0256-4947.51822

Centor, R. M., & Samlowski, R. (2010). Avoiding sore throat morbidity and mortality: When is it not "just a sore throat?", Editorials. American Family Physician, 52(1), 26-28.

Karkos, P. D., Karkos, C. D., Leong, S. C., Sivaji, N, Papadopoulos, D., & Assimakopoulos, A. D. (2010). Lemierre Syndrome: No delays in management. American Journal of Emergency Medicine, 28(1), 844. doi: 10.1016/j.ajem.2009.11.024

Kuppali, K., Livorsi, D., Talati, N. J., & Osborn, M. (2012). Lemierre's Syndrome due to Fusobacterium necrophorum, grand round. Lancet Infectious Diseases, 72(10), 808-815. doi: 10.1016/S 1473-3099(12)70089-0

O'Dwyer, D. N., Ryan, S., O'Keefe, T., Lyons, J., Lavelle, L., & McKone, E. (2011). Lemierre's Syndrome, case report. Irish Journal of Medical Sciences, 108(2), 565-567. doi: 10.1007/ s11845-008-0255-7

Vaid, A., & Kornfeld, M. (2010). Unforgettable? Clinical care conundrum. Journal of Hospital Medicine, 5(8), 486M90. doi:10.1002/jhm.815

Young, B. J., & Steele, R. W. (2010). A teenager with sore throat and neck pain. Clinical Pediatrics, 49(11), 1088-1089. doi:10 .1177/0009922810375981

Questions or comments about this article may be directed to Dorothea Frederick, MSN CNOR RNFA, at dorothea.frederick@ jefferson.edu. She is an Instructor at Jefferson School of Nursing, Philadelphia, PA.

Linda Urwiler is a Nursing Student at Burlington County College, Pemberton, NJ.

Editor's Note: Lemierre's syndrome was featured in the Case Records of the Massachusetts General Hospital in the New England Journal of Medicine, November 20, 2014, Vol. 371, No. 21.

The authors declare no conflicts of interest.

DOI: 10.1097/JNN.0000000000000112
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Author:Frederick, Dorothea; Urwiler, Linda
Publication:Journal of Neuroscience Nursing
Article Type:Clinical report
Geographic Code:1USA
Date:Feb 1, 2015
Words:2197
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