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Subsections
Cerebral Venous and Dural Sinus Thrombosis

Neurology

Cerebral Venous And Dural Sinus Thrombosis

Background

  1. Definition
    • Thrombosis of the cerebral veins and sinuses that will cause obstruction of blood flow and the consequences thereof
  2. Synopsis
    • Thrombosis of dural sinus/cerebral veins (CVT) is an uncommon form of stroke
      • It is distinct from arterial strokes
      • More common in young adults and children
    • Diagnosis and management can be difficult due to
      • Diversity of underlying risk factors
        • For example, a teenager with recent headaches after starting oral contraception or an obtunded man with dilated pupils
      • Absence of a uniform treatment approach
    • Multiple risk factors implicated but only some are reversible

Pathophysiology

  1. Mechanism
    • Incompletely understood
      • Linked classically to the Virchow triad of
        • Stasis of the blood
        • Changes in the vessel wall
        • Changes in the composition of the blood
    • Thrombosis of cerebral veins or dural sinus leading to cerebral parenchymal lesions or dysfunction
    • Occlusion of dural sinus resulting in decreased cerebrospinal fluid (CSF) absorption and ICP
  2. Etiology/Risk Factors
    • Prothrombotic conditions
      • Venous thrombosis (Virchow triad)
      • DIC, thrombotic purpura, thrombocytosis, polycythemia vera
      • Sickle cell, protein C&S deficiencies
    • Thrombophilias
    • Inflammatory bowel disease
      • Ulcerative colitis, Crohn's disease, Bechet's disease
    • Thyroid disease
    • Pregnancy
      • Venous stasis state
      • Complication of epidural blood patch
      • Spontaneous intracranial hypotension
      • Lumbar puncture
    • Severe dehydration
      • Intrinsic hyperviscosity
      • Postpartum - hypovolemic state
    • Oral contraceptives
    • Infection
      • Parameningeal infections (ear, sinus, mouth, face, neck)
    • Substance abuse/drugs
      • Androgen, danazol, lithium, vitamin A, IV immunoglobulin, ecstasy
    • Head trauma
    • Cancer
      • Local compression
      • Hypercoagulable
      • Antineoplastic (tamoxifen, L-asparaginase)
    • Risk factors
      • Acquired
        • Surgery, trauma
        • Antiphospholipid syndrome, cancer, exogenous hormones (OCP)
        • Pregnancy, puerperium
        • Greatest risk
          • 3rd trimester
          • Immediately after childbirth (73%)
          • 1st 4 weeks postpartum
      • Genetic
        • Inherited thrombophilia
      • Cerebral veins by Frequency of Thrombosis (View image)
        • Superior Sagittal sinus (62%)
        • Transverse (lateral) sinus (41-45%)
        • Straight sinus (18%)
        • Cortical veins (17%)
        • Internal jugular (12%)
        • Deep venous system (11%)
  3. Epidemiology
    • Incidence/Prevalence
      • Approximately 5 people per million annually
        • May be as high as 1.32-1.57 per 100,000 person-years
        • Pregnancy: 1 in 2,500-10,000 deliveries
      • Prevalence
        • 0.5-1% of all strokes
        • 75%: predisposed to prothrombotic conditions
        • 78%: Age < 50 yo
    • Mortality/Morbidity
      • Mortality rate of severe CVT remains as high as 34.2%

Diagnostics

  1. History/Symptoms
    • Clinical suspicion and imaging confirmation
      • Clinically may resemble a progressive encephalopathy or viral encephalitis
    • Symptoms due to either
      • ICP
      • Focal brain injury/ICH
    • History
      • Onset
      • Slowly progressive symptoms
        • < 48 hrs (37%)
        • > 48 hrs (56%)
        • Symptom onset to diagnosis - 7 days
      • HA (most common symptom - 90%)
        • Diffuse/progresses in severity days-weeks
        • Thunderclap headache
          • Suggestive of subarachnoid hemorrhage
        • Migraine
      • Lethargy
    • Focal neurologic deficits
      • Altered consciousness
        • Coma
        • Confusion
      • Visual loss
      • Bilateral leg > arm weakness
      • +/- generalized seizures
  2. Physical Exam/Signs
    • Intracranial
      • +/- Bilateral brain involvement
        • Paraparesis
      • Increased ICP
      • Risk of bilateral hemorrhagic infarct and fatal brain edema
    • Ocular/Neuro-Ophthalmic
      • Papilledema and diplopia
    • Head/Scalp
      • Lateral sinus thrombosis
        • Middle ear infection symptoms (fever, ear discharge)
        • Pain, swelling, palpable cord in neck
      • Scalp edema, dilated scalp veins
    • Focal neurological deficits
      • Hemiparesis and aphasia
      • Psychosis
      • Generalized seizures
  3. Labs/Tests
    • CBC, chem panel, pT, pTT
    • D-dimer > 500 ug/L
    • Prothrombotic factors
      • Protein C or S
      • Antithrombin III deficiency
      • Antiphospholipid syndrome
      • Anticardiolipin antibodies
      • Prothrombin G20210A mutation of factor II
        • 2% of whites
        • Elevation of pT levels
      • Factor V Leiden (+)
      • Hyperhomocysteinemia
  4. Imaging
    • Indications for Imaging
      • Lobar ICH of unknown etiology (infarction crossing arterial boundaries)
      • Idiopathic intracranial hypertension
    • MRI T2/MRV (View image)
      • Most sensitive
      • If suspected CVT and normal CT/CTV
        • Negative plain CT/MRI does not rule out CVT (consider venographic studies - CVT)
      • Findings: combination of absence of flow void with signal intensity alteration in dural sinus
    • CT
      • When MRI not available
      • Commonly used as initial neuroimaging test
      • Findings (View image)
        • Hyperdensity of a cortical vein or dural sinus
        • Superior sagittal sinus thrombosis - dense or filled delta sign
    • Invasive Cerebral arteriography/direct cerebral venography
      • Gold standard
      • Reserved when other imaging results inconclusive
  5. Other Tests/Criteria
    • Lumbar puncture
      • Unless meningitis suspected, not helpful
      • opening pressure
  6. Differential Diagnosis
    • Arterial Stroke
    • Idiopathic intracranial hypertension
    • Meningitis
    • Idiopathic intracranial hypotension
    • Brain abscess
    • Brain neoplasm

Treatment

  1. Initial/Prep/Goals
  2. Medical/Pharmaceutical
    • Anticoagulation
      • LMWH (preferred, lower in-hospital mortality and better prognosis)
      • IV heparin (UFH)
        • Bolus of 3000 U + continuous rate
        • pTT - 2x normal
      • If neurologically stable
        • Oral anticoagulation (warfarin) for 3-12 months or lifelong depending on
          • Transient reversible factor
          • Low risk thrombophilia
          • High risk/inherited thrombophilia
          • INR 2-3
        • Oral anticoagulation - mortality 50% within hrs-days of onset
      • Endovascular thrombolysis
    • Aspirin
    • Steroids
    • Antibiotics (if local/systemic infection)
      • Broad spectrum (i.e., ceftriaxone 2 g IV) - infective sinus thrombosis
      • Condition-specific treatment for other infections
    • Fibrinolytic therapy
  3. Surgical/Procedural
    • If severe mass effect/ ICH
      • Consider decompressive hemicraniectomy (lifesaving)
    • No mild mass effect
      • Consider endovascular therapy (with or without mechanical disruption)
    • Direct catheter thrombolysis
    • Balloon-assisted thrombectomy/thrombolysis
    • Catheter thrombectomy

Disposition

  1. Admission criteria
    • All patients
  2. Consult
    • Neurosurgery
  3. Discharge/Follow-up instructions
    • Discharge OCP in women with any history of CVT
    • Patient with previous CVT and recurrent symptoms
      • Repeat CTV/MRV and at 3-6 months
    • Poor long-term prognosis predictors
      • CNS infection
      • Any malignancy
      • Thrombosis of deep venous system
      • Hemorrhage on head CT/MRI
      • Glasgow coma score < 9 (admission)
      • Mental status abnormality
      • Age > 37 yo
      • Male gender
    • 30-day mortality predictors
      • Depressed consciousness
      • Altered mental status
      • Thrombosis of deep venous system
      • Right hemisphere hemorrhage
      • Posterior fossa lesions

References

  1. Stam J. Thrombosis of the Cerebral Veins and Sinuses. N Engl J Med. Jul 21, 2005;352:1791-1798
  2. Ulivi L, Squitieri M, Cohen H, et al. Cerebral venous thrombosis: a practical guide. Pract Neurol. 2020;20:356-367
  3. Liao W, Liu Y, Gu W, et al. Cerebral Venous Sinus Thrombosis: Successful Treatment of Two Patients Using the Penumbra System and Review of Endovascular Approaches. Neuroradiol J. Apr 2015; 28(2): 177-183
  4. Luo Y, Tian X, Wang X. Diagnosis and Treatment of Cerebral Venous Thrombosis: A Review. Front Aging Neurosci. Jan 30, 2018;10(2):1-15
  5. Saposnik G, Barinagarrementeria F. Diagnosis and Management of Cerebral Venous Thrombosis: A Statement for Healthcare Professionals From the American Heart Association/American Stroke Association. Stroke. Feb 3, 2011;42:1158-1192

Contributor(s)

  1. Ho, Nghia, MD

Updated/Reviewed: February 2021