Cerebral venous thrombosis

Manish KC, MBBS (1), Junaid Siddiqui, MD, MRCP (2)
1 - KIST Medical College & Teaching Hospital, Nepal.
2 - Department of Neurology, University of Missouri, Columbia, USA


Introduction

  • Thrombosis of cerebral veins and sinuses
  • Relatively rare and frequently unrecognized condition
  • Potentially severe and fatal condition

Epidemiology

  • 0.5% of all strokes
  • Prevalence: 5/1,000,000 people
  • Common in Asia and the Middle east due to increased rates of pregnancy and infection in these nations
  • Important cause of stroke in young people
  • Common in 20-50 years age group, less common after age 65
  • More common in females (2.9:1) due to estrogen changes, hormone replacement therapy, and pregnancy
  • Superior sagittal sinus thrombosis
    • most common type
    • Frequently presents with bilateral deficits

Etiology

  • Risk Factors: related to factors affecting virchow’s triad (hypercoagulability, endothelial injury and venous stasis)
  • Both hereditary and acquired prothrombotic conditions predispose to CVT
  • Causes and risk factors
    • Hereditary coagulopathies
      • Antithrombin III deficiency
      • Protein C and S deficiency
      • Factor V leiden
      • Factor II gene mutations
      • Methylenetetrahydrofolate reductase gene mutation
      • Von willebrand disease.
    • Acquired coagulopathies
      • Nephrotic syndrome
      • Antiphospholipid antibody syndrome
      • Hyperhomocysteinemia
    • Prothrombotic condition
      • Pregnancy
      • Puerperium
    • Infectious causes
      • Otitis media
      • Sinusitis
      • Meningitis
      • Mastoiditis
    • Drugs
      • Oral contraceptive pills
      • L-asparaginase
      • Corticosteroids
      • Androgens
    • Inflammatory conditions
      • Systemic Lupus erythematosus
      • Sarcoidosis
      • Granulomatosis with polyangiitis
      • Inflammatory bowel disease
    • Hematological disorders
      • Paroxysmal nocturnal hemoglobinuria
      • Primary polycythemia
      • Sickle cell disease
      • Disseminated intravascular coagulation
      • Thrombocythemia
      • Leukemia
    • Trauma
    • Neoplasms
    • Idiopathic

Pathophysiology

  • Cortical vein thrombosis
    • Raised venous and capillary pressure
    • Disruption of blood brain barrier
    • Formation of cerebral edema that develops into hemorrhagic venous infarction
    • Venous infarction causes focal neurological deficits and seizures
  • Cerebral sinus thrombosis
    • CSF outflow obstruction
    • Development of intracranial hypertension
    • Headache and vision problems

Clinical Features

  • 4 prominent clinical syndromes present
    • Isolated intracranial hypertension
    • Focal syndrome
    • Diffuse encephalopathy
    • Cavernous sinus syndrome
  • Headache: Most common
  • Seizures
  • Focal neurological deficit
    • Aphasia
    • Hemiparesis
  • Altered consciousness
  • Loss of vision

Differential Diagnosis

  • Idiopathic intracranial hypertension (pseudotumor cerebri)
  • Arterial ischemic stroke
  • Primary intracerebral hemorrhage
  • Hemorrhagic stroke due to a vascular malformation
  • Meningitis/encephalitis
  • Brain abscess
  • Systemic lupus erythematosus
  • Sarcoidosis
  • Antiphospholipid syndrome
 

Figure 1: Cerebral Sinus Thrombosis on CT Brain

notion image
notion image
Case courtesy of Dr Bruno Di Muzio, Radiopaedia.org, rID: 41031

Diagnosis

  • Combined MRI of brain and Magnetic resonance venography: Gold standard for diagnosis
  • MRI of brain
    • Identifies thrombosed blood vessels
    • Early thrombus formation: Hypodense from deoxyhemoglobin
    • Late thrombus formation: Hyperdense from methemoglobin
  • MR venography
    • empty delta sign (triangular filling defect due to thrombus in sagittal sinus)
  • CT scan of Head and CT venography
    • Inferior to MRI due to bone infarct and radiation exposure
    • Non-contrast CT head: hyperdense cortical veins or dural sinus
    • CT venography: Ideal for subacute or chronic thrombosis due to difference in density of thrombosed sinus
  • Catheter angiography
    • Associated with complications
    • Used only
      • When MRI and CT are inconclusive
      • Endovascular procedure is required
  • D-dimer Test: Increased in current patients with cerebral venous thrombosis. Hence, negative d-dimer in such patients rules out CVT. This does not apply in case of a patient with CVT with a current isolated headache where d-dimer can be normal

Treatment

  • Admit patients to the stroke unit
  • Correct underlying causes like infection and dehydration
  • Antithrombotic treatment
    • Subcutaneous Low molecular weight heparin or
    • IV heparin
  • Worsening of medical condition despite adequate anticoagulation and ruling out other causes of worsening of condition
    • Local IV thrombolysis or
    • Mechanical thrombectomy
  • Prevention of recurrent/future thrombotic events
    • Oral anticoagulation
      • 3-6months: for brief risk factors
      • 6-12 months: idiopathic CVT/mild hereditary thrombophilia
      • Indefinite: recurrent CVT/ severe hereditary thrombophilia
  • Symptomatic treatment
    • Treatment of raised ICP
      • Monitor ICP
      • Elevate head of bed
      • Hyperventilation
      • Sedation
      • Treat with glycerol and mannitol
      • Lumbar puncture in presence of vision threatening papilloedema, drain CSF before initiating heparin
      • Surgery: VP shunt, Lumboperitoneal shunt, optic nerve fenestration surgery
    • Impending herniation: Decompressive hemicraniectomy
    • Antiepileptics in patients with seizures

Complications

  • Hydrocephalus
    • 15% of patients
    • Obstructive type due to basal ganglia and thalamus edema
    • Associated with worse prognosis
  • Intracranial hypertension
    • Headache with or without papilloedema
    • Vision threatening: treat with immediate lumbar puncture, Shunt surgery
  • Transtentorial herniation
    • Major cause of immediate mortality
    • Treatment: Decompressive hemicraniectomy

Prognosis

  • Death rate: 8-10%
  • 80% recover without any sequelae
  • Overall good clinical outcome when compared to arterial stroke
  • Poor prognostic factors
    • Brain infection
    • Presence of any neoplasms
    • Deep vein thrombosis
    • Intracerebral hemorrhage
    • GCS>9 at the time of admission
    • Male gender
    • Age>37 years
    • Altered mental status
  • Death prognostic factors at 30 days
    • Decreased consciousness
    • Deep venous thrombosis
    • Right sided intracranial bleeding
    • Injury at the posterior fossa
  • Major cause of acute mortality
    • Herniation of the brain (transtentorial)
    • Increase in size of hematoma
    • Widespread cerebral edema
    • Pulmonary embolism
    • Status epilepticus

Bibliography

  • Ribes MF. Des recherches faites sur la phlébite. Revue Médicale Française et Etrangère et Journal de Clinique de l’Hôtel-Dieu et de la Charité de Paris 1825; 3: 5–41.
  • Bousser MG, Ferro JM. Cerebral venous thrombosis: an update. Lancet Neurol 2007;6(2):162Y170.
  • Khealani, B. A. et al. Cerebral venous thrombosis: a descriptive multicenter study of patients in Pakistan and Middle East. Stroke 39, 2707–2711 (2008).
  • Stam J (2005) Thrombosis of the cerebral veins and sinuses. N Engl J Med 352:1791–1798.
  • Janghorbani, M. et al. Cerebral vein and dural sinus thrombosis in adults in Isfahan, Iran: frequency and seasonal variation. Acta Neurol. Scand. 117, 117–121(2008).
  • Crassard I, Soria C, Tzourio Ch, et al. A negative D-dimer assay does not rule out cerebral venous thrombosis: a series of 73 patients. Stroke 2005; 36: 1716–19.
  • Ferro JM, Canhao P, Stam J, Bousser MG, Barinagarrementeria F. Prognosis of cerebral vein and dural sinus thrombosis: results of the International Study on Cerebral Vein and Dural Sinus Thrombosis (ISCVT). Stroke 2004; 35: 664–70.
  • Canhão P, Ferro JM, Lindgren AG, Bousser MG, Stam J, Barinagarrementeria F. Causes and predictors of death in cerebral venous thrombosis. Stroke 2005; 36: 1720–25.
  • Piotr R and Barbara K. Cerebral venous and sinus thrombosis. Udar Mo´zgu 2010; 12: 47–50.
  • Wasay, M. et al. Cerebral venous thrombosis: analysis of a multicenter cohort from the United States. J. Stroke Cerebrovasc. Dis. 17, 49–54 (2008).
A Living Pocketbook of Neurology
A Living Pocketbook of Neurology
Applied, Concise, Practical, Up-to-date, Mobile-friendly, peer-reviewed & Open-Access Pocketbook of Neurology and related clinical specialties
Unleash the Digital Healer in You! >> 🌐 https://junaidkalia.com <<
Unleash the Digital Healer in You! >> 🌐 https://junaidkalia.com <<
Learn Digital Health, AI-in-Healthcare, ValueBased Care & More!
🎯 Follow Junaid Kalia MD [Editor-in-Chief]
"If anyone saved a life, it would be as if he saved the life of all mankind"
"If anyone saved a life, it would be as if he saved the life of all mankind"
An AINeuroCare Academy Project - Coaching Clinicians in Digital Healthcare
💡 Learn! 🎥 YouTube 💌 Newsletter 🎓Academy 📝 Blog
👨‍⚕️ Connect! 👥 Linkedin 👋 Facebook 🕊️ Twitter 📸 Instagram

Disclaimer: For Education purposes only. You must NOT rely on the information on this website as an alternative to medical advice from your healthcare provider

CC BY-NC-ND - Attribution-NonCommercial-NoDerivs - AINeuroCare PLLC ©️