Guideline sources The following summarized guidelines for the evaluation and management of cerebral venous thrombosis (CVT) are prepared by our editorial team based on guidelines from the American Society of Hematology (ASH 2023), the European Stroke Organisation (ESO 2017), the Society of Obstetricians and Gynaecologists of Canada (SOGC 2014), and the American Heart Association (AHA/ASA 2011). 1 2 3 4 Calculator Risk score for cerebral venous t Guidelines 1. Diagnostic investigations Diagnostic imaging: https://web.pathway.md/diseases/recc4wg3WuN2Muuhv 1/7 6/29/23, 3:49 AM Cerebral venous thrombosis Pathway g g g As per ESO 2017 guidelines: Consider obtaining MRV or CTV as reliable alternatives to digital subtraction angiography for diagnostic confirmation in patients with suspected CVT. C Consider obtaining CTV as a reliable alternative to MRV for diagnostic confirmation in patients with suspected CVT. C As per AHA 2011 guidelines, obtain imaging of the cerebral venous system in patients with lobar intracranial hypertension of otherwise unclear origin or with cerebral infarction crossing typical arterial boundaries. B Show 8 more Laboratory tests: Obtain the following routine tests in patients with suspected CVT: CBC chemistry panel PT activated PTT. B D-dimer testing: As per ESO 2017 guidelines, consider obtaining D-dimer before neuroimaging in patients with suspected CVT, except in patients with isolated headache or prolonged duration of symptoms (> 1 week) before the test. C As per AHA 2011 guidelines: Consider obtaining D-dimer testing (sensitive immunoassay or rapid ELISA) to help identify patients with low probability of CVT (normal D-dimer levels). C Obtain further evaluation in patients with strong clinical suspicion of CVT, even if D-dimer is normal. Screening for thrombophilia: As per ESO 2017 guidelines: Avoid obtaining thrombophilia screening to reduce death, improve functional outcomes or prevent recurrent venous thrombosis in patients with CVT. D Consider obtaining thrombophilia screening in patients with a high pre-test probability of carrying severe thrombophilia (a personal and/or family history of venous thrombosis, young age at CVT, CVT without a transient or permanent risk factor) to prevent recurrent VTE. E As per AHA 2011 guidelines, screen for potential prothrombotic conditions predisposing to CVT (such as use of contraceptives, underlying inflammatory disease, infectious process) in the initial clinical assessment of patients with CVT. B Show 2 more Screening for occult malignancy: avoid obtaining routine screening for occult malignancy to improve outcomes in patients with CVT. D 2. Medical management https://web.pathway.md/diseases/recc4wg3WuN2Muuhv 2/7 6/29/23, 3:49 AM Cerebral venous thrombosis Pathway Setting of care: consider admitting patients with CVT to a stroke unit for treatment and for prevention of clinical complications. C Anticoagulant therapy, initiation: As per ESO 2017 guidelines, initiate heparin at a therapeutic dose for the treatment of adult patients with acute CVT, including patients with intracerebral hemorrhage at baseline. B Show 2 more As per SOGC 2014 guidelines, initiate therapeutic dose anticoagulation in patients with confirmed CVT. B As per AHA/ASA 2011 guidelines, consider initiating initial anticoagulation with adjusted-dose UFH or weight-based LMWH in full anticoagulant doses in patients with CVT, followed by vitamin K antagonists, regardless of the presence of intracranial hypertension. C Anticoagulant therapy, duration: As per ASH 2023 guidelines: Consider obtaining thrombophilia testing to guide anticoagulant treatment duration after primary treatment in patients with CVT in a setting where anticoagulation would be discontinued. Consider continuing anticoagulation indefinitely in patients with thrombophilia. C Avoid obtaining thrombophilia testing to guide anticoagulant treatment duration after primary treatment in patients with CVT in a setting where anticoagulation would be continued indefinitely. D As per ESO 2017 guidelines: Consider administering OACs (vitamin K antagonists) for a variable period (3-12 months) after CVT to prevent recurrent CVT and other VTE. C Consider offering permanent anticoagulation in patients with recurrent venous thrombosis or with an associated prothrombotic condition with a high thrombotic risk. Consider following specific recommendations for the prevention of recurrent VTE in patients with these conditions. E As per AHA/ASA 2011 guidelines, consider continuing vitamin K antagonists for 3-6 months, with a target INR of 2-3, in patients with provoked CVT (associated with a transient risk factor). C Show 2 more Acetazolamide: As per ESO 2017 guidelines: Avoid using acetazolamide in patients with acute CVT to prevent death or improve functional outcome. D Consider administering acetazolamide if its safety profile is acceptable in patients with isolated intracranial hypertension secondary to CVT causing severe headaches or threatening vision. E As per AHA 2011 guidelines, consider administering acetazolamide in patients with CVT and increased ICP. C https://web.pathway.md/diseases/recc4wg3WuN2Muuhv 3/7 6/29/23, 3:49 AM Cerebral venous thrombosis Pathway Corticosteroids: As per ESO 2017 guidelines: Avoid using corticosteroids in patients with acute CVT to prevent death or improve functional outcome. D Consider administering corticosteroids in patients with acute CVT and Beh et's disease or other inflammatory diseases (such as SLE). C As per AHA 2011 guidelines, do not use corticosteroids in patients with CVT even in the presence of parenchymal brain lesions on CT/MRI, unless needed for another underlying disease. D Antiepileptic drugs: As per ESO 2017 guidelines: Consider initiating antiepileptic drugs to prevent early recurrent seizures in patients with acute CVT with supratentorial lesions and seizures. C Insufficient evidence to recommend prevention of remote seizures. I As per AHA 2011 guidelines, initiate early antiepileptic drugs for a defined duration to prevent further seizures in patients with CVT and a single seizure with parenchymal lesions. B Show 2 more 3. Therapeutic procedures Endovascular interventions: As per ESO 2017 guidelines: Insufficient evidence to recommend thrombolysis in patients with CVT. I Avoid performing thrombolysis in patients with acute CVT with a pretreatment low risk of poor outcome. D As per AHA 2011 guidelines, consider performing endovascular intervention if deterioration occurs despite intensive anticoagulation treatment. C Therapeutic lumbar puncture: As per ESO 2017 guidelines: Insufficient evidence to recommend therapeutic lumbar puncture to improve outcomes in patients with CVT and signs of intracranial hypertension. I Consider performing therapeutic lumbar puncture in patients with CVT and signs of intracranial hypertension, because of a potential beneficial effect on visual loss and/or headache, whenever its safety profile is acceptable. E As per AHA 2011 guidelines, consider performing therapeutic lumbar puncture in patients with CVT and increased ICP if there is progressive visual loss. C 4. Surgical interventions https://web.pathway.md/diseases/recc4wg3WuN2Muuhv 4/7 6/29/23, 3:49 AM Cerebral venous thrombosis Pathway Shunting: As per ESO 2017 guidelines: Avoid performing routine shunting (without other surgical treatment) to prevent death in patients with acute CVT and impending brain herniation due to parenchymal lesions. D Insufficient evidence to recommend shunting to prevent death or improve outcomes in patients with acute or recent CVT and hydrocephalus. I As per AHA 2011 guidelines, consider performing shunting in patients with CVT and increased ICP if there is progressive visual loss. C Decompressive hemicraniectomy: As per ESO 2017 guidelines, perform decompressive surgery to prevent death in patients with acute CVT and parenchymal lesions with impending herniation. B As per AHA 2011 guidelines, consider performing decompressive hemicraniectomy in patients with neurological deterioration due to severe mass effect or intracranial hemorrhage causing intractable intracranial hypertension. C Optic nerve decompression: consider performing optic nerve decompression in patients with CVT and increased ICP if there is progressive visual loss. C 5. Specific circumstances Patients with bacterial infection: administer appropriate antibiotics in patients with suspected bacterial infection and perform surgical drainage of purulent collections of infectious sources associated with CVT when appropriate. B Patients with recurrent disease: consider evaluating for CVT recurrence and intracranial hypertension in patients with a history of CVT complaining of new, persisting, or severe headache. B Pregnant patients: As per ESO 2017 guidelines, consider administering subcutaneous LMWH in pregnant and puerperal patients with acute CVT. C Show 3 more As per SOGC 2014 guidelines, consider administering thromboprophylaxis in future pregnancies following a CVT. C As per AHA 2011 guidelines, continue LMWH in full anticoagulant doses throughout pregnancy in patients with CVT. Continue low molecular weight hepari or vitamin K antagonist with a target INR of 2-3 for at least 6 weeks postpartum (for a total minimum duration of therapy of 6 months). B Show 3 more 6. Follow-up and surveillance https://web.pathway.md/diseases/recc4wg3WuN2Muuhv 5/7 6/29/23, 3:49 AM Cerebral venous thrombosis Pathway Monitoring for vision loss: monitor patients with CVT and increased ICP for progressive visual loss. Treat increased ICP urgently when vision loss is observed. B Indications for specialist consultation: consider obtaining consultation with a physician with expertise in thrombosis to assist in the prothrombotic testing and care of patients with CVT. C Clinical findings Symptoms Past medical history Absence of diplopia Antithrombin III deficiency Facial numbness FVL mutation Headache Protein C deficiency Nausea or vomiting Protein S deficiency Neck pain Prothrombin G20210A mutation Occipital headache Ocular exam Periorbital pain Tinnitus Papilledema Vision loss Neurological exam Abducens nerve palsy Altered mental status Coma Encephalopathy Focal neurologic deficit Homonymous hemianopsia Observed seizure Oculomotor nerve palsy Receptive aphasia Trochlear nerve palsy Diagnostic procedures opening pressure References 1. Gustavo Saposnik, Fernando Barinagarrementeria, Robert D Brown Jr et al. Diagnosis and management of cerebral venous thrombosis: a statement for healthcare professionals from the American Heart Association/American Stroke Association. Stroke. 2011 Apr;42 4 1158 92. Open 2. J M Ferro, M G Bousser, P Canh o et al. European Stroke Organization guideline for the diagnosis and treatment of cerebral venous thrombosis - endorsed by the European Academy of Neurology. Eur J Neurol. 2017 Oct;24 10 1203 1213. Open https://web.pathway.md/diseases/recc4wg3WuN2Muuhv 6/7 6/29/23, 3:49 AM Cerebral venous thrombosis Pathway 3. Chan WS, Rey E, Kent NE et al. Venous thromboembolism and antithrombotic therapy in pregnancy. J Obstet Gynaecol Can. 2014 Jun;36 6 527 53. Open 4. Saskia Middeldorp, Robby Nieuwlaat, Lisa Baumann Kreuziger et al. American Society of Hematology 2023 Guidelines for Management of Venous Thromboembolism: Thrombophilia Testing. Blood Adv. 2023 May 17;bloodadvances.2023010177. Open 5. Jos M Ferro, Helena Bacelar-Nicolau, Teresa Rodrigues et al. Risk score to predict the outcome of patients with cerebral vein and dural sinus thrombosis. Cerebrovasc Dis. 2009;28 1 39 44. Open 6. Campbell Tait, Trevor Baglin, Henry Watson et al. Guidelines on the investigation and management of venous thrombosis at unusual sites. Br J Haematol. 2012 Oct;159 1 28 38. Open 7. Scott M Stevens, Scott C Woller, Lisa Baumann Kreuziger et al. Antithrombotic Therapy for VTE Disease: Second Update of the CHEST Guideline and Expert Panel Report. Chest. 2021 Dec;160 6):e545-e608. Open 8. Paul Monagle, Anthony K C Chan, Neil A Goldenberg et al. Antithrombotic therapy in neonates and children: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. Chest. 2012 Feb;141 2 Suppl):e737S-e801S. Open https://web.pathway.md/diseases/recc4wg3WuN2Muuhv 7/7