EFNS guideline on the treatment of cerebral venous and sinus thrombosis in adult patients

Abstract

Background: Cerebral venous and sinus thrombosis (CVST) is a rather rare disease which accounts for <1% of all strokes. Diagnosis is still frequently overlooked or delayed as a result of the wide spectrum of clinical symptoms and the often subacute or lingering onset. Current therapeutic measures which are used in clinical practice include the use of anticoagulants such as dose-adjusted intravenous heparin or body weight-adjusted subcutaneous low-molecular-weight heparin (LMWH), the use of thrombolysis and symptomatic therapy including control of seizures and elevated intracranial pressure. Methods: We searched MEDLINE (National Library of Medicine), the Cochrane Central Register of Controlled Trials (CENTRAL) and the Cochrane Library to review the strength of evidence to support these interventions and the preparation of recommendations on the therapy of CVST based on the best available evidence. Review articles and book chapters were also included. Recommendations were reached by consensus. Where there was a lack of evidence but consensus was clear we stated our opinion as good practice points. Results and conclusions: Patients with CVST without contraindications for anticoagulation (AC) should be treated either with body weight-adjusted subcutaneous LMWH or with dose-adjusted intravenous heparin (level B recommendation). Concomitant intracranial haemorrhage (ICH) related to CVST is not a contraindication for heparin therapy. The optimal duration of oral anticoagulant therapy after the acute phase is unclear. Oral AC may be given for 3 months if CVST was secondary to a transient risk factor, for 6-12 months in patients with idiopathic CVST and in those with ''mild'' thrombophilia, such as heterozygous factor V Leiden or prothrombin G20210A mutation and high plasma levels of factor VIII. Indefinite AC should be considered in patients with recurrent episodes of CVST and in those with one episode of CVST and ÔsevereÕ thrombophilia, such as antithrombin, protein C or protein S deficiency, homozygous factor V Leiden or prothrombin G20210A mutation, antiphospholipid antibodies and combined abnormalities (good practice point). There is insufficient evidence to support the use of either systemic or local thrombolysis in patients with CVST. If patients deteriorate despite adequate AC and other causes of deterioration have been ruled out, thrombolysis may be a therapeutic option in selected cases, possibly in those without large ICH and threatening herniation (good practice point). There are no controlled data about the risks and benefits of certain therapeutic measures to reduce an elevated intracranial pressure (with brain displacement) in patients with severe CVST. However, in severe cases with impending herniation craniectomy can be used as a life-saving intervention (good practice point).Background and objectives

The Epidemiology and Prognosis of Cerebral Venous Sinus Thrombosis in a Third Level Hospital

“…In our series, the most frequently used acute treatment was LMWH, while in chronic cases the treatment was acenocumarol. These data confi rm what is stated in the guidelines of the European Federation of Neurological Associations (EFNA) on CVT treatment, where it is recommended that these patients, if there are no contraindications for anticoagulation therapy, should be treated with LMWH or UFH [5]. It is also consistent with the recommendations of the American College of Chest Physicians (ACCP), which suggest anticoagulation during the acute and chronic stages of CVT, therefore UFH or LMWH can be used as initial treatment even when there is hemorrhage inside a venous infarction [4].…”

“…For this, both in the short and in the long term, the main treatment option is anticoagulation therapy, either parenterally (UFH or LMWH) or per os (acenocumarol or direct oral anticoagulants) [3][4][5]. In our series, the most frequently used acute treatment was LMWH, while in chronic cases the treatment was acenocumarol.…”

Epidemiology, Endovascular Treatment, and Prognosis of Cerebral Venous Thrombosis: US Center Study of 152 Patients

“…This study, however, was observational and not intended to assess the efficacy and safety of ET compared with standard of care. Only a randomized controlled trial can clarify the role of ET in the treatment of CVT 8. The randomized controlled trial examining the efficacy of ET versus systemic anticoagulation in patients with severe forms of CVT will be able to substantiate our findings 12…”

“…The principal therapy and standard of care of CVT is systemic anticoagulation, even in the presence of intracranial hemorrhage8; subcutaneous low‐molecular‐weight heparin is preferred over intravenous unfractionated heparin 9, 10. The American Heart Association/American Stroke Association (AHA/ASA) scientific statement on CVT suggests that 9% to 13% of patients will have clinical deterioration and poor outcomes despite treatment with anticoagulation, resulting from incomplete recanalization and/or persistent thrombosis 3.…”

“…The American Heart Association/American Stroke Association (AHA/ASA) scientific statement on CVT suggests that 9% to 13% of patients will have clinical deterioration and poor outcomes despite treatment with anticoagulation, resulting from incomplete recanalization and/or persistent thrombosis 3. US and European guidelines recommend endovascular therapies (ETs) if patients with CVT continue to deteriorate despite anticoagulation 3, 8. ETs are increasingly advocated and include mechanical thrombectomy and intrasinus chemical thrombolytic11; however, at the time of the guidelines in 2010 and 2011, evidence for the use of ET was supported mostly by small case series and case reports.…”