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

Treatment of Cerebral Sinus Venous Thrombosis (CSVT)

Initiate anticoagulation immediately upon diagnosis of CSVT with either intravenous unfractionated heparin or subcutaneous low-molecular-weight heparin, even in the presence of intracranial hemorrhage from venous congestion. 1, 2

Initial Anticoagulation Protocol

Start anticoagulation as soon as CSVT is confirmed by CT venography or MR venography—do not delay treatment. 1 The presence of hemorrhagic transformation is not a contraindication, as these hemorrhages result from venous congestion rather than arterial bleeding. 1, 2

Agent Selection

Choose one of the following for acute management:

• Low-molecular-weight heparin (LMWH): 1.0 mg/kg subcutaneously twice daily or 1.5 mg/kg once daily (preferred agent) 2
• Unfractionated heparin (UFH): 5000 IU bolus, then continuous infusion adjusted to maintain aPTT at 1.5-2.5 times baseline 2

Both agents demonstrate equivalent efficacy in adults, with moderate certainty evidence showing reduced mortality (OR 0.35) and severe disability (OR 0.30) compared to no treatment. 3

Pediatric Considerations

For pediatric patients with CSVT, anticoagulation reduces mortality by 64% (RR 0.36; 95% CI 0.16-0.81) and severe CSVT progression by 67% (RR 0.33; 95% CI 0.18-0.58). 4 This translates to 48 fewer deaths and 118 fewer cases of severe CSVT per 1000 children treated. 4

• Anticoagulation is conditionally recommended even in pediatric patients with hemorrhage secondary to venous congestion 3, 4
• Different populations (neonates, infection-associated CSVT, trauma, post-surgical, cancer patients) require consideration of individual bleeding risks 3
• Use anticoagulation alone rather than thrombolysis in pediatric CSVT unless neurologic deterioration occurs despite adequate anticoagulation 3, 4

Transition to Oral Anticoagulation

After clinical stabilization on parenteral therapy, transition to oral anticoagulation with warfarin targeting INR 2.0-3.0 (target 2.5). 1, 2

• Continue parenteral anticoagulation until INR ≥2.0 for at least 24 hours 1
• Warfarin remains the standard oral anticoagulant for CSVT 3, 1

Direct Oral Anticoagulants (DOACs)

While meta-analysis data suggest DOACs demonstrate similar efficacy and safety to warfarin with better recanalization rates in adults 5, current guidelines do not recommend DOACs as first-line therapy for CSVT. 4 DOACs lack pediatric-specific evidence and are not recommended in children. 4

Duration of Anticoagulation

The duration depends on the presence and persistence of risk factors:

Provoked CSVT (Transient Risk Factor)

• 3 months of anticoagulation 1, 2
• Examples include hormonal contraception, pregnancy, acute infection

Idiopathic CSVT or Mild Thrombophilia

• 6-12 months of anticoagulation 2, 6

Severe Thrombophilia, Recurrent CSVT, or Persistent Prothrombotic Conditions

• Indefinite anticoagulation 2, 6

Pediatric Duration

• Minimum 3 months for most cases 1
• 6 weeks for low-risk provoked CSVT 4
• 6-12 months for unprovoked or high-risk CSVT 4
• Consider additional 3 months if ongoing symptoms or persistent occlusion after initial treatment period 1

Thrombolysis and Advanced Interventions

Reserve thrombolysis for patients demonstrating progressive neurologic deterioration despite adequate anticoagulation. 3, 4

• Systemic or catheter-directed thrombolysis may be considered in select cases without intracranial hemorrhage 6
• A randomized adult trial showed higher mortality with endovascular treatment versus anticoagulation alone 3
• Endovascular options in pediatrics depend on patient size and institutional expertise 3

Monitoring and Follow-Up

Obtain follow-up venographic study (CTV or MRV) at 3-6 months after diagnosis to assess recanalization. 1

• Perform early follow-up imaging if persistent or evolving symptoms occur despite medical treatment 1
• Monitor INR regularly to maintain therapeutic range of 2.0-3.0 1, 2

Critical Pitfalls to Avoid

Do not withhold anticoagulation due to intracranial hemorrhage if it results from venous congestion—this is the most common and dangerous error in CSVT management. 1, 2 The hemorrhage is caused by venous hypertension from thrombotic obstruction, and anticoagulation prevents propagation and worsening venous infarction. 3

• Ensure appropriate surgical or medical treatment for associated conditions (e.g., surgical drainage for infection-associated CSVT) alongside anticoagulation 3
• Consider acetazolamide or serial lumbar punctures if intracranial pressure remains severely elevated despite anticoagulation 2
• Screen for underlying prothrombotic conditions including factor V Leiden, prothrombin G20210A mutation, antiphospholipid antibodies, protein C/S deficiency, and antithrombin III deficiency 2