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

Treatment for Cerebral Venous Sinus Thrombosis (CVST)

Immediate anticoagulation with either intravenous unfractionated heparin (UFH) or subcutaneous low-molecular-weight heparin (LMWH) is the first-line treatment for CVST, even in patients with hemorrhagic lesions. 1, 2

Initial Management

• Anticoagulation should be started immediately after diagnosis confirmation, regardless of the presence of intracranial hemorrhage that occurred as a consequence of CVST 3, 2
• Either intravenous unfractionated heparin or subcutaneous low-molecular-weight heparin can be used as initial treatment 1, 2
• Parenteral anticoagulation therapy should be continued until the patient has stabilized clinically 1
• The presence of intracerebral hemorrhage related to CVST is NOT a contraindication to anticoagulation 2, 4
• All patients should be admitted to a stroke unit for close monitoring and specialized care 2

Evidence Supporting Anticoagulation

• Anticoagulation prevents thrombus propagation, increases recanalization chances, and reduces mortality and severe disability 3
• A meta-analysis of two randomized trials (79 patients) showed anticoagulation was associated with:
  • Relative risk of death of 0.33 (95% CI 0.08 to 1.21)
  • Relative risk of death or dependency of 0.46 (95% CI 0.16 to 1.31)
  • Absolute reduction in risk of death or dependency of 13% 1, 5
• A small prospective cohort study of 30 children with CVST reported 3 deaths among 8 untreated children compared with no deaths among 22 treated children 1

Special Considerations

• For patients who demonstrate progressive neurologic deterioration despite adequate anticoagulation, consider:
  • Endovascular thrombectomy
  • Local intrathrombus thrombolytic agent infusion with IV heparin 1, 6
• Dexamethasone may be considered in specific scenarios with significant white matter edema causing mass effect and neurological deterioration (4-8 mg/day oral or IV) 3
• Dexamethasone should not be used routinely in all CVST cases, particularly in asymptomatic patients without significant mass effect 3

Transition to Oral Anticoagulation

• After clinical stabilization, patients should be switched from parenteral anticoagulation to oral anticoagulants 1, 2
• Duration of anticoagulation depends on underlying etiology:
  • 3-6 months for CVST associated with transient risk factors 1, 2, 4
  • 6-12 months for idiopathic CVST and those with mild hereditary thrombophilia 4
  • Indefinite (lifelong) anticoagulation for severe thrombophilia or recurrent thrombosis 2, 4

Management of Complications

• Control of seizures with appropriate anticonvulsants 1
• Management of elevated intracranial pressure 1, 4
• For patients with significant mass effect and brain displacement, consider:
  • Anti-edema treatment (hyperventilation, osmotic diuretics)
  • Surgical decompression (craniectomy) in severe cases 4

Follow-up Recommendations

• Neurological and ophthalmological follow-up is recommended, especially during the first year, due to risk of visual loss from increased intracranial pressure 1
• Investigate underlying prothrombotic conditions, as this affects treatment duration 2
• Monitor for cognitive and neurological sequelae that may require rehabilitation and longer-term therapy 1

Cautions and Pitfalls

• While anticoagulation is generally safe, rebleeding can occur in some patients with pre-existing hemorrhage 7
• The evidence for anticoagulation safety comes from relatively small trials, and the impact of up to 9% of new intracranial hemorrhage cannot be ruled out 7
• Discontinue oral contraceptives in affected patients, as they may increase risk of recurrent CVST 1
• There is insufficient evidence to support the routine use of either systemic or local thrombolysis in all CVST patients 4