What are the current treatment recommendations for cerebral venous sinus thrombosis?

Current Treatment Recommendations for Cerebral Venous Sinus Thrombosis

Immediate anticoagulation with either intravenous unfractionated heparin or subcutaneous low-molecular-weight heparin should be initiated for all patients with cerebral venous sinus thrombosis, even in the presence of intracranial hemorrhage. 1, 2, 3

Initial Anticoagulation Protocol

Start anticoagulation immediately upon diagnosis confirmation—this is the cornerstone of treatment and should not be delayed. 2, 3

First-Line Options:

• Low-molecular-weight heparin (LMWH) - preferred agent: Enoxaparin 1.0 mg/kg subcutaneously twice daily or 1.5 mg/kg once daily 2
• Unfractionated heparin (UFH): Initial bolus of 5000 IU, followed by continuous infusion adjusted to maintain aPTT at 1.5-2.5 times baseline 2, 4

Critical Pitfall to Avoid:

The presence of intracranial hemorrhage secondary to venous congestion is explicitly NOT a contraindication to anticoagulation. 1, 2, 3 This is the most common error in management—withholding anticoagulation due to fear of hemorrhagic complications worsens outcomes. 5 Hemorrhage in CVST results from venous congestion and elevated venous pressure, and anticoagulation prevents thrombus propagation that would worsen venous hypertension. 4, 6

Acute Care Setting and Monitoring

• Admit all patients to a stroke unit or neurocritical care setting for close neurological monitoring every 2-4 hours 2, 3
• Monitor specifically for signs of neurological deterioration, worsening headache, decreased consciousness, or seizure activity 5
• Regular neurological assessment is necessary to detect clinical deterioration 2

Transition to Oral Anticoagulation

Begin oral anticoagulation early, continuing parenteral anticoagulation for a minimum of 5 days and until INR is ≥2.0 for at least 24 hours. 2

Options for long-term anticoagulation:

• Vitamin K antagonists (warfarin) with target INR 2-3 1, 6
• Direct oral anticoagulants (DOACs) are emerging as safe alternatives with similar efficacy and potentially better safety profiles compared to warfarin 7

Duration of Anticoagulation

The duration is determined by the underlying etiology:

• Transient/reversible risk factors (infection, trauma, pregnancy): 3-6 months 1, 2, 3
• Idiopathic CVST or mild thrombophilia: 6-12 months 1, 6
• Severe thrombophilia, recurrent CVST, or persistent prothrombotic conditions: Indefinite (lifelong) anticoagulation 1, 2, 3

Management of Neurological Deterioration

If patients deteriorate despite adequate anticoagulation:

• Endovascular thrombolysis (with or without mechanical disruption) may be considered in severe cases or patients who fail to improve on anticoagulation 1, 4
• Decompressive hemicraniectomy should be considered as a lifesaving procedure for patients with severe mass effect or impending herniation 1
• Thrombolysis is generally reserved for patients with neurological deterioration despite anticoagulation and should be avoided in those with large infarcts and impending herniation 4, 6

Pediatric Considerations

For pediatric patients with CSVT (with or without hemorrhage secondary to venous congestion), anticoagulation is recommended rather than no anticoagulation. 1 However, different populations (neonates, infection-associated CSVT, trauma, surgery, cancer) may have different bleeding risks that should be considered. 1

Anticoagulation alone is preferred over thrombolysis followed by anticoagulation in pediatric patients. 1 Thrombolysis may be considered only when there is neurological deterioration despite anticoagulation. 1

Symptomatic Management

• Seizure control: Antiepileptic drugs should be prescribed for patients with acute seizures and supratentorial lesions 6
• Elevated intracranial pressure: Therapeutic lumbar puncture can reduce intracranial hypertension and relieve symptoms in patients with severe headache and papilledema 6
• Acetazolamide or serial lumbar punctures may be considered if intracranial pressure remains severely elevated despite anticoagulation 5

Prothrombotic Workup

Investigate underlying prothrombotic conditions during hospitalization, but do not delay anticoagulation for extensive thrombophilia workup. 5, 3 This includes screening for:

• Factor V Leiden mutation
• Prothrombin G20210A mutation
• Antiphospholipid antibodies
• Protein C, protein S, and antithrombin III deficiency
• Inflammatory conditions 5

Follow-Up Imaging

• Follow-up CT venography or MR venography at 3-6 months after diagnosis is reasonable to assess for recanalization of the occluded cortical veins/sinuses 2
• Earlier imaging (1-3 months) is indicated if symptoms persist or evolve despite treatment 5

Special Populations

Brain tumor patients: LMWH or direct oral anticoagulants can be used for treatment of established VTE in patients with brain tumors, though anticoagulation confers an increased risk of intracerebral hemorrhage (OR 3.66,95% CI 1.84-7.29). 1 The decision should balance VTE risk against bleeding risk on a case-by-case basis.

Severe renal failure (creatinine clearance <30 mL/min): Use unfractionated heparin followed by early vitamin K antagonists or LMWH adjusted to anti-Xa concentration. 1