Cerebral Venous Thrombosis (CVT) in Adults

Background

• CVT, a rare form of stroke, refers to a thrombosis of the dural sinus and/or cerebral veins.
• CVT generally occurs in younger patients (typically < 50 years old), affects women more than men among young and middle-aged adults, and accounts for 0.5%-1% of all strokes with an incidence of about 5-16 cases per 1 million people per year.
• Common risk factors for CVT include high-risk thrombophilias (for example, antithrombin III, protein C and protein S deficiency, homozygosity for either factor V Leiden or prothrombin G20210A mutations), oral contraceptive use, pregnancy, malignancy, infection, or trauma.
• Complications of CVT include intracranial hemorrhage, intracranial hypertension, and seizures in up to 40% and neurologic worsening after diagnosis (such as, new focal deficit or seizure) in up to 23%, while mortality is 4%-5% in the acute phase.

Evaluation

• Suspect CVT in younger patients (particularly those ≤ 50 years old) who present with acute, subacute, or chronic headache with unusual features, signs of intracranial hypertension, focal neurologic abnormalities (in absence of risk factors), and/or new seizure disorder.
• Considerations for imaging studies to confirm diagnosis of CVT:
  • Patient selection:
    • Perform imaging of the cerebral venous system to exclude CVT in patients with clinical features of idiopathic intracranial hypertension (Strong recommendation).
    • Perform imaging of the cerebral venous system in patients with lobar intracranial hemorrhage of otherwise unclear origin or with cerebral infarction that crosses typical arterial boundaries (Strong recommendation).
    • Consider imaging of the cerebral venous system to exclude CVT in patients with headache associated with atypical features (Weak recommendation).
  • Imaging modalities:
    • Computed tomography (CT) or magnetic resonance imaging (MRI) are useful for initial evaluation, but do not rule out CVT if these tests are normal.
    • Perform computed tomography venography or magnetic resonance venography (CTV or MRV) if CT or MRI are negative and CVT is still suspected or if there is a need to define the extent of CVT (Strong recommendation).
    • Consider catheter cerebral angiography if there is high clinical suspicion of CVT despite an inconclusive CTV or MRV (Weak recommendation).
• Obtain blood tests, including a complete blood count, chemistry panel, prothrombin time, and activated partial thromboplastin time (Strong recommendation).
• Other testing:
  • Consider testing for prothrombotic conditions, which may be beneficial for the management of patients with CVT (Weak recommendation).
  • Consider obtaining the D-dimer level since a normal D-dimer level may identify patients with a low probability of CVT (Weak recommendation).

Management

• Consider admission to a stroke unit for a patient with CVT for treatment or prevention of clinical complications (Weak recommendation).
• Anticoagulation considerations:
  • Consider anticoagulation during acute and chronic phases over no anticoagulant therapy (Weak recommendation).
  • Consider using adjusted-dose unfractionated heparin or weight-based low-molecular-weight heparin followed by vitamin K antagonists, regardless of the presence of intracerebral hemorrhage (Weak recommendation).
  • Consider using low-molecular-weight heparin instead of unfractionated heparin (Weak recommendation).
• Avoid antiseizure medications in most patients without seizures, but administer in patients with a single seizure with parenchymal lesions (Strong recommendation) and consider in patients with a single seizure without parenchymal lesions (Weak recommendation).
• Consider thrombolysis in selected patients who deteriorate despite adequate anticoagulation, such as in patients without intracranial hemorrhage or impending herniation from large hemorrhagic infarcts (Weak recommendation).
• Give appropriate antibiotics and perform surgical drainage in patients with infected CVT (Strong recommendation).
• If intracranial pressure is elevated:
  • monitor for progressive visual loss (Strong recommendation)
  • consider treatment with acetazolamide, lumbar puncture, shunts, or optic nerve decompression (Weak recommendation)
• Surgery and procedures:
  • Consider endovascular intervention if deterioration occurs despite intensive anticoagulation treatment (Weak recommendation).
  • Consider decompressive hemicraniectomy in patients with neurological deterioration, such as impending herniation due to severe mass effect or intracranial hemorrhage causing intractable intracranial hypertension (Weak recommendation).
• Long-term management:
  • Length of continuing anticoagulation is typically 3-12 months, but depends on the presence of underlying conditions.
  • Consider follow-up computed tomography venography or magnetic resonance venography 3-6 months after diagnosis (Weak recommendation).
• For women of fertile age:
  • Consider prophylactic use of low-molecular weight heparin during future pregnancies (which are not contraindicated) and postpartum (Weak recommendation).
  • Consider advising against use of combined hormonal contraceptives (Weak recommendation).