Lemierre syndrome: case report

Abstracts

Lemierre syndrome is a rare disease. It often affects young adults and is most frequently caused by Fusobacterium necrophorum. The initial event is pharyngitis, which extends to the internal jugular vein, serving as source of continuous bacteremia and septic pulmonary emboli. Clinical manifestations include fever, respiratory distress, and swollen cervical lymph nodes. Diagnosis is established based on blood culture or direct blood culture and confirmed by computed tomography and/or duplex scan. Treatment consists of administration of beta-lactamase resistant beta-lactam antibiotics. Surgical exploration is rarely required. A 34-year-old woman with acute oropharyngeal infection presented 48 hours later with prostration, fever, and swollen and painful cervical lymph nodes on the left side of the neck. Chest radiography and tomography demonstrated multiple lung lesions. Computed tomography and duplex scan demonstrated thrombosis of the internal jugular vein, compatible with acute suppurative thrombophlebitis, also known as Lemierre syndrome. The patient received antibiotics and had clinical recovery. A control duplex scan demonstrated partial recanalization of the internal jugular vein.

Venous thrombosis; jugular veins; fusobacterium infections

A síndrome de Lemierre é uma doença rara, mais comum em jovens, causada frequentemente pelo Fusobacterium necrophorum. Inicia-se com faringite e propaga-se até a veia jugular interna, promovendo uma fonte de bacteremia contínua e êmbolos sépticos pulmonares. Manifestações clínicas incluem febre, alterações respiratórias e massa cervical. O diagnóstico é realizado por tomografia computadorizada e duplex scan, além de hemocultura ou cultura direta. O tratamento é realizado com antibióticos beta-lactâmicos resistentes a beta-lactamases, sendo a cirurgia raramente necessária. Paciente do sexo feminino, 34 anos, com quadro de orofaringite, evoluiu em 48 horas com queda do estado geral, febre, aumento de volume e dor em região cervical esquerda. Radiografia e tomografia de tórax evidenciaram múltiplas lesões pulmonares. A tomografia cervical e o duplex scan confirmaram a trombose da veia jugular interna, compatível com tromboflebite supurativa aguda, a síndrome de Lemierre. Após antibioticoterapia, o paciente apresentou melhora do quadro clínico. O duplex scan de controle evidenciou recanalização venosa.

Trombose venosa; veias jugulares; infecções por Fusobacterium

CASE REPORT

Lemierre syndrome: case report

^IServiço de Angiologia e Cirurgia Vascular

^IIServiço de Nefrologia do Hospital de Caridade da Santa Casa de Misericórdia de Curitiba, Pontifícia Universidade Católica do Paraná, PR, Brazil

Correspondence

ABSTRACT

Lemierre syndrome is a rare disease, more common in young adults, frequently caused by Fusobacterium necrophorum. The initial event is pharyngitis, which extends to the internal jugular vein, serving as source of continuous bacteremia and septic pulmonary emboli. Clinical manifestations include fever, respiratory distress, and swollen cervical lymph nodes. Diagnosis is established based on blood culture or direct blood culture and confirmed by computed tomography and duplex scan. Treatment consists of administration of beta-lactamase resistant beta-lactam antibiotics. Surgical exploration is rarely required. A 34-year-old woman with acute oropharyngeal infection presented 48 hours later with prostration, fever, and swollen and painful cervical lymph nodes on the left side of the neck. Chest radiography and tomography demonstrated multiple lung lesions. Computed tomography and duplex scan demonstrated thrombosis of the internal jugular vein, compatible with acute suppurative thrombophlebitis, also known as Lemierre syndrome. After antibiotic therapy, the patient presented clinical recovery. A control duplex scan demonstrated partial recanalization of the internal jugular vein.

Keywords: Venous thrombosis, jugular veins, Fusobacterium infections.

Case Report

A 34-year-old female patient presented with sore throat and shivering. She had no fever, and presented hyperemia and left palatine tonsillar hypertrophy with purulent plaques, receiving benzathine penicillin and symptomatic medication. After 48 hours she returned with sweating, mucous-cutaneous paleness, lips cyanosis, arterial hypotension, tachypnea, tachycardia, and fever. She presented an increased volume in the left cervical region with phlogistic signs, painful ulcers in the tongue and lips herpes. The hemogram accused leukocytosis, left deviation, toxic granulations and Döhle body. Serology for toxoplasmosis, cytomegalovirus and Epstein-Barr virus were negative. Thorax radiography evidenced multiple pulmonary lesions, compatible with septic emboli, verified by computed tomography (Figure 1). The patient presented clinical improvement after antibiotic therapy, but she remained with increased volume in the left cervical region. Cervical echography showed the presence of reactive lymph node enlargement. Cervical computed tomography and duplex scan confirmed the finding of thrombosis of left internal jugular vein (Figure 2). After hospital antibiotic therapy completed, the patient presented a clinical improvement, being discharged from hospital. She remained with oral antibiotic therapy for 4 weeks, having an asymptomatic clinical evolution, and the control duplex scan evidenced partial recanalization of the internal jugular vein (Figures 3 and 4).

Literature Review

Suppurative thrombophlebitis of internal jugular vein, or Lemierre syndrome, is a rare disease, having been described for the first time in 1936, with significant morbidity and mortality, affecting mainly young adults.¹

The most frequent initial event is pharyngitis, generally associated with tonsillar or peritonsillar involvement. The most common originating germen is Fusobacterium necrophorum, with some described cases of Staphylococcus aureus,²Porphyromonas spp.³ and Streptococcus pyogenes.⁴ The infection extends to parapharyngeal space, with the possibility of reaching the internal jugular vein, carotid artery, vague nerve and lymphonodi. Bacteremia from this focus is continuous, as in any endovascular infection. The interval between pharyngeal infection and thrombophlebitis is often shorter than one week.^5,6

Clinical manifestations include fever (> 39º C), shivering, severe prostration, respiratory alterations, with the possibility of pain, hyperesthesia, edema or mass over the mandible angle or across the sternocleidomastoid. Edema or neck rigidity in subjects with recent pharyngitis is observed in 53% of the patients.⁷

Septic emboli for the lung happen regularly (97% of the cases), possibly evolving to pulmonary abscesses and empyema. Metastatic abscesses may occur in other places, generating especially septic arthritis and osteomyelitis.5,6In the presence of septic pulmonary emboli and persistent fever, even with the use of antibiotic therapy, the diagnosis of Lemierre syndrome should be considered. Cases of cerebral infarction, pulmonary abscesses, cavernous sinus thrombosis and hepatic abscesses related to the syndrome have been described.^8-10

Diagnosis should be started by identifying the originating microorganism through blood culture, aspiration of thrombosed vein or direct blood culture.¹¹Definitive diagnosis may be done with computed tomography, phlebography, simple echography or duplex scan. Contrast computed tomography is the most helpful exam for diagnosis, showing edema in soft parts and defects of filing or the thrombus inside the internal jugular vein.⁶Image through nuclear magnetic resonance presents a limited experience, but may be very useful.¹²

Simple echography and mainly duplex scan can demonstrate thrombosis and the extension of the thrombus. Phlebography may show the thrombosis, being used when other methods are unavailable or in cases of diagnostic doubt.

The treatment is based on antibiotic therapy, covering anaerobes, and its minimal duration is 4 weeks or until pulmonary abscesses have disappeared in the tomography. Recommended antibiotics are beta-lactamase-resistant beta-lactam, such as ticarcillin-clavulanate, imipenem, in addition to ampicillin-sulbactam, metronidazol, or clindamycin.^13,14Hyperbaric oxygen therapy was described as assistant treatment with favorable results.¹⁵

Surgical exploration, with ligature and excision of the internal jugular vein is rarely necessary, but surgical drainage of abscesses or pulmonary empyemas may be necessary.⁵The role of anticoagulation is still controversial, with no randomized researches that support its use.⁶

There was a reduction in cases of Lemierre syndromes with the advent of new antibiotics, but the knowledge of the disease is fundamental for differential diagnosis in refractory cases, once proper antibiotic therapy decreases significantly morbidity and mortality.

References

• 1. Baker CC, Petersen SR, Sheldon GF. Septic phlebitis: a neglected disease. Am J Surg. 1979;138:97-103.
• 2. Boga C, Ozdogu H. Lemierre syndrome variant: staphylococcus aureus associated with thrombosis of both the right internal jugular vein and the splenic vein after the exploration of a river cave. J Thromb Thrombolysis. 2007;23:151-4.
• 3. Vandenbos F, Roth S, Girard-Pipau F, Neri D, Boscagli-Melaine A, Montagne M. [Lemierre syndrome due to Porphyromonas spp. in a 21 year-old patient]. Rev Med Interne. 2000;21:909-10.
• 4. Wilson P, Tierney L. Lemierre syndrome caused by Streptococcus pyogenes. Clin Infect Dis. 2005;41:1208-9.
• 5. Sinave CP, Hardy GJ, Fardy PW. The Lemierre Syndrome: suppurative thrombophlebitis of the internal jugular vein secondary to oropharyngeal infection. Medicine (Baltimore). 1989;68:85-94.
• 6. Golpe R, Marin B, Alonso M. Lemierre's syndrome (necrobacillosis). Postgrad Med J. 1999;75:141-4.
• 7. Chirinos JA, Lichtstein DM, Garcia J, Tamariz LJ. The evolution of Lemierre syndrome: report of two cases and review of the literature. Medicine (Baltimore). 2002;81:458-65.
• 8. Shibasaki WY, Yoshikawa H, Idezuka J, Yamazaki M, Onishi Y. Cerebral infarctions and brain abscess due to Lemierre syndrome. Intern Med. 2005;44:653-6.
• 9. Thatcher P. Hepatic abscesses caused by Fusobacterium necrophorum as part of the Lemierre syndrome. J Clin Gastroenterol. 2003;37:196-7.
• 10. Westhout F, Hasso A, Jalili M, et al. Lemierre syndrome complicated by cavernous sinus thrombosis, the development of subdural empyemas, and internal carotid artery narrowing without cerebral infarction. Case report. J Neurosurg. 2007;106(1 Suppl):53-6.
• 11. Andes DR, Urban AW, Acher CW, Maki DG. Septic thrombosis of the basilic, axillary, and subclavian veins caused by a peripherally inserted central venous catheter. Am J Med. 1998;105:446-50.
• 12. Auber AE, Mancuso PA. Lemierre syndrome: magnetic resonance imaging and computed tomographic appearance. Mil Med. 2000;165:638-40.
• 13. Seidenfeld SM, Sutker WL, Luby JP. Fusobacterium necrophorum septicemia following oropharyngeal infection. JAMA. 1982;248:1348-50.
• 14. Nadkarni MD, Verchick J. Lemierre syndrome. J Emerg Med. 2008;28:297-9.
• 15. Hodgson R, Emig M, Pisarello J. Hyperbaric oxygen (HBO2) in the treatment of Lemierre syndrome. Undersea Hyperb Med. 2003;30:87-91.

*

Gustavo Ioshio Handa^I; Giorgio Sfredo Bertuzzo^II; Karin S. Muller^II; Ana Cláudia Dambinski^II; Vinicius Zendrini Buzingnani^I; Lucas Marques Mantovani^I; Andressa Hubar Patriani^I; Denise Krauss^I

Publication Dates

• Publication in this collection
  30 Apr 2010
• Date of issue
  2010

History

• Accepted
  09 Nov 2009
• Received
  02 July 2008