Soundings: Twelve days of bilateral neck pain…

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You are evaluating a 40-year-old woman who complains of 12 days of bilateral neck pain, left greater than right, rhinorrhea, sore throat, cough productive of clear sputum, and subjective fever. She denies any shortness of breath and has chest pain only with coughing. The neck pain is described as a constant stiffness.

Edited by Brady Pregerson, MD

  
altYou are evaluating a 40-year-old woman who complains of 12 days of bilateral neck pain, left greater than right, rhinorrhea, sore throat, cough productive of clear sputum, and subjective fever. She denies any shortness of breath and has chest pain only with coughing. The neck pain is described as a constant stiffness. She had been seen in the ED 5 days earlier for the same symptoms but now also reports 1 day of noticeable swelling of the left side of her neck. Her review of systems is otherwise negative. Her past medical history includes a peptic ulcer and hypertension, for which she currently takes no medications. Family history is positive for a DVT in her mother.

Examination reveals a non-toxic, well-developed woman with an oral temperature of 97.7, a pulse of 86, a respiratory rate of 16 and a blood pressure of 171/110. Her oropharynx is clear without stridor, drooling or trismus, but she has noticeable left neck tenderness and edema just anterior to the sternocleidomastoid muscle. Cardiac and pulmonary examinations are normal, as is the remainder of her physical exam.


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altYou perform a bedside ultrasound of the neck showing the left internal jugular to have multiple hyperechoic shadows, non-compressibility with the patient sitting or lying, and absent flow by Doppler, all compatible with a DVT. (Images 1 & 2 show the left IJ thrombosis and normal comparison view). Despite the emergency physician’s concern about radiation without justification, the admitting physician, a certified test-o-phile, orders a neck CT with contrast, which shows essentially the same thing the duplex did, a venous thrombosis involving the left internal jugular and external jugular veins with extension into the brachiocephalic vein. (Image 3) The patient is started on Lovenox, and is discharged home the next day to continue Lovenox for an additional 7 days along with Coumadin. A hypercoagulability work-up – protein C, protein S, homocysteine levels, lupus anticoagulant – returns negative.

altThrombosis of the internal jugular vein is an under-diagnosed condition that may occur as a complication of head and neck infections, surgery, central venous access, local malignancy, polycythemia, hyperhomocysteinemia, neck massage, and intravenous drug abuse. It is also reported to occur spontaneously. IJ thrombosis itself can have serious complications that include systemic sepsis, abscess formation, necrotizing fasciitis, chylothorax, papilledema, airway edema, and pulmonary embolism. The diagnosis often is very challenging and requires a high degree of clinical suspicion.

altThe symptoms and signs of IJ thrombosis can often be very subtle, making it easy to overlook the diagnosis. Pain and swelling at the angle of the jaw and a palpable cord beneath the sternocleidomastoid muscle both may be absent in a significant minority of patients. The thrombosis may become secondarily infected, producing a septic thrombophlebitis. Suppurative thrombophlebitis of the IJ vein that results from lateral pharyngeal infection is termed Lemierre’s syndrome. It is named after Dr. Andre Lemierre who reported 20 cases in 1936 prior to antibiotics. The disease process is biphasic with an initial pharyngitis followed 4-8 days later by IJ thrombosis, recurrence of fever, and sepsis. The infection spreads via local tissue planes, venules, or lymphatics leading to thrombophlebitis of the IJ vein. The usual organism is Fusobacterium necrophorum. Treatment consists of Penicillin and Flagyl. With widespread antibiotic usage this condition is fortunately now rarely seen.


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Today, with extensive use of the IJ vein for venous access, central venous catheters are the most common underlying cause of IJ thrombosis. Individuals who abuse intravenous drugs can also present with IJ thrombosis secondary to repeated drug injection directly into the IJ vein. Other causes include local malignancy and head, neck, and cardiac surgery. Rare causes include polycythemia, hypercoagulable states and trauma. Spontaneous causes are often secondary to undiagnosed malignancy or a hypercoagulable state.

If the etiology of the IJ thrombosis is not obvious, then a more in-depth investigation of the coagulation system or a search for the cause of a hypercoagulable state should be performed. Currently available assays detect only 10-20% of inherited hypercoagulable states. The most common studies include measurement of levels of protein C, protein S, lupus anticoagulant, and resistance to activated protein C (factor V Leiden). Often a family history or past episodes of arterial thrombosis are present.

Diagnosis is usually made by duplex ultrasonography. Findings include a dilated and incompressible vein, intraluminal clot, no venous enlargement with the Valsalva maneuver, and abnormal Doppler flow. Contrast enhanced CT is considered to be the study of choice for suspected IJ thrombosis and its complications. It provides more information than ultrasound, but exposes the patient to the risks of contrast and radiation. CT scan can demonstrate low-density intraluminal thrombus, soft tissue swelling surrounding the IJ vein, and a distended IJ vein proximal to the thrombus.

Once a diagnosis is made, anticoagulant therapy is indicated to reduce the risk of pulmonary embolism, which is reported to be in the 1-5% range. Indwelling catheters should be removed. In the setting of infection, many patients do well with antibiotics alone with anticoagulant therapy added if there are septic emboli or evidence of clot propagation. Antibiotics should initially provide broad coverage with an emphasis on intra-oral organisms.


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3 Comments

  1. Great to see that you are increasing awareness of this killer disease among Emergency Physicians. My daughter required a craniotomy exactly three years ago–September 13, 2005, to arrest a rapidly-enlarging brain abscess caused by this disease. Thankfully, she survived following after two brain surgeries, the correct diagnosis and proper drug therapy, but sadly this disease continues to claim more lives. Some current estimates place it as a 1 in 40,000-70,000 disease among the most affected groups, rather than 1 in a million.

    Here are some sites that will expand your awareness of Lemierre’s Syndrome still further:

    DB’s Medical Rants/Dr. Robert Centor

    http://www.medrants.com/?s=Lemierre's Syndrome

    Justin’s battle with Lemierre’s Syndrome
    This blog was created for the awareness of Lemierre Syndrome in memory of Justin Rodgers

    http://lemierresyndromejrodgers.blogspot.com/

    Check out the the “Diagnosis” column on Sunday, September 14, 2008, in the New York Times magazine about Lemierre’s Syndrome, written by Dr. Lisa Sanders

    http://topics.nytimes.com/top/news/health/columns/diagnosis/index.html?scp=2&sq=Diagnosis New York Times Magazine&st=cse

    You Tube (3 Parts) Lemierre’s Syndrome

    http://www.youtube.com/watch?v=XmLpeEA5RSs

    http://www.youtube.com/watch?v=XmLpeEA5RSs&feature=related

    http://www.youtube.com/watch?v=oHbafnf89DU&feature=related

  2. It is great to read an article that is a bit outside of norm from what we see everyday. It is very important to be able to differntially diagnose complaints when patients present with oh so familiar, yet different issue.

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