What is the recommended treatment for Cerebral Venous Sinus Thrombosis (CVST)?

Treatment of Cerebral Venous Sinus Thrombosis (CVST)

Immediate anticoagulation is the recommended first-line treatment for cerebral venous thrombosis (CVT), regardless of the presence of intracranial hemorrhage, using either intravenous unfractionated heparin (UFH) or subcutaneous low molecular weight heparin (LMWH). 1

Initial Management Algorithm

1. Confirm diagnosis with MRI T2*-weighted imaging + MRV or CT/CTV if MRI not readily available 2

2. Initiate anticoagulation therapy immediately:

   • IV unfractionated heparin (UFH) with aPTT monitoring, or
   • Subcutaneous LMWH with anti-factor Xa level monitoring 2, 1
   • Note: Intracranial hemorrhage related to CVST is NOT a contraindication for anticoagulation 2, 1

3. Provide supportive care:

   • Control seizures with anticonvulsants if present
   • Manage elevated intracranial pressure
   • Ensure adequate hydration 2

Treatment Based on Clinical Response

If patient shows neurological improvement or remains stable:

• Transition to oral anticoagulation (vitamin K antagonists with target INR 2.0-3.0) 1
• Duration of anticoagulation based on etiology:
  • 3-6 months for transient/reversible risk factors (e.g., infection)
  • 6-12 months for idiopathic CVST or mild thrombophilia
  • Indefinite anticoagulation for recurrent CVST or severe thrombophilia (e.g., antithrombin, protein C or S deficiency) 1, 3

If patient shows neurological deterioration despite anticoagulation:

• Evaluate for severe mass effect or expanding hemorrhage with repeat imaging
• Consider escalation of care:
  • Decompressive hemicraniectomy for severe mass effect (life-saving procedure)
  • Endovascular therapy (thrombolysis with/without mechanical disruption) for patients with contraindications to anticoagulation or failing anticoagulation therapy 2

Special Considerations

Pediatric Patients

• Anticoagulation is safe and effective in children beyond the neonatal period 2, 4
• For older children without hemorrhage, anticoagulation is recommended (consensus from International Pediatric Stroke Study) 2
• Common approach is warfarin for 3-6 months 2
• Anticoagulation is generally not recommended for neonates except in cases of clinical deterioration or radiological evidence of clot propagation 2

Monitoring and Follow-up

• Regular clinical follow-up initially every 1-3 months, then every 3-6 months 1
• Follow-up neuroimaging (CTV or MRV) at 3-6 months to assess recanalization 1
• Neurological and ophthalmological follow-up is recommended, especially during the first year due to risk of visual loss from increased intracranial pressure 2

Potential Pitfalls and Caveats

• While the presence of intracranial hemorrhage related to CVST is not a contraindication for anticoagulation, close monitoring is essential as there is a small risk of rebleeding 5
• The evidence for anticoagulation in CVST comes from relatively small trials, with only limited numbers of patients having baseline hemorrhage 5
• Thrombolysis should be reserved for patients who deteriorate despite adequate anticoagulation, as there is insufficient evidence to support its routine use 6, 3
• Patients with extensive thrombosis involving multiple sinuses may have worse outcomes and might require more aggressive management 2

By following this evidence-based approach, mortality and long-term neurological sequelae from CVST can be significantly reduced, with most patients achieving good functional outcomes when treated promptly and appropriately.