Jugular venous thrombosis

The condition is a serious medical emergency, with a very high fatality rate, if untreated.  Diagnosis is commonly delayed due to its rarity. It is a serious medical emergency with high fatality if untreated. 

Distal septic emboli, especially to the lungs, are very common.

Treatment involves prompt IV antibiotics. The role of anticoagulation remains uncertain.

History

Described by André-Alfred Lemierre (1875-1956) in 1936 in the Lancet. In the pre-antibiotic era, fatality approached 100%.

Epidemiology

• Primarily affects children, adolescents, and young adults.
• More common in the pre-antibiotic era.
• Recent increase due to greater CT imaging use.

Pathology

Primary infection site:

• Common: Palatine tonsils/peri-tonsillar tissue
• Less common: Lung, middle ear, mastoid, teeth, sinuses

Progression:

• Infection spreads into lateral pharyngeal space causing internal jugular vein thrombophlebitis.
• Thrombosis may propagate:
  • Inferiorly into the subclavian vein
  • Superiorly into cavernous/sigmoid/transverse venous sinuses

Sequelae:

• Local: Meningitis
• Disseminated: Lungs (common), joints, liver, muscle, pericardium, brain, skin
• Risk of DIC/septic shock

Organisms

1. Fusobacterium necrophorum (most common, up to 80%)
   • Fusobacterium necrophorum is a non-motile, sporulating gram-negative obligate anaerobe occurring in the normal flora of the pharynx, gastrointestinal tract, and female genital tract.
   • It can become pathogenic, probably because of its toxin production.
   • These bacteria can produce a lipopolysaccharide endotoxin with strong biologic activity, as well as a leukocidin and hemolysin, assisting in destruction of white and red blood cells. Hemagglutinin production augments the fulminant nature of the disease, causing platelet aggregation and septic thrombus formation.
2. Polymicrobial anaerobic bacteraemia
   • Typically anaerobic streptococci and other gram-negative anaerobes
3. Less common: S. aureus and other gram positives

Clinical Features

• Vitals: Fever, possible septic shock
• Neck: Tenderness behind mandible, swelling, lymphadenopathy
• Throat: Pharyngitis, peritonsillar abscess, trismus, drooling
• Cranial nerve palsies (if intracranial extension)
• Septic arthritis: Hip/knee most common
• Liver: Jaundice/hepatomegaly

Investigations

Blood tests: FBE, CRP, U&Es, LFTs, lactate, blood cultures (aerobic/anaerobic)

Throat swab: MCS

ECG: For general assessment

CXR: Look for pulmonary septic emboli (nodules, cavities, effusions)

Ultrasound:

• Detect thrombus in internal jugular vein
• May miss deeper thrombi; Doppler useful

CT with contrast (neck and chest):

• Gold standard
• Detects intraluminal thrombus, septic emboli, primary infection site

MRI/MRA: Alternative imaging

Joint aspiration: If septic arthritis suspected

Management

1. ABC management
2. Analgesia: IV paracetamol, opioids as needed
3. Antibiotics:
   • Piperacillin/tazobactam 4/0.5g IV 8 hourly
   • If anaphylactic to beta-lactams:
     • Clindamycin 600 mg IV 8 hourly + Metronidazole 500 mg IV 8-12 hourly
4. Anticoagulation:
   • Controversial; consider if:
     • Venous sinus thrombosis
     • No improvement with antibiotics
5. Surgical options:
   • IJV ligation/excision
   • Drainage of throat abscess
   • Drainage of metastatic abscesses

Disposition

• All patients require admission and IV antibiotics
• Urgent consultation:
  • ENT
  • Vascular surgery
  • Hematology (anticoagulation)
  • ICU (if clinically indicated)

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References

Publications

• Lemierre A. On certain septicemias due to anaerobic organisms. Lancet. 1936; 227(5874): 701–3
• Eilbert W, Singla N. Lemierre’s syndrome. Int J Emerg Med. 2013 Oct 23;6(1):40.
• Riordan T. Human infection with Fusobacterium necrophorum (Necrobacillosis), with a focus on Lemierre’s syndrome. Clin Microbiol Rev. 2007 Oct;20(4):622-59.
  

FOAMed

• Nickson C. Lemierre syndrome. LITFL
• Cadogan M. André-Alfred Lemierre (1875-1956). LITFL
• Nickson C. Just a sore throat. LITFL
• Bogseth M. Ultrasound Case 097. LITFL